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Atlas of Pediatric

Emergency
Medicine
Notice
Medicine is an ever-changing science. As new research and clinical experience broaden
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Atlas of Pediatric
Emergency SECOND EDITION

Medicine Editor-in-Chief
Binita R. Shah, MD, FAAP
Professor of Pediatrics and Emergency Medicine
SUNY Downstate Medical Center
Director Emeritus, Pediatric Emergency Medicine
Kings County Hospital Center
Brooklyn, New York

Associate Editors
Michael Lucchesi, MD, FACEP John Amodio, MD
Chairman, Department of Emergency Medicine Professor of Radiology
SUNY Downstate Medical Center Chief, Pediatric Radiology
Chief Medical Officer Program Director Emeritus Radiology Residency
University Hospital of Brooklyn SUNY Downstate Medical Center
Brooklyn, New York Kings County Hospital Center
Brooklyn, New York

Assistant Editor
Mark Silverberg, MD, MMB, FACEP
Associate Professor of Emergency Medicine
Associate Residency Director
Department of Emergency Medicine
SUNY Downstate Medical Center
Kings County Hospital Center
Brooklyn, New York

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To my grandchildren Ariya, Roshni, and Kailen
For bringing heaven on earth to my life.
To my children Ronak & Kunal and Toral & Vikas
For carrying the torch of serving humanity through the practice of medicine.
To my husband Rajni P. Shah, MD
For serving as my backbone in pursuit of my professional career for the past 40 years.
To my parents Chanchalben and Ratilal P. Patel, MD
You were my greatest inspiration and strength – I am everything because of you.
Binita R. Shah, MD, FAAP

To Mom and Dad: I am glad that you are in a better place. My memories of you inspire
me and will sustain me until we meet again. To Mike, Jess, and John: I am so fortunate
to have you all in my life. I am so proud of each of you, the men and woman you’ve
become. You are my source of strength and I will continue to work each day to make
you proud of me. To B: How do you thank someone who has saved you time and time
again? You are my best friend, my soul mate, my love. I have learned so much from
you and as I walk through the remainder of my journey, it is a comfort to know that
you and I will be taking each step together.
Michael Lucchesi, MD, FACEP

This book is dedicated to my family, as well as all of my colleagues and residents, past,
present, and future.
John Amodio, MD

I would like to dedicate this book to all of the residents in our amazing Emergency
Medicine training program at SUNY Downstate and Kings County Hospital. They spend
endless hours working hard and seeing patients and sending me MR numbers to make
my job just a little bit easier. Without them, I would never have had such a fabulous
collection of clinical images and radiographs. I would also like to dedicate this book to
my mentors who “raised me” in the field of Emergency Medicine, Dr. Michael Lucchesi,
who was my Program Director, and Dr. Lewis Kohl. Thank you for your inspiration and
motivation.
Mark Silverberg, MD, MMB, FACEP
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CONTENTS

Contributing Authors ................................................... xv Chapter 3


Photo Contributors ..................................................... xxi INFECTIOUS DISEASES ..............................59
Preface ..................................................................... xxix
Vikas S. Shah ■ Binita R. Shah
Acknowledgments .................................................... xxxi
STREPTOCOCCAL PHARYNGITIS AND SCARLET FEVER .........60
Chapter 1 IMPETIGO .............................................................................64
CHILD MALTREATMENT .............................1 ECTHYMA.............................................................................66
Konstantinos Agoritsas ■ Mary Birmingham ERYSIPELAS ..........................................................................68
NECROTIZING FASCIITIS .......................................................70
CHILD ABUSE .........................................................................2 ECTHYMA GANGRENOSUM .................................................72
CUTANEOUS MANIFESTATIONS OF TOXIC SHOCK SYNDROME ...................................................74
CHILD ABUSE ......................................................................6 STAPHYLOCOCCAL SCALDED SKIN SYNDROME..................78
INFLICTED BURNS ...................................................................8 MENINGOCOCCEMIA ..........................................................82
SKELETAL MANIFESTATIONS OF CHILD ABUSE ...................11 ROCKY MOUNTAIN SPOTTED FEVER.....................................86
FEMUR FRACTURES ...............................................................15 CAT-SCRATCH DISEASE ........................................................88
RIB FRACTURES DUE TO ABUSE ............................................16 CHICKENPOX .......................................................................90
SKULL FRACTURES DUE TO ABUSE.......................................18 HERPES ZOSTER ...................................................................92
ABUSIVE HEAD TRAUMA .....................................................20 OROPHARYNGEAL HERPES SIMPLEX VIRUS INFECTION .......94
INFANTICIDE VERSUS SUDDEN HERPETIC WHITLOW ............................................................96
INFANT DEATH SYNDROME ...............................................22 MUMPS ................................................................................97
ROSEOLA INFANTUM ..........................................................98
Chapter 2 MEASLES ...........................................................................100
CONDITIONS MISTAKEN FOR CHILD RUBELLA ............................................................................102
ABUSE AND SEXUAL ABUSE....................25 INFECTIOUS MONONUCLEOSIS .........................................104
Binita R. Shah ERYTHEMA INFECTIOSUM ..................................................108
HAND-FOOT-AND-MOUTH DISEASE .................................110
MONGOLIAN SPOTS ............................................................26 HERPANGINA .....................................................................112
FOLK HEALING PRACTICES...................................................28 CUTANEOUS LARVAE MIGRANS ........................................113
COLD PANNICULITIS ............................................................30 ASCARIASIS .......................................................................114
NEVI OF OTA AND ITO .........................................................32 MALARIA ...........................................................................116
PORT-WINE STAIN ................................................................34
PHYTOPHOTODERMATITIS ...................................................36 Chapter 4
RACCOON EYE .....................................................................38 SEXUAL ABUSE, GYNECOLOGY, AND
HAIR-TOURNIQUET SYNDROME ...........................................40 SEXUALLY TRANSMITTED INFECTIONS ...119
NUTRITIONAL VITAMIN D DEFICIENCY RICKETS ...................42 Amy Suss ■ Sarah A. Rawstron ■ Konstantinos Agoritsas
OSTEOGENESIS IMPERFECTA ................................................46
LICHEN SCLEROSUS ET ATROPHICUS ...................................49 SEXUAL ABUSE AND SEXUALLY
PERIANAL STREPTOCOCCAL DERMATITIS ............................50 TRANSMITTED INFECTIONS ..............................................120
LABIAL ADHESION ..............................................................52 CONDYLOMATA ACUMINATA ..........................................124
URETHRAL PROLAPSE ..........................................................54 TRICHOMONIASIS ..............................................................126
STRADDLE INJURY ................................................................56 CONGENITAL SYPHILIS ......................................................127

vii
viii ■ CONTENTS

PRIMARY AND SECONDARY SYPHILIS ................................130 PULMONARY TUBERCULOSIS.............................................210


GONORRHEA .....................................................................132 MILLIARY TUBERCULOSIS...................................................214
GENITAL HERPES ................................................................134 OBSTRUCTIVE SLEEP APNEA SYNDROME ...........................216
DYSFUNCTIONAL UTERINE BLEEDING ................................136 ASTHMA ............................................................................218
PELVIC INFLAMMATORY DISEASE ......................................138 PRIMARY SPONTANEOUS PNEUMOTHORAX .....................222
ECTOPIC PREGNANCY ........................................................140 PULMONARY EMBOLISM...................................................224

Chapter 5 Chapter 7
CARDIOLOGY .........................................143 DERMATOLOGY .....................................227
Shyam Sathanandam ■ Binita R. Shah Sharon A. Glick ■ Jeannette Jakus
Edited by Shyam Sathanandam ■ Falguni Asrani ■ Kunal M. Shah

Edited by Sharon A. Glick


BRADYCARDIA ...................................................................144
FIRST-DEGREE ATRIOVENTRICULAR BLOCK ........................146 URTICARIA .........................................................................228
SECOND-DEGREE ATRIOVENTRICULAR BLOCK ..................148 ANGIOEDEMA ...................................................................230
THIRD-DEGREE ATRIOVENTRICULAR BLOCK.......................150 SERUM SICKNESS/SERUM SICKNESS–LIKE REACTIONS ......232
LONG QT SYNDROME ........................................................152 ERYTHEMA MULTIFORME...................................................234
SYNCOPE............................................................................155 STEVENS-JOHNSON SYNDROME AND
SINUS TACHYCARDIA ........................................................158 TOXIC EPIDERMAL NECROLYSIS ......................................236
SUPRAVENTRICULAR TACHYCARDIA .................................160 DRUG-INDUCED HYPERSENSITIVITY SYNDROME ................240
ATRIAL FLUTTER .................................................................164 HENOCH-SCHÖNLEIN PURPURA........................................244
PREMATURE VENTRICULAR CONTRACTIONS ......................166 SEBORRHEIC DERMATITIS...................................................246
HYPERCYANOTIC SPELL OF TETRALOGY OF FALLOT ..........168 ALLERGIC CONTACT DERMATITIS ......................................248
DUCTAL-DEPENDENT CARDIAC ATOPIC DERMATITIS...........................................................250
LESIONS/HYPOPLASTIC LEFT HEART SYNDROME ............172 ECZEMA HERPETICUM .......................................................254
INFECTIVE ENDOCARDITIS ..................................................174 PSORIASIS ..........................................................................256
ACUTE PERICARDITIS ..........................................................176 GUTTATE PSORIASIS ...........................................................258
ACUTE MYOCARDITIS.........................................................178 PITYRIASIS ROSEA ..............................................................260
ACUTE RHEUMATIC FEVER .................................................180 GRANULOMA ANNULARE .................................................263
CHEST PAIN........................................................................182 SCABIES .............................................................................264
CONGESTIVE HEART FAILURE .............................................185 TINEA VERSICOLOR ............................................................268
TINEA CAPITIS ....................................................................270
Chapter 6 TINEA CORPORIS ................................................................274
RESPIRATORY DISORDERS ......................189 TINEA CRURIS .....................................................................276
Nooruddin R. Tejani ■ Diana E. Weaver ■ Ari J. Goldsmith TINEA PEDIS .......................................................................278
■ Haidy Marzouk ■ Jessica Stetz TINEA UNGUIUM................................................................280
WARTS ...............................................................................282
TRACHEOBRONCHIAL FOREIGN BODIES ............................190 MOLLUSCUM CONTAGIOSUM ..........................................284
PERTUSSIS ..........................................................................194 ACROPUSTULOSIS OF INFANCY .........................................286
CROUP ...............................................................................195 ERYTHEMA TOXICUM NEONATORUM ...............................287
BACTERIAL TRACHEITIS ......................................................198 EPIDERMOLYSIS BULLOSA .................................................288
EPIGLOTTITIS ......................................................................200 INFANTILE HEMANGIOMA ................................................290
BRONCHIOLITIS..................................................................202 MASTOCYTOSIS .................................................................293
BACTERIAL PNEUMONIA ...................................................205 CAFÉ AU LAIT SPOTS .........................................................296
VIRAL PNEUMONIA ...........................................................208 ERYTHEMA NODOSUM ......................................................298
CONTENTS ■ ix

ACANTHOSIS NIGRICANS ..................................................300 AURICULAR HEMATOMA ..................................................352


HIDRADENITIS SUPPURATIVA .............................................302 EPISTAXIS ...........................................................................354
TRANSIENT NEONATAL PUSTULAR MELANOSIS ...............304 NASAL FOREIGN BODIES ...................................................358
SUBCUTANEOUS FAT NECROSIS OF THE NEWBORN ..........305 ACUTE SINUSITIS ................................................................361
CANDIDA DIAPER DERMATITIS ..........................................306 CHRONIC SINUSITIS ...........................................................364
PERIORAL DERMATITIS .......................................................308 DEEP NECK SPACE INFECTION............................................367
PERITONSILLAR ABSCESS ...................................................370
Chapter 8 LEMIERRE SYNDROME........................................................372
OPHTHALMOLOGY ................................309
Douglas R. Lazzaro ■ Amy Kulak ■ Wayne Scott Chapter 10
Edited by Douglas R. Lazzaro SURGERY AND GENITOURINARY ............377
Jennifer H. Chao ■ Andrea Marmor
ORBITAL CELLULITIS ..........................................................310
PRESEPTAL CELLULITIS .......................................................313 INTUSSUSCEPTION .............................................................378
HORDEOLUM AND CHALAZION .......................................316 HYPERTROPHIC PYLORIC STENOSIS ....................................382
ACUTE CONJUNCTIVITIS .....................................................318 ACUTE NONPERFORATED APPENDICITIS ............................383
OPHTHALMIA NEONATORUM PERFORATED APPENDICITIS................................................385
(NEWBORN CONJUNCTIVITIS) ..........................................320 INCARCERATED INGUINAL HERNIA ...................................386
LEUKOCORIA .....................................................................322 TESTICULAR TORSION ........................................................388
INFANTILE GLAUCOMA .....................................................324 TESTICULAR TRAUMA ........................................................392
CORNEAL FOREIGN BODY..................................................326 EPIDIDYMO-ORCHITIS ........................................................394
CORNEAL ABRASION.........................................................327 TORSION OF THE APPENDIX TESTIS....................................396
CORNEAL ULCER................................................................328 VAGINAL FOREIGN BODY IN A PREPUBERTAL CHILD ........397
HYPOPYON ........................................................................329 CONGENITAL IMPERFORATE HYMEN
HYPHEMA ..........................................................................330 WITH HEMATOMETROCOLPOS .......................................398
SUBCONJUNCTIVAL HEMORRHAGE ...................................332 PARAPHIMOSIS..................................................................400
EYELID LACERATION ..........................................................333 OVARIAN TORSION............................................................402
TRAUMATIC GLOBE RUPTURE ............................................334 MALROTATION AND VOLVULUS .......................................404
CHEMICAL BURNS .............................................................336 UMBILICAL GRANULOMA .................................................405
UMBILICAL HERNIA ...........................................................406
Chapter 9
OTOLARYNGOLOGY ..............................339 Chapter 11
Sydney C. Butts ■ Nira Goldstein ■ Richard Rosenfeld HEMATOLOGY AND ONCOLOGY ..........407
■ Anita Konka ■ Matthew Hanson ■ Miguel Mascaro Scott T. Miller ■ Sreedhar P. Rao ■ Shahina Qureshi
■ Michael C. Singer ■ Christina DiLoreto ■ David H. Burstein
SICKLE CELL ANEMIA WITH FEVER.....................................408
■ Perminder S. Parmar ■ Marika Fraser ■ Haidy Marzouk
SICKLE CELL ANEMIA WITH ACUTE PAIN ..........................410
■ Jessica W. Lim ■ Nur-Ain Nadir ■ Jennifer H. Chao
SICKLE CELL ANEMIA WITH ACUTE CHEST SYNDROME.....414
Edited by Sydney C. Butts
SICKLE CELL ANEMIA AND
ACUTE OTITIS MEDIA .........................................................340 CEREBROVASCULAR DISEASE ..........................................417
COMPLICATIONS OF OTITIS MEDIA ...................................343 SICKLE CELL ANEMIA WITH PRIAPISM ...............................420
OTITIS EXTERNA .................................................................346 SICKLE CELL ANEMIA WITH
PERICHONDRITIS ................................................................348 RIGHT UPPER QUADRANT SYNDROME ............................422
AURICULAR FOREIGN BODY...............................................350 SICKLE CELL ANEMIA AND
TRANSIENT APLASTIC CRISIS............................................426
x ■ CONTENTS

SICKLE CELL ANEMIA AND Chapter 14


ACUTE SPLENIC SEQUESTRATION CRISIS .........................428
ENDOCRINOLOGY..................................515
NEWLY DIAGNOSED IMMUNE THROMBOCYTOPENIA.......430
Glenn D. Harris ■ Irma Fiordalisi
HEMOPHILIA .....................................................................432
■ Irene Mamkin ■ Sonal Bhandari
ABDOMINAL MASS ...........................................................434
LYMPHADENOPATHY ........................................................438 DIABETIC KETOACIDOSIS ...................................................516
OSTEOSARCOMA...............................................................442 ADDISON DISEASE.............................................................522
EWING SARCOMA .............................................................446 CONGENITAL ADRENAL HYPERPLASIA..............................524
ACUTE LYMPHOBLASTIC LEUKEMIA ..................................449 HYPOTHYROIDISM AND MYXEDEMA ................................526
OVARIAN TUMORS ............................................................451 THYROID STORM AND THYROTOXICOSIS ..........................528

Chapter 12 Chapter 15
RHEUMATOLOGY...................................453 GASTROINTESTINAL DISORDERS ............531
Binita R. Shah Kanwal S. Chaudhry
KAWASAKI DISEASE ..........................................................454 ESOPHAGEAL FOREIGN BODIES ........................................532
SYSTEMIC LUPUS ERYTHEMATOSUS...................................458 BATTERY INGESTIONS ........................................................536
NEONATAL LUPUS .............................................................462 RETAINED BONES...............................................................540
RAYNAUD PHENOMENON ................................................464 STOMACH AND LOWER GASTROINTESTINAL
LYME DISEASE ...................................................................466 TRACT FOREIGN BODIES ..................................................542
SHARP OBJECT FOREIGN BODY ..........................................544
Chapter 13 ANORECTAL FOREIGN BODIES ...........................................546
NEUROLOGY ..........................................469 CONSTIPATION ..................................................................548
Geetha Chari ■ Vikas S. Shah ■ Binita R. Shah GASTROINTESTINAL BLEEDING ..........................................550
Edited by Geetha Chari
Chapter 16
BACTERIAL MENINGITIS .....................................................470
NEPHROLOGY ........................................555
RAISED INTRACRANIAL PRESSURE .....................................474
Anil K. Mongia ■ Anup Singh
ALTERED MENTAL STATUS .................................................478
FEBRILE SEIZURES ...............................................................482 PROTEINURIA .....................................................................556
SEIZURES AND EPILEPSY ....................................................484 HEMATURIA .......................................................................558
STATUS EPILEPTICUS ..........................................................487 MINIMAL CHANGE NEPHROTIC SYNDROME .....................561
STROKE IN CHILDREN.........................................................490 ACUTE POSTSTREPTOCOCCAL GLOMERULONEPHRITIS .....564
NEUROCYSTICERCOSIS .......................................................493 URINARY TRACT INFECTION ...............................................566
BELL PALSY ........................................................................496 HEMOLYTIC UREMIC SYNDROME.......................................568
HEADACHE ........................................................................498 OBSTRUCTIVE UROPATHY ..................................................570
BRAIN TUMORS..................................................................501 HYPERTENSIVE EMERGENCIES ............................................572
HYDROCEPHALUS ..............................................................504 NEPHROLITHIASIS ..............................................................574
SPINAL CORD LESIONS (NON-TRAUMATIC) ......................508
GUILLAIN-BARRÉ SYNDROME ............................................511
MYASTHENIA GRAVIS ........................................................513
CONTENTS ■ xi

Chapter 17 Chapter 18
TOXICOLOGY .........................................577 ENVIRONMENTAL EMERGENCIES...........635
Ronak R. Shah ■ Mae De La Calzada-Jeanlouie Mark Silverberg ■ Teresa Bowen-Spinelli
■ Rachel S. Weiselberg ■ Mark Su ■ Chaiya Laoteppitaks
Edited by Mark Su
THERMAL BURNS ...............................................................636
THE POISONED PATIENT: GENERAL APPROACH PERIPHERAL COLD INJURIES ...............................................640
AND GASTROINTESTINAL DECONTAMINATION ..............578 ANAPHYLAXIS ...................................................................643
ACUTE ACETAMINOPHEN TOXICITY...................................585 BROWN RECLUSE SPIDER ENVENOMATION.......................646
ACUTE SALICYLATE TOXICITY ............................................588 BLACK WIDOW SPIDER ENVENOMATION..........................648
IRON TOXICITY ...................................................................590 SCORPION ENVENOMATION .............................................649
OPIOID TOXICITY................................................................592 DOG BITES..........................................................................650
COCAINE TOXICITY ............................................................594 RABIES EXPOSURE AND VACCINE PROPHYLAXIS ..............652
METHEMOGLOBINEMIA ....................................................596 CAT BITES ...........................................................................654
ORGANOPHOSPHATE AND HUMAN BITES ....................................................................656
CARBAMATE INSECTICIDE TOXICITY ................................598 VENOMOUS MARINE ANIMALS:
CASTOR BEAN AND ROSARY PEA INGESTION ...................600 STINGING MARINE ANIMALS—CORALS, SEA
PHENOTHIAZINE TOXICITY ................................................602 ANEMONES, HYDROIDS, AND FIRE CORAL .....................658
ANTICHOLINERGIC DRUG AND PLANT TOXICITY ...............604 STINGING MARINE ANIMALS: BOX JELLYFISH,
ACUTE ISONIAZID TOXICITY...............................................606 TRUE JELLYFISH, AND PORTUGUESE MAN-OF-WAR ........660
CAMPHOR TOXICITY ..........................................................608 STINGING MARINE ANIMALS:
HYDROCARBON AND NAPHTHALENE TOXICITY................609 SPONGES (PHYLUM PORIPHERA) .....................................662
ECSTASY TOXICITY .............................................................612 STABBING MARINE ANIMALS: STARFISH,
KETAMINE TOXICITY...........................................................614 SEA URCHINS, AND CROWN-OF-THORNS STARFISH .......663
DEXTROMETHORPHAN TOXICITY .......................................615 STABBING MARINE ANIMALS: STONEFISH AND
MARIJUANA TOXICITY........................................................616 SCORPIONFISH (FAMILY SCORPAENIDAE) .......................664
ETHYLENE GLYCOL TOXICITY .............................................618 STABBING MARINE ANIMALS: STINGRAY ..........................666
METHANOL TOXICITY ........................................................620 STABBING MARINE ANIMALS:
ETHANOL TOXICITY ...........................................................622 CONE SHELLS (FAMILY CONIDAE)...................................667
PRESCRIPTION DRUGS OF ABUSE .......................................624 BITING MARINE ANIMALS:
BETA-BLOCKERS AND BLUE-RINGED OCTOPUS ..................................................668
CALCIUM CHANNEL BLOCKER TOXICITY .........................626 LIGHTNING INJURIES ..........................................................669
CLONIDINE TOXICITY .........................................................628 SNAKE ENVENOMATIONS .................................................670
CYCLIC ANTIDEPRESSANT TOXICITY ...................................629 ALTITUDE SICKNESS ...........................................................673
LEAD TOXICITY...................................................................631
CAUSTICS ...........................................................................633
xii ■ CONTENTS

Chapter 19 SLIPPED CAPITAL FEMORAL EPIPHYSIS ..............................727


ORTHOPEDICS ........................................675 LEGG-CALVÉ-PERTHES DISEASE .........................................728
Michael Lucchesi ■ Rita Nathawad ■ Diana E. Weaver ANTERIOR CRUCIATE LIGAMENT TEAR...............................729
OSGOOD-SCHLATTER DISEASE ..........................................730
FRACTURES AND FRACTURE CLASSIFICATION ....................676 ACHILLES TENDON RUPTURE .............................................731
TUFT FRACTURES ................................................................685 FOREIGN BODIES AND PENETRATING INJURIES ..................732
PHALANX FRACTURES .......................................................686 HAND INFECTIONS: PARONYCHIA .....................................734
MALLET FINGER .................................................................688 FLEXOR TENOSYNOVITIS ....................................................735
BENNETT FRACTURE AND ROLANDO FRACTURE ................689 BONE AND JOINT INFECTIONS:
BOXER’S FRACTURE ............................................................690 ACUTE OSTEOMYELITIS ...................................................736
FRACTURES OF CARPAL BONES CHRONIC OSTEOMYELITIS .................................................738
(SCAPHOID OR NAVICULAR FRACTURE) ..........................691 OSTEOMYELITIS IN SICKLE CELL DISEASE ..........................740
DISTAL RADIUS FRACTURES ...............................................692 SEPTIC ARTHRITIS ...............................................................742
GALEAZZI FRACTURE DISLOCATION AND NEONATAL OSTEOMYELITIS AND SEPTIC ARTHRITIS .........744
MONTEGGIA FRACTURE DISLOCATION ...........................693 SPINAL TUBERCULOSIS (POTT DISEASE) .............................746
OLECRANON FRACTURE ....................................................694
RADIAL HEAD FRACTURE ...................................................695 Chapter 20
SUPRACONDYLAR FRACTURE .............................................696 TRAUMA ................................................749
HUMERUS FRACTURE .........................................................698 Karen Santucci ■ Bonny J. Baron ■ Sydney C. Butts
CLAVICLE FRACTURE ..........................................................700 ■ Audrey J. Tan ■ Behrad Aynehchi ■ LaMont Jones
SCAPULAR FRACTURE ........................................................701 ■ Baljeet Kaur Purewal ■ Christopher I. Doty
FEMUR FRACTURE...............................................................702
■ Chaiya Laoteppitaks ■ Cynthia L. Benson ■ Ee Tein Tay
PATELLA FRACTURES .........................................................704
■ Ilaaf Darrat ■ Michael Lucchesi ■ Roman Shinder
MAISONNEUVE FRACTURE .................................................705
ANKLE FRACTURE AND SPRAIN .........................................706 HEAD TRAUMA ..................................................................750
CALCANEAL FRACTURE .....................................................708 SKULL FRACTURES..............................................................753
JONES FRACTURE................................................................709 BASILAR SKULL FRACTURE.................................................754
LISFRANC FRACTURE ..........................................................710 ACUTE SUBDURAL HEMATOMA ........................................756
STRESS FRACTURE...............................................................711 EPIDURAL HEMATOMA .....................................................758
DISLOCATIONS: CEREBRAL CONTUSION......................................................759
INTERPHALANGEAL JOINT DISLOCATION .......................712 CEREBRAL CONCUSSION ...................................................760
LUNATE AND PERILUNATE DISLOCATIONS .......................714 NASAL SEPTAL HEMATOMA .............................................761
ELBOW DISLOCATION .......................................................715 NASAL FRACTURES ............................................................762
ANTERIOR AND POSTERIOR GLENOHUMERAL MANDIBLE FRACTURES ......................................................764
JOINT DISLOCATIONS ......................................................716 MIDFACIAL FRACTURES (MAXILLARY/LE FORT,
HIP DISLOCATION ..............................................................718 FRONTAL SINUS, NASOORBITAL ETHMOID)....................767
KNEE DISLOCATION...........................................................719 ORBITAL TRAUMA .............................................................770
ANKLE DISLOCATION ........................................................720 FRACTURES OF THE ZYGOMATIC COMPLEX
PATELLA DISLOCATION .....................................................722 AND ZYGOMATIC ARCH..................................................772
MUSCULOSKELETAL INJURIES: SOFT TISSUE TRAUMA ........................................................774
GAMEKEEPER’S THUMB ...................................................723 LIP LACERATION INVOLVING
NURSEMAID’S ELBOW .......................................................724 THE VERMILION BORDER .................................................777
ACROMIOCLAVICULAR INJURIES .......................................726 TONGUE LACERATION .......................................................778
CONTENTS ■ xiii

TRAUMATIC TEMPOROMANDIBULAR SPLENIC INJURIES ...............................................................812


JOINT DISLOCATION ........................................................779 HEPATIC INJURIES...............................................................814
PENETRATING NECK INJURY ...............................................780 OTHER INTRA-ABDOMINAL TRAUMA
CERVICAL SPINE INJURIES ..................................................782 INCLUDING SEATBELT INJURIES .......................................815
JEFFERSON (4-PART) BURST FRACTURES..............................784 PELVIC FRACTURES .............................................................817
ODONTOID FRACTURES .....................................................785 RING INCARCERATION .......................................................819
TRAUMATIC SPONDYLOLISTHESIS OF C2...........................786 NAIL BED INJURY................................................................820
EXTENSION TEARDROP FRACTURE .....................................787 SUBUNGUAL HEMATOMA ................................................822
ANTERIOR FLEXION FRACTURES DIGITAL AMPUTATION.......................................................824
(FLEXION TEARDROP) ......................................................788 DEGLOVING INJURY ...........................................................826
ATLANTO-OCCIPITAL DISLOCATION .................................789 PENETRATING HAND/FOOT INJURY ....................................828
SPINAL CORD INJURY WITHOUT TRAUMATIC EXTREMITY AMPUTATION ..............................829
RADIOGRAPHIC ABNORMALITY (SCIWORA) ...................790 FISHHOOK REMOVAL ........................................................830
BILATERAL FACET DISLOCATION .......................................791 ZIPPER INJURY ....................................................................831
UNILATERAL FACET DISLOCATION ....................................792
ANTERIOR SUBLUXATION ..................................................793 Chapter 21
SPINAL PROCESS EMERGENCY ULTRASOUND ...................833
(CLAY SHOVELER’S) FRACTURE ........................................794 Dimitrios Papanagnou ■ Michael Secko
DENTAL CARIES AND
DENTOALVEOLAR ABSCESS ............................................795 TRAUMA ............................................................................834
DENTAL TRAUMA ..............................................................796 APPENDICITIS .....................................................................837
THORACIC TRAUMA: INTUSSUSCEPTION .............................................................838
RIB FRACTURES AND FLAIL CHEST ...................................798 PYLORIC STENOSIS .............................................................839
PNEUMOTHORAX AND BILIARY ULTRASOUND .......................................................840
TENSION PNEUMOTHORAX.............................................800 FIRST TRIMESTER PREGNANCY ............................................842
HEMOTHORAX ..................................................................802 PEDIATRIC LUNG ULTRASOUND ........................................844
PULMONARY CONTUSION .................................................804 ABSCESSES AND CELLULITIS ..............................................846
TRAUMATIC AIRWAY OBSTRUCTION .................................805 PEDIATRIC HIP ULTRASOUND ............................................848
AORTIC DISRUPTION ..........................................................806 PEDIATRIC FRACTURES .......................................................849
TRAUMATIC DIAPHRAGM RUPTURE ...................................807
TRACHEOBRONCHIAL TREE INJURY....................................808 Index ......................................................................... 851
ABDOMINAL TRAUMA ......................................................809
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CONTRIBUTING AUTHORS

Konstantinos Agoritsas, MD David H. Burstein, MD


Director, Pediatric Emergency Medicine Fellow, Facial Plastic Surgery Associates
Kings County Hospital Center University of Texas Medical School
Director, Pediatric Emergency Medicine Fellowship Health Science Center at Houston
SUNY Downstate Medical Center Houston, Texas
Brooklyn, New York Chapter 9
Chapters 1, 4
Sydney C. Butts, MD
Falguni Asrani, MD Chief, Facial Plastic and Reconstructive Surgery
Pediatric Dermatology Fellow Assistant Professor of Otolaryngology
Department of Dermatology Department of Otolaryngology
SUNY Downstate Medical Center SUNY Downstate Medical Center
Brooklyn, New York Brooklyn, New York
Chapter 7 Chapters 9, 20
Behrad Aynehchi, MD Jennifer H. Chao, MD, FAAP
Clinical Assistant Instructor Associate Director, Pediatric Emergency Medicine Fellowship
Department of Otolaryngology Clinical Assistant Professor
SUNY Downstate Medical Center SUNY Downstate Medical Center
Brooklyn, New York Kings County Hospital Center
Chapter 20 Brooklyn, New York
Chapters 9, 10
Bonny J. Baron, MD
Associate Professor of Emergency Medicine Geetha Chari, MD
Department of Emergency Medicine Associate Professor of Neurology and Pediatrics
SUNY Downstate Medical Center Program Director
Kings County Hospital Center Clinical Neurophysiology Fellowship
Brooklyn, New York SUNY Downstate Medical Center
Chapter 20 Kings County Hospital Center
Brooklyn, New York
Cynthia L. Benson, DO, MPH Chapter 13
Clinical Assistant Instructor
Department of Emergency Medicine Kanwal S. Chaudhry, MD
SUNY Downstate Medical Center Southside Hospital
Brooklyn, New York Department of Emergency Medicine
Chapter 20 North Shore–Long Island Jewish Health System
Bay Shore, New York
Sonal Bhandari, MD Chapter 15
Assistant Professor of Pediatrics
Division of Pediatric Endocrinology Ilaaf Darrat, MD
Infants and Children’s Hospital of Brooklyn at Maimonides Clinical Instructor
Medical Center and Department of Otolaryngology
SUNY Downstate Medical Center Henry Ford Hospital
Brooklyn, New York Detroit, Michigan
Chapter 14 Chapter 20
Mary Birmingham, MD Mae De La Calzada-Jeanlouie, DO
Assistant Professor of Pediatrics Medical Toxicology Fellow
Weill–Cornell Medical College Department of Emergency Medicine
Assistant Attending Physician North Shore University Hospital
New York Presbyterian/Weill–Cornell Medical Center Manhasset, New York
New York, New York Chapter 17
Chapter 1
Christina DiLoreto, MD
Teresa Bowen-Spinelli, MD Clinical Assistant Instructor
Assistant Professor of Emergency Medicine Department of Otolaryngology
SUNY Downstate Medical Center SUNY Downstate Medical Center
Brooklyn, New York Brooklyn, New York
Chapter 18 Chapter 9

xv
xvi ■ CONTRIBUTING AUTHORS

Christopher I. Doty, MD, FAAEM, FACEP Glenn D. Harris, MD, CDE


Associate Professor of Emergency Medicine Professor of Pediatrics
Residency Program Director Section of Endocrinology and Diabetes
Department of Emergency Medicine East Carolina University, Brody School of Medicine
SUNY Downstate Medical Center Greenville, North Carolina
Kings County Hospital Center Chapter 14
Brooklyn, New York
Chapter 20 Jeannette Jakus, MD, MBA
Pediatric Dermatology Fellow
Irma Fiordalisi, MD Department of Dermatology
Professor of Pediatrics SUNY Downstate Medical Center
Section of Critical Care Brooklyn, New York
East Carolina University, Brody School of Medicine Chapter 7
Director, Pediatric ICU and Sedation Services
Children’s Hospital of Eastern North Carolina LaMont Jones, MD
Greenville, North Carolina Vice Chairman
Chapter 14 Director, Cleft and Craniofacial Clinic
Department of Otolaryngology-Head and Neck Surgery
Marika Fraser, MD Henry Ford Hospital
Clinical Assistant Instructor Detroit, Michigan
Department of Otolaryngology Chapter 20
SUNY Downstate Medical Center
Brooklyn, New York Anita Konka, MD, MPH
Chapter 9 Clinical Assistant Instructor
Department of Otolaryngology
Sharon A. Glick, MD, MS SUNY Downstate Medical Center
Associate Professor of Dermatology and Pediatrics Brooklyn, New York
Residency Program Director Chapter 9
Department of Dermatology
SUNY Downstate Medical Center Amy Kulak, MD
Director of Pediatric Dermatology Clinical Instructor
SUNY Downstate Medical Center Department of Ophthalmology
Kings County Hospital Center SUNY Downstate Medical Center
Maimonides Medical Center Brooklyn, New York
Brooklyn, New York Chapter 8
Chapter 7
Chaiya Laoteppitaks, MD
Ari J. Goldsmith, MD Assistant Program Director
Associate Professor Department of Emergency Medicine
SUNY Downstate Medical Center Albert Einstein Medical Center
Attending, Division of Otolaryngology Philadelphia, Pennsylvania
Maimonides Medical Center Chapters 18, 20
Brooklyn, New York
Chapter 6
Douglas R. Lazzaro, MD, FACS
Professor and Chairman
Nira Goldstein, MD, MPH Department of Ophthalmology
Associate Professor of Otolaryngology The Richard C. Troutman, MD Distinguished Chair in
Department of Otolaryngology Ophthalmology and Ophthalmic Microsurgery
SUNY Downstate Medical Center SUNY Downstate Medical Center
Brooklyn, New York Director of Service
Chapter 9 Kings County Hospital Center/University Hospital of Brooklyn
Brooklyn, New York
Matthew Hanson, MD Chapter 8
Assistant Professor of Otolaryngology
Department of Otolaryngology
SUNY Downstate Medical Center
Brooklyn, New York
Chapter 9
CONTRIBUTING AUTHORS ■ xvii

Jessica W. Lim, MD Nur-Ain Nadir, MD


Assistant Professor of Otolaryngology Clinical Assistant Instructor
Department of Otolaryngology Medical Simulation Resident Director
SUNY Downstate Medical Center Department of Emergency Medicine
Brooklyn, New York SUNY Downstate Medical Center
Chapter 9 Brooklyn, New York
Chapter 9
Michael Lucchesi, MD, FACEP
Chairman, Department of Emergency Medicine Rita Nathawad, MD
SUNY Downstate Medical Center Pediatric Infectious Disease Fellow
Chief Medical Officer Department of Pediatrics
University Hospital of Brooklyn SUNY Downstate Medical Center
Brooklyn, New York Brooklyn, New York
Chapters 19, 20 Chapter 19
Irene Mamkin, MD Dimitrios Papanagnou, MD, MPH, RDMS
Pediatric Endocrinology Attending Director, Medical Simulation and
Saint Barnabas Medical Center Advanced Learning Resource Center
Livingston, New Jersey Assistant Clinical Professor
Chapter 14 Department of Emergency Medicine
SUNY Downstate Medical Center
Andrea Marmor, MD, MSEd Brooklyn, New York
Associate Professor of Clinical Pediatrics Chapter 21
University of California, San Francisco
San Francisco General Hospital Perminder S. Parmar, MD, FACS
San Francisco, California Attending Physician
Chapter 10 Otolaryngology and Head and Neck Surgery
Scripps Clinical Medical Group
Haidy Marzouk, MD La Jolla, California
Attending Physician Chapter 9
Department of Otolaryngology
North Shore–Long Island Jewish Medical Center Baljeet Kaur Purewal, MD
Great Neck, New York Assistant Professor of Ophthalmology
Chapters 6, 9 Department of Ophthalmology
SUNY Downstate Medical Center
Miguel Mascaro, MD Brooklyn, New York
Clinical Assistant Instructor Chapter 20
Department of Otolaryngology
SUNY Downstate Medical Center Shahina Qureshi, MD
Brooklyn, New York Associate Professor of Pediatrics
Chapter 9 Division of Hematology/Oncology
SUNY Downstate Medical Center
Scott T. Miller, MD Kings County Hospital Center
Professor of Clinical Pediatrics Brooklyn, New York
Director, Division of Pediatric Hematology/Oncology Chapter 11
SUNY Downstate Medical Center
Kings County Hospital Center Sreedhar P. Rao, MD
Brooklyn, New York Professor of Clinical Pediatrics
Chapter 11 Director Emeritus
Division of Pediatric Hematology/Oncology
Anil K. Mongia, MD SUNY Downstate Medical Center
Assistant Professor of Pediatrics Brooklyn, New York
Director, Pediatric Dialysis Unit Chapter 11
Division of Pediatric Nephrology
SUNY Downstate Medical Center
Brooklyn, New York
Chapter 16
xviii ■ CONTRIBUTING AUTHORS

Sarah A. Rawstron, MBBS Kunal M. Shah, MD


Associate Professor of Clinical Pediatrics Attending Physician
Weill Medical College of Cornell University Allergy, Asthma and Immunology
Attending, Pediatric Infectious Diseases Mayo Clinic Health System-Mankato
Program Director, Pediatric Residency Program Mankato, Minnesota
Brooklyn Hospital Center Chapter 7
Brooklyn, New York
Chapter 4 Ronak R. Shah, MD
Medical Director of Emergency Medicine
Richard Rosenfeld, MD, MPH Mayo Clinic Health System-Waseca
Professor and Chairman Attending Physician of Emergency Medicine
Department of Otolaryngology Mayo Health System-Mankato
SUNY Downstate Medical Center Mankato, Minnesota
Brooklyn, New York Chapter 17
Chapter 9
Vikas S. Shah, MD
Karen Santucci, MD Pediatric Intensivist
Associate Professor of Pediatrics Kings County Hospital Center
Department of Pediatrics Clinical Assistant Professor
Medical Director and Section Chief SUNY Downstate Medical Center
Pediatric Emergency Medicine Brooklyn, New York
Yale-New Haven Children’s Hospital Chapters 3, 13
New Haven, Connecticut
Chapter 20 Roman Shinder, MD
Assistant Professor of Ophthalmology
Shyam Sathanandam, MD Department of Ophthalmology
Assistant Professor of Pediatrics SUNY Downstate Medical Center
Division of Pediatric Cardiology Brooklyn, New York
Section of Interventional Cardiology Chapter 20
University of Tennessee
Le Bonheur Children’s Hospital Mark Silverberg, MD, MMB, FACEP
Memphis, Tennessee Associate Professor of Emergency Medicine
Chapter 5 Associate Residency Director
Department of Emergency Medicine
Wayne Scott, MD SUNY Downstate Medical Center
Clinical Assistant Professor Kings County Hospital Center
Department of Ophthalmology Brooklyn, New York
SUNY Downstate Medical Center Chapter 18
Kings County Hospital Center
Brooklyn, New York Michael C. Singer, MD
Chapter 8 Director, Division of Thyroid and Parathyroid Surgery
Department of Otolaryngology-Head and Neck Surgery
Michael Secko, MD, RDMS Henry Ford Hospital
Director, Emergency Ultrasound Division Detroit, Michigan
Assistant Clinical Professor Chapter 9
Department of Emergency Medicine
SUNY Downstate Medical Center Anup Singh, MD
Kings County Hospital Center Chief, Pediatric Nephrologist
Brooklyn, New York Department of Pediatrics
Chapter 21 The Children’s Hospital at Saint Peter’s University Hospital
Associate Professor of Pediatrics
Binita R. Shah, MD, FAAP Drexel University College of Medicine
Professor of Pediatrics and Emergency Medicine Philadelphia, Pennsylvania
SUNY Downstate Medical Center Chapter 16
Director Emeritus, Pediatric Emergency Medicine
Kings County Hospital Center
Brooklyn, New York
Chapters 2, 3, 5, 12, 13
CONTRIBUTING AUTHORS ■ xix

Jessica Stetz, MD, MS Ee Tein Tay, MD


Assistant Professor of Emergency Medicine Assistant Professor
Department of Emergency Medicine Departments of Emergency Medicine and Pediatrics
SUNY Downstate Medical Center Mount Sinai Medical Center
Brooklyn, New York New York, New York
Chapter 6 Chapter 20

Mark Su, MD Nooruddin R. Tejani, MD, FAAP


Assistant Professor of Emergency Medicine Director, Pediatric Emergency Medicine
Department of Emergency Medicine Assistant Professor
Hofstra North Shore–LIJ School of Medicine at Hofstra University Department of Emergency Medicine
Director, Fellowship in Medical Toxicology SUNY Downstate Medical Center
North Shore University Hospital Brooklyn, New York
Manhasset, New York Chapter 6
Chapter 17
Diana E. Weaver, MD, FAAP
Amy Suss, MD Assistant Professor of Pediatrics
Director, Division of Adolescent Medicine Department of Pediatrics
Associate Director, Pediatric Residency Program SUNY Downstate Medical Center
Associate Professor of Clinical Pediatrics Director, Pediatric Asthma Program
Department of Pediatrics Acting Director, Pediatric Pulmonology
SUNY Downstate Medical Center Kings County Hospital Center
Brooklyn, New York Brooklyn, New York
Chapter 4 Chapters 6, 19

Audrey J. Tan, DO Rachel S. Weiselberg, MD


Clinical Assistant Instructor Medical Toxicology Fellow
Department of Emergency Medicine Department of Emergency Medicine
SUNY Downstate Medical Center North Shore University Hospital
Brooklyn, New York Manhasset, New York
Chapter 20 Chapter 17
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PHOTO CONTRIBUTORS

Konstantinos Agoritsas, MD Amrit Bhangoo, MD


Director, Pediatric Emergency Medicine Assistant Professor of Pediatrics
Kings County Hospital Center Division of Pediatric Endocrinology
Director, Pediatric Emergency Medicine Fellowship SUNY Downstate Medical Center
SUNY Downstate Medical Center Kings County Hospital
Brooklyn, New York Brooklyn, New York
John Amodio, MD Loring Bjornson, PhD, DABCC, FACB
Professor of Radiology Clinical Chemist
Chief, Pediatric Radiology Department of Pathology and Laboratory Medicine
Program Director Emeritus Radiology Residency North Shore University Hospital
SUNY Downstate Medical Center Manhasset, New York
Kings County Hospital Center
Brooklyn, New York David H. Burstein, MD
Fellow, Facial Plastic Surgery Associates
Angela C. Anderson, MD University of Texas Medical School
Director, Pediatric Pain and Palliative Care Health Science Center at Houston
Consultant, Clinical Toxicology and Pharmacology Houston, Texas
Hasbro Children’s Hospital
Associate Professor of Pediatrics and Emergency Medicine Sydney C. Butts, MD
The Warren Alpert School of Medicine at Brown University Chief, Facial Plastic and Reconstructive Surgery
Providence, Rhode Island Assistant Professor of Otolaryngology
Department of Otolaryngology
Falguni Asrani, MD SUNY Downstate Medical Center
Pediatric Dermatology Fellow Brooklyn, New York
Department of Dermatology
SUNY Downstate Medical Center Mae De La Calzada-Jeanlouie, DO
Brooklyn, New York Medical Toxicology Fellow
Department of Emergency Medicine
Anika Bakster, MD North Shore University Hospital
Clinical Assistant Instructor Manhasset, New York
Department of Emergency Medicine
SUNY Downstate Medical Center Julie Cantatore-Francis, MD
Brooklyn, New York Volunteer Faculty, Department of Dermatology
SUNY Downstate Medical Center
Carlos Barahona, MD Brooklyn, New York
Pediatric Emergency Medicine Fellow
SUNY Downstate Medical Center Charles Catanese, MD
Kings County Hospital Chief Medical Examiner
Brooklyn, New York Orange County, New York
Kirsten Bechtel, MD Haamid Chamdawala, MD
Associate Professor of Pediatrics Chief Resident
Yale School of Medicine Department of Pediatrics
Attending Physician SUNY Downstate Medical Center
Pediatric Emergency Department Brooklyn, New York
Yale–New Haven Children’s Hospital
New Haven, Connecticut Jennifer H. Chao, MD, FAAP
Associate Director, Pediatric Emergency Medicine Fellowship
Cynthia L. Benson, DO, MPH Clinical Assistant Professor
Clinical Assistant Instructor SUNY Downstate Medical Center
Department of Emergency Medicine Kings County Hospital Center
SUNY Downstate Medical Center Brooklyn, New York
Brooklyn, New York

xxi
xxii ■ PHOTO CONTRIBUTORS

Geetha Chari, MD David S. Dinhofer, MD


Associate Professor of Neurology and Pediatrics Clinical Assistant Professor of Radiology
Program Director Head, Emergency Radiology
Clinical Neurophysiology Fellowship SUNY Downstate Medical Center
SUNY Downstate Medical Center Brooklyn, New York
Kings County Hospital Center
Brooklyn, New York Donita Dyalram, MD, DDS
Fellow of Head and Neck Oncology,
Nancy Chase, MD Microvascular Reconstruction
Associate Professor of Pediatrics Department of Oral Maxillofacial Surgery
Division of Pediatric Cardiology University of Maryland Medical Center
University of Tennessee Baltimore, Maryland
LeBonheur Children Hospital
Memphis, Tennessee Irma Fiordalisi, MD
Professor of Pediatrics
Kanwal S. Chaudhry, MD Section of Critical Care
Southside Hospital East Carolina University, Brody School of Medicine
Department of Emergency Medicine Director, Pediatric ICU and Sedation Services
North Shore-Long Island Jewish Health System Children’s Hospital of Eastern North Carolina
Bay Shore, New York Greenville, North Carolina

Thomas Chi, MD, RDMS, FACEP Rachael George, MD


Staff Physician Clinical Assistant Professor
Middlesex Hospital SUNY Downstate Medical Center
Middletown, Connecticut Kings County Hospital Center
Brooklyn, New York
Valeriy Chorny, MD, FAAP
Assistant Professor of Pediatrics Erin Gilbert, MD, PhD, FAAD
Department of Pediatrics Assistant Professor of Dermatology
SUNY Downstate Medical Center SUNY Downstate Medical Center
Brooklyn, New York Brooklyn, New York

Jonathan Cohen, MD M.G.F. Gilliland, MD


Clinical Assistant Instructor Professor
Department of Otolaryngology Assistant Director of Forensic Services and
SUNY Downstate Medical Center Pathology Course Director
Brooklyn, New York East Carolina University, Brody School of Medicine
Vidant Medical Center
John Cragg Greenville, North Carolina
Fish Rock Dive Centre
South West Rocks Radha Giridharan, MD
New South Wales, Australia Clinical Associate Professor of Neurology and Pediatrics
SUNY Downstate Medical Center
Jeanine Daly, MD Brooklyn, New York
Clinical Associate Professor of Dermatology
New York University Yvonne P. Giunta, MD, FAAP
New York, New York Division Director, Pediatric Emergency Medicine
Staten Island University Hospital
Dawn Davis, MD Assistant Professor of Emergency Medicine
Assistant Professor SUNY Downstate Medical Center
Departments of Dermatology and Pediatrics Brooklyn, New York
Mayo Clinic
Rochester, Minnesota

Silvia Delgado-Villalta, MD
Pediatric Pulmonary Fellow
Children’s National Medical Center
Washington, District of Columbia
PHOTO CONTRIBUTORS ■ xxiii

Sharon A. Glick, MD Barry Hahn, MD, FACEP


Associate Professor of Dermatology and Pediatrics Department of Emergency Medicine
Residency Program Director Staten Island University Hospital
Department of Dermatology Staten Island, New York
SUNY Downstate Medical Center
Director of Pediatric Dermatology Michael Hawke, MD
SUNY Downstate Medical Center Professor Emeritus
Kings County Hospital Center Department of Otolaryngology-Head and Neck Surgery
Maimonides Medical Center University of Toronto
Brooklyn, New York Toronto, Canada

Rachelle Goldfisher, MD Robert J. Hoffman, MD, MS


Assistant Professor of Radiology Associate Clinical Professor of Emergency Medicine
Division of Pediatric Radiology Albert Einstein College of Medicine
SUNY Downstate Medical Center New York, New York
Brooklyn, New York
Mark Horowitz, MD, FAAP, FACS
Nira Goldstein, MD, MPH Associate Professor of Urology
Associate Professor of Otolaryngology Director of Pediatric Urology
Department of Otolaryngology SUNY Downstate Medical Center and Staten Island University
SUNY Downstate Medical Center Hospital
Brooklyn, New York Brooklyn, New York

Howard A. Greller, MD, FACEP, FACMT Jeannette Jakus, MD, MBA


Assistant Professor of Emergency Medicine Pediatric Dermatology Fellow
Hofstra North Shore–Long Island Jewish School of Medicine Department of Dermatology
Assistant Fellowship Director SUNY Downstate Medical Center
Medical Toxicology Brooklyn, New York
North Shore University Hospital
Department of Emergency Medicine
Edward C. Jauch, MD, MS, FACEP, FAHA
Manhasset, New York Professor and Interim Director of Emergency Medicine
Division of Emergency Medicine
Christina Guillen, MD Medical University of South Carolina
Assistant Professor of Pediatrics MUSC Children’s Hospital
Pediatric Residency Associate Program Director Charleston, South Carolina
Child Abuse Specialist
Department of Pediatrics
Ryan Jones, MD
SUNY Downstate Medical Center Assistant Professor of Pediatrics
Brooklyn, New York Division of Pediatric Cardiology
University of Tennessee
John Gullett, MD LeBonheur Children Hospital
Assistant Professor of Emergency Medicine Memphis, Tennessee
Co-Director of Emergency Ultrasound
Department of Emergency Medicine Ernesto Jose Jule, MD
University of Alabama at Birmingham Associate Director Emeritus
Birmingham, Alabama Pediatric Emergency Medicine
Kings County Hospital Center
Raavi Gupta, MD Brooklyn, New York
Assistant Professor of Pathology
SUNY Downstate Medical Center Nikita Joshi, MD
Brooklyn, New York Clinical Assistant Instructor
Department of Emergency Medicine
Allysia Guy, MD SUNY Downstate Medical Center
Clinical Assistant Instructor Brooklyn, New York
Department of Emergency Medicine
SUNY Downstate Medical Center Ron Kemper
Brooklyn, New York Cape Coral, Florida
xxiv ■ PHOTO CONTRIBUTORS

Raffi Kapitanyan, MD, FACEP James G. Linakis, PhD, MD


Assistant Professor Professor of Emergency Medicine and Pediatrics
Department of Emergency Medicine Departments of Emergency Medicine and Pediatrics
UMDNJ–Robert Wood Johnson Medical School The Alpert Medical School of Brown University
New Brunswick, New Jersey Rhode Island Hospital/Hasbro Children’s Hospital,
Providence, Rhode Island
Vania Kasper, MD
Chief Resident Eve J. Lowenstein, MD, PhD
Department of Pediatrics Chief of Dermatology
SUNY Downstate Medical Center Brookdale University Hospital
Brooklyn, New York Associate Professor of Dermatology
SUNY Downstate Medical Center
Maria C. Kessides, MD Brooklyn, New York
Chief Resident
Department of Dermatology Michael Lucchesi, MD, FACEP
SUNY Downstate Medical Center Chairman, Department of Emergency Medicine
Brooklyn, New York SUNY Downstate Medical Center
Chief Medical Officer
Ambreen S. Khan, MD University Hospital of Brooklyn
Assistant Professor of Emergency Medicine Brooklyn, New York
SUNY Downstate Medical Center
Kings County Hospital Center Andrea Marmor, MD, MSEd
Brooklyn, New York Associate Professor of Clinical Pediatrics
University of California, San Francisco
Miriam Krinsky, MD San Francisco General Hospital
Pediatric Emergency Medicine Fellow San Francisco, California
SUNY Downstate Medical Center
Kings County Hospital Sashidhar Rao Marneni, MD
Brooklyn, New York Clinical Assistant Professor
SUNY Downstate Medical Center
Daniela Kroshinsky, MD, MPH Kings County Hospital Center
Harvard Medical School Brooklyn, New York
Massachusetts General Hospital
Boston, Massachusetts Brian P. Marr, MD
Associate Attending
Smita N. Kumar, MD, MPH Ophthalmic Oncology Service
Technical Adviser USAID Memorial Sloan-Kettering Cancer Center
Pretoria, South Africa New York, New York
Chaiya Laoteppitaks, MD Robert Mathews
Assistant Program Director Biddulph
Department of Emergency Medicine Stoke-on-Trent
Albert Einstein Medical Center Staffordshire, United Kingdom
Philadelphia, Pennsylvania
Stephen R. McMillan, MD
Douglas R. Lazzaro, MD, FACS Clinical Assistant Instructor
Professor and Chairman Department of Emergency Medicine
Department of Ophthalmology SUNY Downstate Medical Center
The Richard C. Troutman, MD Distinguished Chair in Brooklyn, New York
Ophthalmology and Ophthalmic Microsurgery
SUNY Downstate Medical Center Swati Mehta, MD
Director of Service Clinical Assistant Professor of Pediatrics
Kings County Hospital Center/University Hospital of Brooklyn SUNY Downstate Medical Center
Brooklyn, New York Kings County Hospital Center
Brooklyn, New York
PHOTO CONTRIBUTORS ■ xxv

Scott T. Miller, MD Bipin Patel, MD, FAAP


Professor of Clinical Pediatrics Clinical Professor of Pediatrics,
Director, Division of Pediatric Hematology/Oncology Drexel University College of Medicine
SUNY Downstate Medical Center Chairman, Department of Pediatrics
Kings County Hospital Center Physician-in-Chief
Brooklyn, New York The Children’s Hospital
Saint Peter’s University Hospital
Anil K. Mongia, MD New Brunswick, New Jersey
Assistant Professor of Pediatrics
Director, Pediatric Dialysis Unit Lynne Pinkney, MD
Division of Pediatric Nephrology Assistant Professor of Radiology
SUNY Downstate Medical Center New York University Langone Medical Center
Brooklyn, New York New York, New York
Shella K. Mongia, MD Steven Pulitzer, MD
Associate Pathologist Assistant Professor of Radiology
Integrated Regional Laboratories SUNY Downstate Medical Center
Fort Lauderdale, Florida Director, Division of Neuroradiology
Kings County Hospital Center
Cindy Moran, BS Brooklyn, New York
Quality Assurance Manager
Arkansas State Crime Laboratory Shahina Qureshi, MD
Little Rock, Arkansas Associate Professor of Pediatrics
Division of Hematology/Oncology
Stephen E Nanton, MD SUNY Downstate Medical Center
Clinical Assistant Professor in Pediatrics and Internal Medicine Kings County Hospital Center
Sanford School of Medicine Brooklyn, New York
The University of South Dakota
Director, Pediatric Gastroenterology Morgan E. Rabach, MD
Avera Children’s Hospital Chief Resident
Sioux Falls, South Dakota Department of Dermatology
SUNY Downstate Medical Center
Rita Nathawad, MD Brooklyn, New York
Pediatric Infectious Disease Fellow
Department of Pediatrics Chandrakant Rao, MD
SUNY Downstate Medical Center Clinical Associate Professor
Brooklyn, New York Department of Pathology
SUNY Downstate Medical Center
Trushar Naik, MD, MBA Kings County Hospital Center
Attending Physician, Advocate Medical Group Brooklyn, New York
Department of Emergency Medicine and Internal Medicine
Advocate Christ Medical Center Sudha M. Rao, MD
Oak Lawn, Illinois Director, Pediatric Cardiology
Kings County Hospital Center
Randi Ozaki, MD Clinical Associate Professor of Pediatrics
Clinical Assistant Instructor New York University School of Medicine
Department of Emergency Medicine New York, New York
SUNY Downstate Medical Center
Brooklyn, New York Christy Riley, MD
Resident Physician
Dimitrios Papanagnou, MD, MPH, RDMS University of California, San Francisco
Director, Medical Simulation and Advanced San Francisco, California
Learning Resource Center
Assistant Clinical Professor Rafael Rivera, MD
Department of Emergency Medicine Assistant Professor of Radiology
SUNY Downstate Medical Center New York University Langone Medical Center
Brooklyn, New York New York, New York
xxvi ■ PHOTO CONTRIBUTORS

Jonathan Rochlin, MD Toral Shah, BS, PA-C


Clinical Assistant Professor Division of Gastroenterology
SUNY Downstate Medical Center New York University Langone Medical Center
Kings County Hospital Center New York, New York
Brooklyn, New York
Vikas S. Shah, MD
Richard Rosenfeld, MD, MPH Pediatric Intensivist
Professor and Chairman Kings County Hospital Center
Department of Otolaryngology Clinical Assistant Professor
SUNY Downstate Medical Center SUNY Downstate Medical Center
Brooklyn, New York Brooklyn, New York
Mandakini Sadhir, MD Roman Shinder, MD
Adolescent Medicine Fellow Assistant Professor of Ophthalmology
Children’s Hospital of Wisconsin Department of Ophthalmology
Milwaukee, Wisconsin SUNY Downstate Medical Center
Brooklyn, New York
Shyam Sathanandam, MD
Assistant Professor of Pediatrics Katherine Siamas, MD
Division of Pediatric Cardiology Clinical Instructor
Section of Interventional Cardiology Department of Dermatology
University of Tennessee SUNY Downstate Medical Center
Le Bonheur Children’s Hospital Brooklyn, New York
Memphis, Tennessee
Haseeb A. Siddiqi, PhD
Heather Schultz, MD Director, Diagnostic Immunology and Clinical Parasitology
Clinical Instructor Laboratories
Department of Dermatology Kings County Hospital Center
SUNY Downstate Medical Center Associate Professor
Brooklyn, New York Departments of Medicine, Pathology, Microbiology and
Immunology
Michael Secko, MD, RDMS SUNY Downstate Medical Center
Director, Emergency Ultrasound Division Brooklyn, New York
Assistant Clinical Professor
Department of Emergency Medicine Michael H. Siegel, MD
SUNY Downstate Medical Center Clinical Assistant Professor
Kings County Hospital Center Department of Radiology
Brooklyn, New York SUNY Downstate Medical Center
Brooklyn, New York
Binita R. Shah, MD, FAAP
Professor of Pediatrics and Emergency Medicine Lawrence Silverberg, DPM
SUNY Downstate Medical Center Beth Israel Medical Center
Director Emeritus, Pediatric Emergency Medicine New York, New York
Kings County Hospital Center
Brooklyn, New York Mark Silverberg, MD, MMB, FACEP
Associate Professor of Emergency Medicine
Rajni P. Shah, MD Associate Residency Director
Rheumatologist Department of Emergency Medicine
New Hyde Park, New York SUNY Downstate Medical Center
Kings County Hospital Center
Ronak R. Shah, MD Brooklyn, New York
Medical Director of Emergency Medicine
Mayo Clinic Health System-Waseca Anup Singh, MD
Attending Physician of Emergency Medicine Chief, Pediatric Nephrologist
Mayo Health System-Mankato Department of Pediatrics
Mankato, Minnesota The Children’s Hospital at Saint Peter’s University Hospital
Associate Professor of Pediatrics
Drexel University College of Medicine
New Brunswick, New Jersey
PHOTO CONTRIBUTORS ■ xxvii

Bhuvanesh Singh, MD, PhD Mark Su, MD


Director, Laboratory of Epithelial Cancer Biology Assistant Professor of Emergency Medicine
Director of Speech and Hearing Center and Attending Surgeon Department of Emergency Medicine
Head and Neck Service, Memorial Sloan-Kettering Cancer Center Hofstra North Shore–LIJ School of Medicine at Hofstra University
Associate Professor of Otolaryngology Director, Fellowship in Medical Toxicology
Weill Medical College of Cornell University North Shore University Hospital
Associate Visiting Physician Manhasset, New York
Rockefeller University
New York, New York Audrey J. Tan, DO
Clinical Assistant Instructor
Deeptej Singh, MD Department of Emergency Medicine
Clinical Instructor SUNY Downstate Medical Center
Department of Dermatology Brooklyn, New York
SUNY Downstate Medical Center
Brooklyn, New York Ee Tein Tay, MD
Assistant Professor
Arpan Sinha, MD Departments of Emergency Medicine and Pediatrics
Clinical Instructor Mount Sinai Medical Center
Department of Pediatrics New York, New York
SUNY Downstate Medical Center
Brooklyn, New York Nooruddin R. Tejani, MD, FAAP
Director, Pediatric Emergency Medicine
Mark Spektor, DO, MBA, FACEP Assistant Professor
President and CEO Department of Emergency Medicine
Bayonne Medical Center SUNY Downstate Medical Center
Bayonne, New Jersey Brooklyn, New York
Neil Sperling, MD Svetlana Ten, MD
Associate Professor of Otolaryngology Director of Pediatric Endocrinology
Department of Otolaryngology SUNY Downstate and Infants and Children’s Hospital of Brooklyn
SUNY Downstate Medical Center at Maimonides Medical Center
Brooklyn, New York Associate Professor of Pediatrics
SUNY Downstate Medical Center
Jessica Stetz, MD, MS Brooklyn, New York
Assistant Professor of Emergency Medicine
Department of Emergency Medicine Mihir M. Thacker, MD
SUNY Downstate Medical Center Attending Pediatric Orthopedic Surgeon and Orthopedic Oncologist
Brooklyn, New York Alfred I. DuPont Hospital for Children
Wilmington, Delaware
Michael B. Stone, MD, RDMS Assistant Professor of Orthopedic Surgery and Pediatrics
Director, Division of Emergency Ultrasound Thomas Jefferson University
Department of Emergency Medicine Philadelphia, Pennsylvania
Brigham & Women’s Hospital
Assistant Professor Jonathan D. Thackeray, MD
Harvard Medical School Chief, Division of Child and Family Advocacy
Boston, Massachusetts Nationwide Children’s Hospital
The Ohio State University College of Medicine
Michael A. Stracher, MD Cincinnati, Ohio
Assistant Professor of Orthopaedic Surgery
SUNY Downstate Medical Center Mark K. Thompson, MD, FAANS, FACS
Attending Physician Vice Chairman of Neurosurgery
Brookdale University Hospital and Medical Center SUNY Downstate Medical Center
Brooklyn, New York Chief of Neurosurgery, Kings County Hospital
Brooklyn, New York
xxviii ■ PHOTO CONTRIBUTORS

Matthew Timberger, MD Rachel S. Weiselberg, MD


Clinical Assistant Instructor Medical Toxicology Fellow
Department of Emergency Medicine Department of Emergency Medicine
SUNY Downstate Medical Center North Shore University Hospital
Brooklyn, New York Manhasset, New York

James W. Tsung, MD, MPH Sharon Yellin, MD, FAAP


Associate Professor of Emergency Medicine and Pediatrics Emergency Ultrasound Fellow
Mount Sinai School of Medicine Pediatric Emergency Medicine
New York, New York Department of Emergency Medicine
New York Methodist Hospital
Diana E. Weaver, MD, FAAP Brooklyn, New York
Assistant Professor of Pediatrics
Department of Pediatrics Daniel Zinn, MD
SUNY Downstate Medical Center Assistant Professor of Radiology
Director, Pediatric Asthma Program SUNY Downstate Medical Center
Acting Director, Pediatric Pulmonology Brooklyn, New York
Kings County Hospital Center
Brooklyn, New York
PREFACE

Working in the environment of the emergency department, We have made an attempt to make this second edition
we experienced a plethora of clinical pathology that enhanced even more user friendly than the first. The Atlas features a
and finely tuned our visual diagnosis skills. Armed with a dig- consistent format with concise text regarding Clinical Sum-
ital camera and a consent form, we were able to build a library mary, Emergency Department Treatment and Disposition,
of educational material in the form of clinical pictures, radio- and Pearls. Side by side with this concise, easy-to-read text
graphic images, and fascinating stories. With these assets we is a wealth of images illustrating what these clinical problems
prepared the first edition of the Atlas of Pediatric Emergency look like in real life in an emergency setting. By making the
Medicine and have been humbled and pleased at the enthusi- text higher yield, we have been fortunate to be able to double
astic response of our audience. Based upon that response we the number of images in this new edition, greatly enhancing
have prepared this second edition. the work. Not only have we doubled the amount of images,
The pressures of clinical practice continue to increase with but we have updated the book with the newest imaging tech-
demands of increasing productivity, decreasing reimburse- niques available to all who work in the emergency setting. A
ment, and increasing numbers of patients, all leading to the new section on Emergency Ultrasound is also added; however,
potential to spend more time ordering tests than observing as the medical and lay communities have become “radiation
and examining patients. Thus, the art of visual diagnosis conscious and cautious,” ultrasound and plain radiography
remains an endangered species. Where the bedside experi- still remain the workhorses of pediatric imaging.
ence was once the highlight of our clinical day, the “art” has This Atlas is intended to assist the busy clinician in diagno-
been replaced by mechanics. Our trainees are being schooled sis, work-up, and disposition. It is written for anyone who has
in processing flow and survival mode ideation. the privilege of taking care of acutely ill and injured children.
Sir William Osler once wrote: “Avoid the common and We hope our experience and images will aid those in prac-
fatal facility of reaching conclusions from superficial obser- tice at continuing to hone their visual diagnostic and differen-
vations and being constantly misled by the ease with which tial diagnosis skills, and also that it will stimulate clinicians
our minds fall into the ruts of one or two experiences.” The who are starting their careers to never stop asking questions,
more patients one sees and examines, the better one becomes always strive to improve the art of visual diagnosis, work on
at forming a concise differential diagnosis prior to ordering eliminating any fear of patient contact, and never stop learn-
an expensive, time-consuming, and often invasive work-up. ing from your patients.
The student of visual diagnosis is not only more likely to Ars longa vita brevis (art is long while life is short). With
make the right diagnosis, but is also more likely to avoid this quote, Hippocrates reminds us how much there is to learn
the costly error. We urge our fellow physicians to hold on in a short period and thereby (hopefully) inspire us to be hum-
to this art. By perfecting the tools we were born with and ble, scholarly, and better doctors.
supplementing them with those that we developed, we will
Binita R. Shah, MD, FAAP
continue to have pride in what we do, love our patients, and Michael Lucchesi, MD, FACEP
enjoy our careers. John Amodio, MD
Mark Silverberg, MD, MMB, FACEP

xxix
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ACKNOWLEDGMENTS

We are grateful to all the residents and medical students who also extend our sincere appreciation to Ms. Regina Brown,
rotate through emergency and radiology departments, and to Developmental Editor for preparing the manuscript for pro-
our colleagues and nursing staff of the emergency and radiol- duction, and Ms. Catherine Saggese, Production Supervisor
ogy departments. They constantly stimulate us by challenging and Mr. John Williams, Director of Development and Produc-
us. Learning from each other as we work together has been a tion for shepherding the manuscript through the production
privilege and very rewarding. We thank you all. process. We are especially grateful to Ms. Charu Bansal, the
Most importantly, we are indebted to our patients and their production manager, and her entire team for doing an extraor-
families. In spite of their sufferings, they gave us permission dinary job in layout and production of the final product, tak-
to take their photographs without any reservations. They are ing a complex manuscript and turning it into an elegant book.
the unsung heroes of this book. We salute them for giving us We would also like to thank all the contributing authors
the privilege of taking care of them and for their kindness in and photo contributors for making this book an enormous
allowing us to learn from them and teach others. We trust that encyclopedia of pathology. Their untiring efforts and dedica-
with this book we are in some way repaying the great debt we tion to this book and to the previous edition are responsible
owe to our patients. for this Atlas. We have learned so much from all of you during
We express our heartfelt gratitude to Anne M. Sydor, PhD, this collaboration—Thank you!
Executive Editor, for her countless hours in the creation of Last but not least, we thank the thousands of readers who
this revised edition of the atlas. Her unflinching encourage- have found the previous edition of the Atlas a very valuable
ment and inspiring phone calls were a big motivation (at first resource, for their suggestions and praise inspiring us to take
dreaded, and then understood as a shared commitment to on the challenge of a second edition. We hope that you will
producing the best revision possible). Anne was an asset and find this edition equally valuable.
enormous resource for this challenging task. Because of her
extraordinary editorial diligence, we were able to add 800 + Binita R. Shah, MD, FAAP
more images without compromising the content of the book. Michael Lucchesi, MD, FACEP
Learning many editorial skills from Anne was an extra bonus. John Amodio, MD
Without her help, this book would not come to fruition. We Mark Silverberg, MD, MMB, FACEP

xxxi
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ABUSE
Chapter 1

CHILD
MALTREATMENT
Konstantinos Agoritsas
Mary Birmingham

Subgaleal hematoma

The authors acknowledge the special contributions of Binita R. Shah, MD, to prior edition.

1
CHILD ABUSE

Clinical Summary
In the United States, child abuse is defined as “any recent
act or failure to act on the part of a parent or caretaker which
results in death, serious physical or emotional harm, sexual
abuse, or exploitation” or “an act or failure to act which pres-
ents an imminent risk of serious harm”. The key aspect of
child abuse is maltreatment of a child by parents, guardians,
or caregivers. Risk factors include families with a history of
substance abuse, single-parent households, young parental
age, lack of parental education, previous incidents of domes-
tic violence, socioeconomic constraints, and mental health A
problems. Mechanisms of inflicted injuries range from direct
impact (eg, punching, slapping, or hitting with an object),
abusive head trauma, penetrating injuries, and injuries related
to asphyxiation. Presenting signs and symptoms range from
asymptomatic presentations, such as bruises and contusions,
to symptomatic presentations of seizures, coma, or death
associated with abusive head trauma or blunt abdominal
trauma. Inflicted abdominal injuries include ruptured liver or
spleen, intestinal perforation, duodenal hematoma, pancreatic
injury, and kidney trauma. Skeletal injuries, burns, poisoning,
or Munchausen syndrome by proxy are all possible presenta-
tions for child abuse.

Emergency Department
Treatment and Disposition
The approach to an infant who has been abused or neglected
is not significantly different from the standard care of a child
in the emergency department (ED). Stabilize the patient and
perform a thorough evaluation to exclude immediate life-
threatening or limb-threatening injuries. Once the patient is
stable, obtain a complete history including medical history
(hospitalizations, trauma, chronic disease, etc), family his-
tory, child temperament, developmental delay, substance
abuse, type of household, and socioeconomic constraints.
B
Plot growth parameters of height, weight, and head circum-
ference to exclude failure to thrive. Perform a complete FIGURE 1.1 ■ Child Abuse. (A) Bruise. Bruises are the most
physical examination including ears, scalp, frenula of the common injuries of child abuse. External evidence of inflicted inju-
lip, and tongue since these are uncommon locations for acci- ries may be very subtle as seen in this 3-month-old infant (small
dental trauma. Complete a formal retinal examination in all bruise in the periorbital region) presenting with inconsolable crying.
(B) An oblique fracture of the humerus was detected on skeletal
suspected cases of abuse in patients <12 years of age and in
survey. The humerus and femur are among the most frequently frac-
older children if brain injury is present. tured long bones in abusive injuries. Most common type of abu-
Diagnostic evaluation depends on severity and type of sive fractures are spiral (oblique) or transverse. (Photo contributor:
injury, age of patient, and examination which may include Binita R. Shah, MD.)

2
CHILD ABUSE (CONTINUED) ■ 3

highly suspected. A single x-ray (body gram) is unacceptable.


Obtain anteroposterior (AP) and lateral views of each bone.
The yield from the skeletal survey decreases with increasing
age, as the frequency of occult fractures decreases in older
children (between 2 and 5 years of age). Instead, order appro-
priate radiographs based on physical examination and any
complaints of pain.
Consider radionuclide bone scans, which identify most
fractures within 48 hours of injury and are helpful in infants
and young children with suspected abuse and a negative skel-
etal survey. Bone scans can increase the detection of frac-
tures in locations that are difficult to see radiographically
FIGURE 1.2 ■ Subgaleal Hematoma. Child’s mother admit- (eg, hands, feet, or ribs) and are helpful for detecting recent
ted to forcefully pulling his hair. (Photo contributor: Jonathan fractures (< 7- to 10-day-old rib fractures or subtle diaphyseal
Thackeray, MD.) fractures). They serve as a complementary test to radiogra-
phy, when additional evidence of abusive injuries is required
complete blood count (screening for anemia and platelet to establish the diagnosis.
count), liver function tests (elevation of transaminase seen Order head CT and consider brain magnetic resonance
with liver injury), urine analysis (hematuria with abdominal imaging (MRI) for all patients < 2 years old and for all chil-
injury), and coagulation profile (exclude bleeding disorders). dren with suspected intracranial injury. CT is highly sensitive
Order a radiographic skeletal survey for occult fractures and specific for brain injuries, especially those that require
in all suspected cases of abuse in children < 2 years of age. emergent intervention, and it is readily available and better
The limited skeletal survey can be repeated in 2 weeks to for the evaluation of acute hemorrhage. Brain MRI is best
increase diagnostic yield especially when abnormal or equiv- for fully assessing intracranial injury, including contusions,
ocal findings are found on initial study or when abuse is shear injuries, brain swelling or edema, and intraparenchymal

A B C

FIGURE 1.3 ■ Inflicted Burns. (A) Hot coffee was thrown on this girl by her mother during an argument. (B) Necrotic ulcerated lesion
following burn and multiple bruises were seen in this child. (C) Inflicted burn from a space heater. (Photo contributors: Binita R. Shah, MD
[A, B] and Barry Hahn, MD [C].)
4 ■ CHILD ABUSE (CONTINUED)

that are developmentally appropriate and open ended (eg,


“Can you tell me what happened to your arm?”), rather than
asking leading questions (eg, “Did your mommy hit you?”).
Document the child’s and caregiver’s exact statements about
the injuries, verbatim. Often, it is not even necessary to inter-
view the child other than in the most general, age-appropriate
way and to permit the forensic interviewing by the experts
after the ED evaluation.
Consult with a multidisciplinary team, including a pedia-
trician, child abuse consultant, social worker, and specialists
in pediatric radiology, orthopedics, neurosurgery, surgery,
and ophthalmology as indicated.
Report all suspected abuse to the local child protective ser-
vices as required by law in all 50 states in the United States.
The law requires reporting suspected (not necessarily proven)
cases of physical or sexual child abuse. Mandated reporters
are individuals routinely responsible for a child’s health or
well-being, and may include medical personnel, teachers, day
care workers, and law enforcement professionals. Mandated
reporters who report their suspicions in good faith are pro-
FIGURE 1.4 ■ Duodenal Hematoma; Inflicted Abdominal Injury. tected from lawsuits. A mandated reporter may be prosecuted
Upper GI study demonstrated irregularity and thickened folds of the
for failing to report abuse, and civil malpractice litigation may
second and third portions of the duodenum (arrows) secondary to a
be brought against a physician or other health care practitio-
duodenal hematoma in a child who was repeatedly punched in the
abdomen by his stepfather. There is also dilatation of the more proxi- ner for failure to recognize or diagnose child abuse or child
mal duodenum. (Photo contributor: John Amodio, MD.) sexual abuse.
Document in the medical record with great care with a
very clear, concise, and legible history, examination, and lab-
hemorrhages. Obtain MRI whenever there are positive head oratory and radiologic findings. These records may become
CT findings and in selected cases with a normal CT but with evidence in a criminal prosecution.
high clinical concern for intracranial injury. Also consider If abuse is considered likely, consult child protective ser-
MRI to evaluate for subacute or chronic injury, which may be vice workers and together decide about the safe disposition
missed on a head CT. of the child and the possibility of further harm if the child
Document all physical findings as quickly as possible and returns to the custody of the caregiver in question. Options
with a camera of the highest quality available. First take a include immediate placement in foster care (either with a
picture of the victim’s face, and then other areas. Clearly relative or designated foster parent), or temporary hospital-
identify all pictures, documenting the photographer name, the ization while awaiting arrangements for transport to a safe
date, and time in the chart. Obtain a minimum of two views environment.
of each skin finding: first showing the injury in the context of Refer the victim and their innocent relatives or caregivers
the body region involved and second a close-up of the injury to mental health services to help them cope with the emo-
with a scale (such as a coin or a tape ruler). tional trauma of abuse. Evaluate all other siblings and other
For children old enough to describe what happened, con- minors in the family who are present, if they were also in
duct the interview in a closed environment using questions contact with the alleged perpetrator.
CHILD ABUSE (CONTINUED) ■ 5

FIGURE 1.5 ■ Child Abuse. (A) Abusive head trauma. Axial CT of


the brain demonstrates bilateral subdural collections of high attenu-
ation over the frontal lobes bilaterally (anterior arrows). In addition
there is hemorrhage along the falx posteriorly (posterior arrow).
(B) Axial MR fluid-attenuated inversion recovery (FLAIR) image
of the brain demonstrates high signal over the right frontal lobe
(arrow) consistent with subdural hemorrhage. (C) Single AP view
of the right knee demonstrates a Salter II fracture through the medial
right distal metaphysis consistent with a metaphyseal corner fracture
that was detected in the skeletal survey. (Photo/legend contributors:
B
Konstantinos Agoritsas, MD/Rachelle Goldfisher, MD.)

Pearls 3. Many children who are physically abused may also be


sexually abused. Exclude sexual abuse by taking a thor-
1. Any injury to a minor who presents to a clinician may be
ough history, performing a thorough examination, and
the result of child abuse and the consideration of child
order laboratory studies as indicated.
abuse and/or neglect is paramount when examining a
4. Red flags for child abuse include inconsistent, unex-
child who is potentially injured or abused.
plained, and implausible history; delays in seeking treat-
2. Undress the child completely so that a thorough examina-
ment; and a history of repeated accidents.
tion may be carried out to evaluate for unusual bruises,
marks, burns, and areas of swelling or tenderness.
CUTANEOUS MANIFESTATIONS OF CHILD ABUSE

Clinical Summary accidental trauma, infections associated with petechiae or


purpura, Henoch-Schönlein purpura, folk healing practices
Bruising is one of the most common signs of abuse and may
(eg, cupping), or dermatologic conditions such as hypersen-
be the first presentation of an abused child. Bruises may be
sitivity reactions, cold panniculitis, or phytophotodermatitis.
accidental or inflicted. Consider physical abuse whenever the
history is lacking, changes over time, or is inconsistent with
the injury or developmental stage of the child. Remember Emergency Department
that bruises may be hidden by clothing, and it is important Treatment and Disposition
to examine the child fully undressed. In cases of excessive
Document skin findings from initial presentation with pho-
corporal punishment, bruising may be present in the pattern
tographs and a body diagram if needed and include the color,
of the inflicting object. Multiple bruises in different stages of
shape, pattern, location, and size of each bruise. Involve
healing is also a concerning sign. A Wood’s lamp with the
child protective services in any suspected case of abuse. In
digital camera may improve the visualization of faint bruises
a patient with multiple bruises and no clear history, obtain a
that otherwise might be missed. Differential diagnoses include
CBC and coagulation studies to rule out underlying bleeding
disorders.

FIGURE 1.6 ■ Bite Marks. (A, B) Bite marks lead to distinctive


patterns of bruises and should be suspected when ecchymosis, lac-
erations, or abrasions are elliptical or oval (two arched patterns as
mirror images if both mandibular and maxillary teeth used to bite).
Canine marks are the most prominent (or deep) part of the bite.
The normal distance between maxillary canines in adult humans
is 2.5 to 4 cm, and in a child it is < 3 cm. If the intercanine dis- B
tance is < 3 cm, the bite may have been inflicted by a child; > 3 cm,
it was probably inflicted by an adult. (Photo contributor: Binita FIGURE 1.7 ■ Marks from Objects. (A) Loop and linear marks
R. Shah, MD.) from electrical cord. (B) Patterned bruising inflicted by a hand.

6
CUTANEOUS MANIFESTATIONS OF CHILD ABUSE (CONTINUED) ■ 7

FIGURE 1.7 ■ (Continued) (C) Close-up of multiple linear cuts


inflicted by razor blade on the forearm while the child’s hands
were tied. (Photo contributors: Smitha Kumar, MD [A], Jonathan
B
Thackeray, MD [B] and Binita R. Shah, MD [C].)
Figure 1.8 ■ Inflicted Bruises. (A) Bruises on relatively well-
protected areas suggest inflicted injuries. Purple bruise around the
periorbital region and red bruise on the nasal bridge seen here rep-
resent new bruises. (B) Red bruise and scratch marks are seen on
both cheeks in this infant who was left with his mentally retarded
brother. He also had human bite marks on his trunk. (Photo contribu-
tor: Binita R. Shah, MD.)

Pearls
1. Age of a bruise is indeterminate; location, type of impact
inflicted, and skin color all may affect bruise appearance.
2. It is extremely uncommon for preambulatory children to
bruise. “Those who can’t cruise don’t bruise.”
3. Bruises from normal activity more commonly occur on
bony prominences of the anterior surfaces (eg, forehead,
shins, elbows, knees, lower arms, and dorsum of hands).
FIGURE 1.9 ■ Inflicted Bruises. Ears are not frequently injured 4. Bruises on fleshy or well-protected areas such as ears,
accidentally and bruises are strong indicators of abuse. Ears can cheeks, frenulum, neck, upper arms and trunk, flanks,
be bruised by pulling, pinching, or grabbing and are typically seen thighs, and buttocks suggest inflicted injuries.
on top of the pinna. Pulling the ears can also cause bruises at the
junction of the ear and head posteriorly. (Photo contributor: Binita
R. Shah, MD.)
INFLICTED BURNS

Clinical Summary
Burn injuries may be inflicted or accidental. Splash burns
commonly occur on the face, neck, and chest when the ambu-
lating toddler pulls a hot liquid or object onto themselves.
Usually the most severe burns are seen on the face and shoul-
der, where the liquid hits first, with splash marks away from
the point of maximal contact. Toddlers may also be victims
of forced immersion burns, which often present as a well-
demarcated burn injury on the perineum and/or extremities
with a central area of sparing either on the buttocks or feet
where the child is held against the bottom of the tub. Children
who accidentally come in contact with a hot liquid will usu-
ally present with an indistinct margin and splash marks as the
child reflexively tries to move away. Inflicted burns generally
do not have splash marks because the child is held in position.
Contact burns result from a hot object being held against
the child’s skin. On physical examination, there will usually
be a distinct outline that may be helpful in distinguishing the
object used. Although these may occur accidentally when a
child runs into or pulls an object onto themselves, usually the
outline of the object will be less distinct and more superficial
in these accidental injuries.
Differential diagnosis includes chemical burns, infections
arthropod bites, and dermatologic conditions.

FIGURE 1.11 ■ Inflicted Splash Burns. Hot soup was thrown on


the back of this girl as she tried to run away from her father during
an argument. (Photo contributor: Binita R. Shah, MD.)

FIGURE 1.10 ■ Typical Spill Burn Pattern. Accidental first- and


second-degree burns to the lower face, shoulder, chest, and hand in
this toddler are seen as he pulled the mug filled with hot tea from
a table. In a typical spill burn, the hot liquid usually falls onto the
child’s face and shoulder first (most severe burn seen at these sites),
and as the liquid runs down the body and cools, the burn becomes
less severe creating an arrow-shaped configuration. The presence FIGURE 1.12 ■ Accidental Iron Burn. An accidental burn from an
of clothing makes burns more severe by holding hot liquid in close iron falling on a toddler who pulled it down onto herself. Note less
contact with the skin. Splash marks away from the point of maximal distinct margins and more superficial injuries. (Photo contributor:
contact may be seen. (Photo contributor: Binita R. Shah, MD.) Kirsten Bechtel, MD.)

8
INFLICTED BURNS (CONTINUED) ■ 9

A B

FIGURE 1.13 ■ Inflicted Iron Burns. (A) Burn from iron inflicted on the upper arm. (B) A healed iron burn inflicted to the forearm. (Photo
contributor: Binita R. Shah, MD.)

Emergency Department Pearls


Treatment and Disposition 1. Evaluate carefully every pediatric burn for possible
Patients should be initially managed and resuscitated as any abuse. Listen carefully to the caregiver’s story and exam-
other burn patient, with careful attention to fluid status, pain ine the burn closely to determine plausibility of the story.
management, and infection control. Take a careful history 2. Stocking-and-glove burn patterns are pathognomonic for
with attention to whether the type and degree of injury is child abuse.
consistent with the history given. Document the initial pre- 3. Abusive immersion burns are more common in infants and
sentation of the burn and look for other signs of abuse or toddlers and scalding is the most common mechanism.
neglect in any childhood burn victim. If abuse is suspected, 4. Scalding is the most common mechanism of burn injury
report to child protective services and admit the patient for abused children.
pending further investigation. 5. Accidental burns may also be the result of poor super-
vision, so pay careful attention to the social situation in
every child who presents with a burn.

FIGURE 1.14 ■ Chemical Burns. (A, B) Burn caused by chlorine


bleach accidentally spilled in this infant’s diaper that served as an
occlusive dressing. The incident was reported to child protective
services. After a thorough investigation, this injury was considered
B
to be accidental. (Photo contributor: Binita R. Shah, MD.)
10 ■ INFLICTED BURNS (CONTINUED)

B
A

FIGURE 1.15 ■ Inflicted Burns. (A) Second-degree burns on face,


ear, and head in a toddler. (B) Second-degree burn on foot of an
infant. (C) Second-degree burn over the perineum that extended
on buttocks with sharp demarcation between burned and unburned
areas. Chemical/contact dermatitis secondary to Ex-Lax-containing
senna was a strong consideration (diamond-shaped lesion, sparing of
the perianal region), but history could not be substantiated and the
family was referred to child protective services. (Photo contributor:
C
Binita R. Shah, MD.)

A B

FIGURE 1.16 ■ Contact Burns. (A) A well-defined area of burn inflicted by a heated spoon. (B) Second- and third-degree burns of the lower
extremity inflicted by a hot object in an infant. (Photo contributor: Binita R. Shah, MD.)
SKELETAL MANIFESTATIONS OF CHILD ABUSE

Clinical Summary fractures, and metaphyseal chip fractures), which require


forces larger than what is produced by usual accidental
Skeletal fractures related to child abuse are rarely life-
trauma of infancy. Evaluate all fractures of the rib, scapula,
threatening; however, they may be the first indication of
sternum, or vertebra as these are pathognomic for physi-
child abuse and thus are an important diagnostic tool.
cal abuse. Tibial spiral fractures are usually due to acci-
Inflicted skeletal injures may involve any part of the axial
dental trauma unless they occur in nonambulatory young
and appendicular skeleton and occur in approximately
infants. Other differential diagnosis includes disorders with
40% of physically abused children. Age is the single most
increased tendency to fracture (eg, osteogenesis imperfecta,
important risk factor for abusive skeletal injuries, and 55%
rickets), infections with metaphyseal lesions (eg, congeni-
to 70% are seen in infants <1 year of age. Intracranial and
tal syphilis), and malignancy (eg, leukemia).
intraabdominal injures often coexist with abusive skeletal
injuries; the presence of one indicates the need to evaluate
for the other. Emergency Department
Suspect child abuse when fractures occur in nonam-
bulatory children, patients < 6 months old, those with an
Treatment and Disposition
inconsistent history, or with multiple or repeat fractures. Consider a multidisciplinary team approach including con-
Suspicion of child abuse is high for metaphyseal and sultations with the pediatrician, child abuse consultant,
epiphyseal fractures (eg, bucket handle fractures, corner social worker, and a specialist in pediatric radiology and

FIGURE 1.17 ■ Fractures: Inflicted versus Accidental. (A) Femoral


Shaft Fracture; Abusive Injury. A lateral view of the right femur
demonstrates a spiral fracture with minimal displacement in this
infant brought to the ED with inconsolable crying. The femur is one
of the most frequently fractured long bones in abusive injuries. It
requires a significant force to break the femur; thus a spiral, oblique,
or transverse fracture of the femur is highly suspicious of child abuse
in an otherwise healthy infant. (B) Toddler’s Fracture; Accidental
Injury. A nondisplaced spiral fracture is seen at the distal third of
the tibia in a 12-month-old infant. Toddler’s fracture is usually seen
in the distal third of the tibia in an ambulating or cruising toddler. A
skeletal survey was negative and child abuse was excluded by a thor-
B
ough investigation. (Photo contributor: John Amodio, MD.)

11
12 ■ SKELETAL MANIFESTATIONS OF CHILD ABUSE (CONTINUED)

A B

FIGURE 1.18 ■ Classic Metaphyseal and Epiphyseal Injuries of Child Abuse. (A) Anteroposterior view of the upper extremity demonstrates
a “corner” fracture of the metaphysis (arrow). There is also deformity of the distal shaft of the radius representing an older healing frac-
ture. (Photo contributor: John Amodio, MD). (B) Frontal view of the proximal tibia demonstrates transverse lucency through the metaphy-
sis, consistent with the classic metaphyseal fracture (bucket-handle fracture). Periosteal new bone formation along the shaft of the tibia is
seen. (Reproduced with permission from: Shah BR, Laude TL: Child Abuse and Sexual Abuse. Atlas of Clinical Diagnosis. WB Saunders,
Philadelphia, 2000, p. 36.)

orthopedics. Admission may need to occur prior to comple- 2. Even though diaphyseal fractures are the most common
tion of diagnostic evaluation for safety or critical care; how- type of fractures seen in child abuse, they are not pathog-
ever, when possible, complete the skeletal survey in the ED, nomonic for abusive injuries and can also result from
if possible. Order a full skeletal survey including two views accidental injuries.
of any injuries that require orthopedic management. Report 3. The hallmark finding in fractures caused by abuse is lack
suspected cases of physical abuse to the appropriate child pro- of a plausible explanation.
tection agency. Manage fractures as described in Chapter 19. 4. Order a follow-up skeletal survey 2 weeks after the initial
study to confirm normal anatomic variants or metabolic
Pearls bone diseases that mimic abusive injuries.

1. Skull fractures, rib fractures, and metaphyseal lesions


are the predominant types of abusive injuries seen during
infancy; long bone fractures are the most common skel-
etal injury after 1 year of age.
SKELETAL MANIFESTATIONS OF CHILD ABUSE (CONTINUED) ■ 13

A B C

FIGURE 1.19 ■ Multiple Fractures; Child Abuse. A spiral fracture of the proximal third of right tibia is seen in a 6-month-old infant (A).
A repeat radiograph taken 3 weeks later shows a diffuse periosteal reaction and substantial callus formation (B). A comminuted spiral fracture
of the distal third of the left tibia and a transverse fracture of the left fibula with a subperiosteal new bone formation are seen (C). Toddler or
spiral fractures of the tibia in a child who is not walking or cruising is highly suggestive of abuse. The presence of multiple fractures (either
injuries of different bones or more than one site of fracture within same bone) and/or fractures in different stages of healing suggests child
abuse. (Photo contributor: Binita R. Shah, MD.)
14 ■ SKELETAL MANIFESTATIONS OF CHILD ABUSE (CONTINUED)

FIGURE 1.20 ■ Multiple Fractures; Child Abuse. (A) Anteropos-


terior (AP) view of the left shoulder demonstrates a comminuted
and displaced fracture of the left proximal humeral metaphysis with
widening and disruption of the physis. There is overlying soft tissue
swelling. Periosteal reaction is noted along the distal left clavicle.
(B) AP view of the right clavicle in the same child demonstrates an
acute fracture of the middle third of the right clavicle with inferior
displacement of the distal fracture fragment. (Photo/legend contrib-
utors: Valeriy Chorny, MD/Rachelle Goldfisher, MD.)
B

FIGURE 1.21 ■ Metaphyseal Lesions; Differential of Child Abuse


Fractures. (A) Congenital Syphilis. Frontal view of the upper extrem-
ity demonstrates areas of bone destruction in the metaphyses of the
humerus, radius, and ulna, compatible with congenital syphilis.
(B) Rickets. Frontal radiograph of the wrist demonstrates cupping
and fraying of the metaphyses of the distal radius and ulna. (Photo
contributors: Lynne Pinkney, MD [A] and John Amodio, MD [B].)
FEMUR FRACTURES

Clinical Summary femur fractures for spica cast application and further man-
agement, including evaluation for possible abuse.
Femoral shaft fractures are common diaphyseal fractures in
children that result from falls, motor vehicle accidents, and
sporting injuries. In infants, they may be due to nonaccidental Pearls
injury. Distinguishing abuse from accidental trauma can be
1. A high index of suspicion for nonaccidental injury is
difficult and is often based on clinical suspicion. A history
mandatory when children < 3 years of age (especially
suspicious for abuse, physical or radiographic evidence of
those <18 months) are evaluated for a femur fracture.
prior injury, and age <18 months are risk factors. An infant
2. Spiral femur fractures are concerning for nonaccidental
that is completely nonambulatory (unable to crawl or roll) is
injury in children who are preambulatory.
more likely to have sustained abuse. Associated injuries (eg,
head injuries, bruises) need to be sought. During the first year
of life, as an infant becomes more mobile, the likelihood of
accidental as opposed to nonaccidental injury increases. In a
walking toddler, an isolated femur fracture is more likely to
be accidental than nonaccidental.

Emergency Department
Treatment and Disposition
Femoral shaft fracture in infancy rarely requires operative
intervention. Consult orthopedics for treatment with trac-
tion, spica casting, or a combination of both. Skeletal trac-
tion with delayed spica casting or early and immediate spica
casting have all been advocated. Admit young children with

FIGURE 1.23 ■ Femur fracture; Accidental Trauma. Anteroposterior


FIGURE 1.22 ■ Spiral Fracture of Femur; Child Abuse. Lateral view of the right hip demonstrates a comminuted and angulated
view of the right femur demonstrates a spiral fracture with mini- fracture of the proximal femoral shaft in a child involved in a car
mal displacement in a 43-day-old infant presenting to the ED with accident. Inflicted fractures are more common in infants. Long bone
fussiness. Funduscopic examination also showed retinal hemor- fractures are more likely the result of accidental trauma once chil-
rhages. (Photo/legend contributors: Miriam Krinsky, MD/Rachelle dren become ambulatory. (Photo/legend contributors: Shashidhar
Goldfisher, MD.) Rao Marneni, MD/Rachelle Goldfisher, MD.)

15
RIB FRACTURES DUE TO ABUSE

Clinical Summary are seen on the initial radiographs. Admit all patients with rib
fractures due to child abuse while awaiting additional tests
Rib fractures account for up to 29% of all fractures in abused
for other injuries (as indicated) and child protective agency
children and a higher incidence of rib fractures is reported
investigation. Rib fractures heal rapidly, and do not require
in autopsy findings. Rib fractures are usually seen with
any specific therapy.
violent shaking with anteroposterior thoracic compression
in infants and with direct blows to the chest in older chil-
dren. Involvement of the posterior arc of a rib is most com- Pearls
mon; however, fractures can be seen at any rib site in abuse.
1. Rib fractures are the most common type of thoracic
Posterior rib fractures are due to levering of the posterior
trauma in physically abused children.
neck over the transverse spinous process as the rib cage is
2. Rib fractures are often bilateral, involve multiple levels,
vigorously squeezed. There may be associated anterior and
and frequently involve posteromedial ribs.
costochondral junction fractures. Clinically significant injury
3. Rib fractures are NOT a complication of cardiopulmo-
to the lungs and heart is uncommon in spite of the high fre-
nary resuscitation in infants and young children (unlike
quency of rib fractures in abused children. Other thoracic
adults).
injuries that can be seen rarely with rib fractures include pul-
4. The presence of rib fractures in the absence of any etiol-
monary contusions, pneumothoraces, or pleural effusions.
ogy (eg, severe motor vehicle accident or metabolic bone
disease) should be considered evidence of child abuse
Emergency Department until proven otherwise. These injuries are quite rare even
in the setting of severe accidental trauma in infants.
Treatment and Disposition
5. Children with rib fractures from accidental injuries are
Obtain AP and lateral chest radiographs and right and left significantly older than children with rib fractures related
posterior oblique views to further delineate rib fractures that to abuse.

FIGURE 1.24 ■ Rib Fractures; Abusive Head trauma (Shaken Impact Syndrome). Single anteroposterior view of the chest demonstrates
multiple posterior and lateral rib fractures (arrow) with surrounding callus in the classic “rosary beads” appearance in the same infant
with bilateral subdural hematomas (Figure 1.25). (Photo/legend contributors: Manadakini Sadhir, MD/Rachelle Goldfisher, MD.)

16
RIB FRACTURES DUE TO ABUSE (CONTINUED) ■ 17

A B

FIGURE 1.25 ■ Abusive Head trauma (Shaken Impact Syndrome). (A) Bilateral subdural hematomas. A noncontrast CT image shows a
subtle increased attenuation (density) along the inner table of the right and left frontal bones representing acute hematoma (arrows). (B) MRI
image shows bilateral crescentic collections of high signal (white) along the frontal bones, bilaterally, right greater than left (arrows).
Extracranial hematomas along the frontal bone are also noted in both CT and MRI images. This 5-month-old infant was reported as “falling
off the bed” by mother’s boyfriend. (Photo contributor: David S. Dinhofer, MD.)
SKULL FRACTURES DUE TO ABUSE

Clinical Summary
Child abuse is the most common cause of severe head injury
in children <1 year of age and skull fractures are the second
most common form of abusive skeletal injuries, accounting
for 7% to 30% of all fractures in abused children. Skull frac-
ture is caused by a forceful blow to the head with a solid
object. The absence of a skull fracture does not exclude the
possibility of intracranial injury. Dating of skull fractures is
difficult, as they do not heal with evident callus formation
that is seen with long bone fractures.

Emergency Department
Treatment and Disposition
Order plain radiographs to identify suspected skull fractures,
including AP and lateral views even when cranial CT has been FIGURE 1.26 ■ Skull Fracture. Right lateral view of the skull dem-
performed, because skull fractures coursing in the axial plane onstrates a fracture of the right parietal bone. (Photo contributors:
may be missed with axial CT. Bone scans are not helpful in Valeriy Chorny, MD/Rachelle Goldfisher, MD.)

A B

FIGURE 1.27 ■ Depressed skull Fracture. (A) Single anteroposterior view of the skull demonstrates a depressed skull fracture of the left
parietal bone. (B) Axial CT of the brain with bone windows demonstrates a depressed skull fracture of the left parietal bone. (Photo/legend
contributors: Christina Guillen, MD/Rachelle Goldfisher, MD.)

18
SKULL FRACTURES DUE TO ABUSE (CONTINUED) ■ 19

A B

FIGURE 1.28 ■ Abusive Head Trauma. (A) Comminuted skull fractures. This was most likely produced by flinging the child in a down-
ward trajectory and striking the child’s head against a hard countertop. A fracture of this nature would take tremendous force to produce.
(Reproduced with permission from: Catanese C: Color Atlas of Forensic Medicine and Pathology. Taylor and Francis Group, CRC. 2010,
p. 174.) (B) An epidural hematoma with midline shift is shown in a different child presenting in coma with signs of herniation. (Photo
contributor: Binita R. Shah, MD.)

identifying skull fractures. Order cranial CT without contrast 2. Skull fractures that occur in infants after simple acci-
for initial evaluation of all suspected inflicted head injuries. dental falls (eg, falls < 4 feet, falling off of a bed or
Isolated skull fractures require no specific therapy and are table) are typically single, linear, nondiastatic, and most
benign in the majority of cases. Patients need continuing fol- commonly involve the parietal bone. Fractures associ-
low-up by their primary care provider for development of a ated with simple accidental falls are also not associated
leptomeningeal cyst. with intracranial injury.
3. Suspect abuse in a child presenting with a history of
minor trauma with depressed, diastatic, or complex
Pearls or multiple fractures, particularly those involving the
1. No pattern of skull fracture is pathognomonic of child occipital bone.
abuse; however, skull fractures that are multiple, bilat-
eral, or nonlinear or those that cross suture lines are more
likely to be due to abuse than to an accident.
ABUSIVE HEAD TRAUMA

Clinical Summary
Abusive head trauma is a leading cause of death in infants and
often causes long-term neurologic disabilities in survivors.
Shaken impact syndrome (SIS; violent shaking of an infant
often in combination with blunt impact) is one of the impor-
tant mechanisms of abusive head trauma. Cardinal signs of
SIS include brain injury (subdural hematomas and cerebral
edema), retinal hemorrhages, and skeletal injuries. Cervical
spine injuries may be seen in severe cases. However, because
blunt impact trauma may also occur alone or in combination
with shaking, inclusive terminology (abusive head trauma)
is preferred when describing abusive head injuries. Because
there is often no history of trauma, or a vague history of a very
minor trauma, a high degree of suspicion is necessary when
assessing the need for imaging in an infant or very young
child suspected of abusive head trauma. Victims may pres-
ent with cardiac arrest, apnea, seizures, or with altered men-
tal status but also may have subtle or nonspecific symptoms,
such as irritability, vomiting, or sleepiness. Focal neurologic FIGURE 1.30 ■ Rib Fractures; Abusive Head Trauma. Single
signs may or may not be present; thus, it may be very easy to anteroposterior view of the chest demonstrates multiple bilateral rib
fractures with callus formation that were incidentally detected on
this radiograph in the same infant (Figure 1.22). Endotracheal tube
and enteric tube are also seen. (Photo/legend contributors: Christina
Guillen, MD/Rachelle Goldfisher, MD.)

incorrectly confuse these patients with common pediatric ill-


nesses. In addition, often there are no other signs of external
trauma on examination or very subtle bruising that is initially
overlooked.

Emergency Department
Treatment and Disposition
Perform initial stabilization and resuscitation as indicated
and admit to pediatric intensive care unit (PICU). Order
a head CT for the evaluation of possible subdural or sub-
arachnoid hematomas, or multiple subdural hematomas of
differing ages. Consider plain films and MRI to evaluate
FIGURE 1.29 ■ Abusive Head Trauma. A noncontrast CT of the for skull fractures, which may or may not be seen depend-
brain shows hyperdense area along the right tentorium extending ing on the type of force inflicted and can be missed by CT
into the posterior interhemispheric fissure, along the falx and over scan. MRI is a better tool than CT to assess for diffuse
the convexity, suggestive of an acute subdural hematoma. This
axonal injury but often not easily accessible in the emer-
3-month-old infant was brought to the ED with lethargy and signs
of Cushing triad with bulging anterior fontanelle and absence of any gency setting. Consult a multidisciplinary team, including
external injuries. Subsequently, the father confessed to shaking the child abuse consultant, social worker, ophthalmology (eye
infant. (Photo contributor: Binita R. Shah, MD.) examination for retinal hemorrhages) and neurosurgery,

20
ABUSIVE HEAD TRAUMA (CONTINUED) ■ 21

FIGURE 1.32 ■ Retinal Hemorrhages. Severe, diffuse prereti-


nal and intraretinal blot hemorrhages characteristic of child abuse.
Hemorrhages such as these strongly correlate with inflicted injury.
(Reproduced with permission from: Christian CW, Lane WG:
Ophthalmic involvement in non accidental trauma. In: Hertle RW,
Schaffer DB, Foster JA (eds). Pediatric Eye Disease. Color Atlas
and Synopsis. McGraw-Hill, New York, 2002, p. 106.)

3. Carefully examine for bruising in hidden areas, bulging


fontanelles, or long-bone tenderness in every infant with
nonspecific symptoms to rule out inflicted head injury in
FIGURE 1.31 ■ Epidural Hematoma. Axial CT of the brain dem- a subtle presentation, noting, however, that usually there
onstrates a lenticular-shaped area of high attenuation over the left are no signs of external trauma.
parietal lobe consistent with an epidural hematoma (arrow) in an 4. Consult ophthalmology for a dilated examination in
infant presenting with lethargy and vomiting. He also had multiple
every suspected victim of inflicted head injury. Careful
bruises. (Photo contributor: Rachelle Goldfisher, MD.)
documentation of the extent and location of retinal hem-
orrhages can help distinguish between abusive and non-
as indicated (eg, patient may need surgical evacuation of abusive causes of head injury.
epidural hematoma). Obtain a full skeletal survey includ-
ing skull films to evaluate for other injuries. Obtain tests to
evaluate for internal organ injury, including CBC; liver and
renal function tests; amylase, lipase, and creatine kinase
analysis; urinalysis; and coagulation studies.

Pearls
1. Minor household falls rarely result in life-threatening
injuries. Maintain a high index of suspicion for patients
who present with a history of short falls and multiple or
complex skull fractures or subdural hemorrhage on CT FIGURE 1.33 ■ Optic Nerve Sheath Hemorrhages; Abusive Head
Trauma (Shaken Impact Syndrome). Eye globes showing hemor-
scans.
rhages. Retinal hemorrhages were also noted on microscopic exami-
2. The classic triad of subdural hematoma, retinal hemor- nations. (Photo contributor: Charles Catanese, MD; Reproduced
rhages, and posterior rib fractures is present in only a with permission from: Catanese C. Color Atlas of Forensic Medicine
minority of abused patients. and Pathology. Taylor and Francis Group, CRC; 2010:171.)
INFANTICIDE VERSUS SUDDEN INFANT DEATH SYNDROME

Clinical Summary Provide the accurate history to the medical examiner after
carefully interviewing caretaker and family members who
Sudden infant death syndrome (SIDS) is a sudden death of an
were present at the scene. A thorough examination of the
infant <1 year of age, which remains unexplained after a thor-
dead infant should be done by either a child maltreatment
ough case investigation, including performance of a complete
specialist or pediatrician with expertise in child maltreat-
autopsy, examination of the crime scene, and review of the
ment. All of the findings must be conveyed to the medical
clinical history. The incidence of SIDS is more common in
examiner.
non-Hispanic black and American Indian/American natives
Suspect possible intentional suffocation or infanticide
than non-Hispanic white infants. Although it was thought to
with any of the following: a previous history of recurrent
rarely occur in the first month of life, approximately 10% of
episodes of cyanosis, apnea, or apparent life-threatening
cases occur in the first month of life and peaks between 3
event (ALTE) while under the care of the same person, age
and 4 months of age. Up to 90% of cases occur in less than
at death > 6 months, or previous unexplained and unex-
6 months of age. Contributory factors for SIDS include
pected deaths of one or more siblings or simultaneous or
sleeping in a prone position or sleeping on soft material or
nearly simultaneous death of twins or previous death of
a soft surface, young maternal age, maternal smoking dur-
infants under the care of the same unrelated person, or dis-
ing or after pregnancy, lack of prenatal care, prematurity, low
covery of blood on the infant’s nose or mouth in association
birth weight, male gender, overheating, and overwrapping.
with ALTEs.
Infanticide is an incident of child abuse that is fatal during
In the absence of any external evidence of trauma, a pre-
infancy. Estimates of the incidence of infanticide among cases
liminary diagnosis of “probable SIDS” can be given to a
diagnosed as SIDS vary between <1% and 5%. It is impos-
previously healthy infant who has died suddenly and without
sible to distinguish at autopsy between SIDS and accidental
any explainable cause. Postmortem findings in fatal child
or intentional suffocation with a soft object.
abuse most often reveal cranial injuries (eg, subdural hema-
Postmortem studies that will be done by the medical
toma, intracerebral hemorrhage), intra-abdominal trauma
examiner include radiographic skeletal survey, toxicologic
(eg, liver laceration, hollow viscus perforation, intramural
screening, metabolic screening for inborn errors of metab-
hematoma), burns, and drowning or toxic exposure.
olism and tissue analysis of the brain, liver, kidney, heart,
muscle, adrenal glands, and pancreas.

Emergency Department Management


Once the infant is pronounced dead, approach the family
with an empathetic, compassionate, supportive, and non-
accusatory manner while attempting to learn more about
the circumstances surrounding the infant’s death. Note the
reaction of the caregivers or parents and try to obtain any
observations made by the first-response teams and docu-
ment in the medical record (eg, position of the infant; body
temperature and rigor; the presence of any marks on the
body prior to CPR administration; the type of crib or bed
and any defects and the type of mattress [firm versus a
water bed]; the presence of a fluffy blanket or comforter,
FIGURE 1.34 ■ Marks Due to Child Abuse. Note the multiple
soft stuffed toys or pillows in the crib; the amount of cloth-
angulated contusions on the child’s back consistent with a belt
ing on the baby; the room temperature and type of ventila-
buckle strike. This child was brought in cardiopulmonary arrest to
tion and heating) even though a thorough investigation of the ED. He had multiple blunt force injuries to his head, back, and
the death scene will be done by law enforcement officers. extremities. (Photo contributor: Binita R. Shah, MD.)

22
INFANTICIDE VERSUS SUDDEN INFANT DEATH SYNDROME (CONTINUED) ■ 23

FIGURE 1.35 ■ Infanticide. (A) Abdominal bruise. An infant


brought in with cardiopulmonary arrest was found to have this bruise
on the abdominal wall. The mother gave a history of finding the
baby “not breathing” 4 hours after she had fed the baby. (B) Rib
fractures. Multiple rib fractures and liver laceration were found at
autopsy. (Photo contributors: Binita R. Shah, MD [A] and Charles
B
Catanese, MD [B].)

Pearls 3. Suspect SIDS when a previously healthy infant appar-


ently dies during sleep, prompting an urgent call for
1. An autopsy must be performed on any infant who dies
emergency services. Often the history reveals that the
suddenly and unexpectedly. Infant deaths without post-
infant fed fine before going to sleep, and the infant was
mortem examination should not be attributed to SIDS.
found in the same position in which he or she had been
2. Excluding child abuse through a thorough investigation
placed at bedtime.
in every sudden and unexplained death can help protect
surviving and subsequent siblings.
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MISTAKEN ABUSE
Chapter 2

CONDITIONS
MISTAKEN FOR
CHILD ABUSE AND
SEXUAL ABUSE
Binita R. Shah

“Raccoon eyes”

25
MONGOLIAN SPOTS

Clinical Summary Tyndall effect (when light strikes the surface of the lesion,
red wavelengths of light are absorbed and blue wavelengths
Mongolian spots (also referred to as blue-gray macule of
are reflected back from the brown melanin pigment from
infancy) are the most frequently encountered birthmarks in
the dermis). More than 95% of African American and 80%
neonates. Melanin-containing melanocytes in the dermis are
of Asian infants are born with Mongolian spots. The skin
present (migrational arrest) and the distinctive blue color,
lesion is flat (macular), slate-gray, bluish-gray or brown,
characteristic of dermal melanin, occurs as a result of the
and consists of poorly circumscribed, single or multiple

FIGURE 2.1 ■ Mongolian Spots. (A) Bluish-gray macular lesions, present since birth, are seen on the back and buttocks (the most common
location) of an otherwise healthy African American infant. (B) Lesion on the anterior chest wall in the same infant. (Photo contributor: Binita
R. Shah, MD.)

26
MONGOLIAN SPOTS (CONTINUED) ■ 27

lesions ranging in size from a few millimeters to several


centimeters. The most common location is the sacrum and
buttocks (90%), but lesions may occur anywhere, including
the back, shoulders, or flank. Mongolian spots fade gradu-
ally and are resolved by age 5 to 6 in about 96% of cases.
Differential diagnosis of Mongolian spots includes acciden-
tal or inflicted injury and other forms of dermal melanocy-
tosis (eg, nevus of Ito or Ota; see page 32).

Emergency Department
Treatment and Disposition
Mongolian spots do not lead to any symptoms and require
no treatment. Family needs to be reassured about the benign
nature of these lesions.

Pearls
1. Mongolian spots can be mistaken for ecchymosis result-
ing from inflicted injuries. Mongolian spots are non-
tender unlike ecchymosis, which may be tender.
FIGURE 2.2 ■ Mongolian Spot. Multiple areas of bluish discol-
2. An ecchymotic skin lesion undergoes sequential color
oration are seen on the back and buttocks in a Caucasian infant,
changes and resolves within a few days, whereas in whom the incidence of Mongolian spots is uncommon (<10%),
Mongolian spots do not undergo similar color changes and these lesions may be mistakenly attributed to inflicted bruises.
and fade spontaneously over a period of years. (Photo contributor: Binita R. Shah, MD.)

FIGURE 2.3 ■ Mongolian Spot. An infant brought to the ED in cardiopulmonary arrest had these lesions thought to be Mongolian spots;
however, inflicted bruises from child abuse were in the differential diagnosis. The medical examiner would typically incise areas of skin
discoloration to exclude underlying contusions from inflicted injuries. (Photo contributor: Binita R. Shah, MD.)
FOLK HEALING PRACTICES

Clinical Summary appear. Moxibustion is also practiced among the Chinese.


It is a form of acupuncture. Burning sticks of incense, yarn,
Folk healing practices are used by various cultures to treat ill-
cigarettes, or cones of the herb Artemisia are used to make
ness. Coin rubbing (also known as Cao Gio, scratch the wind,
small circular burns on the skin at therapeutic points. Maquas
or coining) is practiced among Southeast Asians (Vietnamese
is used by Arabs, Jews, and Bedouins. Hot metal spits or coals
and Cambodians) when a child is sick. Warm oil (eg, tiger
are applied to areas of disease or over a traditional “draining
balm or mentholated oil) is applied over the affected region
point” to produce burns. Caida de mollera (fallen fontanelle)
of the body, and then it is vigorously rubbed in with the edge
is used by Mexicans and Hispanics. An attempt is made to
of a coin. This is an attempt to rid the body of “ill wind” to
elevate the fontanelle in infants who are dehydrated by hold-
reduce fever and chills. Cupping (ventosa) is used in some
ing the infant upside-down. Retinal hemorrhages may be seen
Eastern European, Latin American, and Russian cultures to
with this practice and it resembles shaken impact syndrome.
reduce congestions. Alcohol is applied to the inner rim of the
cupping glass and ignited with cotton soaked in alcohol. The
cup is applied to the skin after the flame is extinguished. As Emergency Department
it cools, a vacuum forms in the cup causing an ecchymotic Treatment and Disposition
lesion at the site. Spooning (spoon rubbing or quat sha) is used
These practices are not followed to injure the child, but rather
among the Chinese to relieve pain and headache. The skin
are performed with the belief that they will help the child heal
is scratched with a porcelain spoon until ecchymotic lesions
or recover from an illness. Ignorance of these folk healing
practices on part of the medical team results in allegations
of physical abuse, and trust between the medical team and
the family may be irrevocably damaged. Consultation with

FIGURE 2.4 ■ Coin Rubbing (Cao Gio). Linear, bilateral, and sym-
metrical lines of purpura in the intercostal spaces in a Cambodian
boy produced by rubbing with a hot coin in an effort to cure FIGURE 2.5 ■ Coin Rubbing (Cao Gio). This Cambodian boy with
fever. (Reproduced with permission from: American Academy of linear patterns of petechiae and purpura on the neck was referred
Pediatrics: The Visual Diagnosis of Child Physical Diagnosis. The to the ED by the school nurse to “rule out” child abuse. The com-
C. Henry Kempe National Center on Child Abuse and Neglect. mon sites in coin rubbing are along bony prominences and include
American Academy of Pediatrics, Elk Grove Village, IL, 1994, spine, neck, and intercostal spaces. (Photo contributor: Binita R.
p. 12.) Shah, MD.)

28
FOLK HEALING PRACTICES (CONTINUED) ■ 29

a child abuse specialist may help in difficult cases. Educate


parents about the injurious nature of these practices and sug-
gest alternative approaches to treating illness.

Pearls
1. Folk healing practices (also known as pseudo-battering)
may be mistaken for inflicted injuries from child abuse.
2. Physicians should become familiar with folk healing
practices in their community.

B FIGURE 2.7 ■ Inflicted Child Abuse Bruises. Differential Diag-


nosis of Cupping and Coin Rubbing. (A) Belt buckle mark from
FIGURE 2.6 ■ Cupping (Ventosa). (A) Several round identical pur- inflicted injury. (B) Close-up of belt buckle mark. Geometric shapes
puric lesions are seen on the back because of cupping that was used or patterns to bruises suggest child abuse. (C) Bruises inconsistent
to relieve back pain. First- or second-degree burns may also be seen with the stated history or multiple bruises in different stages of heal-
in cupping. The common sites are back, abdomen, and chest. (B) ing (suggestive of repeated abuse), unusual locations (eg, genitalia,
Close-up of purpura produced by cupping. (Photo contributor: Binita buttocks, neck) suggest child abuse. (Photo contributor: Binita R.
R. Shah, MD.) Shah, MD.)
COLD PANNICULITIS

Clinical Summary of the mouth) and may be unilateral or bilateral. Panniculitis


lesions resolve spontaneously in 2 to 3 weeks without scar-
Cold panniculitis is an inflammation of subcutaneous fat after
ring. Differential diagnosis includes child abuse, subcuta-
prolonged exposure to cold or prolonged application of a cold
neous fat necrosis from other etiology (eg, hypercalcemia,
object to any area of the skin (eg, ice packs applied to the
trauma), buccal cellulites, giant urticaria, contact dermatitis,
face of an infant to control supraventricular tachycardia or
or frostbite.
ice packs applied to the lower extremities after vaccination).
It is believed to occur solely because of the inherent proper-
ties of infant body fat with a higher percentage of saturated Emergency Department
fatty acids (compared with older children and adults) and an Treatment and Disposition
increased propensity to solidify with prolonged exposure to
Cold panniculitis is self-limiting and does not require spe-
a cold object. The degree of fat necrosis is inversely related
cific therapy except reassuring caregivers. Recurrence is
to the age of the patient. Popsicle panniculitis is caused by
common and parents must be educated about the condition.
sucking on ice, seen especially in infants who while sucking
Dermatology consultation and a skin biopsy (nonspecific
on the popsicles or ice cubes do not move them around in the
lobular adipocyte necrosis at the dermal–epidermal junction
mouth (as done by adults) keeping the cold object in contact
with a surrounding mixed inflammatory infiltrate seen) may
with buccal fat for a prolonged period.
aid in doubtful cases.
Popsicle panniculitis is commonly seen in the summer.
Typically there are no systemic signs like fever or leukocyto-
sis. The skin lesion is red to purplish indurated, discrete nod- Pearls
ules or plaques with mild tenderness and sometimes itching. 1. A history of skin exposure to a cold object followed by
Lesions are seen in the perioral area (adjacent to the corners a skin lesion supports the diagnosis of cold panniculi-
tis. (Important: Panniculitis arises within hours to 1 to
2 days after the exposure to a cold object).
2. Popsicle panniculitis lesions due to sucking on ice are
seen adjacent to the corners of the infant’s mouth.

A B

FIGURE 2.8 ■ Cold Panniculitis. (A) An infant was referred to “rule out” child abuse when this erythematous and indurated lesion near the
corner of the mouth was seen during a well baby visit. It was thought to be a red bruise produced by pinching. A history of sucking on an ice
cube 2 days prior to appearance of this lesion was subsequently obtained. (Photo contributor: Binita R. Shah, MD.) (B) Bilateral erythema-
tous, indurated lesions adjoining each side of the mouth after sucking on a popsicle. (Reproduced with permission from: Shah BR, Laude T:
Atlas of Pediatric Clinical Diagnosis. WB Saunders, Philadelphia, 2000, p. 176.)

30
COLD PANNICULITIS (CONTINUED) ■ 31

A B

FIGURE 2.9 ■ Cold Panniculitis versus Buccal Cellulitis. (A) An afebrile, very playful infant with an erythematous, indurated, and mildly
tender lesion on the cheek was admitted with a diagnosis of child abuse versus buccal cellulites. A history of putting ice packs on his cheeks
because of an extreme heat wave 2 days prior to appearance of the rash was obtained subsequently. (B) Erythematous, indurated, and tense
swelling with severe tenderness was seen in this highly febrile and irritable child. Blood culture was positive for Streptococcus pneumoniae.
(Photo contributor: Binita R. Shah, MD.)

A B

FIGURE 2.10 ■ Cold Panniculitis. (A) A young infant presented with an erythematous, indurated, linear “skin rash,” thought to be a bruise
inflicted by a long object (eg, a ruler or belt), and the family was referred to child protective services. The mother said that her 6-year-old son
was eating a popsicle (like that shown in Figure 2.10B), and had placed the popsicle on the thigh of the infant. A skin biopsy confirmed the
diagnosis of cold panniculitis, and charges were subsequently dropped against the family. (Photo contributor: Binita R. Shah, MD.)
NEVI OF OTA AND ITO

Clinical Summary Pearls


Nevus of Ota and Ito arise from dermal melanocytes and 1. Nevi of Ota and Ito may be mistaken for ecchymoses due
are present at birth in about 50% of cases. A second peak to inflicted child abuse injuries. However, these nevi are
occurs during puberty. The skin lesion is a light to dark nontender unlike ecchymoses, which may be tender.
brown, blue-black, or bluish-gray macule with a mottled or 2. An ecchymotic lesion undergoes sequential color changes
specked appearance. It increases in intensity and size with and resolves within a few days. These nevi do not undergo
time and persists for life. They are more common in Asians similar color changes and are permanent lesions.
(about 75% of cases; most commonly found in the Japanese), 3. These nevi are unilateral, present in a specific anatomic
in African Americans, and in girls. Nevi of Ota occur on the location and have a speckled or mottled appearance
face, forehead, zygomatic region, periorbital area, cheek, unlike Mongolian spots, which are uniform in appearance
nose, or eye. Ipsilateral eye involvement (ocular melano- and fades spontaneously over the years.
sis) is commonly seen in moderate to severe cases and may
involve the sclera (most common), conjunctiva, cornea, iris,
retina, and optic nerve. Other mucous membranes and tis-
sues (ear canal, tympanic membrane, pharynx, hard palate,
nasal mucosa, buccal mucosa) may be rarely involved. Nevi
of Ito occur on the supraclavicular region, shoulder, upper
arm, and neck. Differential diagnosis of these nevi includes
postinflammatory hyperpigmentation, café-au-lait spot, or
Mongolian spots.

Emergency Department
Treatment and Disposition
The diagnosis is made by typical clinical appearance. These
nevi do not require any intervention in the ED. Patient can
be referred to a dermatologist for evaluation and therapies
that may include laser surgery or masking with cosmetics.
If ocular melanosis is present, glaucoma may occur (mela-
nocytes in the ciliary body of the anterior chamber may
impede normal flow of fluid), and patients also need peri-
odic funduscopic examinations by an ophthalmologist.
FIGURE 2.11 ■ Nevus of Ota. Unilateral blue-gray pigmentation
involving the face and periorbital area (ophthalmic and maxillary
divisions of the trigeminal nerve). (Photo contributor: Binita R.
Shah, MD.)

32
NEVI OF OTA AND ITO (CONTINUED) ■ 33

FIGURE 2.12 ■ Nevus of Ota versus Inflicted Bruise from Child


Abuse. (A) Close-up of patient (Figure 2.11) showing blue-gray pig-
mentation with a specked appearance and involvement of the sclera.
(B) Bluish and red areas of discolorations are seen around the eye
of a patient who was punched in both eyes by her stepmother. She
also had subconjunctival hemorrhage in her other eye and numerous
bruises in different stages of healing. (Photo contributor: Binita R.
B
Shah, MD.)

FIGURE 2.13 ■ Nevus of Ito. An incidental finding of blue-gray pigmentation involving the scapular and deltoid region in a child with
Gianotti Crosti syndrome. (Photo contributor: Sharon A. Glick, MD.)
PORT-WINE STAIN

Clinical Summary Emergency Department


A port-wine stain (PWS), also known as nevus flammeus, is a Treatment and Disposition
congenital vascular malformation of dermal capillaries pres- PWS is diagnosed clinically and does not require any inter-
ent at birth. Most commonly they are seen on the face or neck vention in the ED. Refer the patient to a dermatologist for
but may occur anywhere. The lesion is pink to purple, varied evaluation for laser therapy. Radiologic studies (as clinically
in size and shape, and sharply circumscribed. Large lesions indicated) may be ordered to evaluate patients with Sturge-
follow a dermatomal distribution and rarely cross the midline. Weber syndrome presenting to the ED with seizures and may
During infancy and childhood the lesion is macular; as age include CT scan and/or MRI. Such patients will need appro-
increases, it may become darker in color, papular or nodu- priate referrals to neurologist and ophthalmologist.
lar, causing disfigurement. Elevated skin lesions may bleed
spontaneously.
PWS may occur as an isolated birthmark (majority of Pearls
patients) or be associated with a syndrome, including Sturge- 1. PWS can be confused with inflicted bruises from child
Weber syndrome (PWS in the distribution of the V1-V2 abuse.
branch of the trigeminal nerve, intracranial calcifications, 2. An ecchymotic skin lesion undergoes sequential color
seizures, glaucoma, etc) and Klippel-Trenaunay syndrome changes and resolves within a few days. PWS does not
(PWS over an extremity, hemangiomas, varicosities, and limb undergo similar color changes; however, PWS may
hypertrophy of the involved extremity). Differential diagnosis become darker in color and more nodular over the years.
of PWS includes capillary hemangioma and other vascular 3. PWS does not regress spontaneously and persists for a
malformations (eg, salmon patch). lifetime.

FIGURE 2.14 ■ Port-Wine Stain. Pink-purple macular lesions FIGURE 2.15 ■ Port-Wine Stain. Most common location of PWS is
on the buttocks led to referral of this infant to the ED to “rule out” the face. It may be confused with a red or purple bruise inflicted by
child abuse. These lesions were present since birth. Location of abuse, especially when it is very extensive as shown. When a PWS
these lesions can mimic abuse as one of the typical sites for inflicted is localized to the trigeminal area of the face (especially around the
bruises are buttocks and lower back. (Photo contributor: Binita R. eyelids), a diagnosis of Sturge-Weber syndrome must be considered.
Shah, MD.) (Photo contributor: Binita R. Shah, MD.)

34
PORT-WINE STAIN (CONTINUED) ■ 35

A B

FIGURE 2.16 ■ Port-Wine Stain; Klippel-Trenaunay Syndrome (KTS). PWS on the foot and extending onto the leg and thigh in an infant
with KTS. (A, B). PWS associated with hemihypertrophy of the extremity in a child with KTS. Skin discoloration and “swollen” (hypertro-
phied) extremity may be mistaken for inflicted injuries (C). (Photo contributors: Sharon A. Glick, MD [A, B] and Binita R. Shah, MD [C].)
PHYTOPHOTODERMATITIS

Clinical Summary drinks or making lemonade. A history of helping parents


to make such drinks or spilling juice on the body during
Phytophotodermatitis (PPD) is an acute phototoxic eruption
outdoor activities may be present. In temperate countries,
following contact with certain plants, fruits, or vegetables
cases are often seen among children playing outdoors dur-
containing photosensitizing compounds and exposure to
ing summer, when psoralens are most abundant in wild and
sunlight. Examples of plants causing phototoxic reactions
garden plants.
include limes, lemons, celery, parsnip, fig, fennel, parsley,
Berloque dermatitis refers to dermatitis that results from
dill, mangoes, carrots, and meadow grass. PPD occurs only
application of a psoralen-containing perfume (eg, oil of ber-
with contact with a photosensitizing furocoumarin (pso-
gamot), followed by UV exposure. The patient develops a
ralens) compound that readily penetrates the epidermis
rash at the exact areas where the perfume was applied.
and subsequent exposure to UV radiation (wavelengths
Acute presentation of PPD after inflammation can be
greater than 320 nm). PPD is common among food han-
a mild reaction with erythema or a severe reaction that
dlers (eg, salad makers and grocery workers) and the most
includes tingling of exposed skin and erythema followed
common compounds are limes and lemons used in mixing
by edema and vesiculation (after 24 hours; similar to severe
sunburn). Bullous formation lasting for days may occur.
Presentation after resolution of inflammation includes des-
quamation followed by hyperpigmentation. Configuration
of lesions may be puzzling or unusual patterns like streaks
(eg, from brushing against a plant), drip marks or haphazard
lines (eg, from spilling celery juice), hand prints (eg, child
touched with hands soaked with lemon juice), or crisscross-
ing linear streaks of erythema, vesicles, and bullae (eg, due
to meadow grass rubbing on the skin). The lesions of PPD
are sharply limited to areas of sun exposure, at the sites of
contact (eg, mouth, face, arms, legs) and seen during hot
and humid sunny days. Ecchymoses from inflicted bruises
due to child abuse is an important differential diagnosis.

FIGURE 2.17 ■ Phytophotodermatitis. Erythematous rash with FIGURE 2.18 ■ Phytophotodermatitis. Bizarre streaky hyperpig-
some areas of hyperpigmentation developed in this child who was mentation on the torso can be mistaken for resolving bruises. A his-
accidentally splashed with lemonade at the beach. (Photo contribu- tory of mixing lemonade at the beach confirmed the impression of
tor: Binita R. Shah, MD.) PPD. (Photo contributor: Binita R. Shah, MD.)

36
PHYTOPHOTODERMATITIS (CONTINUED) ■ 37

Emergency Department Pearls


Treatment and Disposition 1. Phytophotodermatitis is an example of photo-irritant con-
Symptomatic relief may be required for acute presenta- tact dermatitis.
tions, including baths with colloidal oatmeal (eg, Aveeno), 2. Commonest produce containing the offending com-
emollients (eg, Aquaphor or Vaseline), and topical steroids. pounds are limes and lemons used in mixing drinks or in
Systemic therapy as indicated may include analgesics, anti- making lemonade.
histamines (eg, hydroxyzine), and systemic steroids (eg, 3. Phytophotodermatitis may be easily overlooked when
prednisone for 5-14 days). Patients presenting with hyperpig- a patient presents with erythema and vesicles following
mentation do not require any treatment and it will fade over a contact with plants or foods thought by parents to be
period of weeks to months. irrelevant. Diagnosis can be easily made by taking a thor-
ough history.
4. Phytophotodermatitis may be mistaken for child abuse
because of its unusual patterns of cutaneous lesions.

A B

FIGURE 2.19 ■ Phytophotodermatitis. (A) An infant with hyperpigmentation that corresponds to mother’s hands is seen. (Reproduced
with permission from: Shah BR and Laude T: Atlas of Pediatric Clinical Diagnosis. WB Saunders, Philadelphia, 2000, p. 240.) (B) The
mother, whose hands were wet with lime juice, picked the infant who was unclothed on a hot, humid sunny day. (Photo contributor: Binita
R. Shah, MD.)
RACCOON EYE

Clinical Summary fractures) or unilateral or bilateral (eg, direct eye trauma).


Other differential diagnosis of raccoon eye includes coagu-
“Raccoon” eye refers to periorbital ecchymosis and results
lopathy and metastatic neuroblastoma with orbital involve-
from subgaleal bleeding, bleeding from a fracture site in the
ment (see Figure 8-4).
anterior portion of the base of skull (basilar skull fracture)
or from a blow to the forehead. In an upright child, bleed-
ing from the subgaleal space or forehead leaks ventrally Emergency Department
into the facial soft tissues alongside of the nose and into
Treatment and Disposition
the periorbital space. Darkening of the soft-tissue swelling
and eyes (ecchymosis) are seen about 1 to 3 days after the A raccoon eye due to accidental forehead hematoma or sub-
trauma because of degradation of blood products. Because galeal bleeding is usually a benign finding and only requires
of the loose connection of the eyelid skin and underlying reassurance. Neurosurgery consultation will be required for
subcutaneous tissues, dramatic ecchymosis can be seen with raccoon eye due to basilar skull fracture (see management of
mild blunt trauma. The initial subgaleal hematoma may be basilar skull fracture; page 754).
masked by the child’s hair, while a forehead hematoma may
be seen or palpated. Raccoon eyes from basilar skull frac-
ture are usually seen 4 to 6 hours after the traumatic event.
Pearl
The other signs of basilar skull fracture that may be seen are 1. Raccoon eye of accidental trauma can be mistaken for
retroauricular ecchymosis (Battle’s sign), hemotympanum, inflicted trauma from child abuse; however, the orbital
cerebrospinal leaks (otorrhea or rhinorrhea) or VII nerve rim and ecchymotic shiner is nontender in raccoon eye
palsy. Raccoon eye can be bilateral (eg, forehead hematoma, and there is evidence of earlier forehead trauma or basilar
subgaleal bleeding, basilar skull fracture, LeFort II and III skull fracture.

A B

FIGURE 2.20 ■ Raccoon Eye. (A) Periorbital ecchymosis in a child after an accidental fall out of a shopping cart 3 days earlier and hitting
the left side of his head. He was referred to “rule out” child abuse as teacher questioned the mother’s story that this severe swelling around
the eye appeared 2 days after the fall (and not seen immediately after the fall). (B) Note the absence of any other injuries in the periorbital
region or subconjunctival hemorrhage. (Photo contributor: Binita R. Shah, MD.)

38
RACCOON EYE (CONTINUED) ■ 39

A B

FIGURE 2.21 ■ Inflicted Eye Trauma due to Child Abuse versus Raccoon Eyes due to Accidental Trauma. (A) Subconjunctival hemor-
rhage with periorbital ecchymosis and abrasion of the lower eyelid in a patient who was punched in the eye by his father. Other signs of
trauma like laceration, eyelid swelling, tenderness, or hyphema may be also seen with direct eye trauma. (Photo contributor: Binita R. Shah,
MD.). (B) Raccoon eyes 3 days after patient hit her forehead against the bed railing while jumping. Note the absence of subconjunctival
hemorrhage or other signs of direct eye trauma. (Reproduced with permission from: Shah BR: Atlas of Pediatric Clinical Diagnosis. WB
Saunders, Philadelphia, 2000, p. 46.)

FIGURE 2.22 ■ Raccoon Eye. Extensive subgaleal bleed-


ing with unilateral raccoon eye are seen in a toddler after an
accidental fall while running and hitting right side of the scalp.
Severity of such bleeding can be easily mistaken for inflicted
injuries. Bleeding disorders also need to be excluded in such
cases. (Photo contributor: Bipin Patel, MD.)
HAIR-TOURNIQUET SYNDROME

Clinical Summary Emergency Department


Hair-tourniquet syndrome (HTS) occurs when fibers of Treatment and Disposition
hair or thread are wrapped around an appendage produc- As the encircling hair dries out, it shrinks, cutting through
ing tissue necrosis and is frequently an accidental injury. the skin and becoming enveloped, making diagnosis difficult.
A constricting hair or thread decreases lymphatic drainage, Prompt removal of the constricting hair or fibers is required
and lymphedema subsequently impedes venous drainage, to prevent tissue loss. Sometimes the end of the hair or fiber
leading to more edema and eventually preventing arterial is visible and can be used to facilitate its removal; otherwise
flow to the appendage. This obstruction leads to necrosis the wound needs exploration to dissect and remove all the
and tissue loss if not promptly relieved. strands. A meticulous hunt to remove every single strand is
Common sites of HTS include toes, fingers, penis, clito- required before discharging the patient.
ris, or labia. Involvement of toes or fingers is seen in young
infants (birth to 2 years). Penile-tourniquet syndrome is seen
in infants up to 6 years, and clitoral-tourniquet syndrome is Pearls
seen in older girls (8 years to adolescence). Symptoms may 1. Consider HTS in any infant presenting with irritability or
include inconsolable crying or irritability (young infants) inconsolable crying or swelling of an appendage includ-
and odd gait or genital pain (older children). The physical ing the penis or clitoris.
exam may show erythema and swelling of the involved area 2. Even though most cases of HTS are unintentional, the
(early presentation) to gangrene and amputation (late pre- possibility of child abuse must be considered, especially
sentation). Complications are variable depending on dura- if there is a delay in seeking medical care.
tion of strangulation and include amputation of the digit, 3. Deliberate wrapping of hair around the penis to stop wet-
clitoris, or penis and partial or complete transection of the ting has been reported to be a form of punishment used by
urethra. Differential diagnosis of HTS include child abuse some care-takers.
or self-inflicted (eg, older girls with clitoral-tourniquet 4. A cursory examination of digits or other appendages may
syndrome). fail to identify the problem in an infant presenting with
irritability or inconsolable crying. A thorough physical
examination of all digits (after removal of the socks and
mittens) and genitalia is required.

A B

FIGURE 2.23 ■ Hair-Tourniquet Syndrome. (A) An infant presenting with inconsolable crying was found to have a hair wrapped around
a digit that was erythematous and edematous. (B) One or more hairs may be wrapped around one or more times, so a thorough inspection is
required after removal of hair to be sure no strands remain. (Photo contributor: Binita R. Shah, MD.)

40
HAIR-TOURNIQUET SYNDROME (CONTINUED) ■ 41

FIGURE 2.24 ■ Hair-Tourniquet Syndrome. A hair wrapped around a toe in an infant with irritability. A full sepsis workup was done before
this was discovered (Pearl: Evaluate thoroughly including examining all digits and genitalia in any infant presenting with irritability or
inconsolable crying). (Photo contributor: Binita R. Shah, MD.)

FIGURE 2.25 ■ Penile-Tourniquet Syndrome. Erythema and edema of the glans penis was produced by a hair wrapped around the glans.
With extreme swelling of the glans and edema of the coronal sulcus as a result of venous and arterial occlusion, constricting strands of hair
may be difficult to see (as in this photo). Hair was deliberately wrapped around the penis to stop bedwetting in this patient as a punishment
by his mother. (Photo contributor: Michael Stracher, MD.)
NUTRITIONAL VITAMIN D DEFICIENCY RICKETS

Clinical Summary failure to thrive, hypocalcemic seizures or tetany (latent or


manifest) are other features of NR.
Rickets is caused by undermineralization of the cartilagi-
nous epiphyseal growth plate (present only in childhood),
resulting in excessive accumulation of unmineralized matrix Emergency Department
(osteoid). The normal product of serum Ca × PO4 concentra-
tion (each measured in mg/dL) is 40; rickets occurs when
Treatment and Disposition
the Ca × PO4 product drops below 30. Nutritional vitamin D Laboratory tests suggestive of NR include either low
deficiency rickets (NR) results from a deficiency of vitamin (early) or normal serum Ca (secondary hyperparathyroid-
D metabolites, including “Sunshine deficiency” (inadequate ism), invariably a low PO4, and an elevated alkaline phos-
exposure to sunlight, or factors preventing UV light penetra- phatase value. Liver enzymes, serum albumin, blood urea
tion like industrial pollution, darkly pigmented skin, abun- nitrogen, and creatinine values help in excluding underly-
dant clothing), dietary vitamin D deficiency (eg, exclusive ing liver or kidney disease as a cause of rickets. Additional
breast-feeding without vitamin D supplementation, strict tests for confirmation of NR include an elevated serum para-
vegan diet), and fat malabsorption (eg, celiac disease, extra- thyroid hormone (PTH) and a decreased serum calcidiol
hepatic biliary atresia). (25-hydroxycholecalciferol) values.
Rachitic deformities that are seen on physical examina- A single anteroposterior (AP) view of the knee in chil-
tion include bow-legs, prominent wrists and ankles, rachitic dren <3 years of age (femur and tibia being the most rap-
rosary, Harrison groove (weakened ribs pulled by muscles, idly growing bones in infants leading to accentuated rachitic
producing flaring over the diaphragm), craniotabes, frontal changes) or a single AP view of the wrist in older children is
bossing with craniosomatic disproportion, knock-knee (genu obtained. Radiographic changes suggestive of rickets include
valgum), or kyphoscoliosis. Generalized muscular hypotonia, cupping of metaphyses (concave deformity of the end of the

A B

FIGURE 2.26 ■ Rachitic Deformities. (A) Prominent wrist. Prominent enlargement of the wrist results from excessive accumulation of
osteoid. This African American infant was exclusively breast-fed and did not receive any vitamin D supplementation. This finding can be
mistaken for abusive injury (eg, swelling due to fracture). (B) Bowing of the forearm with prominent wrist. (Photo contributor: Binita R.
Shah, MD [A, B].)

42
NUTRITIONAL VITAMIN D DEFICIENCY RICKETS (CONTINUED) ■ 43

C D

FIGURE 2.26 ■ (Continued ) (C) Bow-legs (Genu varum). (D) Kyphosis. Vertebral softening due to rickets leading to kyphosis is seen in
a malnourished child who was fed a strict vegan diet (boiled vegetables and rice) with no dairy products. Child abuse/neglect needs to be
excluded in such patients. (Photo contributor: Binita R. Shah, MD [C, D]). (E) Rachitic rosary (enlargement of costochondral junctions).
(Reproduced with permission from: Shah BR and Laude TL: Atlas of Pediatric Clinical Diagnosis. WB Saunders, Philadelphia, 2000, p. 382.)
44 ■ NUTRITIONAL VITAMIN D DEFICIENCY RICKETS (CONTINUED)

long bone shaft, instead of the normal convex or flat appear- Pearls
ance), fraying of metaphyses (indistinct, shaggy borders),
1. Metaphyseal changes of rickets can be mistaken for skel-
widening of metaphyses, generalized osteopenia, thinning of
etal injuries of abuse. Presence of rachitic changes on the
the cortex, pseudofractures, and bowing deformities of long
physical examination and lack of evidence of abusive
bones. Endocrine consultation may be requested, if diagnosis
injuries will help to differentiate the two.
is uncertain.
2. Lucent epiphyseal-metaphyseal junctions seen in rick-
Patients presenting with symptomatic or asymptomatic
ets may resemble the “bucket-handle” fractures of child
hypocalcemia require hospitalization for correction of hypo-
abuse. However, widening of the metaphyseal plates dis-
calcemia and initiation of vitamin D therapy. Asymptomatic
tinguishes rickets radiographically from abuse-related
normocalcemic patients can be referred to a primary care phy-
fractures.
sician or endocrinologist for the treatment of rickets.

FIGURE 2.27 ■ Rickets. Anteroposterior (AP) view of the knee (left; pretreatment) shows metaphyseal widening, cupping, and fraying
of the distal femur and proximal tibia and fibula. Normally the epiphyses should be “hugging” the metaphyses; the increased distance seen
between the metaphyses and epiphyses (radiolucent zone) is due to the presence of radiolucent osteoid. A repeat radiograph (right) 2 weeks
following the vitamin D therapy shows healing rickets. (Photo contributor: Binita R. Shah, MD.)
NUTRITIONAL VITAMIN D DEFICIENCY RICKETS (CONTINUED) ■ 45

FIGURE 2.28 ■ Differential of Child Abuse and Rickets. (A) Child


abuse. Anteroposterior radiograph of the leg shows lucency travers-
ing the distal tibia (arrow), extending to the corners, and indicative
of fracture—known as the classical metaphyseal lesion. (B) Rickets.
Bilateral and usually symmetrical pattern of skeletal involvement
with widening of physis, and metaphyseal fraying distinguishes rick-
ets radiographically from abuse-related fractures. (Photo contribu-
A
tors: John Amodio, MD [A] and Binita R. Shah, MD [B].)
OSTEOGENESIS IMPERFECTA

Clinical Features skin and ligaments of joints (hands, feet, knees), and wor-
mian bones (small, irregular bones seen along the cranial
Osteogenesis imperfecta (OI) is a genetic disorder of connec-
sutures) and spine changes (scoliosis, kyphosis, codfish ver-
tive tissue seen in all racial and ethnic groups. It results from
tebrae). Fractures in OI occur following a slight trauma or
an abnormal quantity or quality of type I collagen, a primary
spontaneously in an active child.
component of the extracellular matrix of the bone and skin.
Seven clinical subtypes of OI (type I to VII) are seen with Emergency Department
variable manifestations. Genetic inheritance is autosomal
dominant in the majority of the subtypes. The triad of OI
Treatment and Disposition
includes fragile bones, blue sclerae, and deafness (conduc- Serum calcium, phosphorus and alkaline phosphatase val-
tive hearing loss seen in adolescents and adults). Other fea- ues, and coagulation profile tests are normal in a child with
tures include easy bruising, fragile skin, growth retardation OI. Radiographic features of OI include osteopenia, thin
with short stature, defective dentinogenesis, hyperlaxity of cortices, bowing, angulation of healed fractures and normal

A B

FIGURE 2.29 ■ Osteogenesis Imperfecta (OI). (A) An infant


with multiple angular deformities with OI, type I (the most com-
mon type of OI). (Reproduced with permission from: Shah BR and
Laude TL: Atlas of Pediatric Clinical Diagnosis. WB Saunders,
Philadelphia, 2000, p. 382.) (B) AP view of the lower extremity in
a different patient with OI, type I, demonstrates diffuse osteopenia,
bowing deformity of right femur, and fractures in different stages
of prolonged healing. Note the multiple growth arrest lines of the
distal femora, frequently seen in OI. (C) AP view of the chest in a
patient with OI, type I, shows diffuse osteopenia and multiple right
rib deformities compatible with healing fractures. (Photo contribu-
C
tor: John Amodio, MD [B, C].)

46
OSTEOGENESIS IMPERFECTA (CONTINUED) ■ 47

callus formation at the sites of recent fractures. Most frac- with increased bone fragility. Osteotomies may be needed
tures in OI are situated in the diaphysis. Consultation with to straighten bone, and intramedullary rods may be inserted
a radiologist is valuable in a difficult case presenting with to maintain correct alignment and repair deformities arising
multiple fractures and when a diagnosis of child abuse can- both from fractures and from progressive bowing or bending
not be excluded. Orthopedic consultation in the ED may of the skeleton. OI patients need a close follow-up by ortho-
be required depending on the type and location of the frac- pedics and their primary care physicians. Multidisciplinary
tures. Goals of therapy include splinting or casting of frac- management with referrals as indicated to a dentist, a
tures and maximizing comfort and function. Management geneticist, an endocrinologist, and a social worker for fam-
of fractures in OI patients with moderate severity is no dif- ily support are also needed. In doubtful cases, diagnosis of
ferent from management of fractures in otherwise normal OI can be confirmed by collagen biochemical studies or a
children. Protective orthotics may be needed in children skin punch biopsy.

A B

FIGURE 2.30 ■ Osteogenesis Imperfecta (OI). (A) Bilateral blue


sclera noted at birth in a patient with OI, type II. (Reproduced with
permission from: Shah BR and Laude TL: Atlas of Pediatric Clinical
Diagnosis. WB Saunders, Philadelphia, 2000, p. 382). (B) An infant
with OI, type II, with deformities noted at birth (type II presents with
multiple fractures in utero/perinatal period). (C) AP view of an infant
with OI, type II, shows multiple congenital fractures involving the
upper and lower extremities and ribs, with small lung capacity. (Photo
C
contributors: Binita R. Shah, MD [B] and John Amodio, MD [C].)
48 ■ OSTEOGENESIS IMPERFECTA (CONTINUED)

Pearls
1. Osteopenia is insufficient bone mass resulting from
reduced bone production, increased breakdown of bone,
or both. Osteoporosis is a syndrome resulting from osteo-
penia that can lead to increased bone fragility and suscep-
tibility to fractures and skeletal deformities.
2. OI or “brittle bone disease” is the most common osteopo-
rosis syndrome in childhood.
3. OI is less common than child abuse. Milder forms of OI
often get misdiagnosed as child abuse. Multiple fractures
with angular deformities of the long bones, wormian
bones, osteopenia, dentinogenesis imperfecta, and a posi-
tive family history will help to differentiate the two.
4. A history incompatible with injury, the hallmark of
abuse, may also be present in OI. Absence of other signs
of abuse or fractures that occur in different environments
point toward OI.
5. Spiral or transverse fractures of long bones are more
common in OI than metaphyseal fractures; however,
metaphyseal fractures resembling abuse are also seen.
FIGURE 2.31 ■ Osteogenesis Imperfecta. AP view of the skull
demonstrates a “cracked egg shell” appearance secondary to
Wormian bones (excess sutural bones). (Photo contributor: John
Amodio, MD.)

A B C

FIGURE 2.32 ■ Osteogenesis Imperfecta. (A) Severe bowing and deformities of both upper and lower extremities are seen in this girl with
OI, type III. She had normal sclera, triangular facies, and severe bone fragility leading to multiple fractures from early infancy. (B) Radiograph
of the lower extremity showing generalized osteopenia, thinned cortices, and marked angular deformities of the bones. (C) Dentinogenesis
imperfecta showing transparent yellow, prematurely eroded teeth. (Photo contributor: Binita R. Shah, MD.)
LICHEN SCLEROSUS ET ATROPHICUS

Clinical Summary atrophy, becoming depressed plaques with a wrinkled sur-


face. The location of lesion in girls is vulvar, perianal, and
Lichen sclerosus et atrophicus (LSA) is characterized by dis-
perineal skin (most common site). Atrophic plaques may
tinctive white lesions on the genitalia. It is predominantly
lead to shrinkage of the labia and stenosis of the introitus.
seen in girls (F:M ratio 10:1) and in boys it is called balani-
In boys, the lesion is on glans and undersurface of prepuce.
tis xerotica obliterans. LSA occurs most commonly between
Extragenital lesions appear as atrophic depigmented patches
1 and 13 years of age. Most lesions appear spontaneously,
with cigarette paper–like wrinkling of the surface and sites
but some may be induced by trauma. Borrelia burgdorferi or
include neck, axillae, trunk, periumbilical, and flexor surfaces
other similar strains may be involved in the development or
of wrists and around eyes.
as a trigger.
Differential diagnosis include vitiligo, postinflammatory
Pruritus, burning, constipation, dysuria, dyspareunia, vagi-
hypopigmentation, sexual abuse, bacterial vulvovaginitis, and
nal discharge preceding vulvar lesions (girls), and phimosis
intertrigo.
and recurrent balanitis (boys) are common presenting symp-
toms. The skin lesion starts as ivory-colored shiny, indurated
papules that become confluent irregular plaques, which may Emergency Department
develop hemorrhagic bullae at their margins. Later lesions Treatment and Disposition
Topical steroid cream is the initial treatment and patient needs
to be referred to a dermatologist or a primary care physician
for ongoing treatment and follow-ups. LSA usually resolves
spontaneously without sequelae in about two-thirds of chil-
dren around puberty; about one-third will have persistence of
LSA into adulthood.

Pearls
1. LSA is one of the most common dermatitides mistaken
for sexual abuse, especially when it presents with purpura
of the vulva.
2. LSA lesions are atrophic, porcelain-white plaque around
the anus and vulva forming a figure-8 or hourglass con-
figuration. This most distinctive pattern is seen in prepu-
bertal females as well as in adults.
FIGURE 2.33 ■ Lichen Sclerosus et Atrophicus. White atrophic
3. Nearly all affected boys with LSA present with phimo-
plaque with erythema in an hourglass distribution surrounding the
vagina and perirectal area. This patient complained of pruritus. sis in a previously retractable foreskin with an atrophic
(Photo contributor: Sharon A. Glick, MD.) depigmented plaque at the tip of the penis.

49
PERIANAL STREPTOCOCCAL DERMATITIS

Clinical Summary
Perianal streptococcal dermatitis (PSD) is a superficial bacte-
rial infection caused by group A β-hemolytic Streptococcus
(GABHS) in the perianal area. PSD is also referred as peri-
anal streptococcal cellulitis. Chronic PSD may present with-
out any signs of cellulitis; thus, the preferred term is PSD. It
primarily occurs in children between 6 months and 10 years
of age. About 70% of cases involve boys. Intrafamily spread
can occur (eg, family members bathing together). Siblings
may be either asymptomatic with only GABHS-positive cul-
tures, or may develop similar dermatitis.
Perianal dermatitis and pruritus are the most common pre-
senting symptoms followed by painful defecation with rec-
tal pain, perianal pain, constipation, blood-streaked stools,
and anal discharge. Systemic symptoms are usually absent.
During the acute phase (<6 weeks), the perianal area may
show bright red erythema (without induration) that is tender
and confluent from anus outward. Size varies from a few to
several centimeters. During the chronic phase, patients may
present with painful perirectal fissures, dried mucoid dis-
charge leading to irritation and excoriation, proctocolitis,
or bleeding with little or no erythema. Psoriasiform plaques
with yellow peripheral crust may also be seen. Infection may
involve penis or vulva.
Differential diagnosis includes sexual abuse, candidiasis, A
contact, atopic or seborrheic dermatitis, chronic diaper der-
matitis, psoriasis, pinworm infestation, inflammatory bowel
disease, or local trauma (heavy wiping).
PSD is diagnosed by its characteristic distribution and con-
firmed by a culture obtained from the perianal area. A specific
request should be made of the laboratory to look for GABHS.
Culture the index patient and family members as indicated. A
moderate to heavy growth of GABHS will confirm the diag-
nosis (children with asymptomatic perianal colonization have
light growth of GABHS). Direct antigen studies for GABHS
may also help (sensitivity about 90%; false-negative results
seen early in the course). A throat culture may also be posi-
tive for GABHS. Rarely perianal dermatitis may be caused by B
Staphylococcus aureus.
FIGURE 2.34 ■ Perianal Streptococcal Dermatitis. (A) This young
girl was brought to “rule out” sexual abuse. This rash was present for
several weeks and she received different ointments without response.
(B) A close-up showing perianal erythema, fissuring, and excoria-
tion with crusting at the periphery. A rectal culture was positive for
GABHS. (Photo contributor: Binita R. Shah, MD.)

50
PERIANAL STREPTOCOCCAL DERMATITIS (CONTINUED) ■ 51

Emergency Department Pearls


Treatment and Disposition 1. PSD is a bright-red, sharply demarcated perianal derma-
PSD should be treated with systemic antibiotic therapy aug- titis caused by GABHS.
mented by topical therapy. Drug of choice is penicillin given 2. PSD often gets mistakenly diagnosed as sexual abuse or
either orally or parenterally. Amoxicillin may be used instead contact or atopic dermatitis.
of penicillin (better-tasting preparation). Erythromycin or 3. Patients with PPD are subjected to treatments for a vari-
clindamycin is used for patients who are allergic to penicillin. ety of different diagnoses without success. Failures to
These alternative antibiotic treatments may also be helpful respond to prior therapy with topical antifungal or corti-
for patients who have not responded to a course of penicil- costeroids or therapy for pinworms are important clues in
lin, or who are infected with S aureus. Topical therapy is suspecting PSD.
with mupirocin ointment. The patient needs to be followed 4. Undiagnosed and inappropriately treated patients with
by the primary care physician for monitoring of response to PSD develop perianal fissures, bleeding, pain during
therapy and to document bacteriologic cure posttreatment. defecation, and constipation.
Recurrences can occur.

FIGURE 2.35 ■ Perianal Streptococcal Dermatitis. A young male child was referred for evaluation of sexual abuse. He had a history of
constipation associated with painful defecation. A rectal culture was positive for GABHS. (Photo contributor: Binita R. Shah, MD.)
LABIAL ADHESION

Clinical Summary diaper rash, and use of harsh soaps are common contributory
factors. Other etiologies include lichen sclerosus, atopic or
Labial adhesion is fusion of the labia minora, usually extend-
seborrheic dermatitis, herpes or pinworm infection, or sexual
ing from an area immediately inferior to the clitoris to the
abuse.
fourchette. The extent of the adhesion is variable and is thick-
Labial adhesion occurs most commonly between 3 months
est posteriorly. Labial fusion and labial or vulvar agglutina-
and 6 years of age (peak: 13 to 23 months); however, it can
tion or synechiae are synonyms commonly used. This is an
occur at any age. It is rare in newborns (exposure to high lev-
acquired condition and occurs as a result of labial abrasion
els of maternal estrogens) and in postpubertal premenopausal
that culminates in medial surfaces of labia minor adhering to
women. The majority of patients are asymptomatic and it is
one another followed by epithelialization. Fusion occurs sec-
usually accidentally noted by a parent or by a physician dur-
ondary to local inflammation in the presence of the hypoes-
ing examination. Symptoms of urinary tract infection like
trogenic state of a preadolescent child (inflamed/injured
dysuria and frequency may be present (related to pooling of
epithelium is more likely to fuse in a low-estrogen environ-
urine in the vagina leading to recurrent vulvovaginitis). Other
ment). Nonspecific vulvovaginitis, poor perineal hygiene,
symptoms include urinary retention or altered urinary stream.
Patients with labial adhesions have normal female genita-
lia; however, hymen and introitus are obscured because of
adhesions. With complete adhesions, the urethra is usually
not visualized. Urine may be seen dribbling at the anterior
end (just posterior to the clitoris). Labial adhesions may also
reoccur.
Differential diagnosis includes imperforate hymen, sexual
abuse, ambiguous genitalia, congenital absence of the vagina,
and scarring following female circumcision.

FIGURE 2.36 ■ Labial Adhesion. A young, asymptomatic girl was


accidentally found by her parents to have “abnormal looking private
parts.” With complete adhesions, the introitus is not seen and this
is often interpreted by parents as “absence of vagina.” It may also
appear that there is no urethral opening. A thin line of central raphe
FIGURE 2.37 ■ Labial Adhesion. Almost complete adhesions are
(arrow) where the labia are fused is seen. Visualization of a midline
seen in this infant, with a very small opening seen near the posterior
raphe excludes the diagnosis of imperforate hymen. (Photo contribu-
fourchette (arrow). This patient presented with acute urinary reten-
tor: Binita R. Shah, MD.)
tion. (Photo contributor: Binita R. Shah, MD.)

52
LABIAL ADHESION (CONTINUED) ■ 53

Emergency Department pigmentation) and caregiver needs to be educated about this.


If the patient presents with acute urinary symptoms, a gentle
Treatment and Disposition
attempt to separate the labia with a Calgiswab can be made
Spontaneous resolution (without therapy) occurs in the (only if adhesions appear to separate easily) after applying
majority of children (>80%) over the course of a year. With 5% lidocaine ointment or 2.5% prilocaine cream.
endogenous estrogen production and the vaginal pH becom- Labial adhesions can result from sexual abuse, but in
ing more acidic at puberty, labial adhesions almost always the absence of other evidence, the presence of adhesions
resolve spontaneously. No treatment except reassurance is alone is not diagnostic of sexual abuse. Consider possibility
indicated for patients with asymptomatic adhesions. General of sexual abuse in girls (especially older) presenting with
measures include reassurance to the family about the pres- adhesions many years after the toilet training or without
ence of normal female genitalia, improving perineal hygiene any prior history of adhesions or any predisposing skin con-
(eg, regular changing of diapers and thorough wiping after dition or presenting with thick adhesions that are difficult
each bowel movement from anterior to posterior), removal to treat.
of irritants like caustic soaps, treatment of diaper dermatitis,
loose-fitting cotton underwear, and sitz baths. Estrogen cream
may be considered for patients with nearly complete adhe-
Pearls
sions with or without urinary symptoms or for patients with 1. A thin vertical raphe (line of adherence) in the midline
significant parental anxiety. Patients will need a follow-up where the labia are fused is pathognomonic for labial
with the primary care physician. A small quantity of estrogen adhesions.
cream is applied topically with gentle traction twice a day 2. Labial adhesions should not be separated manually by
for 3 weeks, then at bedtime for 3 weeks. An additional few force; it is painful and the resulting raw surfaces have a
weeks of application of an inert ointment (eg, petroleum jelly) greater tendency to reagglutinate.
keeps the labia apart while healing is completed. Prolonged 3. Patients with labial adhesions have normal female geni-
use of estrogen cream should be discouraged because of talia; however, hymen and introitus are obscured because
side effects (eg, breast enlargement and/or tenderness, vulvar of adhesions.

FIGURE 2.38 ■ Female Circumcision (Infibulation). This 10-year-old girl underwent excision of her clitoris and labia minora (as ritual
female circumcision) during her early childhood in Africa. Scarred tissue and a narrowed introitus following surgery may mimic labial adhe-
sion or trauma due to sexual abuse. The practice of female circumcision is seen in Africa, Middle East, and Muslim populations of Indonesia
and Malaysia. The type of mutilation ranges from simple excision of the prepuce of the clitoris to complete excision of all elements of the
vulvar region, leaving a very small opening for the passage of urine and menstrual fluid. Female circumcision is illegal in Western countries
and is classified as child abuse in some European countries. (Photo contributor: Swati Mehta, MD.)
URETHRAL PROLAPSE

Clinical Summary blood flow with subsequent edema and erythema or purplish
appearance of the mucosa. Thrombosis and necrosis of the
Urethral prolapse is an eversion of the distal urethral mucosa
mucosa may result if the process is not corrected.
protruding through the urethral meatus and may be partial
Urethral prolapse is seen almost exclusively in premen-
or complete. Predisposing factors include increased intra-
archeal girls, with a peak incidence between 4 and 10 years
abdominal pressure (eg, violent coughing, constipation) in
of age, and about 95% of cases are African Americans. The
a hypoestrogenic state and preceding trauma (eg, straddle
most common presenting symptom is painless bleeding or
injury or sexual abuse). Constriction of the prolapsed mucosa
spotting on the underwear (90% of patients). Other symptoms
at the urethral meatus may lead to impairment of the venous
may include dysuria and/or urinary frequency (due to urethral
inflammation), difficulty voiding or urinary retention (pro-
lapse may occlude urethral meatus) or perineal discomfort.
Patient may also be completely asymptomatic and prolapse
may be noted on routine examination.
Clinical signs of a prolapsed urethra include cherry-red
doughnut or prolapsed cervix-like circular, nontender mass at
the introitus. It may appear as a friable rosette of red or hem-
orrhagic tissue, may be ulcerated or gangrenous (necrotic)
or infected. A centrally located urethral meatus may not be
visible in the presence of severe edema or strangulation.
Differential diagnosis of urethral prolapse includes hema-
toma of hymen from sexual abuse, straddle injury, hemato-
metrocolpos, prolapsed ectopic ureterocele or urethral polyp,
urethral cysts, condylomata acuminata, hemangioma, and
A sarcoma botryoides.

Emergency Department
Treatment and Disposition
Urethral prolapse is a clinical diagnosis. Conservative ther-
apy is used for mild cases without necrotic mucosa. This
includes warm sitz baths and emollient cream. If indicated,
topical antibacterial therapy or topical estrogen therapy (the
distal urethra is estrogen dependent) twice a day for 10 to
14 days can be used. It usually resolves spontaneously or
with the above therapy in 2 to 3 weeks. Urologic or surgical
consultation is required for patients with necrotic mucosa
or recurrent prolapse. Indications for surgery (excision and
B
reapproximation of the mucosal edges or CO2 laser ther-
FIGURE 2.39 ■ Urethral Prolapse. (A) Blood-stained underwear apy) include prolapse with necrotic mucosa at the time of
from an asymptomatic 4-year-old African American girl is shown. presentation, persistent prolapse with failed medical ther-
Painless bleeding or spotting on underwear, the most common apy, recurrent symptomatic prolapse, or urinary retention.
symptom of urethral prolapse, often gets mistaken for vaginal bleed-
Recurrences may be observed weeks to months after the
ing or hematuria or rectal bleeding. (B) Urethral prolapse is usually a
complete circle forming a doughnut-shaped, hyperemic, edematous initial resolution following medical therapy. Recurrence is
mass obscuring the vaginal introitus. Center of the “doughnut” is the uncommon after surgery.
urethral orifice. (Photo contributor: Binita R. Shah, MD.)

54
URETHRAL PROLAPSE (CONTINUED) ■ 55

Pearls bladder through the central dimple of the mass or observa-


tion of the child during voiding will aid in the diagnosis.
1. Urethral prolapse is the most common cause of apparent
3. Clinical findings of urethral prolapse can be mistakenly
vaginal bleeding in premenarcheal girls.
attributed to sexual abuse, especially when prolapsed
2. Urethral prolapse presents as an interlabial mass and is
mucosa has become hemorrhagic and friable.
the only lesion that has a circular mass of tissue surround-
4. Urethral prolapse cannot be reduced manually.
ing the urethral meatus. If in doubt, catheterization of the

FIGURE 2.40 ■ Urethral Prolapse. The prolapse may be large enough to conceal the introitus. Retraction of the vulva gently in a downward
and lateral direction or placing the child in a knee-chest position may help in appreciating the anatomy clearly, with the vaginal introitus seen
as a separate structure posterior to the prolapsed urethra. (Photo contributor: Binita R. Shah, MD.)

FIGURE 2.41 ■ Urethral Prolapse. A doughnut-shaped, edematous mass with hemorrhagic mucosa obscuring the vaginal introitus is seen.
This finding can be mistakenly attributed to trauma caused by sexual abuse. (Photo contributor: Binita R. Shah, MD.)
STRADDLE INJURY

Clinical Summary majora and labia minora. Other sites may include clitoral
hood, clitoris, periurethral tissue, or posterior fourchette, but
Straddle injuries are the most common type of accidental
hymen or vagina are usually not involved (unless accidental
injuries to the genitalia and rarely involve penetration. Soft
penetrating injury). Typical injuries are linear abrasions, bruis-
tissues get crushed between the pubic bone (eg, pubic sym-
ing, or hematoma of labia majora or minora. Small posterior
physis, ischiopubic ramus) and a hard object during impact,
fourchette tears may be seen. Swelling, pain, and bleeding are
such as accidentally falling on a furniture arm, bed, bicycle
common symptoms. Findings in the boys include ecchymosis
crossbar, balance beam, fence, toilet seat, concrete wall, or
or minor laceration of scrotum and/or penis. Scrotal contents
playground equipment.
may be contused or crushed. Injury to anterior urethra is sug-
Straddle injuries are usually unilateral and involve the
gested by blood at the meatus, periurethral or perineal swell-
anterior portion of external genitalia in both girls and boys.
ing, and difficulty voiding.
Injuries to internal genital structures or the anus are rare (pro-
Differential diagnosis of straddle injuries includes self-
tected by soft tissues of the buttocks, bones of the pelvis, and
inflicted injuries, which are exceedingly rare in children.
labia in girls). The most common location in girls is the labia
Normal masturbation in girls is clitoral or labial and does not
cause genital injury. Self-inserted foreign bodies also do not
cause injuries to the hymen.

FIGURE 2.42 ■ Straddle Injury. Laceration of the labia majora FIGURE 2.43 ■ Straddle Injury. A laceration of the vaginal wall
extending into periurethral tissue secondary to a fall onto the bar of is evident here at 11:30 after this teenager crashed her bicycle into
a bicycle is seen in this girl. Her injury was unilateral and did not another rider. Look for other signs of trauma and try to confirm the
involve the urethra, hymen, or vagina. (Photo contributor: Binita R. story with bystanders to make sure it is not fabricated to cover up the
Shah, MD.) abuse. (Photo contributor: Mark Silverberg, MD.)

56
STRADDLE INJURY (CONTINUED) ■ 57

Emergency Department to take sitz baths. Voiding in bath of warm water also may
ease the dysuria. Local application of antibiotics may help in
Treatment and Disposition
promoting healing. Child abuse specialist or child protective
During the evaluation, other associated injuries including services may be consulted prior to discharge if the diagnosis
injury to the urethra must be excluded. Lacerations may be is unclear.
obscured because of swelling. Examination may be limited
as a result of intense pain and fear, and the patient may need
procedural sedation. If internal injuries cannot be excluded in Pearls
girls, examination under anesthesia and evaluation by a pediat- 1. Straddle injuries are the most common type of accidental
ric surgeon or gynecologist is required. If clinically indicated, injuries involving the genitalia and may be difficult to
a scrotal ultrasound to assess testicular injuries and a retro- differentiate from injuries due to sexual abuse. A plau-
grade urethrogram to assess urethral injuries (extravasation) sible explanation and physical examination that sup-
may be ordered. Boys will need evaluation by urologist for ports the history are important in arriving at the correct
urethral, testicular (eg, hematoma, rupture) or scrotal injuries diagnosis.
(eg, lacerations extending through the dartos). Hematomas 2. Suspect sexual abuse if there is a lack of correlation
and superficial lacerations are managed with supportive between history and clinical findings, evidence of injuries
care and resolution occurs spontaneously. Patient’s ability in other areas, genital injuries in nonambulatory children,
to void without any difficulty must be confirmed before the extensive injuries, vaginal, perianal, rectal, or hymenal
discharge. To minimize discomfort, patient can be advised injuries without a clear history of penetrating trauma.

FIGURE 2.44 ■ Straddle Injury. Unilateral scrotal swelling with


bruising and a small abrasion is seen in this boy who fell on a bal-
FIGURE 2.45 ■ Straddle Injury. This scrotal hematoma was sus-
ance beam. Scrotal ultrasound showed a hematoma but the testes
tained after consensual rectal intercourse involving a foreign body
were not involved. (Photo contributor: Binita R. Shah, MD.)
in a teenager; although abuse needs to be considered. Notice also
the laceration of the anterior rectal commissure. (Photo contributor:
Mark Silverberg, MD.)
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INFECTION
Chapter 3

INFECTIOUS
DISEASES
Vikas S. Shah
Binita R. Shah

Ecthyma
gangrenosum

59
STREPTOCOCCAL PHARYNGITIS AND SCARLET FEVER

Clinical Summary sign (an accentuation of the rash in skin folds with fine line
of petechiae). Desquamation may occur after 7 to 10 days
“Strep throat” is acute tonsillopharyngitis caused by
and usually continues for 2 to 3 weeks, but can last up to
Streptococcus pyogenes (group A β-hemolytic streptococci
2 months. When desquamation occurs, it begins as fine flakes
[GABHS]) in the majority, and occasionally by serogroups
on the face, then precedes over the trunk, hands, and feet.
C and G. Strep throat is seen among schoolchildren (5–15
The extent and duration of desquamation are directly related
years) with a peak incidence during the first few years of
to the intensity of the rash. Scarlet fever rash is caused by
school. The incubation period is 2 to 4 days. Presenting
one or more of several erythrogenic exotoxins produced by
symptoms are abrupt onset with high fever, sore throat, pain
GABHS strains and occurs in children who lack the immu-
on swallowing, malaise, headache, and abdominal pain.
nity to the exotoxin. The portal of entry is oropharynx in the
Pharynx is beefy red; palate and uvula may be edematous,
majority and rarely a surgical wound (surgical scarlet fever).
reddened, and covered with petechiae. Tonsils are erythema-
Most cases occur in children between 2 and 8 years of age in
tous, enlarged with patches of a gray-white exudate giving a
winter and early spring.
“strawberry” appearance (intensity of exudates either absent
Both streptococcal pharyngitis and scarlet fever are self-
in mild cases to distinct membrane covering entire pharynx
limited diseases. Fever abates within 3 to 5 days (in absence
in severe cases). Tender anterior cervical lymphadenopathy
of suppurative complications) with resolution of all signs
at the angles of the mandibles is palpable.
and symptoms within 1 week. Tonsillar hypertrophy and
Scarlet fever rash may appear 12 to 24 hours after the onset
lymph nodes take several weeks to return to their usual size.
of fever. The patient’s forehead and cheeks are flushed with
Suppurative complications include acute cervical lymph-
circumoral pallor and a rash of tiny papules that appears like
adenitis, peritonsillar cellulitis or peritonsillar abscess, ret-
a “sunburn with goose pimples,” has the texture of sandpa-
ropharyngeal abscess, acute otitis media, acute sinusitis,
per, and blanches prominently with pressure. The rash begins
mastoiditis, pneumonia, toxic shock syndrome (TSS), intra-
in the axilla, groin, and neck and generalizes, sparing the
cranial complications (eg, meningitis, brain abscess), bacte-
palms, soles, and face within 24 hours. Other findings include
remic spread with metastatic infection (eg, septic arthritis,
strawberry tongue, exudative tonsillopharyngitis, and Pastia
osteomyelitis), and nonsuppurative complications include
acute rheumatic fever (ARF) and acute glomerulonephri-
tis. Differential diagnosis of exudative tonsillopharyngitis
includes viral infections (eg, adenovirus, Epstein-Barr virus,

FIGURE 3.1 ■ Exudative Tonsillopharyngitis. Exudative tonsillo-


pharyngitis in an adolescent presenting with high fever, sore throat,
and tender anterior cervical adenopathy and a throat culture positive FIGURE 3.2 ■ Acute Suppurative Cervical Lymphadenitis. Both
for GABHS. Intensity of exudates can be absent in mild cases to for- throat culture and lymph node aspirate culture were positive for
mation of a membrane covering the entire pharynx in severe cases. GABHS in this girl presenting with anterior cervical lymphadenitis.
(Photo contributor: Mark Silverberg, MD.) (Photo contributor: Binita R. Shah, MD.)

60
STREPTOCOCCAL PHARYNGITIS AND SCARLET FEVER (CONTINUED) ■ 61

A B

FIGURE 3.3 ■ Scarlet Fever. (A) Erythematous scarlatiniform rash with sore throat and fever were seen in this child. Note the prominence
of the rash in the neck, axillas, and groin (all pressure sites). (B) In dark-skinned patients, the scarlet fever rash may be palpated more eas-
ily as “sandpaper texture” than seen. Note the accentuation of the rash in the neck area and absence of conjunctival injection and red lips
(characteristic features of Kawasaki disease that also presents with scarlatiniform rash). (Photo contributors: Bipin Patel MD [A] and Binita
R. Shah, MD [B].)

herpes simplex virus [shallow tonsillar ulcers with gray exu- adequate sample) or rapid antigen detection test as accurate
dates), other bacteria (diphtheria, tularemia), and Candida clinical differentiation of viral and GABHS pharyngitis is not
(immunosuppressed patients). Differential diagnosis of scar- possible. A positive rapid antigen test is diagnostic; a negative
let fever includes Kawasaki disease, staphylococcal scarla- test requires a throat culture. Consider starting therapy after
tina, staphylococcal scalded skin syndrome, viral exanthems, a positive rapid antigen detection test; withholding therapy
mycoplasma infection, TSS, drug hypersensitivity reactions, for 24 to 48 hours while awaiting throat culture results does
severe sunburn, and Arcanobacterium haemolyticum infec- not increase the risk of ARF. Prescribe antibiotics (before
tion (tonsillopharyngitis with scarlatiniform rash; seen in throat culture result) for patients with any of the following:
adolescents and young adults). prior history of ARF, scarlet fever, suppurative complications
(eg, peritonsillar cellulitis or abscess), or patients unavail-
Emergency Department able for follow-up. Prescribe penicillin V (drug of choice)
orally for 10 days (emphasize the importance of completing
Treatment and Disposition the full 10-day course regardless of clinical improvement to
Confirm diagnosis by either performing a throat culture prevent ARF). If compliance is an issue, alternatives include
(vigorously swab tonsils and posterior pharynx to obtain an amoxicillin (single daily dose for 10 days) or benzathine
62 ■ STREPTOCOCCAL PHARYNGITIS AND SCARLET FEVER (CONTINUED)

A B

FIGURE 3.4 ■ “Strawberry Tongue” of Scarlet Fever. (A) White


strawberry tongue (edematous red papillae projecting through white-
coated tongue is seen on the second day of illness in a child with scar-
let fever). (B) The real “red strawberry tongue”? (C) Red strawberry
tongue (as white coating disappears, red tongue is seen studded with
prominent papillae that stand out). Other differential of strawberry
tongue includes Kawasaki disease and toxic shock syndrome (TSS;
Staphylococcus aureus–mediated TSS and Streptococcal-mediated
C
TSS). (Photo contributor: Binita R. Shah, MD.)
STREPTOCOCCAL PHARYNGITIS AND SCARLET FEVER (CONTINUED) ■ 63

FIGURE 3.6 ■ Desquamation as Presenting Sign of Scarlet Fever.


This child was brought to emergency department because of this
prominent (and frightening to parents) desquamation seen in both
hands and lower extremities. He had a sore throat and low-grade
fever with “goose bumps” 9 days before this finding. Desquamation
may be a presenting sign of scarlet fever (if the initial acute phase is
mild and overlooked). Large sheaths of epidermis may be shed from
the palms and soles in a glove-like pattern, exposing new and tender
epidermis beneath. (Photo contributor: Binita R. Shah, MD.)

Pearls
1. Most common clinical illness produced by GABHS is
acute tonsillopharyngitis.
2. Coryza, cough, conjunctivitis, hoarseness, diarrhea,
discrete ulcers, or stomatitis in a child with pharyngi-
tis strongly suggests a viral etiology and not GABHS
infection.
B
3. Exudative pharyngitis in children <3 years of age is
FIGURE 3.5 ■ Desquamation of the Hand and Foot in Scarlet Fever. rarely of streptococcal etiology and diagnostic studies for
(A) and (B) Photographs taken on the eighth day of illness in a child GABHS are not recommended routinely.
with scarlet fever. (Photo contributor: Binita R. Shah, MD.) 4. Scarlet fever occurs most commonly in association with
pharyngitis and rarely with pyoderma or an infected wound.
Tonsillopharyngitis is absent in surgical scarlet fever.
penicillin G (BPG) or mixtures of BPG with procaine penicil-
5. Skin tenderness is absent in scarlet fever, as opposed
lin (to reduce pain) given intramuscularly as a single dose.
to the prominent skin tenderness of staphylococcal
For patients allergic to penicillin, clarithromycin, azithromy-
scarletina.
cin, or clindamycin are alternatives. Children can return to
school after 24 hours of antibiotic therapy. Routine posttreat-
ment follow-up culture is not needed.
IMPETIGO

Clinical Summary little surrounding erythema, and tend to occur on traumatized


skin (eg, insect bite, abrasion), commonly in hot, muggy sum-
Impetigo is a contagious superficial skin infection occurring
mer months. Exposed body surfaces such as the face, nose,
in two forms: bullous and nonbullous (accounts for >70% of
mouth, or extremities are most commonly affected. Pruritus
cases). Bullous impetigo is caused by Staphylococcus aureus,
is common and scratching causes lesions to spread through
whereas the nonbullous form is caused by either group A
autoinoculation. Regional lymphadenopathy is common, but
β-hemolytic streptococcus (GABHS or S pyogenes), S aureus
constitutional symptoms are absent or minimal.
or both. Nonbullous impetigo is most commonly seen in pre-
Bullous impetigo is most commonly seen in newborns,
schoolers and begins as small vesicles progressing to pustules
infants, and preschoolers. The bacterial reservoir is the upper
that extend radially with satellite lesions. Vesiculopustular
respiratory tract (eg, nose, conjunctiva) of asymptomatic car-
lesions rupture, exposing a red, moist base and purulent dis-
riers who spread the pathogen to the child’s skin. Lesions
charge, which forms thick, yellow, or honey-colored adherent
result from exfoliative toxin produced by bacteria at the con-
crusts. Lesions are usually painless, asymptomatic and have
tact site and begin as vesicles that progress to flaccid bullae
with little or no surrounding erythema and clear to cloudy
contents. Rupture leaves a narrow rim of scale at the edge
of shallow, moist, red erosions (scalded skin appearance).
Nikolsky sign is absent (unlike staphylococcal scalded skin
syndrome). Lesions may be few and localized (usually cov-
ered areas) or numerous and widely scattered. Common sites
are trunk, perineum, buttocks, and extremities. Lesions heal
without scarring. There is no seasonal predilection.
Complications include acute poststreptococcal glomerulo-
nephritis (impetigo by GABHS) and rarely serious second-
A
ary infections (eg, osteomyelitis, septic arthritis). Differential
diagnosis of nonbullous impetigo includes ecthyma, herpes
simplex virus infection, varicella, tinea corporis, and local-
ized acute pustular psoriasis. In contrast, differential diag-
nosis of bullous impetigo includes bullous arthropod bites,
poison ivy, child abuse (abusive burn injury), autoimmune
blistering disease (eg, linear IgA dermatosis, pemphigus), and
epidermolysis bullosa.

Emergency Department
Treatment and Disposition
Diagnosis is clinical, though the aspirate from the lesion may
be cultured and Gram stain smear obtained. Though usually
self-limited, untreated infection may last for weeks or months.
Advise patients and caregivers to wash the affected area with
antibacterial soap once or twice a day, and remove crusts,
B which can be softened by soaking area with a wet cloth com-
press, before applying topical therapy. Prescribe topical mupi-
FIGURE 3.7 ■ Non bullous Impetigo. (A) Exposed body surfaces
rocin ointment for localized impetigo (bullous or nonbullous)
such as the face (around the nose and mouth) are the most common
sites. (B) Lesion with a honey-colored crust on the upper extremity.
to eradicate the infection and prevent person-to-person spread.
Removal of the crust reveals bright red, shiny erosions. (Photo con- For generalized impetigo, or if there is involvement of mul-
tributor: Binita R. Shah, MD.) tiple family members, prescribe systemic antibiotics covering

64
IMPETIGO (CONTINUED) ■ 65

A B

FIGURE 3.8 ■ Bullous Impetigo. (A) An intact bullous lesion filled with pus is seen in a 10-day-old neonate. He also had several round,
ruptured erosions with a moist, red surface that were surrounded by a narrow ring of scale. Intact or ruptured bullae are round or oval in shape.
(Photo contributor: Binita R. Shah, MD.) (B) An infant with numerous flaccid bullae filled with pus. (Reproduced with permission from: Shah
BR and Laude TL: Atlas of Pediatric Clinical Diagnosis. WB Saunders, Philadelphia, 2000, p. 93.)

mixed pathogens (GABHS and S aureus). Depending on the Pearls


prevalence of methicillin-resistant or methicillin-sensitive
S aureus in the community, consider oral penicillinase-resistant 1. A pathognomonic finding of bullous impetigo is a ring of
β-lactam drugs (eg, dicloxacillin), a first- or second-generation scale at the periphery of an eroded lesion with a varnished
cephalosporin, clindamycin or doxycycline (children >8 years surface.
of age), or trimethoprim-sulfamethoxazole (for bullous impe- 2. Impetigo is a highly contagious infection and spread is
tigo). Refer patients with recurrence for evaluation and treat- facilitated by poor hygiene.
ment of nasal carriage of S aureus. 3. Staphylococci can be isolated from bullous impetigo
lesions even though culture of staphylococcal scalded
skin syndrome lesions is negative.

FIGURE 3.9 ■ Bullous Impetigo. A 7-month-old infant was referred


to the emergency department with this lesion that was thought to
be an inflicted burn injury (child abuse). Aspiration of the lesion FIGURE 3.10 ■ Bullous Arthropod Bites; Differential Diagnosis
showed gram-positive cocci in clusters on Gram stain and culture of Bullous Impetigo. Close-up of bullous lesions on upper extremity
was positive for Staphylococcus aureus. Posttraumatic paronychia (exposed area) due to arthropod bites. These lesions can be mistaken
due to S aureus should also be considered in the differential diagno- for either second-degree burns or bullous impetigo. (Photo contribu-
sis. (Photo contributor: Binita R. Shah, MD.) tor: Binita R. Shah, MD.)
ECTHYMA

Clinical Summary Emergency Department


Ecthyma is a skin infection caused by group A β-hemolytic Treatment and Disposition
streptococci (GABHS or S pyogenes) and/or S aureus. Unlike
Diagnosis is clinical. Culture of the exudate (beneath an
impetigo, which is superficial, ecthyma involves deeper lay-
unroofed crust) reveals GABHS and S aureus. Advise wash-
ers of the skin (the entire thickness of the epidermis to the
ing the affected area with antibacterial soap and removal of
upper reaches of the dermis). Ecthyma is seen in children of
the crust (facilitated by softening the crust by soaking with
all ages and more frequently during summer. Predisposing
a wet cloth compress). Prescribe topical mupirocin therapy
factors include preceding minor skin trauma (eg, abrasions,
until all lesions have cleared. Depending on the prevalence
insect bites), conditions associated with pruritus (eg, scabies,
of methicillin-resistant or methicillin-sensitive S aureus in
pediculosis), poor hygiene, and malnutrition. Lesions begin
the community, antibiotic options include oral penicillinase-
as vesicles or vesiculopustules with an erythematous base and
resistant β-lactam drugs (eg, dicloxacillin) or a first- or sec-
rupture to form crusts and erode through the epidermis into
ond-generation cephalosporin, clindamycin or doxycycline
the dermis, forming indolent “punched-out” ulcers with ele-
(children >8 years of age).
vated margins. Ulcers become elevated and obscured by thick,
dark greenish-yellow tightly adherent crusts that contribute
to the persistence of the infection. Lesions are discrete and Pearls
round and the most common site is lower legs. Lesions spread
1. Thick crusts and underlying ulcers differentiate ecthyma
by autoinoculation and heal with scarring (unlike impetigo).
from non bullous impetigo.
Complications include cellulitis (untreated ecthyma) and
2. Ecthyma can be mistakenly attributed to cigarette burns
poststreptococcal glomerulonephritis. Differential diagnosis
of child abuse.
includes cigarette burns (due to child abuse) and ecthyma
gangrenosum (Pseudomonas aeruginosa infection).

FIGURE 3.11 ■ Ecthyma. An enlarging, slightly painful lesion which started as a “blister” on the forearm (without any prior history
of trauma). Culture grew S pyogenes. Ecthyma is a discrete, round lesion with a dark crust in the center surrounded by a rim of shallow
ulceration. (Photo contributor: Andrea Marmor, MD.)

66
ECTHYMA (CONTINUED) ■ 67

FIGURE 3.12 ■ Ecthyma. Multiple ecthyma lesions on the neck. (Photo contributor: Binita R. Shah, MD.)

A B

FIGURE 3.13 ■ Ecthyma Versus Cigarette Burn. Ecthyma (A) may be confused with cigarette burns (B). Ecthyma lesions are frequently of
different sizes and are associated with crusting. Cigarette burns are circular punched-out lesions of uniform size with an average diameter of
about 8 to 10 mm and have a smooth well defined edge. (Photo contributor: Binita R. Shah, MD [A]; Photo B. Reproduced with permission
from: American Academy of Pediatrics: The Visual Diagnosis of Child Physical Diagnosis. The C. Henry Kempe National Center on Child
Abuse and Neglect. Elk Grove Village, IL, American Academy of Pediatrics, 1994, p. 25.)
ERYSIPELAS

Clinical Summary malnutrition, and immunocompromised states. Onset is usu-


ally abrupt with constitutional symptoms (eg, fever, chills,
Erysipelas is a distinct skin infection involving the upper-
malaise). Common sites are extremities (usually associated
most layers of the subcutaneous tissue and cutaneous
with a wound) and face (usually associated with pharyn-
lymphatic vessels. It is caused by group A β-hemolytic strep-
gitis). Initiating lesion is frequently inconspicuous and
tococci (GABHS) in the majority and rarely by groups G,
spreads peripherally with a raised, advancing border. The
C, and B streptococci and occurs most frequently in infants,
lesion is fiery red or salmon colored, hot, tender, tense, and
young children, older adults, and debilitated patients. The
indurated well-circumscribed plaque (sometimes with peau
organism gains access to the deeper layers of the skin
d’orange appearance) with a sharp demarcated border dis-
through breaks (eg, abrasions, lacerations, wounds, chronic
tinguishing it from the surrounding normal tissue. Reddish
ulcers). Predisposing factors include lymphedema, local
lymphatic streaks projecting from the margins of the lesion
lymphatic dysfunction, venous stasis, diabetes mellitus,
toward regional nodes may be noted. Intense edema may
lead to formation of vesicles or tense bullae that later rup-
ture and crust (bullous erysipelas). A “butterfly” appearance
with involvement of both cheeks and nasal bridge may occur
with involvement of the face. Regional lymphadenopathy

FIGURE 3.15 ■ Butterfly Rash of Erysipelas. A rapidly spreading


(<12-hour duration), tender, erythematous, indurated facial rash was
seen in this girl with very high fever and leukocytosis. The sharp
demarcation between erythema and the normal surrounding skin is
FIGURE 3.14 ■ Erysipelas. A rapidly spreading tender facial rash evident. Erysipelas may create a “butterfly” appearance (mimicking
and high fever were complaints of this girl. She had scratch marks the rash of systemic lupus erythematosus) on the face. She gave a his-
on her cheek (portal of entry). The sharp demarcation between the tory of falling off the monkey bar 2 days prior to appearance of this
salmon-red erythema and the normal surrounding skin is evident. rash. Within 6 months after this infection, she presented again with
Marking the margins of the erythema with ink (as seen in the photo) a second episode of erysipelas at the same site. (Reproduced with
helps in following the clinical course (progression or regression) of permission from: Shah BR, Santucci K, Tunnessen W: Erysipelas.
the infection. (Photo contributor: Binita R. Shah, MD.) Arch Pediatr Adolesc Med 1995;149:55.)

68
ERYSIPELAS (CONTINUED) ■ 69

FIGURE 3.16 ■ Erysipelas. Erythematous rash with classic honey-


colored crust is seen in this facial erysipelas caused by GABHS.
(Photo contributor: Rajni P. Shah, MD.)

may be present. Desquamation of involved skin may occur


5 to 10 days into the illness. Complications are same as
with any GABHS infection (see page 60). Erysipelas pro-
duces lymphatic obstruction leading to impaired lymphatic
drainage causing lymphedema, which predisposes to recur-
rent episodes of erysipelas frequently at the same site and
permanent local swelling. Differential diagnosis includes
cellulitis, erysipelas-like presentation due to other bacterial FIGURE 3.17 ■ Cellulitis; Differential Diagnosis of Erysipelas.
infection (eg, S pneumoniae, S aureus), necrotizing fasciitis, Cellulitis spreads more slowly and extends more deeply into the
and contact dermatitis. subcutaneous tissues; hence, the borders of cellulitis are ill-defined
(in contrast to erysipelas). Group A β-hemolytic Streptococcus and
Staphylococcus aureus are common pathogens causing cellulitis.
Emergency Department Streptococcus pneumoniae and Haemophilus influenzae can cause
buccal cellulites associated with bacteremia in unimmunized young
Treatment and Disposition children. (Photo contributor: Binita R. Shah, MD.)
Diagnosis is clinical. Hospitalize patients with infection
involving the face with systemic signs for intravenous anti-
biotics (eg, ceftriaxone) for at least 48 to 72 hours. Consider
complete blood count (leukocytosis with a left shift), a blood affected part, and cool, wet dressings as supportive therapy.
culture, and/or a culture by aspiration from the advancing Extension into deeper soft tissues is rare and surgical débride-
margin of the lesion. If cellulitis due to S aureus cannot be ment is not necessary.
excluded by examination, depending on the prevalence of
methicillin-resistant or methicillin-sensitive S aureus in the
community, antibiotic options include penicillinase-resistant
Pearl
β-lactam drugs (eg, oxacillin) or clindamycin. Prescribe 1. Erysipelas is commonly referred as St Anthony’s fire
oral antibiotics for patients with milder presentations with a because it spreads rapidly like a fire, affecting a very
close follow-up. Advise rest, immobilization, elevation of the large area of the skin within a short period of time.
NECROTIZING FASCIITIS

Clinical Summary (some cases) may be present and there may be loss of feeling
on the skin. The lesion expands rapidly with undermining of
Necrotizing fasciitis (NF; also known as streptococcal gan-
overlying skin (important clue for diagnosis), progressing to
grene, flesh-eating bacteria syndrome, hospital gangrene, or
necrosis and gangrene. Signs of toxicity include (high fever,
necrotizing erysipelas or necrotizing cellulites) is a life-threat-
malaise, altered sensorium, shock). Fournier’s gangrene is NF
ening bacterial infection of the fascia and subcutaneous tis-
of perineum, scrotum, and penis. Predisposing factors include
sues. Patients present with an erythematous, indurated, tender,
infections (eg, varicella), contaminated surgical wounds,
ill-defined plaque (often mistaken for cellulitis) that manifests
IV drug or alcohol abuse, and immunocompromised states.
disproportionate pain and tenderness. The lesion is dusky
Bacterial myonecrosis refers to involvement of the muscle.
purple with vesicles/bullae sometimes filled with maroon
Various etiologies include S pyogenes (most common patho-
or violaceous fluid. Subcutaneous emphysema or crepitus
gen), groups B, C, or G streptococci, S aureus, P aeruginosa,

C D

FIGURE 3.18 ■ Necrotizing Fasciitis. (A) A 9-day-old neonate presented with high fever, moaning, and slightly indurated swelling with blu-
ish discoloration on the back. (B) Within 12 hours, there was vesiculation and purplish discoloration. This photograph was taken 8 hours fol-
lowing the first surgical exploration and débridement and shows the underlying muscle and necrotic borders. (C) Close-up showing necrotic
borders and pus over the underlying muscle. Both blood and tissue cultures grew Staphylococcus aureus. (D) Multiple surgical explorations
and débridement were performed followed by skin grafting during recovery. (Photo contributor: Binita R. Shah, MD.)

70
NECROTIZING FASCIITIS (CONTINUED) ■ 71

anaerobes (eg, Bacteroides, Peptostreptococcus, Clostridium aspiration from the involved area (may show presence of pus and
spp) and polymicrobial (gram-negative bacilli [eg, Escherichia bacteria on Gram stain), and send aspirated material and blood
coli, Proteus spp] and anaerobic bacteria). The majority of culture for both aerobic and anaerobic pathogens. Consider
cases occur as extensions from localized nearby skin infec- radiograph of the affected area (may show presence of subcuta-
tions that expand along fascial planes, leading to edema, vas- neous gas, which mandates rapid surgical exploration; however,
cular injury, and thrombosis resulting in widespread necrosis absence of soft tissue gas does not exclude diagnosis). Consider
of the superficial fascia, adjoining tissues, and deeper layer computed tomographic scan, ultrasound, or magnetic resonance
of dermis. Associated compression or destruction of nerves imaging for diagnosis in patients with early signs (when diag-
innervating skin results in anesthesia. Differential diagno- nosis is unclear); however, these modalities should never delay
sis includes erysipelas, cellulitis, and purpura fulminans. immediate surgical exploration in patients with obvious signs.
Complications include multiorgan failure with oliguria, acute Hospitalize all suspected cases of NF to ICU for ongoing man-
respiratory distress syndrome, disseminated intravascular agement. Repeat débridement within 24 to 48 hours may be
coagulation, myocardial failure, and fluid and electrolyte dis- necessary because of continued spread of the infection into sur-
turbances (extravasation of intravascular fluid into tissues). rounding tissues. Other treatment modalities may include hyper-
baric oxygen.
Emergency Department
Treatment and Disposition Pearls
Necrotizing fasciitis is a medical emergency. Consult surgery 1. Sine qua non of NF is fascial necrosis with widespread
emergently for surgical exploration, biopsy (frozen-section undermining of the skin. The ability to pass a sterile
biopsy allows rapid evaluation of the pathology to confirm the instrument along a plane superficial to the deep fascia
diagnosis and identification of pathogens underlying the intact without resistance is indicative of NF.
skin), débridement of all necrotic tissue, and fasciotomy. Consult 2. Presence of an indurated “wooden” plaque adjacent to
infectious disease specialist and begin empiric broad-spectrum infected skin coupled with extreme pain in an area of
IV antibiotics (eg, ampicillin-sulbactam or ampicillin with either rapidly advancing edema and inflammation are important
clindamycin or metronidazole and vancomycin). Obtain CBC, clues.
comprehensive metabolic panel, and coagulation profile (leuko- 3. Marked systemic toxicity and pain out of proportion to
cytosis with immature cells, anemia, hyponatremia, hypocalce- the local findings (in contrast to the usual patient with
mia, hypoproteinemia, evidence of disseminated intravascular cellulitis) suggest NF.
coagulation, and elevated values of blood urea nitrogen, cre-
atinine, and liver enzymes may be noted). Perform fine-needle

A B

FIGURE 3.19 ■ Necrotizing Fasciitis; Fournier’s Gangrene. (A, B) Coronal and axial CT images demonstrate air within the soft tissues
of the left perineal region (arrows) compatible with necrotizing fasciitis. (Photo/legend contributors: Nikita Joshi, MD/John Amodio, MD.)
ECTHYMA GANGRENOSUM

Clinical Summary S aureus, S pyogenes), thermal burns, pyoderma gangreno-


sum, purpura fulminans, and brown recluse spider bite.
Ecthyma gangrenosum is a pathognomonic cutaneous mani-
festation of Pseudomonas aeruginosa septicemia; other skin
lesions include subcutaneous nodules, vesicular lesions, gan- Emergency Department
grenous cellulitis, and petechiae rash. Ecthyma gangrenosum Treatment and Disposition
usually occurs as a result of bloodborne metastatic seeding
to the skin from the bacteremia, or rarely as a primary lesion Stabilize patient based on abnormal vital signs (patients with
without bacteremia. Predisposing factors for Pseudomonas ecthyma gangrenosum are usually septic). Hospitalize to ICU
septicemia include immunosuppression (eg, malignancy, for IV antibiotic therapy and general supportive care including
diabetes, malnutrition, cystic fibrosis), debilitated preterm wound care. Begin antibiotic therapy with an aminoglycoside
newborns, severe thermal burns, disorders leading to severe (eg, tobramycin, gentamicin) and an antipseudomonal penicil-
neutropenia or pancytopenia, hospitalized patients on inten- lin (eg, ticarcillin, piperacillin). Obtain CBC (usually neutro-
sive antibiotic treatment, or those with indwelling urinary, penia especially with septicemia), comprehensive metabolic
arterial, or venous catheters. Skin lesions begin as hemor- panel, coagulation profile, blood culture and needle aspiration
rhagic (purpuric) macular, vesicular, pustular, or bullous and culture of the lession (Gram’s stain may show presence
lesions and evolve into punched-out necrotic ulcers with of bacteria). Consult dermatologist if diagnosis is unclear; a
raised edges with a rim of erythema. Lesions remain localized skin biopsy may show numerous bacteria invading the blood
or more typically extend over several centimeters ultimately vessels (invasion of media and adventitia of vein walls deep in
becoming a round, indurated, painless lesion with a char- the dermis with very few inflammatory cells).
acteristic central grey-black eschar. Either a single ulcer or
multiple (noncontiguous) ulcers may be seen. Common sites Pearl
include perineal or gluteal region, extremities, trunk, face,
intertriginous areas, and axilla. Lesions take a few weeks to 1. The characteristic findings of ecthyma gangrenosum are
heal. Differential diagnosis includes ecthyma gangrenosum– punched-out ulcers with a black eschar, surrounded by a
like lesions in sepsis from other pathogens (eg, Candida spp, rim of erythema.

72
ECTHYMA GANGRENOSUM (CONTINUED) ■ 73

A
B

C D

FIGURE 3.20 ■ Ecthyma Gangrenosum. (A) Purpuric macular lesions involving the perineum, buttocks, and trunk in a malnourished infant
(fed only diluted soy formula) presenting in septic shock. (B), (C), and (D) Close-up of lesions showing progression to necrotic ulcers with
dense black eschars in the center. Note the surrounding erythematous halo with raised edges. Both blood and wound cultures were positive
for Pseudomonas aeruginosa. (A, B, C. Reproduced with permission from: Secord A, Milla C, Shah BR: Picture of the month. Ecthyma
gangrenosum. Am J Dis Child 1993;147:795; and Photo D. Reproduced with permission from: Shah BR and Laude TL: Atlas of Pediatric
Clinical Diagnosis. WB Saunders, Philadelphia, 2000, p. 99.)
TOXIC SHOCK SYNDROME

Clinical Summary inconspicuous, with absent signs of inflammation in skin or


wound lesions in nonmenstrual TSS.
Toxic shock syndrome (TSS) is a potentially fatal systemic
Complications of TSS include acute renal failure, acute
infection by toxin-producing strains of either S aureus
respiratory distress syndrome, overwhelming sepsis, DIC,
(S aureus–mediated TSS) or S pyogenes (S pyogenes–
and multiorgan failure leading to death. Differential diag-
mediated TSS). Diagnosis is made using published crite-
nosis includes Kawasaki disease, scarlet fever, septic shock
ria. S pyogenes–mediated TSS occurs commonly in young
from other etiology (eg, meningococcemia, Rocky mountain
children. Predisposing factors include varicella, HIV infec-
spotted fever, gram-negative sepsis), measles or atypical
tion, diabetes mellitus, IV drug use, and invasive infections
measles, ehrlichiosis, leptospirosis, and drug hypersen-
(eg, bacteremia, pneumonia, osteomyelitis, endocarditis).
sitivity (eg, Stevens-Johnson syndrome, toxic epidermal
S aureus–mediated TSS is seen less commonly in chil-
necrolysis).
dren and most cases occur in women of age 15 to 34 years
(reflecting association with menses). Nonmenstrual cases
occur in people of all ages and in both sexes. Predisposing Emergency Department
factors include use of tampons during menstruation, foreign
body placements (eg, contraceptive sponge, central lines),
Treatment and Disposition
primary S aureus infections (eg, cellulitis, endocarditis), Assess and stabilize vital signs, including fluid resuscita-
postoperative wound infections, skin or mucous membrane tion and pressor agents, as indicated for septic shock. It may
disruptions (eg, burns, insect bites) and vaginal or pharyn- be impossible to distinguish clinically both forms of TSS;
geal colonization. Onset may be abrupt or preceded by a begin empiric antibiotic therapy that includes antistaphylo-
prodromal illness of fever, chills, and myalgias. Rash (seen coccal agents (eg, vancomycin and nafcillin) and a protein
almost always in first 24 hours of illness) may be faint and synthesis–inhibiting agent (eg, clindamycin). Obtain CBC,
often mistaken for flush associated with fever. Beefy, red blood culture, cultures from the site of infection (skin or soft
hyperemia of mucous membranes, conjunctival injection, tissues), vaginal culture (for suspected menstrual TSS), serum
strawberry tongue, erythema, and edema of palms and soles electrolytes, BUN, creatinine, liver enzymes, and radio-
(findings similar to Kawasaki disease) are often seen. Pelvic logic studies (as indicated). Consult surgery for exploration,
exam is usually normal except for subtle erythema and biopsy, and resection of necrotic tissue in NF, drainage of
edema of the skin of the inner thigh and perineum in men- the infected sites, or removal of foreign bodies. Hospitalize
strual TSS. The focus of the staphylococcal infection may be patients to ICU for further management.

A B

FIGURE 3.21 ■ Streptococcal Toxic Shock Syndrome. (A) An 8-year-old girl presented with fever, hypotension, oliguria, and infected vari-
cella lesions with multiorgan involvement. Note also the bilateral periorbital edema. Her blood culture was positive for S pyogenes. (B) Close-up
of her nose showing honey-colored crusted lesions that are characteristic of S pyogenes impetigo. (Photo contributor: Binita R. Shah, MD.)

74
TOXIC SHOCK SYNDROME (CONTINUED) ■ 75

A B

FIGURE 3.22 ■ Cellulitis in Streptococcal Toxic Shock Syndrome (TSS). (A) Cellulitis involving the thigh and lower leg was seen in this
child with AIDS. He presented with septic shock and multiorgan failure with a blood culture positive for S pyogenes. (B) Cellulitis of abdomi-
nal wall in an adolescent patient presenting with TSS. (Photo contributor: Binita R. Shah, MD.)

Pearls 3. Do not use clindamycin alone as an initial empiric ther-


apy, as 1% to 2% of S pyogenes organisms in the United
1. Fever, rash, desquamation, hypotension, and multiorgan
States are resistant to clindamycin.
involvement are the hallmarks of TSS.
4. Women with menses-related TSS may experience one or
2. TSS can be confused with many other causes of fever
more recurrent episodes. Advise against use of tampons
with mucocutaneous manifestations.
and barrier methods of contraception.

FIGURE 3.23 ■ Desquamation in Streptococcal Toxic Shock Syndrome (TSS). Shown here is desquamation that developed on the seventh
day of the illness in an adolescent girl admitted with streptococcal TSS. (Photo contributor: Haamid Chamdawala, MD.)
76 ■ TOXIC SHOCK SYNDROME (CONTINUED)

FIGURE 3.24 ■ Staphylococcal Toxic Shock Syndrome (TSS).


(A, B) Diffuse erythroderma with innumerable pustular lesions, high
fever, vomiting, and diarrhea were the presenting complaints of this
patient with TSS. Her blood culture was positive for S aureus. (C)
Close-up showing pustular lesions. Culture of these pustules was
C
negative for S aureus. (Photo contributor: Binita R. Shah, MD.)
TOXIC SHOCK SYNDROME (CONTINUED) ■ 77

TABLE 3.1 ■ STREPTOCOCCAL TOXIC SHOCK SYNDROME: CLINICAL CASE DEFINITION


Confirmed case Presence of all laboratory and clinical findings below
Probable case Positive culture from nonsterile site and all clinical findings below (and no other cause
for illness identified)
Laboratory findings Isolation of Streptococcus pyogenes
From a normally sterile site (eg, blood, cerebrospinal fluid, peritoneal fluid, tissue
biopsy specimen)
From a nonsterile site (eg, throat, sputum, vagina, surgical wound, or superficial skin lesion)
Clinical findings
Hypotension Systolic blood pressure ≤90 mm Hg in adults or <5th percentile for age in children
Multisystem involvement Renal Creatinine ≥2 mg/dL for adults or two times or more the upper limit
(at least two of following) of normal for age
Coagulopathy Platelets ≤100,000/mm3 or disseminated intravascular coagulation
Hepatic Serum alanine and aspartate aminotransferases or total bilirubin
values 2 times or more the upper limit of normal for age
Pulmonary Acute respiratory distress syndrome
Skin Erythematous macular rash that may desquamate
Soft tissue Necrosis, including necrotizing fasciitis or myositis, or gangrene

TABLE 3.2 ■ STAPHYLOCOCCAL TOXIC SHOCK SYNDROME: CLINICAL CASE DEFINITION


Probable case Meets laboratory criteria below and has 4–5 of the clinical findings listed below
Confirmed case Meets laboratory criteria and all 5 clinical findings below, including desquamation,
unless the patient dies before desquamation occurs.
Laboratory criteria Negative results on following tests (if obtained)
Blood, throat, or cerebrospinal fluid cultures (blood culture may be positive for
Staphylococcus aureus)
Serologic tests for Rocky Mountain spotted fever, leptospirosis, or measles
Clinical findings
Fever 38.9°C (102.0°F) or greater
Rash Diffuse macular erythroderma (nonpruritic)
Desquamation 1 to 2 weeks after onset (particularly palms, soles, fingers, toes)
Hypotension Systolic BP ≤90 mm Hg for adults
<5th percentile for age for children <16 years of age
Orthostatic drop in diastolic BP ≥15 mm Hg from lying to sitting
Orthostatic syncope or orthostatic dizziness
Multisystem involvement Gastrointestinal Vomiting or diarrhea at onset of illness
(three or more of the following) Muscular Severe myalgia or creatinine phosphokinase >2 times the upper
limit of normal
Mucous membrane Vaginal, oropharyngeal, or conjunctival hyperemia
Renal Blood urea nitrogen or serum creatinine level >2 times upper
limit of normal or urinary sediment with ≥5 white blood cells
per high-power field in absence of urinary tract infection
Hepatic Total bilirubin, aspartate, or alanine aminotransferase
level >2 times the upper limit of normal
Hematologic Platelet count ≤100,000/mm3
Central nervous Disorientation or alterations in consciousness without focal
system–related neurologic signs when fever and hypotension are absent
STAPHYLOCOCCAL SCALDED SKIN SYNDROME

Clinical Summary 62% <2 years of age). Infection in older children and adults is
related to a decreased renal clearance of the toxin (eg, patients
Staphylococcal scalded skin syndrome (SSSS), also known as
with renal insufficiency) or immunosuppression (eg, HIV
Ritter disease (SSSS of the newborn) and Lyell disease, is a
infection).
toxin-mediated disease caused by Staphylococcus aureus that
Patients present with a sudden onset of fever and irrita-
produces exfoliative toxins (ETA and ETB). S aureus colo-
bility. Prominent crusting around the eyes and mouth occurs
nizes mucous membranes of the nasopharynx, eyes, and other
early. Pharyngitis, conjunctivitis, or superficial erosions of
areas (eg, umbilical stump, circumcision site), producing a
lips may be noted but intraoral mucosal surfaces are spared.
localized infection. Toxin produced in these areas circulates
The rash begins with an exquisite tenderness of the skin
and acts specifically at the zona granulosa of the epidermis,
(eg, infant cries when held) as a diffuse erythematous scarla-
leading to characteristic exfoliation. SSSS is most commonly
tiniform eruption (sandpaper texture, accentuation in perioral
seen in infants and young children (90% of cases <6 years;

A C

FIGURE 3.25 ■ Staphylococcal Scalded Skin Syndrome (SSSS). (A) and (B) Radial sunburst crusting and fissuring around the orifices
(mouth, eyes, nose) are hallmarks of SSSS (first day of rash in this patient). Note the absence of any mucous membrane involvement of the
mouth or eyes (unlike prominent mucous membrane involvement of TEN/Stevens-Johnson syndrome). Within 24 hours after these photo-
graphs were taken, the skin over the trunk and perineum started wrinkling and flaccid bullae developed, followed by exfoliation in sheets
revealing a red scalded-looking surface. (C) Large areas of exfoliation in a different patient with SSSS revealing a scalded-looking surface is
shown. The borders of the exfoliating skin look like wet tissue paper. (Photo contributor: Binita R. Shah, MD, [A, B]; Photo C. Reproduced
with permission from: Shah BR and Laude TL: Atlas of Pediatric Clinical Diagnosis. WB Saunders, Philadelphia, 2000, p. 95.)

78
STAPHYLOCOCCAL SCALDED SKIN SYNDROME (CONTINUED) ■ 79

and flexural areas). Within 24 to 48 hours, the skin wrinkles toxic epidermal necrolysis [TEN]), toxic shock syndrome,
and flaccid bullae develop, followed by exfoliation in sheets epidermolysis bullosa, and pemphigus.
revealing a moist, red, shiny scalded-looking surface. Borders
of exfoliating skin are rolled like wet tissue paper. Nikolsky
sign is positive (slight rubbing of normal-looking adjacent Emergency Department
skin results in blistering). Exfoliation can spread to entire
body surface area with severe disease. Drying of exfoli-
Treatment and Disposition
ated areas occur with flaky desquamation lasting 3 to 5 days Obtain cultures from the colonized sites like mucous mem-
and rash heals without scarring in 7 to 14 days. An associated branes of nasopharynx, conjunctiva, or umbilical stump in
staphylococcal infection such as impetigo or purulent con- neonates (typically blood culture and cultures of the skin and
junctivitis may be present. intact bullae are negative for staphylococci). Consult derma-
Complications include fluid and electrolyte losses (loss of tology if diagnosis is unclear. A skin biopsy specimen (or a
epidermal barrier) leading to hypovolemia, faulty tempera- frozen section of an induced peel for a rapid diagnosis) will
ture regulation, cutaneous infection (cellulitis), pneumonia, help to distinguish between SSSS and TEN. Admit all patients
and septicemia. Differential diagnosis includes streptococ- with widespread erosions and denuded skin for monitoring
cal scarlet fever, staphylococcal scarlatina or staphylococcal of fluid and electrolyte deficits and IV antibiotic therapy.
scarlet fever (some cases of SSSS do not progress beyond Depending on the clinical situations and prevalence of meth-
staphylococcal scarlatina [forme fruste of SSSS], bullous icillin-resistant and methicillin-sensitive S aureus (MRSA or
impetigo, drug eruptions (eg, Stevens-Johnson syndrome or MSSA) in the community, initial empiric antibiotic options

FIGURE 3.26 ■ Staphylococcal Scalded Skin Syndrome. (A)


A 15-month-old infant presented with pustular lesions with cel-
lulitis on the thumb, fever, and generalized scarlatiniform rash
associated with skin tenderness. Subsequently he developed exfo-
liation in sheets, revealing a scalded-looking surface. Photograph
taken on the third day after hospitalization (B) shows drying of
exfoliated area with flaky desquamation. (Photo contributor:
B
Binita R. Shah, MD.)
80 ■ STAPHYLOCOCCAL SCALDED SKIN SYNDROME (CONTINUED)

FIGURE 3.27 ■ Staphylococcal Scalded Skin Syndrome in a Neonate (Ritter Disease). Following circumcision at birth, this 1-week-old
neonate presented with extreme irritability, fever, numerous pus-filled intact bullae with some exfoliating lesions, and purulent conjunctivitis.
Within 48 hours after these photos, generalized exfoliation over the entire body was seen. (Photo contributor: Binita R. Shah, MD.)
STAPHYLOCOCCAL SCALDED SKIN SYNDROME (CONTINUED) ■ 81

A B

FIGURE 3.28 ■ Staphylococcal Scalded Skin Syndrome. (A, B) Superficial desquamation of the palm and dorsal foot is seen in a 2-month-
old febrile infant (photos taken on second day of illness). Intact blisters followed by denuded plaques developed on the neck, abdomen, and
back. Nikolski sign was positive. Eye and blood cultures were positive for S aureus. (Photo contributor: Katherine Siamas, MD.)

vary, for example, vancomycin and nafcillin (life-threatening Pearls


with septicemia) or clindamycin (without septicemia with
substantial rates of MRSA and low prevalence of clindamycin 1. Radial sunburst crusting and fissuring around the orifices
resistance) or vancomycin (without septicemia with substan- (mouth, eyes, nose) are hallmarks of SSSS.
tial rates of MRSA and substantial prevalence of clindamycin 2. Nikolsky sign is a hallmark of SSSS, though this is also
resistance). For penicillin-allergic patients, give vancomycin. seen in Stevens-Johnson syndrome, TEN, and pemphigus
Usual duration of therapy is 5 to 7 days; bacteremic or immu- vulgaris.
nocompromised patients require a longer duration of therapy. 3. Early distinction between SSSS and TEN is extremely
Treat patients with localized SSSS with oral antibiotic therapy important. Therapy for SSSS includes antistaphylococ-
(eg, trimethoprim-sulfamethoxazole) with a close follow-up. cal antibiotics, whereas discontinuation of the offending
Topical antibiotic is not required. Advice gently moistening drug, and aggressive supportive therapy in a burn unit
and cleaning the skin with isotonic saline or Burow solution, may be lifesaving in TEN.
applying an emollient to provide lubrication and avoiding wet
dressings (may cause further drying and cracking).

TABLE 3.3 ■ COMPARISON OF STAPHYLOCOCCAL SCALDED SKIN SYNDROME (SSSS)


AND TOXIC EPIDERMAL NECROLYSIS (TEN)

Skin Skin Biopsy Finding


Age Cause Tenderness Findings Level of Split Treatment
SSSS Infants S aureus Always Exfoliating skin white Granular layer Antibiotics
No scarring within epidermis
TEN Older Drugs Sometimes Necrosis Full-thickness Discontinue drug;
children Frequent scarring epidermis provide care in
burn unit
MENINGOCOCCEMIA

Clinical Summary
Meningococcal infections are caused by Neisseria meningiti-
dis. Invasive infection usually results in meningococcemia,
meningitis or both. Strains belonging to groups A, B, C, Y,
and W-135 account for most infections. Risk factors include
inherited or acquired terminal complement deficiencies (C5
to C9), properdin deficiency, hypogammaglobulinemia,
or asplenia (anatomic or functional). Children 2 years of
age or younger (peak attack rate: infants <1 year) are most
often affected, with another peak in adolescents (15 through
18 years of age). Incubation period is 1 to 10 days. Presenting A
signs and symptoms may be nonspecific mimicking a viral
infection (eg, fever, chills, myalgias/weakness, headache), or
septic shock, lethargy, coma, convulsion, signs of meningitis
(nuchal rigidity, Kernig sign, and/or Brudzinski sign) or signs
of multiorgan failure. Initially rash may begin as urticaria,
macules or maculopapules, or petechiae that may coalesce
to form larger ecchymotic areas with ischemic necrosis.
Common sites for the rash are the trunk and extremities
(may involve any area), mucous membranes (eg, palpebral
conjunctivae), and clusters at pressure points. Differential
diagnosis of purpura and petechial lesions includes septice-
mia with gram-negative or gram-positive organisms, entero-
viral infections (eg, echovirus or coxsackie virus), Rocky
Mountain spotted fever, Henoch-Schönlein purpura, pete-
chiae from intractable coughing or vomiting, trauma (child
abuse, accidental), factor deficiency (eg, hemophilia), mea-
sles/atypical measles, idiopathic thrombocytopenic purpura,
thrombocytopenia from other etiology (eg, leukemia), drug
reactions (eg, sulfonamides, penicillins), bacterial endocar-
ditis, gonococcemia, epidemic typhus, Ehrlichia canis infec-
tion, and Stevens-Johnson syndrome. B

Complications include disseminated intravascular coag- FIGURE 3.29 ■ Meningococcemia. (A) Fever and petechial erup-
ulation (DIC), purpura fulminans, cardiac (eg, myocarditis, tion (discrete pinpoint nonblanching lesions measuring 1 to 2 mm
congestive heart failure), pulmonary (eg, pneumonia, lung in diameter) associated with septic shock were the presenting signs
abscess), neurologic sequelae from meningitis (eg, subdu- in this child with meningococcemia. Use of a glass slide (as shown
ral effusion or empyema, brain abscess) and Waterhouse- here) helps in determining if the rash is blanching or not. (B) An
infant presenting with petechiae, septic shock and DIC with menin-
Friderichsen syndrome (bleeding into adrenals, shock,
gococcemia. (Photo contributor: Binita R. Shah, MD.)
coma, and death). Poor prognostic factors include hypoten-
sion, hypothermia, leucopenia, thrombocytopenia, absence
of meningitis, purpura fulminans, petechiae <12 hours
before presentation, seizures, or shock on presentation.

82
MENINGOCOCCEMIA (CONTINUED) ■ 83

FIGURE 3.30 ■ Purpura Fulminans of Meningococcemia. Purpuric lesions of <10 hours’ duration seen on the foot of an 18-month-old infant
who presented with septic shock with DIC with meningococcemia. (Photo contributor: Binita R. Shah, MD.)

Emergency Department coagulation profile (elevated prothrombin time, partial throm-


boplastin time, D-dimer, fibrin degradation products, and
Treatment and Disposition
decreased plasma fibrinogen with DIC), serum electrolytes,
Prompt recognition, stabilization, and immediate initiation BUN, creatinine, and liver enzymes (multiorgan failure),
of antibiotic therapy are essential in reducing morbidity and and urinalysis. Obtain cultures from blood and skin lesion
mortality. Stabilize vital signs, including fluid resuscitation scrapings. Gram stain of a petechial skin lesion may also
and pressor agents, as indicated for septic shock. Begin IV be positive. Perform lumbar puncture (LP) only in a stable
antibiotic therapy with cefotaxime or ceftriaxone and van- patient. With meningitis, cerebrospinal fluid (CSF) findings
comycin (meningococcemia and meningococcal meningitis usually will show increased protein and decreased glucose
are indistinguishable clinically from diseases caused by other levels, pleocytosis (predominance of polymorphonuclear leu-
bacterial pathogens, including S pneumoniae). Do not delay kocytes), and a positive culture. Hospitalize patient to ICU;
in initiating therapy for completion of diagnostic studies. droplet precautions in addition to standard precautions are
Obtain CBC (leukocytosis or leukopenia, thrombocytopenia), recommended until 24 hours after initiation of therapy.
84 ■ MENINGOCOCCEMIA (CONTINUED)

FIGURE 3.31 ■ Purpura Fulminans. Purpura fulminans is a cutaneous infarction and/or acral gangrene due to DIC. It can be seen in any
overwhelming bacterial sepsis. Lesions of purpura fulminans are usually seen at the distal extremities, areas of pressure, and lips, ears, nose,
and trunk. (Photo contributor: Binita R. Shah, MD.)

Advise chemoprophylaxis for close contacts (risk of con- Pearls


tracting meningococcal infection among close contacts is
500 to 800 times the rate for the general population). Throat 1. N meningitidis has become the leading cause of bacterial
and nasopharyngeal cultures are of no value for decid- meningitis in children as vaccination for H influenzae,
ing who should receive prophylaxis. Antibiotic prophy- type b, and S pneumoniae has become available.
laxis is given within 24 hours of diagnosis of primary case. 2. A transient maculopapular rash mimicking a wide variety
Chemoprophylaxis options include rifampin (drug of choice of viral exanthems may be a presenting sign of menin-
for children) or ceftriaxone, and rifampin, ceftriaxone, or gococcemia. Conduct a careful examination of skin and
ciprofloxacin for adults. Refer to the American Academy of mucous membranes for petechiae (a common harbinger
Pediatrics Red Book for details of dosing and categories of of meningococcemia).
“close contacts.” Health care workers who did not have direct 3. Classic signs of meningococcal infection (fever with pete-
exposure to the patient’s oral secretions do not need to panic, chiae or purpura) are seen in only 70% of cases. Patients
and they do not need chemoprophylaxis. with meningococcal meningitis may also lack CSF abnor-
malities on initial LP (even with a positive CSF culture).
Thus, infections by N meningitidis must be considered
even in the absence of skin lesions or CSF abnormalities.
MENINGOCOCCEMIA (CONTINUED) ■ 85

FIGURE 3.32 ■ Henoch-Schönlein Purpura; Differential Diagnosis


of Meningococcemia. Symmetrically distributed palpable purpuric
B
lesions on both lower extremities and buttocks (usually below the
waist) are characteristic features of HSP. Other associated findings FIGURE 3.33 ■ Petechiae from Other Etiology; Differential
(eg, arthralgia/arthritis, abdominal pain, hematuria) also help in dif- Diagnosis of Meningococcemia. Sudden eruption of petechiae
ferentiating HSP from meningococcemia. Patients with HSP are also associated with subconjunctival hyperemia appeared only on the
usually afebrile and not sick-looking and the platelet count is usually face in this afebrile girl following several violent episodes of vomit-
elevated or normal. (Photo contributor: Binita R. Shah, MD.) ing due to food poisoning. The remainder of her examination was
normal. Petechiae on the face and upper thorax can occur in healthy
children following intractable episodes of coughing or vomiting. In
contrast, meningococcal infections would cause generalized distri-
bution of petechiae. (Photo contributor: Binita R. Shah, MD.)
ROCKY MOUNTAIN SPOTTED FEVER

Clinical Summary
Rocky Mountain Spotted Fever (RMSF) is an acute febrile
exanthematous illness caused by Rickettsia rickettsii, which
are transmitted by either dog tick, wood tick, or Lone Star tick,
which are systemically infected, and transmit it to humans
while feeding. RMSF is the second most common (after Lyme
disease) tick-borne infection in the United States. RMSF is
a misnomer; cases are reported from all parts of the United
States. Risk factors include exposure to dogs, residence in
wooded areas, and male gender. The tick bite is painless and
often goes unrecognized. About 60% of cases are preceded
by the removal of an attached and engorged tick. Incidence is A
highest in children aged 5 to 9 years and during the months of
April to October. The incubation period ranges from 2 to 14
days, and there may be a prodrome of nonspecific symptoms
(mimics viral infection). Classically, patients present with
severe headache, myalgia, and bilateral calf pain and may
have photophobia, abdominal pain, vomiting, and diarrhea,
with abrupt onset of fever and chills. The rash occurs 2 to 4
days after the onset of illness beginning on wrists and ankles
and spreading to extremities and trunk within 24 hours; the
palms and soles are nearly always affected. Rash begins as
erythematous maculopapular lesions that blanch on pressure
and becomes petechial and may become confluent with areas B
of necrosis. The severity of the rash varies from mild eva-
nescent to severe generalized. Involvement of the scrotum
and vulva is a diagnostic clue. Desquamation may be seen in
severe areas. Conjunctivitis, lymphadenopathy, hepatospleno-
megaly, jaundice, periorbital or peripheral edema may also be
present.
Differential diagnosis of RMSF includes meningococ-
cemia, bacterial sepsis from other pathogens (eg, S pneu-
moniae), Henoch-Schönlein purpura, maculopapular rash
from viral exanthems (eg, echovirus, Epstein-Barr virus),
toxic shock syndrome, idiopathic thrombocytopenic purpura,
measles or atypical measles, drug-induced hypersensitivity
C
reactions (eg, sulfonamides, penicillins), secondary syphilis,
and Kawasaki disease. FIGURE 3.34 ■ Rocky Mountain Spotted Fever (RMSF). (A, B,
C) Erythematous maculopapular eruption that started on the distal
extremity and subsequently became petechial associated with fever,
Emergency Department severe headache, and myalgia were seen in this adolescent boy.
Treatment and Disposition A history of camping during summer vacation in North Carolina
12 days before onset of this rash was obtained. These photographs
Obtain CBC (a normal or low WBC with bandemia, throm- were taken on the fifth day of the illness. As seen here, the palms and
bocytopenia, anemia), comprehensive metabolic panel (hypo- soles are nearly always affected in RMSF. He also had edema of both
natremia, hypoalbuminemia, elevated serum values of CPK, hands and feet. (Photo contributor: Binita R. Shah, MD.)

86
ROCKY MOUNTAIN SPOTTED FEVER (CONTINUED) ■ 87

FIGURE 3.36 ■ Henoch-Schönlein Purpura (HSP); Differential


FIGURE 3.35 ■ Meningococcemia; Differential Diagnosis of Rocky
Diagnosis of Rocky Mountain Spotted Fever (RMSF). Symmet-
Mountain Spotted Fever (RMSF). Purpura with septic shock was the
rically distributed palpable purpuric lesions on both lower extremi-
presentation of this adolescent girl. Differentiation between RMSF
ties and buttocks (usually below the waist) are characteristic features
and meningococcemia is difficult based on clinical presentation
of HSP. Patients with HSP are also usually afebrile, not sick-looking
alone. (Photo contributor: Vania Kasper, MD.)
like those with RMSF, and the platelet count is usually elevated or
normal. (Photo contributor: Anup Singh, MD.)

and liver enzymes may be seen). If clinically indicated, per-


form lumbar puncture (CSF exam may be normal or show straight out without twisting. If fingers are used, protect them
pleocytosis with an elevated protein and normal glucose val- with tissue and avoid squeezing the body of the tick followed
ues). Obtain specific serologic antibody titers (a fourfold or by thoroughly washing hands after removal.
greater rise in titer between the acute and convalescent phases
are diagnostic; however, it takes 7 to 10 days into the course
of illness to yield results). RMSF is a curable but potentially Pearls
fatal disease. An important factor in patient survival is early 1. Centripetal spread is the hallmark, with rash first appear-
diagnosis and therapy. Admit critically ill patients after sta- ing on wrists and ankles followed by spreading to trunk
bilization of vital signs, including restoration of fluid and within hours.
electrolyte abnormalities. Begin empiric therapy with IV 2. The classic triad of fever, rash, and history of tick bite is
doxycycline (drug of choice) and a third-generation cephalo- present in only 60% to 70% of patients. Neither absence
sporin to cover for N meningitidis. Other alternative includes of tick exposure nor absence of rash excludes the diagno-
tetracycline (for patients >8 years of age; tetracycline staining sis. A “spotless” form of RMSF occurs in approximately
of teeth is dose-related; doxycycline is less likely to cause 5% of children infected with R rickettsii.
dental staining than other tetracyclines). Consult dermatology 3. The most significant risk factor for death is a delay of
if diagnosis is unclear. Diagnosis of RMSF can be confirmed appropriate antibiotic therapy; do not withhold therapy
rapidly (usually between 4 and 8 days) in patients with a rash. while awaiting confirmation by the serology tests.
Immunofluorescent staining or PCR of skin biopsy samples 4. Differentiation between RMSF and meningococcemia is
demonstrate R rickettsii. For the removal of a tick, grasp difficult based on clinical presentation alone.
the tick with fine tweezers close to the skin and pull gently
CAT-SCRATCH DISEASE

Clinical Summary inoculation site, though occasionally multiple lymph nodes are
involved. The most commonly involved sites are axilla, cer-
Cat-scratch disease (CSD) is a zoonotic bacterial infection
vical, submandibular, preauricular, epitrochlear, femoral, and
caused by Bartonella henselae following a cat scratch (espe-
inguinal lymph nodes. The nodes are tender, warm, erythema-
cially kittens or feral cats). Infection is transmitted by cutane-
tous, and indurated and the lymphadenopathy usually lasts 1 to
ous inoculation. Transmission between cats occurs by the cat
2 months, but can last up to 1 year. Spontaneous suppuration is
flea. Most reported cases occur in people aged <20 years. A
seen in 10% to 40% of cases.
history of a recent contact with a cat is present in >90% of
In atypical CSD, there is Parinaud oculoglandular syn-
cases. Even though many siblings may play with the same kit-
drome (involvement of conjunctiva with granuloma but
ten, only one family member is usually affected. Occasionally
without any discharge or pain, involvement of ipsilateral pre-
clusters of CSD may be seen in the same family within weeks
auricular lymph node, and may involve ipsilateral subman-
of one another. The incubation period is 7 to 12 days (from
dibular or cervical lymphadenopathy).
time of scratch to appearance of skin papule) or 5 to 50 days
Rare manifestations of CSD include persistent fever, skin
(skin lesion to regional lymphadenopathy). There may be visi-
rashes (eg, erythema nodosum, maculopapular rashes), aseptic
ble signs at the inoculation site, including a scratch or bite with
meningitis, encephalitis, hepatitis, pneumonia, microabscess
one or more small red papules or pustules, though this is often
in liver and spleen, osteolytic lesions, and thrombocytopenic
overlooked because of small size. Typically patients present
purpura. Differential diagnosis of unilateral lymphadenopa-
with regional lymphadenopathy of 1 to 5 cm or more and is the
thy includes bacterial adenitis, viral infections (eg, infectious
most prominent and common symptom, occurring in >90% of
mononucleosis), malignancy (eg, lymphoma), typical or atyp-
cases. Low-grade fever and other constitutional symptoms (eg,
ical mycobacterial infection, tularemia, brucellosis, histoplas-
malaise, anorexia, fatigue, headache) may be present. Usually,
mosis, toxoplasmosis, and lymphogranuloma venereum.
there is involvement of a single lymph node that drains the

FIGURE 3.37 ■ Cat-Scratch Disease. Axillary lymphadenitis (with tenderness, erythema and warmth) of 3 weeks’ duration is shown in a
4-year-old child who received a kitten as a Christmas gift 2 months before the onset of this swelling. He gave a history of playing roughly and
receiving several scratches on his arm by the kitten. A needle aspiration was performed to relieve the symptoms (usually needle aspiration
of suppurative nodes is preferred over I & D to avoid prolonged drainage and possible scarring). (Photo contributor: Binita R. Shah, MD.)

88
CAT-SCRATCH DISEASE (CONTINUED) ■ 89

FIGURE 3.38 ■ Bacterial Adenitis/Abscess; Differential Diagnosis of Cat-Scratch Disease. Axillary lymphadenitis (tenderness, erythema,
and warmth) and fever of 3 days’ duration were the presenting complaint of this child who was prescribed dicloxacillin. An abscess developed
requiring I & D and culture was positive for Staphylococcus aureus. (Photo contributor: Binita R. Shah, MD.)

Emergency Department or immunocompromised hosts). Choices include oral (eg,


azithromycin, trimethoprim-sulfamethoxazole, ciprofloxa-
Treatment and Disposition
cin) and parenteral antibiotics (eg, gentamicin). Educate fam-
Consult infectious disease specialist for advice on diagnos- ilies that cats are usually healthy and require no treatment,
tic tests (eg, indirect fluorescent antibody test for detection no need to get rid of the cat and on prevention of CSD (eg,
of serum antibody to antigens of Bartonella species or poly- avoid playing roughly with cats/kittens, declawing, careful
merase chain reaction assay) and management. Consider hand washing after handling the cat, immediately washing
ultrasonography for assessing lymph nodes’ size and sup- sites of cat scratches/bites and not allowing cats to lick open
puration. CSD is a self-limited infection and management cuts or wounds).
is primarily symptomatic. Lymphadenopathy may persist
for 2 to 4 months, but spontaneous resolution is the rule.
Consider direct needle aspiration of pus to relieve discom-
Pearls
fort with suppurative nodes; avoid incision and drainage 1. Chronic regional lymphadenitis is the hallmark of CSD.
(I & D). Consider antibiotic therapy for ill patients with sys- 2. Consider CSD in patients with adenopathy, fever, mal-
temic symptoms (eg, patients with large painful adenopathy aise, and history of a feline contact.
CHICKENPOX

Clinical Summary
Chickenpox or varicella is an acute exanthematous illness
caused by varicella-zoster virus (VZV), carried only by
humans and spread by person-to-person contact or airborne
droplets of infected respiratory tract secretions. Maternal var-
icella infection can lead to transplacental in utero infection.
Incubation period is 10 to 21 days (less in the immunocom-
promised) and there is usually no prodrome in children. Rash
and constitutional symptoms (eg, low-grade fever, headache)
occur simultaneously and the rash lasts 5 to 7 days (longer
in immunocompromised and may become hemorrhagic). The A
rash is vesicular with centripetal distribution, involving the
scalp and mucous membranes (eg, conjunctiva, oropharynx).
Lesions are round or oval and progress from macule to papule
to vesicle to pustule, followed by crusting; different stages of
lesions can be seen together in the same anatomic area (unlike
smallpox). Central umbilication of lesions occurs with heal-
ing. Children receiving intermittent courses of corticosteroids
(eg, for asthma) may have more severe course. Differential
diagnosis of other papulovesicular eruptions includes herpes
zoster, coxsackie virus infections (eg, hand-foot-and-mouth
disease), drug eruptions (eg, Stevens-Johnson syndrome),
eczema herpeticum, eczema vaccinatum, rickettsialpox, bul-
lous impetigo, impetigo contagiosum, arthropod bites/papular
urticaria, dermatitis herpetiformis, and smallpox.
Complications (more common in immunocompromised)
include secondary skin infections by S aureus and/or S pyo-
genes (eg, cellulitis, impetigo); streptococcal TSS; necrotizing
fasciitis; CNS (eg, meningoencephalitis, cerebellar ataxia),
cardiac (eg, myocarditis), hematologic (eg, acute thrombo-
cytopenia, hemorrhagic chickenpox), renal (eg, glomerulone-
phritis), and bone (eg, reactive arthritis), bronchopneumonia;
and hepatitis.

Emergency Department
Treatment and Disposition
B
Diagnosis is clinical. Laboratory tests (eg, Tzanck smear,
polymerase chain reaction, or direct fluorescent antibody FIGURE 3.39 ■ Chickenpox. (A, B) These figures show the pres-
test to identify VZV from vesicular fluid) are rarely required. ence of lesions in different stages (papule, vesicle, pustule, and
crusted lesion) with a centripetal distribution in a child with chick-
Chickenpox is a benign, self-limiting disease in otherwise
enpox. Lesions are also seen on mucous membranes involving the
healthy children, and oral acyclovir is not recommended for lips and oropharynx. (Photo contributor: Binita R. Shah, MD.)
routine use. Acyclovir (given within 24 hours of onset of

90
CHICKENPOX (CONTINUED) ■ 91

FIGURE 3.40 ■ Chickenpox. Appearance of chickenpox is some-


times described as “dew drops on a rose petal”; the classic vesicles are
surrounded by erythema. (Photo contributor: Binita R. Shah, MD.)

rash) can be considered in those at increased risk for moder-


ate to severe varicella (eg, patients >12 years of age; patients
receiving either long-term salicylates or short, intermittent, FIGURE 3.41 ■ Bullous Varicella. Intact pus-filled bullae resulting
from secondary infection with Staphylococcus aureus were seen on
or aerosolized courses of corticosteroids; and patients with
the trunk of a child with chickenpox. Close-up of pus-filled bullous
chronic cutaneous or pulmonary disorders). Chickenpox is lesion is shown. (Photo contributor: Binita R. Shah, MD.)
highly contagious (most contagious from 1 to 2 days before
the onset of rash until all the lesions have dried or crusted).
Secondary cases occur in 98% of susceptible persons when prescribing antipruritic medications that cause drowsiness, as
exposed. Varicella-zoster immune globulin (VeriZIG for this side effect may mask lethargy and drowsiness related to
passive immunoprophylaxis) is recommended (given within complications of chickenpox (eg, meningoencephalitis).
96 hours of exposure) for contacts at risk of developing severe
varicella. Refer to American Academy of Pediatrics Red Book
for recommendations on antiviral therapy and VeriZIG. Admit
Pearls
immunocompromised patients for IV antiviral therapy. Do 1. Varicella-zoster virus remains in a latent state in the dor-
not prescribe salicylates (or salicylates-containing products) sal root ganglion cells, and may get reactivated (as the
to children with varicella (association with Reye syndrome). immunity wanes), resulting in herpes zoster.
Consider colloidal oatmeal baths and antipruritic lotions like 2. Chickenpox is more severe in adolescents, adults, and
pramoxine for the pruritus and discomfort. Be careful when immunocompromised hosts.
HERPES ZOSTER

Clinical Summary
Herpes zoster or shingles is an acute vesiculopustular erup-
tion occurring in dermatomal distribution caused by varicella-
zoster virus (VZV) and is unusual in children.
A prior clinical or subclinical episode of varicella is a pre-
requisite for the occurrence of herpes zoster. After primary
infection has occurred, the virus lies dormant in a sensory gan-
glion; reactivation of the latent virus results in herpes zoster. Up
to 20% of those with history of varicella develop herpes zoster
during their lifetime, and multiple attacks can occur (eg, HIV
patients). Young infants may develop herpes zoster without prior
clinical varicella if in utero exposure occurred. Zoster begins
with pain and tingling within the dermatome and progresses to
sharp, dull, or burning pain, which precedes the rash by 48 to 72
hours (range 1–10 days). Pain is minimal when zoster occurs in
children and there is usually a lack of systemic symptoms. The
rash begins as erythematous macules and papules that progress
to vesicles (12–24 hours) to pustules (3–4 days), then crusting
FIGURE 3.43 ■ Herpes Zoster. An 8-month-old infant with herpes
(7–10 days). Vesicles are grouped and umbilicated on an ery- zoster on C5 and C6 dermatomes. This infant never had chickenpox,
thematous base in a linear distribution, and erupt along one to but his mother had varicella during the last trimester of pregnancy.
three dermatomes supplied by a spinal or cranial nerve (most (Reproduced with permission from: Shah BR and Laude TL: Atlas of
common site: thoracic T5-T10 [~75% of cases]). Lesions do not Pediatric Clinical Diagnosis. WB Saunders, Philadelphia, 2000, p. 55.)
cross the midline in immunocompetent hosts.

Ramsay Hunt syndrome is a specific manifestation of


zoster that affects geniculate ganglion (facial or auditory
nerves) and involves the pinna, auditory canal, tympanic
membrane, and anterior two-thirds of the tongue (decreased
taste). Patients present with ipsilateral Bell palsy, tinnitus,
deafness, vertigo, hearing loss, and otalgia. In herpes zoster
ophthalmicus, the ophthalmic branch of the trigeminal nerve
is affected and rashes occur on the eyelids; in addition, con-
junctivitis, keratitis, iridocyclitis, secondary glaucoma, ocular
muscle palsies, ptosis, mydriasis, and panophthalmitis may
occur. Hutchinson sign (involvement of nasociliary division
of ophthalmic nerve with vesicles on the side and top of the
nose) may be seen. Vesicles occur on the palate, uvula, and
tonsillar fossa when the maxillary branch of the trigeminal
nerve is involved and on the buccal mucosa, floor of the
mouth, and anterior part of the tongue when the mandibu-
lar branch is involved. Disseminated zoster (generalized rash
with fever) and visceral dissemination (pneumonia, hepatitis,
meningoencephalitis) may be seen in patients with persistent
FIGURE 3.42 ■ Herpes Zoster. Herpes zoster involving the T5 der-
depression of cell-mediated immunity. CNS involvement may
matome. Lesions do not cross the midline in an immunocompetent occur and includes asymptomatic CSF lymphocytic pleocy-
child. (Photo contributor: Binita R. Shah, MD.) tosis with normal or elevated protein value or symptomatic

92
HERPES ZOSTER (CONTINUED) ■ 93

A C

FIGURE 3.44 ■ Herpes Zoster. (A, B, C) Vesicular eruptions were present on the face, lips, anterior two-thirds of the tongue, pinna, auditory
canal, and tympanic membrane of this girl. She did not have facial nerve palsy. Note that lesions do not cross the midline. (Photo contributor:
Binita R. Shah, MD.)

meningoencephalitis (eg, headache, vomiting, photophobia, test to identify VZV from vesicular fluid) are rarely required.
altered sensorium), transverse myelitis, and zoster pare- Admit immunocompromised patients for IV antiviral therapy
sis (motor weakness and atrophy in associated myotome). and observe airborne/contact precautions for duration of the
Postherpetic neuralgia (pain persisting for >6 weeks after dis- illness. Immunocompetent children usually do not need any
appearance of the rash) is uncommon in children. Differential treatment and can be discharged with instructions to wash
diagnosis includes dermatomal herpes simplex infections (eg, hands after touching lesions and cover lesions. If lesions can-
anogenital herpes), contact dermatitis, grouped arthropod not be covered, instruct caregivers to keep them home from
bites, and localized bacterial or viral skin infections. school/daycare until lesions are crusted. Consult ophthalmol-
ogy for patients with zoster ophthalmicus.

Emergency Department
Treatment and Disposition Pearl
Diagnosis is clinical. Laboratory tests (eg, Tzanck smear, 1. A patient with herpes zoster can transmit varicella, but
polymerase chain reaction, or direct fluorescent antibody not herpes zoster, to a susceptible host.
OROPHARYNGEAL HERPES SIMPLEX VIRUS INFECTION

Clinical Summary Emergency Department


Oropharyngeal herpes simplex virus (HSV) infections man- Treatment and Disposition
ifest as acute herpetic gingivostomatitis, herpes labialis or
Diagnosis is clinical and tests are usually not required.
pharyngitis. Type 1 infections usually occur above the waist
Tzanck smear is positive in both HSV and varicella zoster
and type 2 usually below the waist; HSV-1 or HSV-2 can
virus (VZV) infections. Culture of fluid from vesicles or
be found in either site, depending on the source of infec-
scrapings of the lesion or polymerase chain reaction assay
tion. Transmission is by skin-to-skin, mucosa-to-mucosa, or
will differentiate between HSV and VZV infections when
skin-to-mucosa contact. Most primary HSV infections are
necessary. Admit immunocompromised patients for IV
asymptomatic and virus persists for life in a latent form in a
antiviral therapy (refer to American Academy of Pediatrics
sensory ganglion. Reactivation may follow several triggers,
Red Book guidelines). For immunocompetent patients,
including febrile illness, emotional or physical stress, debili-
antiviral therapy is not routinely recommended. Advise
tating activities. In adolescents, primary HSV manifests as
pharyngitis (shallow tonsillar ulcers with or without gray
exudates).
Gingivostomatitis commonly affects children aged 1 to
3 years, who present with high fever, foul breath, inability to
eat or drink, dehydration, drooling, and irritability. Lesions
begin as vesicles that rapidly rupture turning into shallow
gray ulcers (1–3-mm size) on an erythematous base. The
ulcers are extremely painful, friable, and bleed easily. The
gums are swollen, ulcerated, erythematous, friable, and bleed
easily; gingivitis may precede appearance of mucosal vesi-
cles. Lesions are typically located on the anterior tongue, buc-
cal mucosa, gums, hard and soft palate; lesions may extend
A
to lips, chin, or neck (even in an immunocompetent child).
Tender cervical and submandibular/submental lymphade-
nopathy may be present. Gingivostomatitis is self-limited and
usually resolves in 5 to 7 days.
Herpes labialis or fever blisters have a prodrome of pain,
burning, tingling, or itching that lasts a few hours; systemic
symptoms are unusual. The lesions begin within 24 to 48 hours
after prodrome as papule progresses to vesicle to ulcer followed
by crusting (within 3 to 4 days) and healing (within 5 to 10
days). The lesions are painful, especially at the vesicular stage,
and are typically found at the vermilion border of lips, muco-
cutaneous junction of the perioral region; lesions are localized
in a nondermatomal distribution and recur at the same location
B
or closely adjacent areas.
Differential of gingivostomatitis and herpes labialis FIGURE 3.45 ■ Recurrent Herpes Labialis and Recurrent
includes herpangina, hand-foot-and-mouth disease, aph- Erythema Multiforme (EM). (A) HSV is the most common etiol-
thous stomatitis (recurrent ulcers with rim of erythema and ogy of EM and recurrences of EM are common with recurrences
of HSV infections. (B) Typical annular erythematosus lesions (tar-
gray exudates), and zoster ophthalmicus. Differential of her-
get lesions) with duskiness in the center are seen on the face of
pes pharyngitis includes acute exudative tonsillopharyngitis this child who had herpes labialis (drying HSV lesions seen around
from other etiologies (eg, S pyogenes, adenovirus, Epstein- the lower lip) 5 days prior to the onset of EM. (Photo contributor:
Barr virus). Binita R. Shah, MD.)

94
OROPHARYNGEAL HERPES SIMPLEX VIRUS INFECTION (CONTINUED) ■ 95

A B

FIGURE 3.46 ■ Herpes Labialis. (A) The hallmark of herpes simplex virus skin infections is grouped vesicles on an erythematous base as
seen here on the mucocutaneous junction of the lip. (B) Recurrent herpetic lesions in a patient with HIV infection. The lesions began with
vesicles and healed with crusting. This patient also had lesions on the tongue and buccal mucosa. (Photo contributor: Binita R. Shah, MD.)

supportive therapy including antipyretics, hydration, and Pearls


dietary modifications (eg, cool drinks, popsicles, and soft
diet; avoid citrus juices). Do not prescribe oral anesthetics 1. In children, most common manifestation of primary HSV
(eg, lidocaine viscous) because of the risk of toxicity via infection is gingivostomatitis, and of recurrent HSV
absorption through oral mucosa. Exclude children with gin- infection is herpes labialis.
givostomatitis with drooling and active lesions from child 2. Recurrent HSV infections may be associated with recur-
care centers. rent episodes of erythema multiforme.
3. Herpetic gingivostomatitis may be confused with herpan-
gina. Vesicles are usually not seen on the buccal mucosa
and anterior portion of the mouth in herpangina.

FIGURE 3.47 ■ Herpetic Gingivostomatitis. A child presenting FIGURE 3.48 ■ Tzanck Smear of Herpetic Infection. Giemsa stain
with high fever, refusal to eat, and sores in the mouth. (Photo con- of vesicle contents demonstrating multinucleated giant cells (fused
tributor: Binita R. Shah, MD.) virally infected keratinocytes) is indicative of a herpetic infection.
(Reproduced with permission from: Kane KS, Ryder JB, Johnson
RA, et al. Herpes simplex virus. In: Color Atlas & Synopsis of
Pediatric Dermatology. New York, McGraw-Hill, 2002, p. 544.)
HERPETIC WHITLOW

Clinical Summary for pain as indicated. Topical penciclovir cream may be


applied to affected areas every 2 hours. Acyclovir may be
Herpetic whitlow is a cutaneous HSV infection of the ter-
used for severe infections.
minal phalanx of the fingers or thumb. It is an occupational
hazard (eg, dentists) that may also occur in infants and young
children; first episode may accompany herpetic gingivosto- Pearls
matitis. After the primary infection, HSV remains dormant
1. Herpetic whitlow is characterized by grouped umbili-
in the sensory ganglia and can recur in the same location.
cated, vesiculopustular lesions on an erythematous base
Onset is abrupt with pain or paresthesia at the site. Skin lesion
on the distal fingertips or thumb. It is commonly confused
begins as groped vesicles and/or pustules on an erythema-
with a bacterial paronychia or felon.
tous base, subsequently becoming a painful, erythematous,
2. Do not incise or drain herpetic whitlow; surgical débride-
swollen lesion that usually persists for 7 to 10 days (range,
ment may exacerbate the condition.
1–3 weeks). Common sites are terminal phalanx of finger or
thumb. One or more fingertips may be involved. Fever, lym-
phangitis, and regional lymphadenopathy may accompany the
lesion. Differential diagnosis includes contact dermatitis and
dyshidrotic eczema.

Emergency Department
Treatment and Disposition
Diagnosis is clinical. Tzanck smear (see Figure 3-48) or a
needle aspiration of the lesion for HSV culture will confirm
the diagnosis. It usually resolves without any specific ther-
apy. Supportive therapy includes antipyretics and analgesics

FIGURE 3.49 ■ Herpetic Whitlow. A 4-month-old infant present-


ing with clusters of vesiculopustular lesions on an erythematous base
on the finger. This infant’s mother had “cold sores” 7 days prior to FIGURE 3.50 ■ Herpetic Whitlow. Herpes simplex virus infec-
developing this rash. Culture of the lesion was positive for HSV-1. tion like this often gets mistaken for paronychia. (Photo contributor:
(Photo contributor: Binita R. Shah, MD.) Binita R. Shah, MD.)

96
MUMPS

Clinical Summary parotid swelling (eg, sarcoidosis, parotid duct stone, and long-
term wind instrument use). Differential diagnosis of mumps
Mumps is an acute systemic paramyxovirus infection char-
orchitis includes epididymo-orchitis from other etiology (eg,
acterized by swelling of the parotid and other salivary
sexually transmitted diseases), and testicular torsion (espe-
glands. History of exposure to a similar illness is important
cially when orchitis precedes parotitis). Differential diagnosis
as humans are the only known natural hosts and the virus
of mumps meningoencephalitis includes meningoencephalitis
is acquired by direct intimate contact with oropharyngeal
from other viruses (eg, enterovirus).
secretions (droplets). Incubation period ranges from 12 to 25
days after exposure. There is a 1 to 2-day prodrome of fever,
headache, malaise, anorexia, and abdominal pain. Bilateral Emergency Department
(70% of cases) or unilateral painful, nonerythematous parotid Treatment and Disposition
gland swelling follows. This pushes the ear lobe upward and
outward, obscuring the mandibular angle. The opening of Diagnosis is clinical and no laboratory tests are usually
Stensen duct may be red and edematous. Ingestion of sour required. Serum amylase is elevated in approximately 70%
liquids may increase pain in the parotid gland. Submaxillary of cases of parotitis, with peak values during the first week of
glands may be involved as well. Symptoms occur with (or illness. Treatment is supportive for the majority. Resolution
closely follows) parotitis. Rarely, only submaxillary glands of parotitis takes approximately 10 days. Prescribe support
are involved. The opening of Wharton duct may be red and of the testicle, ice packs, and pain management for mumps
edematous. Sublingual glands are least frequently involved, orchitis with follow-up. Admit patients with meningitis (or
and bilateral swelling is seen in the submental region and in meningoencephalitis) for supportive management. Mumps
the floor of the mouth. is contagious; exclude children from school for 5 days from
Complications include CNS involvement (eg, meningitis onset of parotid gland swelling. Advise standard as well as
or encephalitis), orchitis or epididymo-orchitis (seen among droplet precautions for contagious illness.
postpubertal adolescents and young adults). Other rare com-
plications include arthritis, oophoritis, cerebellar ataxia, Pearls
transverse myelitis, pancreatitis, myocarditis, and deafness.
Differential diagnosis of mumps parotitis includes paroti- 1. Mumps presents as either unilateral or bilateral mildly
tis from other viruses (eg, HIV, parainfluenza, or coxsackie tender parotid gland swellings.
viruses), bacterial parotitis (eg, S aureus), lymphadenitis (eg, 2. Purulent parotitis is unilateral; extremely tender and
anterior cervical or preauricular adenitis) and other causes of purulent drainage from Stensen duct may be noted.

A B

FIGURE 3.51 ■ Parotitis. (A) Mumps. Parotid swelling in an unimmunized child who immigrated from Trinidad 14 days before the appearance
of this swelling. The parotid gland is normally not palpable. Note swelling extending below the angle of the mandible. (B) Human immunodefi-
ciency virus parotitis. Children with HIV can develop parotitis usually without much symptoms. Typically, the parotid gland is firm, nontender,
and chronically enlarged. Involvement can be unilateral or bilateral. (Photo contributor: Binita R. Shah, MD.)

97
ROSEOLA INFANTUM

Clinical Summary period though there may be a bulging anterior fontanelle and
lymphadenopathy (cervical, occipital). After the fever drops
Roseola infantum (also known as exanthem subitum, or rose-
(12–24 hours) and rarely as it is dropping, a rash of pink- to
ola or sixth disease) is an acute exanthematous illness caused
rose-colored nonpruritic macules or maculopapules occurs
by human herpesvirus-6 (HHV-6; majority of cases) or HHV-
on the trunk and then spreads to the face and extremities.
7, for which humans are the only known natural hosts. Infants
Lesions blanch on pressure and do not become vesicular, pus-
may have apparent recurrence that is actually sequential
tular, or petechial. Lesions usually remains discrete and the
infection by the two viruses. Roseola cases occur through-
rash is evanescent, lasting from a few hours to 1 to 3 days,
out the year without any seasonal predilection. About 95% of
after which it clears completely without pigmentation or des-
cases occur at 6 to 36 months of age (peak: between 6 and 15
quamation. CNS complications include febrile seizures dur-
months). The incubation period is 7 to 15 days. Usually there
ing the febrile prodromal stage (seen in 10%–15% of cases),
is no prodrome or mild rhinorrhea and pharyngeal and con-
meningoencephalitis, or encephalitis. Differential diagnosis
junctival injection. Illness begins with an abrupt-onset fever
includes other viral infections (eg, enteroviral infections,
that rises rapidly to 39°C to 41°C and remains elevated con-
infectious mononucleosis, and rubella), drug hypersensitivity
sistently or intermittently for 3 to 6 days, after which time it
reactions, and Kawasaki disease.
drops precipitously. Most infants look well during the febrile

FIGURE 3.52 ■ Roseola Infantum. Diffuse erythematous maculopapular eruptions that developed after 3 days of intermittently high fever (up
to 40°C) in this well-appearing 8-month-old infant who was hospitalized because of bulging fontanelle. All the cultures were negative including
CSF. A diagnosis of roseola infantum was made when he developed this rash. Note the absence of conjunctival injection and red lips (characteristic
features that are often seen in a highly febrile infant with Kawasaki disease). (Photo contributor: Binita R. Shah, MD.)

98
ROSEOLA INFANTUM (CONTINUED) ■ 99

Emergency Department stage except reassuring the family about the benign course of
roseola.
Treatment and Disposition
Diagnosis of roseola is clinical based on the age, classic his-
tory, and clinical findings. During the eruptive phase, no
Pearls
laboratory tests are required as classic roseola is rarely con- 1. Roseola is the most common exanthem of infants
fused with other viral exanthems, and a specific diagnosis of <2 years of age.
HHV-6 is not usually necessary. During the preeruptive, highly 2. The sine qua non of roseola is appearance of rash at defer-
febrile stage of roseola, many infants undergo sepsis workup, vescence; thus, roseola often gets diagnosed retrospec-
receive antibiotic therapy awaiting culture results, and even tively once the typical febrile course has been observed,
hospitalization (especially infants presenting with a bulging followed by appearance of the rash at defervescence.
anterior fontanelle). CSF examination in infants with HHV- 3. Roseola is frequently misdiagnosed as drug hypersensitiv-
6-associated febrile seizures is usually normal. A diagnosis ity rash, as these infants are frequently prescribed antibiot-
of roseola is often established in these infants retrospectively ics during the febrile preeruptive phase. Detailed history of
when they develop the rash. No therapy is required during this rash in relation to fever will establish the correct diagnosis.

FIGURE 3.53 ■ Roseola Infantum. Diffuse erythematous maculopapular eruption that developed after 4 days of high fever in this very
happy-looking 18-month-old toddler. He was treated for occult bacteremia as an outpatient. He continued to spike fever in spite of antibiotic
therapy and blood and urine cultures were negative. He was rushed to the ED when this rash appeared on the fifth day (he was afebrile). A
diagnosis of roseola infantum was made (Important: A toddler should not be eating raisins due to the risk of choking and foreign body aspira-
tion) (Photo contributor: Binita R. Shah, MD.)
MEASLES

Clinical Summary involving palms and soles. On the face and trunk, the rash
becomes confluent and initially blanches on pressure; after
Measles (also known as Rubeola or first disease) is a highly
3 to 4 days the rash becomes brownish and does not blanch
contagious exanthematous illness caused by a paramyxovirus.
because of capillary hemorrhages. The rash peaks at the
The incubation period is 8 to 12 days and there is a prominent
height of fever between the fourth and sixth day of illness and
prodrome of fever, cough, coryza, conjunctivitis (the 3 C’s)
resolves in order of appearance between the seventh and ninth
with significant photophobia. On the third day of illness, ery-
day. Following the rash, a fine, brawny desquamation is seen
thematous maculopapular lesions occur along the hairline and
over sites of most extensive involvement (but not on palms
spread down the trunk and extremities to the hands and feet,
or soles, differentiating it from scarlet fever and Kawasaki

A B

FIGURE 3.54 ■ Measles. (A, B, C) An 8-month-old child presented


with fever, coryza, and marked conjunctivitis associated with intense
photophobia. Confluent, erythematous, and maculopapular rash on
the face developed on the fourth day of fever. He developed measles
during the measles outbreak seen in New York City in 1993. (Photo
contributor: Binita R. Shah, MD [A, B]; Photo C. Reproduced with
permission from: Shah BR and Laude TL: Atlas of Pediatric Clinical
C
Diagnosis. WB Saunders, Philadelphia, 2000, p. 61.)

100
MEASLES (CONTINUED) ■ 101

rickettsial infections, other viral exanthems (eg, adenovirus,


infectious mononucleosis, and enteroviruses).

Emergency Department
Treatment and Disposition
Diagnosis is usually clinical. Report case immediately to the
local health department for assistance in verifying the diag-
nosis. Serum IgM antibody to measles is one of the simplest
methods to confirm diagnosis. Additional tests (eg, measles
IgG antibody acute and convalescent titers) may be recom-
mended. Obtain CBC (leukopenia with a relative lymphocy-
FIGURE 3.55 ■ Koplik’s Spots of Measles. White spots on the hard tosis seen in uncomplicated measles). CSF examination in
palate of a patient with measles. (Reproduced with permission from:
suspected encephalitis will show mild pleocytosis with pre-
Mei Kane KS, Ryder JB, Jonson RA et al: Measles virus. In: Color
Atlas & Synopsis of Pediatric Dermatology. New York, McGraw-
dominance of lymphocytes with increased protein and nor-
Hill, 2002, p. 585.) mal glucose values. Admit infants, malnourished children, or
patients with complications (eg, pneumonia, croup, encepha-
litis). Measles is self-limiting, and treatment is supportive.
disease). Koplik spots are a pathognomonic mucocutaneous Patients are contagious from 3 to 5 days before to 4 days after
exanthem of white papular dots on an erythematous buccal the appearance of the rash. Immunocompromised patients are
mucosa seen opposite lower molars that may spread to involve contagious for the duration of the illness (prolonged excre-
entire buccal and labial mucosa. Koplik spots are seen 2 days tion of the virus in respiratory tract secretions). Vitamin A
before and 2 days after the rash and are transient. Absence of therapy has been associated with a reduction in morbidity and
Koplik spots does not exclude diagnosis. Modified measles mortality. Currently, the World Health Organization recom-
is milder (eg, afebrile course, nonconfluent rash with shorter mends vitamin A for all children with acute measles, regard-
duration) in infants <9 months old because of a transplacental less of their country of residence. Follow CDC guidelines for
antibody or administration of immunoglobulin to an exposed administration of the vaccine and immune globulin (IG) in
individual. Complications include otitis media, pneumonia susceptible individuals following exposure to measles.
(interstitial pneumonitis from measles virus or bronchopneu-
monia from secondary bacterial infection), cervical adenitis,
encephalomyelitis, laryngotracheobronchitis, myocarditis, Pearls
pericarditis, exacerbation of tuberculosis, “black” measles 1. Measles is characterized by a prominent prodrome of
(hemorrhagic measles, purpura fulminans with disseminated cough, coryza, and conjunctivitis (the 3 C’s) followed by
intravascular coagulation), corneal ulceration, and blindness. confluent erythematous maculopapular rash.
Differential diagnosis includes Kawasaki disease, drug erup- 2. Immunocompromised children may not develop charac-
tions (eg, Stevens-Johnson syndrome), meningococcemia, teristic rash.
RUBELLA

Clinical Summary lymphadenopathy involving postauricular and suboccipital


nodes occurs (most common sites), but can be generalized.
Rubella (also known as German measles or three-day mea-
Complications include transient polyarthralgia and polyar-
sles) is an exanthematous infection caused by rubella virus.
thritis in adolescent females, purpura (thrombocytopenic or
Humans are the only source of infection, which is transmit-
nonthrombocytopenic purpura), and congenital rubella syn-
ted through direct or droplet contact from nasopharyngeal
drome. Differential diagnosis includes other viral exanthems
secretions. Many cases are subclinical; clinical cases are
(eg, infectious mononucleosis, enteroviral infections) and
mild and now seen in susceptible teenagers and young adults.
drug eruptions.
Incubation period varies from 14 to 21 days. Coryza, cough,
conjunctivitis (without photophobia) and sore throat may
occur in adolescents. In children, the prodrome is absent and Emergency Department
rash is the first sign of illness. Fever is absent or low-grade
during rash (unlike measles). Palatal enanthem (petechiae or
Treatment and Disposition
rose spots) may be seen. Rose-pink maculopapular rash starts Rubella is a difficult clinical diagnosis as similar rash is seen
on the face and neck, becoming generalized within 24 to 48 in many other viral infections. Laboratory diagnosis is impor-
hours. Lesions are discrete (unlike striking confluent rash of tant when rubella is suspected in pregnant women or new-
measles). By the third day, rash on the face disappears and borns. Consult infectious disease specialist (tests may include
is present only on the extremities. Eruption usually resolves isolation of virus from nasopharynx or serology like rubella-
by the end of the third day, followed by fine brawny desqua- specific IgM antibody and a fourfold rise or seroconversion
mation (with intense eruption) or no desquamation. Tender between acute and convalescent IgG serum titers). Rubella

FIGURE 3.56 ■ Congenital Rubella Syndrome (CRS). An infant with glaucoma, microcephaly, and patent ductus arteriosus was born to
an unimmunized mother who contracted rubella during the first trimester of pregnancy. Other eye findings of CRS include cataract, anterior
uveitis, and micro-ophthalmia. (Photo contributor: Binita R. Shah, MD.)

102
RUBELLA (CONTINUED) ■ 103

FIGURE 3.57 ■ Rubella. (A, B) Discrete erythematous rose-pink papular rash over the face and subsequently spreading to the trunk in an
otherwise well-appearing 16-month-old infant who was not immunized for rubella. The patient also had significant postauricular and suboc-
cipital adenopathy. Adenovirus and enterovirus infections can cause exanthema that mimics rubella. (Photo contributor: Binita R. Shah, MD.)

infection is asymptomatic and requires no therapy in the major- Pearl


ity of patients who recover without any sequelae. Patients with
1. Rubella causes a 3-day discrete maculopapular rash asso-
arthritis will require therapy directed at pain relief. Children
ciated with prominent tender enlargements of postauricu-
with postnatal rubella should be excluded from schools and
lar, suboccipital, and cervical lymph nodes.
child care centers for 7 days after the onset of the rash.
INFECTIOUS MONONUCLEOSIS

Clinical Summary (moderate enlargement in ∼50%), and skin rash (∼5% to


15% of cases), which can be morbilliform, scarlatiniform,
Infectious mononucleosis (IM) is caused by Epstein-Barr
macular, petechial, urticarial, or erythema multiforme–like.
virus (EBV) in >90% of cases. Five to 10% of IM-like illness
Complications include upper airway obstruction, splenic rup-
is caused by cytomegalovirus, Toxoplasma gondii, adenovi-
ture, hematologic (eg, thrombocytopenia, hemolytic anemia),
rus, viral hepatitis, and HIV. IM is named for mononuclear
cardiac (eg, myocarditis), disseminated lymphoproliferative
lymphocytosis with atypical lymphocytes that accompanies
disease, and chronic fatigue syndrome. Differential diagno-
the illness. IM occurs in all age groups with variable clini-
sis includes streptococcal pharyngitis, leukemia, other viral
cal expression. In young children, it is often asymptomatic
exanthems, viral hepatitis, and diphtheria.
or indistinguishable from other infections. In adolescents and
young adults, the presentation is more classic and is transmit-
ted by oral secretions by close contact such as kissing (hence Emergency Department
called “kissing disease”) or exchange of saliva from child to
child (eg, child care centers). The period of communicabil-
Treatment and Disposition
ity is indeterminate. The incubation period is 30 to 50 days. Obtain CBC with peripheral smear, which will show leu-
Patients present with fever, myalgias, headache, anorexia, kocytosis with predominant lymphocytosis and atypical
chills, and sore throat with moderate to severe exudative ton- lymphocytes usually >10% (other viral infections usually
sillopharyngitis associated with bilateral anterior and pos- <10%). Consider Monospot test (usually negative in children
terior cervical lymph adenopathy (nodes mildly tender to <4 years; heterophil antibody test identifies about 85% of
palpation); submandibular, axillary, inguinal, or epitrochlear cases of IM in older children during the second week of ill-
nodes may also be involved. Other features include hepatic ness), and if required, EBV serology tests (eg, IgM and IgG
manifestations (transient elevations of liver enzymes [∼80% antibodies to viral capsid antigen [VCA], antibody to early
to 90%]), hepatomegaly (∼10% to 15%), splenomegaly antigen [EA], and antibody to EBV nuclear antigen [EBNA]).

FIGURE 3.58 ■ Exudative Tonsillopharyngitis of Infectious Mononucleosis. Marked white exudates on the tonsils of a child with IM.
(Reproduced with permission from: Mei Kane KS, Ryder JB, Jonson RA, et al: Epstein-Barr virus. Color Atlas & Synopsis of Pediatric
Dermatology. New York, McGraw-Hill, 2002, p. 576.)

104
INFECTIOUS MONONUCLEOSIS (CONTINUED) ■ 105

FIGURE 3.59 ■ Infectious Mononucleosis. Cervical lymphadenopathy and mouth breathing are noted in this child who presented with
difficulty breathing, fever, and sore throat. His monospot was positive and had 25% atypical lymphocytes on the peripheral smear. (Photo
contributor: Binita R. Shah, MD.)

FIGURE 3.60 ■ Infectious Mononucleosis. Cervical and submental lymphadenopathy (arrow) and mouth breathing are seen in this
child who presented with “worsening of his snoring” sore throat, and fever. For upper airway obstruction due to IM see also Fig. 6.39. (Photo
contributor: Binita R. Shah, MD.)
106 ■ INFECTIOUS MONONUCLEOSIS (CONTINUED)

FIGURE 3.61 ■ Ampicillin (or Other Penicillin) Related Rash in Infectious Mononucleosis. (A) This generalized erythematous papular
rash was precipitated by amoxicillin, which was given to this patient 5 days earlier for exudative tonsillopharyngitis (presumed to be “strep
throat”). Fever continued while on amoxicillin. He also had splenomegaly and atypical lymphocytosis. Administration of ampicillin (or
other penicillin) produces a rash in up to 90% to 100% of patients with IM. The rash may be pruritic, erythematous, or copper-colored
maculopapular rash that begins 5 to 10 days after the drug is begun and lasts up to 1 week. Involvement can be extensive on trunk, including
involvement of palms and soles. (B) Amoxicillin rash in a different patient with IM. This rash is not an allergic reaction to penicillin. True
penicillin-allergic skin manifestations include urticaria, erythema multiforme, and Stevens-Johnson syndrome/toxic epidermal necrolysis.
(Photo contributors: Binita R. Shah, MD [A] and Andrea Marmor, MD [B].)

IM is typically self-limited and patients recover without to avoid contact sports or heavy lifting until full recovery
specific therapy. Admit patients with complications such of splenomegaly and refer such patients to the primary
as upper airway obstruction. Corticosteroid therapy is not care physician for monitoring. Educate family about signs
routinely recommended; however consider a short course of of splenic rupture (eg, sudden-onset abdominal pain, espe-
prednisone given orally for 7 days with subsequent tapering cially left-sided; vomiting; pallor; lethargy; or fatigue [signs/
if there is upper airway obstruction. Advise family/patient symptoms of hemorrhage]).
INFECTIOUS MONONUCLEOSIS (CONTINUED) ■ 107

FIGURE 3.62 ■ Splenic Rupture; Infectious Mononucleosis. CT scan of the abdomen demonstrates grade 4 laceration of the spleen, extend-
ing to hilum with smaller areas of laceration. Active extravasation was also noted on a different CT image. This 16-year old adolescent male
presented with abdominal pain, left shoulder pain and feeling dizzy following trauma to his left side of the abdomen while wresting in the
school. He had a syncopal episode while in the ED. He had sore throat 2 weeks prior to this. Splenic rupture is a rare but serious complication
leading to hemorrhage, shock, and death. Incidence of rupture is highest in 2nd or 3rd week of illness. Rarely this may a first sign of IM as
seen in this patient. (Photo/legend contributors: Vikas S. Shah, MD/John Amodio, MD.)

Pearls 3. Streptococcal pharyngitis mimics IM; tender tonsillar


and cervical adenopathy and absence of hepatospleno-
1. Classic IM is an acute illness characterized by a triad of
megaly may help in differentiating between the two.
fever, exudative pharyngitis, and cervical adenopathy.
Suspect IM when a patient with “strep throat” does not
2. IM spectrum varies from asymptomatic infection to fatal
improve within 48 to 72 hours after antibiotics.
infection; most frequent causes of mortality are neuro-
4. Ampicillin or amoxicillin given to patients with IM
logic complications, splenic rupture, or upper airway
causes nonallergic morbilliform rash that is often mis-
obstruction.
taken for penicillin allergy.
ERYTHEMA INFECTIOSUM

Clinical Summary Complications include transient aplastic crisis in patients


with hemolytic anemias (eg, sickle cell disease, thalassemia).
Erythema infectiosum (also known as fifth disease) is a
Adolescent and young adults (more commonly females) may
exanthematous illness caused by human parvovirus B19.
develop symmetrical peripheral polyarthropathy (hands,
Sporadic cases are seen throughout the year, while sea-
wrists, knees, ankles) that may last 2 to 4 weeks. Neurologic
sonal peaks occur in late winter and spring, most commonly
complications (eg, meningitis, encephalitis), thrombocytope-
affecting school-aged children. The incubation period
nic purpura, and hydrops fetalis due to intrauterine infection
ranges from 4 to 21 days. The prodrome of myalgia and
of the fetus are other complications. Differential diagnosis
headache may be absent or mild; patients are usually afe-
includes other viral exanthems (eg, enteroviral infections),
brile (or low-grade fever) presenting with a rash. The dis-
drug eruptions, and collagen vascular diseases (patients pre-
ease occurs in three stages. First is a rash of red flushed
senting with arthralgia/arthritis).
cheeks with circumoral pallor (slapped-cheek appearance)
lasting 1 to 4 days. This is followed by a symmetric, ery-
thematous maculopapular rash beginning on the trunk and Emergency Department
spreading to the arms, buttocks, and thighs. The palms and Treatment and Disposition
soles usually are spared and the rash is more prominent on
extensor surfaces. The rash is often pruritic. As the rash Diagnosis is clinical based on the exanthema and no labo-
clears spontaneously, there is central clearing, making the ratory tests are required for immunocompetent children. For
rash appear reticulated or lace-like. Desquamation does not patients with hemolytic anemias, obtain CBC with reticulo-
occur and the rash resolves within 3 weeks. However, in the cyte count. Presence of anti-B19 IgM antibody on a single
third stage of the disease, there is recurrent waxing and wan- serum specimen confirms acute or recent infection. IgG anti-
ing of rash (for weeks or sometimes months) precipitated bodies are detected a few days after IgM antibodies and per-
by a variety of stimuli (eg, sunlight, vigorous exercise, hot sist for years. The disease is self-limited and only supportive
shower, stress). therapy is required for immunocompetent patients. Children

FIGURE 3.63 ■ Slapped-Cheek Appearance of Erythema Infectiosum. Slapped-cheek appearance in a child with parvovirus B19 infection.
(Reproduced with permission from: Mei Kane KS, Ryder JB, Jonson RA, et al: Human parvovirus B19. Color Atlas & Synopsis of Pediatric
Dermatology. New York, McGraw-Hill, 2002, p. 579.)

108
ERYTHEMA INFECTIOSUM (CONTINUED) ■ 109

FIGURE 3.64 ■ Erythema Infectiosum. Lacy, reticulated rash on the body of a child with EI. (Reproduced with permission from: Shah BR,
Laude TL: Erythema infectiosum. In: Atlas of Pediatric Clinical Diagnosis. Philadelphia, WB Saunders, 2000, p. 70.)

do not need isolation and exclusion from school or day care as Pearl
they no longer are contagious once the rash appears. Educate
1. Slapped-cheek appearance in an otherwise well-appear-
family about possible recurrence of the rash. No specific ther-
ing child is pathognomonic of EI.
apy is necessary for arthritis except pain management. Admit
patients with hemolytic anemias presenting with aplastic
crisis for close monitoring and possible transfusion.

FIGURE 3.65 ■ Papular Purpuric “Gloves and Socks” Syndrome in Parvovirus B19 Infection. This syndrome is characterized by pete-
chiae on the hands and feet that appear in a distinct gloves-and-socks distribution (with a clear line of distinction between rash and non-rash
areas at wrists and ankles). It is usually seen in older children and adolescents with parvovirus B19 infection. (Photo contributor: Binita R.
Shah, MD.)
HAND-FOOT-AND-MOUTH DISEASE

Clinical Summary buccal mucosa, hard palate, gingivae, lips). Elliptical or oval
gray-roofed vesicles with erythematous rims varying in size
Hand-foot-and-mouth disease (HFMD) is a clearly recog-
from 3 to 7 mm (up to 2 cm) and in number from a few to
nizable coxsackie or enterovirus infection commonly seen
a hundred are seen. Skin lesions are bilateral and symmet-
during late summer and early fall in temperate climates and
ric on hands and feet and usually are asymptomatic, whereas
often occurring in epidemics. HFMD is a highly contagious
mouth lesions are painful. Nonvesicular papular, macular, or
illness; modes of transmission include fecal-oral, respiratory
petechial lesions may be seen on the buttocks (most com-
route, and from fomites. The incubation period ranges from
mon site), upper thighs, and knees. Myocarditis, meningo-
4 to 7 days. Constitutional symptoms (eg, fever, diarrhea,
encephalitis, and aseptic meningitis are rare complications.
anorexia) usually precede skin lesions. Location of lesions
Differential diagnosis includes other viral exanthems with
include hands/palms (dorsal and palmar surface, sides of fin-
vesicular lesions (eg, varicella), herpes gingivostomatitis,
gers), feet (sides of feet and toes, plantar surface of soles),
herpangina, aphthous stomatitis, arthropod bites, and allergic
and mouth (anterior portion of mouth involving tongue and
contact dermatitis.

FIGURE 3.66 ■ Hand-Foot-and-Mouth Disease. Typical elliptical or oval-shaped papulovesicular lesions with erythematous rims are seen
on the hand and foot (A) and (B),

110
HAND-FOOT-AND-MOUTH DISEASE (CONTINUED) ■ 111

FIGURE 3.66 ■ (Continued) ulcers surrounded by a rim of erythema are seen in the mouth (C). A nonvesicular maculopapular rash (D) is
also seen on the buttocks which is the most common site. Similar lesions may also be seen on the upper thighs and knees (arms, legs, and face
are less commonly involved). (Photo contributor: Binita R. Shah, MD.)

Emergency Department Pearls


Treatment and Disposition 1. HFMD is characterized by vesicular lesions in the mouth,
Diagnosis is made clinically, and no laboratory tests are usu- hands, and feet. However, all three anatomic areas need
ally performed. Treatment is supportive with antipyretics/ not be affected. The mouth is most frequently involved,
analgesics (as indicated) with attention to adequate hydration. followed by hands than feet.
Soft diet and cool, noncitrus liquids are often well tolerated 2. Do not use lidocaine viscous topically for pain relief in
in the presence of oral ulcerations. Advise regarding personal the mouth. Systemic toxicity can occur due to absorption
hygiene including hand washing to prevent spread of the of the lidocaine from the buccal mucosa.
infection. HFMD usually is a self-limited disease with lesions
lasting 2 to 7 days and healing without scarring.
HERPANGINA

Clinical Summary Emergency Department


Herpangina is a characteristic enanthem produced by entero- Treatment and Disposition
viruses like coxsackie A, coxsackie B, and echoviruses,
Diagnosis is made clinically, and usually no laboratory tests
affecting most commonly children between 3 and 10 years
are performed. Treatment is symptomatic, with attention
old. It is typically seen during summer or early fall in temper-
to maintaining good hydration. Soft diet and cool, noncit-
ate climates. Constitutional symptoms (eg, fever, vomiting,
rus liquids are often well tolerated in the presence of oral
sore throat, dysphagia) may be present. Discrete vesicles and/
ulcerations.
or ulcers (1–2 mm in diameter) surrounded by an erythema-
tous ring occur on the anterior tonsillar pillars (most frequent
site), soft palate, tonsils and uvula, and posterior pharyngeal Pearls
wall. Number of lesions varies from a few to many, lasting for
1. Herpangina causes tiny vesicles and ulcers on the poste-
about 1 week. The course is benign in the majority of cases,
rior pharynx. HSV gingivostomatitis causes ulcers in the
without any complications. Differential diagnosis includes
posterior and anterior oropharyngeal mucosa involving
herpes simplex virus (HSV) gingivostomatitis and hand-foot-
tongue, buccal mucosa, and gums which are swollen and
and-mouth disease.
may bleed with minor trauma.
2. Hand-foot-and-mouth disease causes oval-shaped vesi-
cles with erythematous rims on hands, feet, and mouth
associated with nonvesicular papular, macular, or pete-
chial lesions that may be seen on buttocks, upper thighs,
and knees.

FIGURE 3.67 ■ Herpangina. Typical yellowish-white ulcers or vesicles that measure usually about 1 to 2 mm in diameter surrounded by an
erythematous base are seen. Typically in herpangina, these lesions are seen on the soft palate, tonsils, and uvula. Gingiva, tongue, and buccal
mucosa are spared (unlike herpetic gingivostomatitis in which gingiva, tongue, and buccal mucosa are involved). (Reproduced with permis-
sion from: Greenberg MI (ed): Greenberg’s Text Atlas of Emergency Medicine. Lippincott Williams and Wilkins, Philadelphia, 2005, p. 156.)

112
CUTANEOUS LARVAE MIGRANS

Clinical Summary months even without treatment, as the larvae die. For symp-
tomatic relief, prescribe oral albendazole or ivermectin.
Cutaneous larvae migrans (CLM, also known as creeping
eruption or sandworm disease) is a skin infestation caused
most commonly by the larvae of the dog or cat hookworm
Pearl
(eg, Ancylostoma braziliense) and rarely by human nematodes 1. An advancing serpiginous growing tunnel in the skin asso-
(eg, Ancylostoma duodenale). A braziliense larvae infect dogs ciated with intense pruritus is pathognomonic of CLM.
and cats by burrowing through the skin. Adult hookworms
reside in the intestine and shed eggs in feces that are deposited
on the ground. The eggs develop into infectious larvae outside
the body in places protected from desiccation and temperature
extremes, such as sandy, shady areas around beaches or under
houses. The larvae penetrate human skin to initiate infection.
CLM is commonly seen in people who come into contact with
soil contaminated with cat and dog feces (eg, children, garden-
ers, sunbathers, playing in sandboxes, walking barefoot on a
beach, and working in tight crawlspaces under houses). Larvae
migrate through the skin (at the plane of the epidermal-dermal
junction) and advance from several millimeters to a few cen-
A
timeters a day, producing serpiginous tracks. Larval activity
can continue for weeks to months; however, the organisms are
unable to penetrate the basement membrane, and die within
months because they cannot complete their life cycle in the
human host. CLM occurs worldwide. Skin lesion begins as a
stinging or tingling sensation and reddish papule at the site of
larval penetration with an intense localized pruritus. A serpigi-
nous, tortuous, slightly raised track (sometimes with vesicles
and bullae) is seen in the upper epidermis; such tracks can grow
1 to 2 cm/day (larva typically located 1 to 2 cm in front of ser-
piginous eruption). New areas of involvement may appear every
few days. Lesions may be single or numerous (if large areas of
the body are exposed, eg, sunbathing). Systemic symptoms are
absent. Common sites (any area may be affected) include foot,
buttock, hand, and genitalia. Rare presentations include Löffler
syndrome (pneumonitis with a large burden of parasites), myo-
sitis, and eosinophilic enteritis (larvae reaching the intestine).
Peripheral eosinophilia may be present. Skin biopsy is not nec-
essary for the diagnosis (eosinophilic inflammatory infiltrate
without migrating parasite seen). Differential diagnosis includes
tinea infections, contact dermatitis, granuloma annulare, and
cutaneous pili migrans (embedded hair or creeping hair).

B
Emergency Department
Treatment and Disposition FIGURE 3.68 ■ Cutaneous Larva Migrans. Erythematous, intensely
pruritic, tortuous, and serpiginous tracks of migrating larva in a
Diagnosis is clinical. CLM is usually a self-limited disease. 6-year-old patient with a history of walking barefoot on one of the
Spontaneous cure occurs usually in several weeks to a few Caribbean islands. (Photo contributor: Binita R. Shah, MD.)

113
ASCARIASIS

Clinical Summary intussusception and acute intestinal obstruction (seen in chil-


dren with heavy infection). Stressful conditions (eg, fever,
Ascariasis (roundworm) is a nematode infestation by Ascaris
illness) stimulate adult worm migration. Allergic reactions
lumbricoides that occurs at all ages, most commonly in pre-
(due to absorption of toxins from products of living or dead
school- and early school–aged children. Adult worms live
worms) include asthma, hay fever, and urticaria. Differential
freely in the lumen of the small intestine (commonly in the
diagnosis of pulmonary ascariasis with eosinophilia includes
jejunum and middle ileum) for up to a year, shedding eggs in
asthma and Löffler syndrome from other parasites (eg, toxo-
the stool, leading to fecal-oral transmission. Low standards
cariasis, strongyloidiasis, hookworm); differential of ascaria-
of hygiene, poor sanitation, and use of human feces as fer-
sis-induced GI diseases include pancreatitis or appendicitis or
tilizer are contributing factors. The majority of patients are
cholecystitis from other etiologies.
asymptomatic. Pulmonary signs and symptoms occur as lar-
vae migrate through the lungs and include fever, shortness of
breath/dyspnea, substernal pain, wheezing/asthma, hemopty- Emergency Department
sis, rales, Löffler syndrome (transient pulmonary infiltrates),
and peripheral eosinophilia. Gastrointestinal signs and symp-
Treatment and Disposition
toms caused by adult worms in the small intestine include Identification of ova on direct smear of a fresh stool specimen
abdominal pain (persistent or recurrent) and distension, or of adult worms in vomitus or feces (often brought to the
vomiting (with or without bile), malabsorption, and diarrhea. ED by caretakers) confirms diagnosis. If infection is uncer-
Worms may be seen in vomitus or stools. Worm migrations tain, examine direct smear of stool samples on three con-
at different sites may cause ascending cholangitis, acute pan- secutive mornings. If biliary ascariasis is suspected, obtain
creatitis, acute appendicitis, diverticulitis, gallstones (dead ultrasound of the biliary tree. Prescribe orally albendazole as
worms as nidus for stone), perforation, peritonitis, volvulus, a single dose or mebendazole for 3 days. Consult surgery if

FIGURE 3.69 ■ Ascaris lumbricoides. This worm was vomited in the ED by a patient presenting with abdominal pain and several episodes of
nonbilious vomiting. Roundworm is the largest intestinal nematode parasite of humans. Sites from which adult worms may be passed include
the rectum, nose (after migration through the nares), and from the mouth in vomitus. Characteristic features of roundworms include tapered
ends, length between 15 and 40 cm (females tend to be larger), and white or reddish-yellow in color. (Photo contributor: Binita R. Shah, MD.)

114
ASCARIASIS (CONTINUED) ■ 115

FIGURE 3.70 ■ Ascariasis. Plain radiograph of the abdomen showing a curvilinear radiopaque density in the bowel lumen in the left iliac
region (this radiopaque density represents a roundworm, as this patient gave a history of passing roundworms in the stool). He presented with
clinical signs of intestinal obstruction. He responded to conservative management and passed several worms after receiving mebendazole
therapy. (Photo contributor: Binita R. Shah, MD.)

patient presents with acute intestinal obstruction and admit Pearls


for conservative management (eg, IV hydration, nasogastric
suctioning), which may resolve symptoms. Surgical inter- 1. Ascariasis is the most prevalent human helminthic
vention may be still needed for obstruction or volvulus. In infestation.
a patient with a mixed infestation, treat ascariasis first; treat- 2. Small diameter of the intestinal lumen and propensity
ment of other parasites first may stimulate a large worm bur- to acquire large worm burdens predisposes children for
den to migrate simultaneously, causing obstruction. acute intestinal obstruction.
MALARIA

Clinical Summary and hepatomegaly may occur and anemia/pallor, thrombo-


cytopenia, leucopenia, leukocytosis, and jaundice may be
Malaria is a protozoan infection transmitted to humans by
present. Different species of Plasmodium cause specific
the bite of nocturnal-feeding Anopheles mosquitoes. Four
symptoms.
species of Plasmodium that cause infection include P falci-
parum, P ovale, P vivax, and P malariae (most commonly
P falciparum and P vivax). Less commonly, transmis- Emergency Department
sion occurs through transfusion, contaminated needles or
syringes, or transplacentally. Malaria affects all ages, and
Treatment and Disposition
infections can be acute or chronic. Though Anopheles mos- Obtain CBC, comprehensive metabolic panel, a thick and
quitoes are native to the United States, most cases here are thin peripheral smear (thick smear to detect organisms in
imported. Following a 1- to 3-week incubation period in the erythrocytes, and thin smear to determine species). Obtain
liver, Plasmodium enters a 48- to 72-hour asexual eryth- multiple smears per day and if possible at least one during or
rocyte cycle depending on the species. This explains the right after a fever spike. Consult infectious disease special-
periodicity of high fever and shaking chills (febrile parox- ist and/or Centers for Disease Control (CDC) for treatment
ysms) that are the classic presentation. Sweats, rigors, head- (http://www.cdc.gov/malaria/faq.htm). The percentage of
ache, and muscle aches are also seen. At first, the fever is erythrocytes harboring parasites and identification of species,
irregular followed by paroxysms alternating with periods of both detected from a thin peripheral smear, help determine
fatigue in a cyclic pattern (as the infection becomes syn- therapy. Admit any patient presenting with severe malaria
chronized). Paroxysms coincide with rupture of schizonts. with signs of CNS or other end-organ involvement, shock,
Nausea, vomiting, diarrhea, abdominal pain, arthralgia, and metabolic abnormalities (eg, acidosis, hypoglycemia), or par-
back pain may be present with paroxysms. Splenomegaly asitemia >5% to the ICU for monitoring and therapy.

A B

FIGURE 3.71 ■ Plasmodium falciparum Malaria: Giemsa-Stained Thin Smear of Peripheral Blood. (A) Multiple ring forms of P falciparum
(arrow). Note that many of the RBCs are infected and some have two ring forms. Some of the rings have double chromatin dots. (B) Banana-
or crescent-shaped gametocytes (arrow) of P falciparum. Note that the RBC membrane has been stretched to enclose the gametocyte. This
child immigrated from Africa 2 weeks before his illness. (Photo contributor: Haseeb A. Siddiqi, PhD.)

116
MALARIA (CONTINUED) ■ 117

TABLE 3.4 ■ SPECIFIC SYMPTOMS CAUSED BY DIFFERENT PLASMODIA

Organism Incubation Period (days) Periodicity of Fever Other Specific Symptoms


P ovale and P vivax 12–18 Schizonts rupture every Hypersplenism (risk for splenic rupture)
(benign tertian P vivax can be 6–12 months 48 hours, resulting in Relapse (up to 3 to 5 years after primary
malaria) daily fever spikes and infection)
paroxysms
P falciparum 9–14 Less apparent Cerebral malaria (fever up to 106°F to
(malignant tertian 108°F, delirium, coma, seizures, pupillary
malaria) changes, retinal hemorrhages, hemiplegia,
and absent or exaggerated deep tendon
reflexes)
Coagulopathy
Noncardiogenic pulmonary edema,
respiratory failure
Hypoglycemia, metabolic acidosis,
blackwater fever (massive intravascular
hemolysis, hemoglobinemia, hemoglobin-
uria [“black urine”])
Acute hepatic and renal failure
Algid malaria (overwhelming infection:
hypotension, hypothermia, pallor, vascular
collapse, and death)
P malariae (benign 18–40 Schizonts rupture every Chronic asymptomatic parasitemia (for
quartan malaria) 72 hours, resulting in several years), nephrotic syndrome
alternate-day or every-
third-day fever spikes
and paroxysms

Pearls 3. Diagnosis of P falciparum malaria is a medical emergency.


4. P vivax and P ovale parasites may cause relapse months
1. Presume malaria unless proven otherwise in children
to years after initial infection.
with unexplained fever or systemic illness and history of
5. Malaria is preventable with chemoprophylaxis and mea-
travel or emigration from an endemic area within the pre-
sures to prevent contact with mosquitoes (eg, protective
vious year.
clothing, repellents).
2. Repeat peripheral smear every 12 to 24 hours over
72 hours if clinical suspicion is strong and an initial
smear is negative.
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GYNECOLOGY
Chapter 4

SEXUAL ABUSE,
GYNECOLOGY,
AND SEXUALLY
TRANSMITTED
INFECTIONS
Amy Suss
Sarah A. Rawstron
Konstantinos Agoritsas

Condyloma
acuminata

The authors acknowledge the special contributions of Binita R. Shah, MD, to prior edition.

119
SEXUAL ABUSE AND SEXUALLY TRANSMITTED INFECTIONS

Clinical Summary
Child sexual abuse occurs when a dominant, more powerful
person involves a depending, developmentally immature child
or adolescent in sexual activities for his or her own or others’
sexual stimulation or gratification. This includes child pornog-
raphy and prostitution, and ranges from nontouching abuses to
direct genital, anal, or oral-genital contact. About 1% of children
experience some form of sexual abuse each year. Perpetrators
are usually male (75% to 90%) and usually involve adults or
minors known to the child. Abuse by family members or known
acquaintances often involves multiple episodes over weeks to
years, whereas abuse by strangers tend to be a single episode.
FIGURE 4.1 ■ Condylomata Acuminata (Genital Warts Due to
Most cases are detected when the child discloses the abuse.
Sexual Abuse). Lesions are seen in the perianal area of this 3-year-
Sexually abused children may present with a variety of general old male child who was abused by his maternal uncle. Genital warts
and/or nonspecific symptoms and signs (eg, sleep disturbances, are soft, flesh-colored, elongated lesions that develop around muco-
abdominal pain, enuresis, encopresis, phobias). Physical find- cutaneous junctions and intertriginous areas (eg, perianally, mucosae
ings are often absent even when the perpetrator admits to pen- of female genitalia). Condylomata acuminata must be differentiated
from condylomata lata as both occur in the same areas. (Photo con-
etration of the child’s genitalia. Many types of abuse leave no
tributor: Binita R. Shah, MD.)
physical evidence, and injuries to the mucosa heal quickly and
thus leave no physical evidence in most cases.

Emergency Department their influence or distraction are minimized. Maintain a “tell


me more” or “and then what happened approach.” Explain
Treatment and Disposition the physical examination to the child before conducting it and
Obtain a history in those older than age 3 years, and avoid avoid any additional emotional trauma. Examination should
leading and suggestive questions or showing strong emotions be immediate if the patient presents within 72 hours of the
such as disbelief or shock in the absence of the parent so that alleged sexual abuse, and must include checking for acute

TABLE 4.1 ■ IMPLICATIONS OF COMMONLY ENCOUNTERED STDs FOR THE DIAGNOSIS AND REPORTING
OF SEXUAL ABUSE OF INFANTS AND PREPUBERTAL CHILDREN

STD Confirmed Sexual Abuse Suggested Action


Gonorrhea* Diagnostic† Report‡
Syphilis* Diagnostic Report
HIV|| Diagnostic Report
Chlamydia* Diagnostic† Report
Trichomoniasis Highly suspicious Report
Condylomata acuminata* Suspicious Report
Genital herpes Suspicious Report§
Bacterial vaginosis Inconclusive Medical follow-up
*
If not perinatally acquired and rare nonsexual vertical transmission is excluded.

Use definitive diagnostic methods such as culture.

To local agencies as required to receive reports of suspected sexual abuse.
§
Unless there is a clear history of autoinoculation. Herpes virus types 1 and 2 are difficult to differentiate using current techniques.
||
If not perinatally or transfusion acquired.
Reproduced with permission from: American Academy of Pediatrics: The evaluation of sexual abuse in children: Clinical report Kellog N and Committee on
Child Abuse and Neglect. Pediatrics 2005;103:509.

120
SEXUAL ABUSE AND SEXUALLY TRANSMITTED INFECTIONS (CONTINUED) ■ 121

A B

FIGURE 4.2 ■ Condylomata Acuminata (Gental Warts) due to Sexual Abuse. (A, B) A cauliflower-like mass present for 1 year on the
perineum of this 10-year-old girl is shown. She was repeatedly sexually abused by her stepfather since the age of 8. Condylomata start as
pinhead papules that are pink, red, or skin colored. Lesions remain either solitary or develop into grapelike clusters and may coalesce in the
rectal or perineal area to form a large cauliflower-like mass. (Photo contributor: Binita R. Shah, MD.)

FIGURE 4.4 ■ Genital Herpes due to Sexual Abuse. Extremely


painful ulcerative lesions (HSV type 2 culture positive) were seen
in an 8-year-old girl who was repeatedly abused by her uncle in
the past 2 weeks. Genital herpes is uncommon in children. Lesions
usually appear after an incubation period of 2 to 20 days after expo-
sure. Except for perinatal transmission at birth, most HSV-2 genital
infections are sexually transmitted. (Photo contributor: Binita R.
FIGURE 4.3 ■ Condyloma Lata due to Sexual Abuse. Flat-topped,
Shah, MD.)
round-oval nodular lesions and plaque (formed by papules that
coalesce) with a wide base are seen around the anus and genitalia
in a 6-year-old girl who was repeatedly abused by her stepfather.
Unlike condylomata acuminata, these lesions are flat and not cov-
ered by digitate vegetations. (Reproduced with permission from
Shah BR, Laude T: Atlas of Pediatric Clinical Diagnosis. WB
Saunders, Philadelphia, 2000, p. 40.)
122 ■ SEXUAL ABUSE AND SEXUALLY TRANSMITTED INFECTIONS (CONTINUED)

FIGURE 4.5 ■ Gonococcal Conjunctivitis. An 8-year-old girl with profuse mucopurulent discharge. Her eye, pharyngeal, and rectal cultures
were positive for Neisseria gonorrhea. Investigation among the family members led to a 21-year-old uncle with gonococcal urethritis, and
subsequently he confessed abusing this girl. (Reproduced with permission from Shah BR, Laude T: Atlas of Pediatric Clinical Diagnosis.
WB Saunders, Philadelphia, 2000, p. 40.)

injury or bleeding, vaginal discharge, possible presence of for asymptomatic prepubertal children being evaluated for
sexually transmitted infections (STIs) and the possibility of possible child sexual abuse. In contrast, adolescents should
pregnancy in adolescent girls who have reached menarche. be encouraged to receive prophylaxis for STIs. Use a multi-
If more than 72 hours have passed since the alleged abuse, disciplinary approach involving local or regional child abuse
the exam can be scheduled at the earliest time that is com- consultants and a social worker. Once sexual abuse is iden-
fortable for the child, the investigative team, and the physi- tified, all siblings should be examined and close follow-up
cian. Perform a thorough physical examination, including arranged within 1 to 2 weeks to detect new infections and to
mental and emotional status with special attention to areas complete counseling and treatment for other STIs. Consider
that are involved in sexual activity. The supine frog-leg posi- mental health assessment and treatment as necessary.
tion is most often employed and well tolerated by prepuber-
tal girls. It is important also to view the hymen in a second
position if a concerning physical finding is seen. The prone
Pearls
knee-chest position often allows better visualization of the 1. A normal physical examination does not exclude abuse;
hymen, the fossa navicularis, and the posterior fourchette. Do diagnostic findings are seen in 5% of victims.
not perform speculum or digital examinations in a prepuber- 2. Physical examination should not result in additional emo-
tal child. A digital rectal examination is usually not neces- tional trauma.
sary. Conduct universal screening of postpubertal patients 3. Vaginal, rather than cervical, samples are adequate for
for gonorrhea, Chlamydia, syphilis, HIV, or other STIs. In testing of STIs in prepubertal children.
asymptomatic prepubertal patients, the yield of positive 4. Diagnosis is often made on the basis of the history; a child’s
cultures is very low, and appropriate cultures and serologic statement of abuse is often the most important evidence.
tests are recommended when epidemiologically indicated or 5. Presume sexual abuse until proven otherwise in a child
when history and/or physical findings suggest oral, genital, presenting with STIs.
or rectal contact. Prophylaxis for STIs is not recommended
SEXUAL ABUSE AND SEXUALLY TRANSMITTED INFECTIONS (CONTINUED) ■ 123

A B

FIGURE 4.6 ■ Injuries due to Sexual Abuse. (A) Fresh bleeding and blood clots at the introitus and a superficial laceration in the posterior
fourchette were seen in this 4-year-old girl who was sexually abused by her uncle. (B) A hematoma at the introitus and fresh anal injuries with
significant anal dilation were seen in this 4-month-old infant brought to the ED with cardiopulmonary arrest. These injuries occurred while
infant was being cared by mother’s boyfriend. Posterior rib fractures, splenic rupture, and retroperitoneal hematoma were other injuries seen
in this infant. (Photo contributor: Binita R. Shah, MD.)

FIGURE 4.7 ■ Infantile Hemangioma Mimicking Sexual Abuse. FIGURE 4.8 ■ Jacquet Diaper Dermatitis Mimicking Sexual Abuse.
A 7-month-old infant with hemangioma with central erosions This severe erythematous erosive eruption is in the differential of
of the gluteal cleft with extension to the perineum. Evaluation diaper dermatitis and can mimic sexual abuse. (Photo contributor:
of this midline hemangioma revealed a tethered cord and left- Julie Cantatore, MD.)
sided duplex ureter, thus fulfilling criteria for PELVIS syndrome.
Because of perianal location and erosion, this can be mistak-
enly attributed to sexual abuse. (Photo contributor: Sharon A.
Glick, MD.)
CONDYLOMATA ACUMINATA

Clinical Summary Oncogenic or high-risk HPV types (eg, HPV types 16


and 18) cause cervical cancers and other anogenital cancers.
Condyloma acuminata (genital warts) are skin-colored, fleshy
Persistent infection is the strongest risk factor for the develop-
lesions on mucocutaneous junctions and intertriginous areas,
ment of precancers and cancers. HPV types 6 and 11 account
which can coalesce to large cauliflower-like lesions. Locations
for 90% of genital warts. Two HPV vaccines are licensed in
include perianal area, glans penis, scrotum, labia or posterior
the United States: a bivalent vaccine (Cervarix) containing
introitus, vagina, cervix, anus, urethra, mouth, nose, and eyes.
HPV types 16 and 18 and a quadrivalent vaccine (Gardasil)
Symptoms may include pruritus, burning, bleeding, and pain
containing HPV types 6, 11, 16, and 18. HPV DNA testing
depending on location and size. Condyloma acuminate is
is not recommended as it does not alter clinical management.
caused by human papillomavirus (HPV), which is prevalent
Differential diagnosis includes condylomata lata, pearly
and insidious: >50% of sexually active people are infected
penile papules, and skin tags.
and most infections are asymptomatic, unrecognized, or sub-
clinical and self-limited. Transmission, occurs by sexual and
nonsexual contact activity. Genital warts in children require
Emergency Department
careful consideration as about 50% are the result of sexual Treatment and Disposition
abuse. Nonsexual acquisition is usually seen in children <3 Untreated, visible warts can resolve on their own, remain
years old and may be suggested by warts on the hands (with unchanged, or increase in size or number. Treatment is aimed
autoinoculation patient transferring warts to mouth, genitals, at relief of symptoms and many factors influence treatment
anal area), a mother with hand warts, sexual play among chil- selection, including size, number, anatomic site, morphol-
dren, absence of other signs of sexual abuse, and warts distant ogy, patient preference, convenience, adverse effects, and
from the anus or introitus. provider experience. The response to therapy is influenced

FIGURE 4.9 ■ Condylomata Acuminata. Condylomata lesions


grouped into grapelike clusters in a sexually active adolescent girl.
These can be pink, red, or skin colored, with a smooth or velvety FIGURE 4.10 ■ Condylomata Acuminata due to Sexual Abuse.
surface and soft consistency. Lesions may coalesce in the perineal Venereal warts in a 10-year-old girl who was abused by uncle.
area to form a large cauliflower-like mass. (Photo contributor: Binita (Photo contributor: Binita R. Shah, MD.)
R. Shah, MD.)

124
CONDYLOMATA ACUMINATA (CONTINUED) ■ 125

FIGURE 4.11 ■ Condylomata Acuminata. Perianal warts on a


young boy evaluated for sexual abuse (treated with topical gentian
violet). Infection was most likely due to vertical transmission from
mother at birth. (Photo contributor: Sharon A Glick, MD.)

B
by the presence of immunosuppression and compliance with
therapy. No treatment has been shown to be superior to any FIGURE 4.12 ■ Condylomata Acuminata. Hundreds of warts
other and no single treatment is ideal for all patients or all on mons pubis (A) and few lesions on breast (B) of a young teen-
warts. Patient-applied therapies and provider-administered ager with HIV. Warts may occur more frequently and in greater
number in the immunocompromised. (Photo contributor: Morgan
therapies are available. Patient-applied treatment regimens
Rabach, MD.)
for external genital warts include podofilox 0.5% solution
or gel or imiquimod 5% cream or sinecatechins 15% oint-
ment. Refer the patient to dermatology if diagnosis is uncer-
tain, immunocompromised patient, warts are fixed, indurated,
pigmented, ulcerated, or bleeding. Refer for provider-applied
treatment regimens (eg, cryotherapy with liquid nitrogen, sur-
gical removal, curettage, or electrosurgery).

Pearls
1. Examination of sexual partners is not required; partners
are usually infected at the time one person is diagnosed
with HPV infection even in the absence of visible warts.
2. Exclude sexual abuse in children with genital warts.
3. Consult a specialist for management of intraanal warts as
FIGURE 4.13 ■ Pearly Penile Papules; Differential Diagnosis of
patients may also have warts on the rectal mucosa.
Condyloma Acuminata. Dome-shaped or hairlike projections on
4. Laryngeal papillomatosis has been seen in infants born the penile corona are small angiofibromas, and represent normal
to mothers with HPV infection, delivered vaginally or by variants that do not require treatment. (Photo contributor: Mark
cesarean section. Silverberg, MD.)
TRICHOMONIASIS

Clinical Summary Treat patients with metronidazole (2 g orally in a single


dose or 500 mg orally twice a day for 7 days) or Tinidazole
Trichomonas vaginalis infection is almost always sexually
(2.0 g orally in a single dose). Both of these regimens have
transmitted with peak prevalence rates between ages of 16
high cure rates of about 95%. Intravaginal or topical metroni-
to 35 years. The most common site of infection is the vagina,
dazole gel has not been shown to be effective and is not rec-
followed by periurethral glands and urethra. Patients present
ommended. Certain strains of T vaginalis can have diminished
with pruritus, dysuria, dyspareunia, lower abdominal pain,
susceptibility to metronidazole. If treatment failure occurs
vaginal discharge, postcoital bleeding, edema and excoriation
with single-dose metronidazole and reinfection is excluded,
of the external genitalia, bartholinitis, and urethritis.
treat with metronidazole for a 7-day regimen or tinidazole.
Treatment of all sex partners is advisable. Because of the high
Emergency Department rate of reinfection among patients in whom trichomoniasis is
diagnosed, refer the patient to the primary care physician for
Treatment and Disposition
follow-up.
Obtain vaginal secretions for microscopic detection of
Trichomonas (specificity of 60%–70%). Tests like OSOM
Trichomonas Rapid Test or AFFIRM VP III (nucleic acid
Pearls
probe test) performed on vaginal secretions have sensitiv- 1. T vaginalis is one of the most common STDs in the
ity of >83% and specificity >97%. Culture of Trichomonas United States.
is still the most sensitive and specific method of diagnosis. 2. Up to 25% to 50% of girls may be asymptomatic, whereas
PCR assay for T vaginalis in vaginal or endocervical swabs, up to 90% of boys are symptomatic.
and in urine from both men and women is available (sensitivi- 3. Symptomatic girls typically have diffuse, malodorous,
ties ranging from 88% to 97%; specificity from 98% to 99%). yellow-green vaginal discharge with vulvar irritation.
T vaginalis has not been found to infect oral sites, and rectal 4. Cervicitis is the most common signs of infection and
prevalence appears low; therefore, oral and rectal testing is “strawberry spots” may be seen.
not recommended. 5. Adverse pregnancy outcomes with trichomoniasis include
premature rupture of membranes, preterm delivery, and
low birth weight.

FIGURE 4.14 ■ Trichomonas. Saline wet mount demonstrating oval-bodied, flagellated trichomonads. They are similar in size to leukocytes
and can be distinguished from them by their motility and presence of flagella. (Reproduced with permission from Hansfield HH: Atlas of
Sexually Transmitted Diseases. McGraw-Hill, New York, 1992.)

126
CONGENITAL SYPHILIS

Clinical Summary serology at delivery (serology often negative in very early


syphilis and becomes positive later) or when the mother’s
Congenital syphilis occurs in babies born to mothers with
syphilis status was unknown or not reported. Diagnosis relies
untreated or inadequately treated syphilis during pregnancy.
on clinical findings and reactive syphilis serology (nontrepo-
Transplacental transmission of spirochetes, Treponema pal-
nemal antibody tests like the Venereal Disease Research
lidum, can occur at any stage of pregnancy. Congenital syphi-
Laboratory [VDRL] and rapid plasma reagin [RPR] tests),
lis is divided into early (diagnosed at <2 years of age) and
and confirmed by a treponemal test (fluorescent treponemal
late (diagnosed >2 years of age). Clinical manifestations
antibody, absorbed [FTA-ABS] and Treponema pallidum
are listed in Table 4.2. Infants may present with congenital
particle agglutination [TP-PA]). Diagnosis in neonates is dif-
syphilis after nursery discharge if the mother has very early
ficult, because serologic results in the baby reflect maternal
untreated and asymptomatic primary syphilis with negative
antibody status and lack of clinical findings at birth in many

A A

B FIGURE 4.16 ■ Congenital Syphilis. Desquamation over the


hands and feet (A, B) in a neonate. (Photo contributor: Binita R.
FIGURE 4.15 ■ Congenital Syphilis. Erythematous maculopapular Shah, MD.)
round or oval lesions are seen on the hands and feet (A, B) of a neo-
nate. These lesions fade to coppery brown in color. (Photo contribu-
tor: Binita R. Shah, MD.)

127
128 ■ CONGENITAL SYPHILIS (CONTINUED)

FIGURE 4.17 ■ Snuffles of Congenital Syphilis. Rhinitis of con- FIGURE 4.18 ■ Condyloma Lata. A single flat-topped, round-oval
genital syphilis manifests weeks after birth. Nasal discharge is highly plaque (formed by papules that coalesce) with a wide base is seen
infectious. Postinflammatory hypopigmentation is also seen in this around the anus in this infant. Unlike condylomata acuminata, these
infant due to chronic irritation from nasal discharge. VDRL test was lesions are flat and not covered by digitate vegetations. (Photo con-
positive with 1:1024 titer. (Photo contributor: Binita R. Shah, MD.) tributor: Binita R. Shah, MD.)

babies. Recommended tests include complete blood count


(CBC), cerebrospinal fluid (CSF) examination for VDRL,
cell count and chemistry, liver enzymes, long bone x-rays
(usually lower extremities), abdominal ultrasound, and an
ophthalmologic examination.

Emergency Department
Treatment and Disposition
Hospitalize all infants with suspected congenital syphilis for
either IV penicillin or IM procaine penicillin therapy to be
given for 10 days. Therapy is started in the hospital, though
IM procaine penicillin can be continued on outpatient basis
after diagnostic tests are completed.

Pearl
FIGURE 4.19 ■ Congenital Syphilis. Papulosquamous round lesions
1. Most patients with congenital syphilis are asymptom- were seen in this infant who also had hepatosplenomegaly, jaundice,
atic at birth, and develop clinical findings within the first elevated liver enzymes, thrombocytopenia, generalized lymphade-
3 months of life. nopathy, and periostitis. (Photo contributor: Binita R. Shah, MD.)
CONGENITAL SYPHILIS (CONTINUED) ■ 129

TABLE 4.2 ■ CLINICAL MANIFESTATIONS OF CONGENITAL SYPHILIS

Early Congenital Syphilis Late Congenital Syphilis


(Presentation <2 years of age) (Presentation >2 years of age)

General Intrauterine growth restriction, lymphadenopathy, Mental retardation, saddle nose,


pneumonitis, snuffles rhagades
Liver Hepatosplenomegaly, jaundice
Kidney Nephrotic syndrome
Skin Erythematous maculopapular or papulosquamous or
palmoplantar scaling, vesiculobullous hemorrhagic
lesions on palms and soles (rare), condyloma lata,
and moist patches on lips and mouth
Bone Metaphysitis, osteochondritis, periostitis, osteomyelitis, Hutchinson teeth, frontal bossing, saber
Wimberger sign (bilateral medial tibial metaphyseal shins, and Clutton joints
osseous destruction)
Blood Hemolytic anemia, thrombocytopenia, leucopenia,
leukocytosis
Brain Aseptic meningitis (CSF pleocytosis, elevated CSF Eighth nerve deafness, neurosyphilis
protein)
Eye Chorioretinitis, uveitis, glaucoma Interstitial keratitis

FIGURE 4.20 ■ Wimberger’s Sign in Congenital Syphilis. Note FIGURE 4.21 ■ Bony Lesions in Congenital Syphilis. Metaphysitis,
metaphyseal lesion along the medial surface of the tibia. These osteochondritis, and periostitis are pathognomonic and babies with
lesions are seen bilaterally and are highly suggestive of congenital such lesions may present with pseudoparalysis of Parrot (irritabil-
syphilis. (Photo contributor: Binita R. Shah, MD.) ity and refusal to move the involved arm or leg due to severe pain).
These bone findings are in all extremities (not only in the limb that is
painful). (Photo contributor: Binita R. Shah, MD.)
PRIMARY AND SECONDARY SYPHILIS

Clinical Summary
Acquired syphilis is contracted through direct sexual con-
tact with ulcerative lesions of skin or mucous membranes
of infected people, and has three stages: primary, secondary,
and tertiary. Primary syphilis presents with a round, firm,
and painless skin ulcer that has a smooth border and a rub-
bery base. Chancre occurs at the site of contact 10 to 90 days
postexposure and heals spontaneously over 3 to 6 weeks. The
lesion is usually on the glans penis, cervix, or vagina and less
commonly on the lips, nipple, and tongue. Associated pain-
less regional lymphadenopathy may occur 1 to 2 weeks later.
Secondary syphilis presents 4 to 10 weeks after the chancre
(primary chancre still present in 10% of patients) usually with
a rash that is symmetric and generalized on the trunk, extremi- FIGURE 4.22 ■ Primary Syphilis. This teenager showed up in the
ties, palms, and soles. Lesions vary and may be polymorphic ED because his girlfriend “made him see a doctor to get the rash
maculopapular annular, papulopustular, psoriasiform, or fol- on his penis checked out.” This totally painless ulcer with raised
licular; the lesions are often copper colored and nonpruritic. edges is typical for primary syphilis. (Photo contributor: Mark
Silverberg, MD.)
A flu-like syndrome, generalized lymphadenopathy, and sple-
nomegaly may also occur as may condylomata lata (highly
infectious raised white/gray lesions in warm moist areas like transmitted and may be seen in sexually active adolescents.
perineal and anal). Some patients have mucous patches in the Diagnosis is based on typical skin lesions and confirmed by
mouth, alopecia of beard, scalp, or eyebrows. Lesions resolve scraping moist lesions and immediately examining the sample
in 3 to 12 weeks. Tertiary (or late)-stage infection refers to with darkfield microscopy. If a darkfield microscope is not
gumma formation (skin, bone, or viscera) and cardiovascular available, a slide of the moist material can be made and sent to
involvement (aortitis). Neurologic infection can occur with any a laboratory for staining with specific T pallidum immunofluo-
stage of syphilis. Primary and secondary syphilis are sexually rescent antibody. The diagnosis of syphilis (see also page 127)

A B

FIGURE 4.23 ■ Secondary Syphilis. (A, B) Copper-colored papulosquamous nonpruritic characteristic lesions of secondary syphilis were
present on both palms and soles in a 6-year-old girl who was placed in different foster homes since birth and was sexually abused on numer-
ous occasions. (Photo contributor: Binita R. Shah, MD.)

130
PRIMARY AND SECONDARY SYPHILIS (CONTINUED) ■ 131

requires both nontreponemal (eg, RPR) and treponemal (eg, are treated with either doxycycline or tetracycline for 14 days
FTA-ABS) testing. Standard testing is usually a nontrepone- (see the Centers for Disease Control and Prevention’s [CDC’s]
mal screening test followed by a confirmatory treponemal test STD treatment guidelines, 2010). Admit younger children
if positive. Nontreponemal antibody titer correlates with dis- with suspected primary or secondary syphilis for further
ease activity and usually become nonreactive after treatment. evaluation including lumbar puncture and review of the birth
Most patients with reactive treponemal tests will remain posi- mother’s medical records to determine if the child has con-
tive for their lifetime regardless of treatment or disease activity. genital or acquired syphilis. Follow-up requires repeat non-
RPR is reactive in only about 80% of patients with primary treponemal serologic testing 6 and 12 months after treatment.
syphilis at presentation, although darkfield microscopy will be
positive. Request test with dilutions of serum in order to pre- Pearls
vent a false-negative test due to a prozone phenomenon (most
1. All cutaneous lesions of secondary syphilis are infec-
common in secondary syphilis with high titers). False-positive
tious. Relapses can occur up to 1 year after untreated
nontreponemal test results may occur due to various medical
primary infection.
conditions. All patients who have syphilis should be tested for
2. Parenteral penicillin is the preferred drug for treatment of
HIV infection. Diagnosis of primary or secondary syphilis in a
all stages of syphilis.
young child is evidence of sexual abuse and the child needs to
3. Sexual abuse must be suspected in any child with acquired
be evaluated for other STDs and treated if necessary.
syphilis.

Emergency Department
Treatment and Disposition
Treat primary and secondary syphilis with one dose of IM
benzathine penicillin (50,000 units/kg up to a maximum of
2.4 million units). Nonpregnant patients allergic to penicillin

B
FIGURE 4.24 ■ Condyloma Lata. Flat-topped round to oval plaques
(formed by papules that coalesce) with a wide base are seen around FIGURE 4.25 ■ Secondary Syphilis. (A, B) Diffuse papulosqua-
the anogenital area are seen in a 5-year-old boy who was repeatedly mous lesions with the involvement of palms and soles were seen in
sexually abused by his stepfather. Unlike condylomata acuminata, this sexually active adolescent patient. (Reproduced with permission
these lesions are flat and not covered by digitate vegetations. (Photo from Shah BR, Laude T: Atlas of Pediatric Clinical Diagnosis. WB
contributor: Binita R. Shah, MD.) Saunders, Philadelphia, 2000, p. 107.)
GONORRHEA

Clinical Summary
Gonorrhea (Gonococcal infections; GC) is one of the most
common reportable sexually transmitted diseases (STDs)
and is often asymptomatic. Incidence is highest among
adolescents. Clinical manifestations are similar to those
of Chlamydia trachomatis, and coinfection is frequent.
Susceptible sites include mucosal columnar epithelial areas
(eg, the cervical transition zone in adolescent girls), pharynx,
rectum, and conjunctiva. Infants of mothers with GC infec-
tion are at risk for ophthalmia neonatorum, scalp abscesses at
sites of fetal monitor sites, rhinitis, pneumonia, or anorectal
infections. Disseminated gonococcal infection (DGI) is more
prevalent in girls (ratio 4:1), especially those who are preg-
nant or had menses within the previous 7 days. Other risk
factors for DGI include pharyngeal gonorrhea, complement
deficiency, and immune system diseases such as systemic
lupus erythematosus. Other DGI complications include men-
ingitis, endocarditis, osteomyelitis, pneumonia, and hepatitis.

Emergency Department
Treatment and Disposition
Gonorrhea is diagnosed either by Gram stain (gram-negative
intracellular diplococci), culture (eg, Thayer-Martin cul-
ture plate, chocolate agar), and nucleic acid amplification
techniques (NAATs; sensitivities and specificities >95%).
Specimen swabs can be taken from nonsterile areas such the FIGURE 4.26 ■ Gonorrhea. Purulent discharge that grew N. gonor-
urethra, vagina, and cervix. The preferred method of testing rhoeae was seen in this sexually active adolescent girl presenting
with dysuria and vaginal discharge. (Photo contributor: Binita R.
from nongenital sites (eg, rectum, pharynx, conjunctiva) is cul-
Shah, MD.)
ture because some NAATs can cross-react with nongonococ-
cal Neisseria species in both throat and rectum. NAAT testing
can also be done on urine samples. In patients with suspected
DIG, obtain blood cultures and culture mucosal surfaces.
Treat according to current CDC STDs guidelines and with
azithromycin (1 g orally in a single dose) or doxycycline
(100 mg orally twice daily for 7 days), if coinfection with chla-
mydia is not ruled out. Treat uncomplicated GC with cephalo-
sporins (cefixime 400 mg orally in a single dose or ceftriaxone
250 mg IM in a single dose). Do not use quinolone as there are
quinolone-resistant N gonorrhoeae. Admit patients with DGI for
IV therapy. Refer to CDC guidelines for the current treatment
of complicated gonococcal infections, including extragenital
sites, DGI, and infections acquired during pregnancy. Instruct
patients to refer their sex partners for evaluation and treatment. FIGURE 4.27 ■ Gonorrhea. Purulent discharge that grew N. gon-
Follow-up referrals for counseling and patient education should orrhoeae was seen in this sexually active adolescent male. (Photo
be provided. contributor: Binita R. Shah, MD.)

132
GONORRHEA (CONTINUED) ■ 133

FIGURE 4.29 ■ Bartholin Gland Abscess; Gonococcal Infection.


A Bartholin gland abscess is a Bartholin glandular or ductal cyst
infection usually caused by polymicrobial agents (eg, Neisseria
gonorrhoeae, Chlamydia trachomatis, Escherichia coli, Proteus
mirabilis, and anaerobes including Bacteroides fragilis and
Peptostreptococcus spp.). (Photo contributor: Binita R. Shah, MD.)

FIGURE 4.28 ■ Bartholin Gland Abscess; Gonococcal Infection.


Dysuria, difficulty sitting and walking were the complaints of this
sexually active adolescent female. An extremely tender, fluctuant
mass with surrounding edema and erythema of the labia majora
was seen secondary to Bartholin gland abscess requiring incision
and drainage. The culture grew N. gonorrhoeae. Bartholin’s glands
are located bilaterally at the posterior introitus and drain through
ducts that empty into the vestibule at approximately the 4 o’clock
and 8 o’clock positions. These glands are normally pea-sized and
are neither palpable nor visible unless infected or inflamed. (Photo
FIGURE 4.30 ■ Disseminated Gonococcal Infection. Swelling of
contributor: Binita R. Shah, MD.)
the phalanges of the right thumb and hemorrhagic rash in an adoles-
cent girl. Her blood culture was positive for N. gonorrhea. (Photo
contributor: Binita R. Shah, MD.)

Pearls
1. Treatment failures most often represent reinfection.
4. Symptomatic girls present with abnormal vaginal dis-
2. Many infections are asymptomatic (thus screening is
charge, intermenstrual bleeding, menorrhagia, or dysuria.
important).
5. DGI is characterized by arthritis/arthralgia, tenosynovi-
3. Symptomatic boys present with genitourinary infection with
tis, and dermatitis (arthritis-dermatitis syndrome).
dysuria, urinary frequency, and purulent urethral discharge.
6. Long-term sequelae in women include tubal scarring,
infertility, and ectopic pregnancy.
GENITAL HERPES

Clinical Summary
Genital herpes is a chronic lifelong herpes simplex virus
(HSV-1 and HSV-2) infection that is sexually transmitted.
Approximately 70% to 95% of genital infections are caused
by HSV-2. Most patients infected with HSV-2 have not been
diagnosed with genital herpes. Many have mild or unrec-
ognized infections and intermittently shed virus into the
genital tract. Painful vesicular or ulcerative lesions may be
present and viral shedding is highest at this time; however,
lesions may not be present and viral shedding can still occur.
Recurrences are less symptomatic than primary infections.
Incidence of HSV infection increases rapidly after puberty,
reaching a peak in the third decade of life.

Emergency Department
Treatment and Disposition
Take a history and perform a physical exam with atten-
tion to previous episodes, potentially infected partners, and
presence of lesions as these may help establish the diagno-
sis. Obtain cell culture (differentiates between HSV-1 and
HSV-2) or PCR for HSV DNA, noting that sensitivity of cul- FIGURE 4.31 ■ Herpetic Vulvovaginitis. A sexually active ado-
ture declines as healing of lesions progresses. PCR assays lescent female patient presented with dysuria, urinary retention, and
are more sensitive and is the test of choice for detecting ulcerated lesions that were surrounded by erythema and edema. Note
some of the lesions have coalesced, producing large ulcerated areas
HSV in spinal fluid. Failure to detect HSV does not indicate
and bleeding. This was her first episode of HSV infection. (Photo
absence of infection, because viral shedding is intermittent. contributor: Binita R. Shah, MD.)
Type-specific HSV serology might be useful for recurrent
genital symptoms or atypical symptoms with negative HSV
cultures. Treat all patients with a first episode with systemic
antiviral drugs such as acyclovir, valacyclovir, or famciclo-
vir. These agents can partially control signs and symptoms
when used to treat the first clinical episode or recurrent epi-
sodes (when given within 1 day of onset of symptoms) or
used as daily suppressive therapy. However, these drugs do
not eradicate latent virus and, when stopped, do not affect
risk, frequency, or severity of recurrences when medication is
stopped. Admit patients with severe disease or complications
(eg, disseminated infection, pneumonitis, hepatitis, meningi-
tis, or encephalitis) for IV acyclovir therapy. Inform patients
with genital herpes that sexual transmission can occur during
asymptomatic periods. Advise patients to abstain from sexual
activity with uninfected partner(s) when lesions or prodro-
FIGURE 4.32 ■ Genital Herpes. This teenager came to the ED with
mal symptoms are present and explain the risk of neonatal these very painful sores a few weeks after having sex without a con-
HSV infection. Pregnant patients should inform health care dom. These painful eroded lesions on a red base are quite typical for
genital herpes. (Photo contributor: Mark Silverberg, MD.)

134
GENITAL HERPES (CONTINUED) ■ 135

FIGURE 4.34 ■ Herpes Simplex Neonatorum. Infection in the neo-


nate is usually acquired from maternal labial lesions. Fifty percent of
infants born to mothers with primary HSV-2 of the genitalia develop
neonatal HSV infection. Localized infection of skin presents with
grouped vesicles (seen here) or pustules with varying numbers of
lesions seen on the scalp, torso, or extremities usually between
third and sixth days of life; however, lesions may be present at birth
(ascending infection) with premature rupture of membranes or with
prolonged labor. (Photo contributor: Binita R. Shah, MD.)

providers who care for them during pregnancy, and cesar-


ean section is recommended if genital lesions or prodromal
symptoms are present during delivery.

Pearls
1. HSV is the cause of 90% of all vesiculoulcerative lesions
B of the genitalia.
2. Genital herpes is a recurrent, incurable viral disease.
FIGURE 4.33 ■ Herpetic Vulvovaginitis; Sexual Abuse. (A)
3. Genital herpes has the ability to become latent and then
Extremely painful ulcerative lesions (culture proven HSV type 2)
were seen in this girl who was repeatedly abused by her uncle in recur. Latency is lifelong but is interrupted by periods of
the past 2 weeks. HSV lesions usually appear after an incubation viral reactivation leading to silent viral shedding.
period of 2 to 20 days after exposure. Genital herpes is uncommon
in children. Most HSV-2 genital infections are sexually transmitted
in children (except for perinatal transmission at birth). (B) These
very painful sores on the vulva in this foster child prompted a sex-
ual abuse workup. Foster mother’s teenage son was found to be the
perpetrator (Photo contributors: Binita R. Shah, MD [A] and Mark
Silverberg, MD [B].)
DYSFUNCTIONAL UTERINE BLEEDING

Clinical Features Emergency Department


Dysfunctional uterine bleeding (DUB) or irregular and/ Treatment and Disposition
or prolonged vaginal bleeding occurs as a result of endo-
Obtain a through history focused on menstrual history, sex-
metrial sloughing, in the absence of structural pathology
ual history (including past STDs, pelvic inflammatory dis-
or anomaly and is usually due to anovulation. Up to 80%
ease [PID]), history of systemic illness (eg, kidney or liver
of menstrual cycles are anovulatory in the first year after
disease), endocrine history (symptoms of hypothyroidism
menarche and become ovulatory an average of 20 months
or hyperthyroidism or hyperandrogenism), use of hormonal
after menarche. Normal menstrual cycles during adoles-
contraception or exogenous hormones, family history of poly-
cence are 21 to 40 days long, with 2 to 8 days of bleed-
cystic ovarian disease, or bleeding diathesis.
ing (20–80 mL of blood loss per cycle). DUB is a common
Order a pregnancy test, CBC, STD screening if applicable,
menstrual problem during adolescence, and when severe,
thyroid function tests, coagulation profile, liver enzymes,
can result in life-threatening anemia. Menorrhagia is pro-
blood urea nitrogen, creatinine, type, and cross-match (if
longed or heavy uterine bleeding that occurs at regular inter-
transfusion is anticipated). Obtain pelvic ultrasound to evalu-
vals. Metrorrhagia is uterine bleeding that occurs at irregular
ate for possible structural anomalies in a virginal patient who
intervals. Menometrorrhagia is prolonged or heavy bleeding
cannot tolerate a pelvic examination or if a mass is palpated
that occurs at irregular intervals.
on pelvic examination or if the pregnancy test is positive.

FIGURE 4.35 ■ Extreme Pallor in Dysfunctional Uterine Bleeding.


(A, B) A 13-year-old adolescent girl (menarche at 12 years) pre-
sented with a history of bleeding for 19 days, feeling tired, weak,
pale (Hgb/Hct: 4.8/15.1), tachycardic with orthostatic changes.
Pallor of her hand is compared with an examiner’s hand. (Photo con-
A
tributor: Binita R. Shah, MD.)

136
DYSFUNCTIONAL UTERINE BLEEDING (CONTINUED) ■ 137

FIGURE 4.36 ■ Ectopic Pregnancy; Differential Diagnosis of FIGURE 4.37 ■ Incomplete Abortion; Differential Diagnosis of
Vaginal Bleeding. A transvaginal coronal image of an ectopic ges- Vaginal Bleeding. An adolescent patient came to ED with contin-
tational sac (E) adjacent to the ovary (Ov) is seen in an adolescent ued lower abdominal pain, vaginal bleeding, and passage of clots for
girl presenting with cramping abdominal pain with vaginal bleeding. >1 week. Pelvic examination showed blood in the vaginal vault with
(Photo contributor: Trushar Niak, MD.) dilated cervical os. A transvaginal image of the uterus (UT) with
complex fluid and echoes in the lower uterine segment (between the
calipers) demonstrates products of conception. She was taken to the
operating room for dilation and curettage without any further com-
plications. (Photo contributor: Steve Mcmillan, MD.)
Evaluate the patient for hemodynamic stability. Once the
bleeding is found to be uterine, the evaluation focuses on
determination of its cause. Severe bleeding with hemody-
namic instability requires immediate intervention with IV C. Patients with Hgb levels less than 10 g/dL: If the patient
fluids and/or blood transfusions. is hemodynamically stable, reliable, and able to tolerate
Treatment for DUB is based on the level of anemia. the oral contraceptive, management is as stated above.
Otherwise, hospitalization is indicated until the bleeding
A. Patients with Hgb levels of at least 12 g/dL: Reassure,
stops.
prescribe multivitamin with iron, advise patient to keep
menstrual calendar, and refer to a primary care physician.
B. Patients with Hgb levels between 10 and 12 g/dL: Begin a Pearls
35-µg combined oral contraceptive pill to be taken every
1. DUB is a diagnosis of exclusion. Pregnancy, endocri-
6 to 12 hours for 24 to 48 hours until the bleeding stops,
nopathies, blood dyscrasias, STDs, and uterine pathology
along with an antiemetic if necessary for nausea and
must be excluded.
vomiting. Advise tapering oral contraceptive to one pill
2. The most common reason for DUB during adolescence is
daily by day 5. Begin a new 28-day pill packet and inform
anovulatory cycles secondary to a physiologically imma-
the patient that a withdrawal bleed is likely when the pla-
ture hypothalamic-pituitary-gonadal axis.
cebo pills are taken. Patients usually will need combined
oral contraceptive pill for 3 to 6 months; thus refer to a
primary care physician or adolescent medicine specialist.
PELVIC INFLAMMATORY DISEASE

Clinical Summary Differential diagnosis of PID includes appendicitis, compli-


cations of pregnancy, urinary tract infection, reproductive
PID is an ascending polymicrobial genital tract infection usu-
tumors or cysts, adnexal torsion, inflammatory bowel disease,
ally found in sexually active females and includes endome-
ureteral stones, or calculi.
tritis, parametritis, pelvic peritonitis, salpingitis, oophoritis,
tubo-ovarian abscess (TOA), and perihepatitis. Inflammatory
disruption of the cervical barrier facilitates movement of Emergency Department
microorganisms from the vagina into the normally sterile
environment of the uterus. The risk of PID is highest among
Treatment and Disposition
sexually active females during adolescence (nearly 33% of Begin empiric treatment with broad-spectrum antibiotics in
all cases of PID); women <25 years of age account for 70% sexually active young women with pelvic or lower abdominal
of cases. Risk factors for girls and young women are younger pain, if no cause other than PID can be identified and one or
age, multiple sex partners, unprotected sex, younger age at more of the following minimum criteria are present on pelvic
first intercourse, or previous PID. Menstruation or recent examination: cervical motion tenderness or uterine tenderness
uterine instrumentation (eg, abortion) is believed to facili- or adnexal tenderness. Oral temperature >101°F, abnormal
tate ascension of microorganisms into the upper genital tract. cervical or vaginal mucopurulent discharge, elevated ESR or

B
A

C D

FIGURE 4.38 ■ Tubo-Ovarian Abscess. (A) Plain film of pelvis shows air within uterine cavity (black arrow) and air-fluid level in left
adnexa region (white arrow) secondary to left tubo-ovarian abscess. The abscess contained anaerobic gas-producing organisms, which
entered the uterus in a retrograde fashion. (B, C) Corresponding sonographic images show high reflective echoes (air), within the uterus (u)
and complex collection, containing highly reflective echoes (air) within the left adnexal region (LAD). (D) CT slice of pelvis demonstrates
a large left adnexal tubo-ovarian abscess. (Photo contributor: Zinn Daniel, MD.)

138
PELVIC INFLAMMATORY DISEASE (CONTINUED) ■ 139

UT

TOA

FIGURE 4.40 ■ Intrauterine Device; A Predisposing Factor For


Pelvic Inflammatory Disease. A transvaginal image of the uterus
(UT) shows a hyperechoic linear foreign body within the endometrial
stripe consistent with an intrauterine device (IUD), which increases
TOA the risk of PID. (Photo contributor: Michael Secko, MD, RDMS.)
Ov

single-dose IM ceftriaxone plus doxycycline given orally with


or without metronidazole. Some recommend that all patients
be admitted for IV antibiotics. Those with mild or moderate
clinical severity may be treated on an outpatient basis. Admit
B those in whom surgical emergencies (eg, appendicitis) cannot
be excluded, who are pregnant, or do not respond to or are
FIGURE 4.39 ■ Tubo-Ovarian Abscess. (A) A transabdominal
sonogram from a phased array probe reveals sagittal view of the unable to follow or tolerate an oral regimen and those with
uterus with a cystic adnexal mass posterior to the uterus. (B) A severe illness, nausea, vomiting, or high fever or TOA.
transvaginal sonogram reveals complex adnexal structure with an
adjacent cystic structure with internal septations. (Photo contributor:
Michael Secko, MD, RDMS.) Pearls
1. All women who have PID should also be tested for HIV
infection.
elevated CRP, laboratory documentation of cervical infection 2. Neisseria gonorrhoeae and Chlamydia trachomatis are
with N gonorrhoeae or C trachomatis also support the diag- the most common causative agents of PID, but vaginal
nosis of PID. and enteric organisms (eg, anaerobes, Escherichia coli,
Recommended treatment regimens (refer to CDC STDs genital mycoplasmas, G vaginalis, Streptococcus agalac-
guidelines) include IV regimen of cefotetan or cefoxitin plus tiae) contribute to its pathogenesis.
doxycycline. Metronidazole or clindamycin can be added 3. Complications of PID include peritonitis, perihepatitis
to this regimen with TOA because it provides more effec- (Fitz-Hugh and Curtis syndrome), TOA, and adhesions
tive anaerobic coverage. An outpatient regimen includes a leading to infertility, ectopic pregnancy, and chronic pain.
ECTOPIC PREGNANCY

Clinical Summary previous tubal infections (eg, PID), previous ectopic preg-
nancy, prior tubal surgery, tubal adhesions from previous
Ectopic pregnancy, the implantation of a fertilized egg in
appendicitis or abdominal pelvic surgery. Patients present
the fallopian tube instead of the endometrium (uterus),
with mild vaginal bleeding to massive hemorrhage. Patients
is the leading cause of maternal morbidity in the first tri-
with an acutely ruptured ectopic pregnancy present with
mester of pregnancy, and the most common risk factors are

OV
B

EG

EG

A B

FF

C D

FIGURE 4.41 ■ Ectopic Pregnancy. (A) Longitudinal image of pelvis shows ectopic gestation (EG) outside of the uterus (U). B is bladder.
(B) Longitudinal image of EG in left adnexal region, separate from left ovary (OV) and surrounded by hemorrhage (H). (C) Transvaginal
image demonstrates fetal pole in EG sac, in left adnexal region. The gestation was viable. (D) Longitudinal image of right kidney shows free
fluid (FF) in Morrison pouch. (Photo contributor: Rachelle Goldfisher, MD.)

140
ECTOPIC PREGNANCY (CONTINUED) ■ 141

about 5 weeks of pregnancy is the first significant finding on


ultrasound suggestive of an IUP; however, a definitive diag-
nosis of IUP should be deferred until a yolk sac is present.
Serial measurements of serum β-human chorionic gonado-
tropin (β-hCG) also helps; it will increase by at least 66%
every 48 hours in a normal pregnancy within the first 30 days
after implantation. If β-hCG levels are unchanged or increas-
ing more slowly than normal, the pregnancy is abnormal (it
may be an abnormal IUP or it may be an ectopic pregnancy).
Pregnancies that have lower than normal β-hCG levels are
more likely to be ectopic.
Ectopic pregnancy is a surgical emergency. Unstable
patients require aggressive resuscitation with fluid and blood
followed by surgery. Begin transfusion if clinically indicated,
FIGURE 4.42 ■ Tubo-Ovarian Abscess; Differential Diagnosis of
Ectopic Pregnancy. A noncontrast CT scan through pelvis shows a but do not delay surgery for transfusion. Emergency surgery
large, lobulated, predominantly hypodense cystic mass that is seen is necessary to stop the bleeding and try to preserve at least a
posterior and to the right of the uterus, and displacing the uterus portion of the affected fallopian tube, if it is not completely
anteriorly in an adolescent girl presenting with severe abdominal ruptured. Oophorectomy is not indicated unless the ovary is
pain. (Photo contributor: Binita R. Shah, MD.)
bleeding uncontrollably or has other pathology. If ectopic
pregnancy is probable or possible, management depends on
presence of risk factors, serial measurements of serum β-hCG
sudden onset of extreme sharp or stabbing unilateral pain levels, and results of transvaginal ultrasound over a period of
as well as shoulder pain. Other acute symptoms include diz- days. These patients should be followed in consultation with
ziness or loss of consciousness from acute intraperitoneal an obstetrician on an outpatient basis.
hemorrhage. Differential diagnosis of ectopic pregnancy
includes threatened or incomplete abortion, molar preg-
nancy, ruptured corpus luteum cyst, adnexal torsion, urinary
Pearls
tract infection, appendicitis, PID, and ureteral calculi.
1. Ectopic pregnancies must be considered in pregnant
patients with pelvic pain, particularly early in gesta-
Emergency Department tion and if associated with abnormal uterine spotting or
Treatment and Disposition bleeding.
2. Ectopic pregnancies are diagnosed by transvaginal ultra-
Obtain ultrasound to determine if there is intrauterine preg-
sound if there is no IUP.
nancy (IUP), which excludes the diagnosis of ectopic preg-
nancy in most cases. The appearance of a gestational sac at
This page intentionally left blank
CARDIOLOGY
Chapter 5

CARDIOLOGY
Shyam Sathanandam
Binita R. Shah

Ectopia cordis
Omphalocele

143
BRADYCARDIA

Clinical Summary ECG is necessary to exclude second-degree or complete


heart block (CHB), and findings include a slow heart rate with
Bradycardia is a heart rate below normal range for age with
P waves that may or may not be visible. QRS duration is nor-
many etiologies. Bradycardia of <60 beats per minute (bpm)
mal or prolonged (depending on the location of the intrinsic
associated with poor systemic perfusion is clinically signifi-
cardiac pacemaker). Dissociation of P waves and QRS is seen
cant. Signs include poor skin perfusion with pallor, cyanosis,
in CHB. P wave with a normal PR interval preceding each
cool mottled extremities, prolonged capillary refill, thready,
QRS complex is seen in sinus bradycardia.
weak, or absent peripheral pulses, and discrepancy in volume
between peripheral and central pulses. Patients are irritable, Emergency Department
lethargic, confused, or have decreased level of consciousness.
Severe bradycardia may cause respiratory difficulty, decreased
Treatment and Disposition
pulse pressure of >20 mm Hg, hypotension (decompensated Stabilize ABCs and observe and reassess patients with
shock), and decreased or no urine output. There are sev- bradycardia not associated with evidence of poor systemic
eral types of bradyarrhythmias including sinus bradycardia, perfusion and admit for continued observation. Consult
sinus node arrest with a slow junctional or ventricular escape cardiology for asymptomatic bradycardia from drug inges-
rhythm, and atrioventricular (AV) block. tion, complete AV block, acquired or congenital heart

FIGURE 5.1 ■ Sinus Bradycardia. (A) 12-lead ECG showing a heart


rate of 50 bpm. The patient is in sinus rhythm as every QRS is pre-
ceded by a P-wave, a QRS complex follows every P-wave, and the
P-wave axis is normal. This could be a normal phenomenon in well-
trained athletes. (B) An ECG from a different patient also with a slow
heart rate. Note the P-wave axis in leads II, III, and aVF. The P-waves
are inverted in these leads, suggesting this is not a sinus-rhythm. This
patient is in an ectopic atrial rhythm, which is usually normal. (Photo
B
contributor: Shyam Sathanandam, MD.)

144
BRADYCARDIA (CONTINUED) ■ 145

TABLE 5.1 ■ NORMAL HEART RATE RANGES


AND ETIOLOGIES OF BRADYCARDIA
Awake heart rate
Age in bpm (mean)
Newborn 80–180 (130)
1 week to 3 months 80–180 (140)
3 months to 2 years 80–160 (130)
2 to 10 years 65–130 (80)
10 years to adult 55–90 (75)
Etiologies of Bradycardia
Respiratory
• During CPR (intubated patient): mnemonic DOPE
Dislodged endotracheal tube (ET) (eg, esophageal or
migration to right main stem bronchus)
Obstructed ET tube (mucus plug)
Pneumothorax (inadequate ventilation)
Equipment failure (lack of oxygen leading to hypoxemia)
• Pneumomediastinum
Cardiovascular
• Excessive vagal stimulation (eg, induced by suctioning)
• Cardiomyopathy FIGURE 5.2 ■ Differential Diagnosis of Bradycardia. Subarachnoid
• Postoperative atrial cardiac surgery for CHD—check hemorrhage (SAH) Presenting with Cushing Triad. A noncontrast
• Inflammatory myocarditis head CT scan shows hyperdensity in the basal cisterns in the area of
circle of Willis, with blood in the fourth ventricle (findings typical
Other
of SAH) in an adolescent boy presenting to the emergency depart-
• Increased intracranial pressure (Cushing triad:
ment with sudden onset of severe headache, increasing lethargy,
bradycardia, hypertension, irregular respirations)
and obtundation. Patient had bradycardia, irregular respirations,
• Hypothyroidism
and hypertension (Cushing triad secondary to increased intracranial
Important contributory causes: H’s and T’s of CPR: pressure). (Photo contributor: Mark Silverberg, MD.)
Hypoxemia Tension pneumothorax
Hypovolemia Tamponade
drug of choice. Atropine usually is not effective for hypoxic-
Heart block Toxins/poisons/drugs
Hypothermia (eg, organophosphates, ischemic induced bradycardia. Vagally induced bradycardia
Head injury clonidine, beta-blockers, usually resolves once the stimulation is withdrawn. Atropine
Hyperkalemia calcium channel blockers) is very effective if used prophylactically before vagal stimula-
tion in procedures such as endotracheal intubation.
Pacing is not helpful in children with bradycardia second-
disease (CHD), or patients with refractory bradycardia that
ary to postarrest hypoxic-ischemic myocardial insult. It may
require pacing.
be helpful for bradycardia caused by acquired or CHD caus-
If cardiorespiratory compromise is present, emergent
ing CHB or sinus node dysfunction. Options include transcu-
intervention to establish a patent airway and assist breathing
taneous, transvenous, or transesophageal pacing.
with delivery of 100% oxygen. Perform chest compressions if
the patient fails to improve (patient remains unresponsive or
flaccid with poor systemic perfusion), the heart rate remains
Pearls
<80 bpm in neonates or <60 bpm in infants and children. If 1. Asymptomatic sinus bradycardia can occur as a normal
there is no response to effective oxygenation and ventila- variant during deep sleep and is also common in highly
tion, give epinephrine. A continuous epinephrine, isuprel, trained athletes.
or dopamine infusion titrated to effect may be required. For 2. Most common causes of symptomatic bradycardia are
symptomatic bradycardia due to vagal stimulation, choliner- hypoxemia and vagal stimulation, and if not corrected
gic drug toxicity, or primary AV block, give atropine as the may degenerate into full cardiac arrest.
FIRST-DEGREE ATRIOVENTRICULAR BLOCK

Clinical Summary Emergency Department


First-degree AV block is prolongation of the PR interval Treatment and Disposition
beyond the norm for age. PR interval is determined by the Consult cardiology and hospitalize patients with first-degree
point of conduction from the sinus node to the onset of QRS. heart block associated with underlying serious heart disease
The PR interval and QRS duration are age-dependent mea- (eg, acute rheumatic fever, myocarditis) for evaluation and
sures of AV conduction. Impaired conduction is classified as appropriate therapy.
first-degree, second-degree, or third-degree heart block. The
normal PR interval changes with both age and heart rate, but
it is more age dependent. The AV node is usually the site Pearl
of conduction delay, although it may occur in the atrium or 1. First-degree AV block alone does not lead to hemo-
the infranodal level. ECG findings of first-degree AV block dynamic compromise and does not need any specific
includes a regular rhythm that originates in the sinus node therapy.
with a prolonged PR interval and a normal QRS morphology,
and etiologies are mentioned in Table 5.3.

FIGURE 5.3 ■ First-Degree Heart Block. A 12-lead ECG demonstrates a heart rate of 94 bpm and the rhythm is sinus. The PR interval mea-
sures 160 ms, demonstrating first-degree AV block in a patient presenting with acute rheumatic fever (ARF). Prolongation of the PR interval
is among the minor modified Jones criteria required for the diagnosis of ARF. The PR interval is measured from the beginning of the P-wave
to the beginning of the QRS complex. (Photo contributor: Shyam Sathanandam, MD.)

146
FIRST-DEGREE ATRIOVENTRICULAR BLOCK (CONTINUED) ■ 147

FIGURE 5.4 ■ Hyperkalemia Presenting with First-Degree Heart Bock. A 12-lead ECG obtained on a 15-year old boy who presented
to the emergency department with respiratory distress and syncope. The ECG shows sinus rhythm, 92 bpm, first-degree AV block (PR =
0.21 sec), broad notched P-waves, left bundle branch block, diffuse ST abnormalities, and tall tented T-waves. His serum potassium level was
7.6 secondary to renal failure. (Photo contributor: Shyam Sathanandam, MD.)

TABLE 5.2 ■ ATRIOVENTRICULAR BLOCKS TABLE 5.3 ■ FIRST-DEGREE ATRIOVENTRICULAR


(AV) BLOCK
• First-degree AV block: Conduction of all atrial impulses
into the ventricles, but AV conduction is slower than • First-degree AV block is present if:
normal Infants: PR interval >0.14 second
• Second-degree AV block: Intermittent AV conduction Children: PR interval >0.16 second
(some atrial impulses reach the ventricles and others are Adolescents: PR interval >0.2 second
blocked)
Conditions Associated with First—Degree AV Block
• Third-degree AV block: Complete interruption of AV
conduction • Healthy persons (with normal hearts)
• Increased vagal tone (of any etiology)
• Cardiomyopathy
• Untreated congenital heart disease (eg, atrial septal
defect, ventricular septal defect)
• Acute rheumatic fever
• Therapy with antiarrhythmic agents (eg, digitalis,
beta-blockers, calcium channel blockers)
• Postoperative congenital heart disease
• Myocarditis (of any etiology)
• Hyper- or hypokalemia, hyper- or hypocalcemia,
hypomagnesemia, hypoglycemia
SECOND-DEGREE ATRIOVENTRICULAR BLOCK

Clinical Summary newly diagnosed patients, those with postcardiac surgery heart
block, and symptomatic patients (eg, syncope or dizzy spells)
Second-degree AV block is when one or more (but not all)
as they may need pacemaker implantation. Treat the underly-
of the atrial impulses fail to conduct to the ventricles because
ing cause in patients with Mobitz type I AV block. The prog-
of impaired conduction. Two distinct types of second-degree
nosis of Mobitz type II AV block associated with a wide QRS
AV blocks are type I, second-degree AV block (also known
complex may progress to complete heart block (CHB). If
as Mobitz I or Wenckebach periodicity) and type II second-
signs of hypoperfusion are present with slow ventricular rates,
degree AV block (also known as Mobitz II). Mobitz type I
give atropine emergently. Alternatively, Isoproterenol can
heart block can occur as a normal variant (without any symp-
be used with caution (eg, in patients with digoxin toxicity).
toms) in children. Causes of second-degree AV block include
Transcutaneous cardiac pacing may be required for patients
myocarditis, atrial septal defect, acute rheumatic fever,
who do not respond to atropine. A permanent transvenous
Ebstein anomaly, cardiomyopathy, structural congenital heart
cardiac pacemaker is required if symptoms of presyncope or
disease, drug toxicity (eg, digitalis, beta-blockers), increased
syncope occur.
vagal tone (any etiology), and following surgery near the AV
junction. Patients with Mobitz type II may present with syn-
cope or heart failure. Diagnosis is confirmed by ECG. A chest Pearls
radiograph may show cardiomegaly, depending on the under-
1. Mobitz type I heart block is often transient (eg, seen with
lying etiology (eg, myocarditis, cardiomyopathy).
myocarditis).
2. Mobitz type II heart block is usually permanent, and
Emergency Department implies structural damage to the infranodal conducting
system.
Treatment and Disposition
Evaluate patient and consult cardiology to determine the
underlying etiology for the second-degree heart block. Admit

FIGURE 5.5 ■ Mobitz Type I Second-Degree Heart Block or Wenckebach Periodicity. A 12-lead ECG demonstrates a progressive prolonga-
tion of the PR interval seen on the ECG on consecutive beats followed by a blocked P-wave. This is because of the decremental property of
the AV node. After the dropped QRS complex, the PR interval resets and the cycle repeats giving the impression of paired QRS complexes.
(Photo contributor: Shyam Sathanandam, MD.)

148
SECOND-DEGREE ATRIOVENTRICULAR BLOCK (CONTINUED) ■ 149

FIGURE 5.6 ■ Mobitz Type II Second-Degree Heart Block. A rhythm strip demonstrating 3:1 AV block. The atrial rate as measured by the
P-P interval is 100/min, but only every third P-wave is followed by a QRS complex. The PR interval is normal, suggesting the level of block
is not at the level of the AV node or above the AV node. This type of second-degree AV block called Mobitz type II block is more pathologic
as the site of the block is in the more distal conduction system (His-Purkinje system). (Photo contributor: Shyam Sathanandam, MD.)

TABLE 5.4 ■ SECOND-DEGREE ATRIOVENTRICULAR (AV) HEART BLOCK


Mobitz Type I AV Block (Wenckebach Block)
Characteristic features:
• The P-P interval remains constant.
• Progressive prolongation of the PR interval over several beats until one QRS complex drops (usually only a single atrial
impulse is blocked).
• After the dropped beat, AV conduction returns to normal.
• Normal QRS morphology
• Above cycle repeats itself; however, the number of beats in each cycle may not be constant.
• Some (but not all) atrial impulses fail to conduct to the ventricle.
• Atrial rate is greater than the ventricular rate.
• Site of the block is almost always at the AV node.
• Normal AV node exposed to very fast atrial rates leads to Wenckebach phenomenon.
• AV block is often due to reversible depression of AV nodal conduction.
• No hemodynamic compromise and progression to CHB is unlikely.
Mobitz Type II AV Block
Characteristic features:
• Sudden AV conduction failure
• QRS drops after a normal P wave
• One or more beats may be nonconducted at a single time.
• Absence of preceding prolongation of the PR interval in normal conducted beats (thus, PR interval remains constant
before and after the nonconducted atrial beats)
• QRS interval may be prolonged (unlike Mobitz type I block).
• Site of block is in the more distal AV conduction system (His-Purkinje system).
• Rhythm may progress to CHB.
THIRD-DEGREE ATRIOVENTRICULAR BLOCK

Clinical Summary failure, evidence of hydrops) with CHB with ventricular rates
≤50 bpm require cardiac pacing. Adrenergic agents (epineph-
Third-degree AV block, or CHB, occurs when no impulses
rine or isoproterenol) or a vagolytic agent (atropine) may be
from the atria reach the ventricles. Because the impulse is
tried to increase the heart rate while awaiting placement of
blocked, an accessory pacemaker will typically activate the
the pacemaker. Cardiac pacing (transthoracic, transcutaneous,
ventricles (an escape rhythm). Third-degree heart block is rare
or transvenous) is also required in symptomatic patients with
in the pediatric age group. Signs and symptoms in patients
CHB and CHD. Temporary pacing may be required in post-
with an otherwise normal heart vary. Patients may be brady-
operative CHB following surgery for CHD.
cardic and asymptomatic. Older children may present with
syncope (syncope from a high-degree AV block not related
to positional changes or exertion is called a Stokes-Adams Pearls
attack). Older infants may present with night terrors, irritabil-
1. Autoimmune disease accounts for 60% to 70% of all
ity, or tiredness with frequent naps. Acquired heart block is
cases of congenital CHB.
frequently symptomatic, or present with syncope, congestive
2. About 25% to 33% of cases of CHB occur in patients
heart failure (CHF), shock, or sudden death. Peripheral pulses
with associated structural heart disease.
may be prominent secondary to large compensatory stroke
3. Complete heart block may not present at birth in infants
volume. Chest radiograph may show cardiomegaly secondary
born to mothers with systemic lupus erythematosus
to increased diastolic ventricular filling.
(SLE), and may develop within the first 3 to 6 months
after birth. Unlike other manifestations of neonatal lupus
Emergency Department that resolve, CHB is permanent and patients often require
cardiac pacing.
Treatment and Disposition
4. An implantable pacemaker is used to prevent sudden
Admit symptomatic patients (eg, syncope, CHF) or those death in symptomatic patents with CHB.
newly diagnosed with CHB. Symptomatic newborns (eg, heart

FIGURE 5.7 ■ Complete Heart Block. A 12-lead ECG demonstrates a third-degree AV block with an atrial rate of 92/min and the ventricular
rate of 42/min. There is no constant relationship between the P-waves and the QRS complexes demonstrating atrioventricular dissociation.
(Photo contributor: Shyam Sathanandam, MD.)

150
THIRD-DEGREE ATRIOVENTRICULAR BLOCK (CONTINUED) ■ 151

TABLE 5.5 ■ THIRD-DEGREE ATRIOVENTRICULAR HEART BLOCK


Characteristic features:
• Failure of conduction of atrial impulses to the ventricles
• Atrioventricular (AV) dissociation (atria and ventricles beat completely independently and P waves and QRS complexes
have no constant relationship)
• Ventricles are paced by an escape pacemaker at a rate slower than the atrial rate.
• QRS duration may be prolonged or may be normal if the heartbeat is initiated high in the bundle of His (generally,
lower the location of the pacemaker within the ventricular conduction system, slower the heart rate and wider the QRS
complexes).
Some examples of congenital or acquired diseases leading to complete heart block
• Infants born to mothers with systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis, or Sjögren syndrome
(autoimmune destruction of AV tracts by maternally derived IgG antibodies)
• Complex congenital heart anomaly (eg, common AV canal)
• Postsurgical repair of congenital heart disease involving the ventricular septum
• Myocarditis
• Long QT syndrome
• Lyme disease
• Digoxin toxicity
LONG QT SYNDROME

Clinical summary oxygen and ventilation as needed. Intravenous lidocaine,


or amiodarone, or procainamide can be used. If the above
Long QT syndrome (LQTS) is a pathologically prolonged
medical therapy fails, synchronized cardioversion is used
corrected QT interval that may be acquired, but more often
for stable patients with VT with palpable pulses and defi-
is congenital and inherited. Prolongation of the QTc interval
brillation for patients with pulseless VT as per PALS
results from a prolongation of the refractory period of the
protocols.
myocardium (during which the atrium is still firing at its reg-
Consult cardiology for any patient with LQTS. ECG
ular pace), leading to torsades de pointes (“R-on-T phenom-
features of LQTS include low heart rate for age, notched
enon,” a malignant form of ventricular tachycardia [VT]).
T waves, and T-wave alternans. Stress testing (exertional
Patients present with syncope or presyncope episodes (recur-
LQTS), or 24-hour Holter monitoring (intermittent LQTS)
rent, often precipitated by exercise, fright, or being startled),
may help in arriving at the final diagnosis. Long-term goals
seizures or episodes of paroxysmal VT (often with torsades
of therapy of LQTS include beta-blockade (propranolol
de pointes morphology that can progress to ventricular fibril-
is drug of choice) to prevent VT from progressing to ven-
lation and sudden death). LQTS may be an incidental ECG
tricular fibrillation and sudden death and to blunt heart
finding. There may be a family history of sudden death in
rate response to exercise. Therapy should be continued
young relatives, syncope with exercise or emotional stress,
for life. A pacemaker may be required in some patients to
seizures, arrhythmias, and hearing impairment (congenital
overcome profound bradycardia (a common association).
LQTS). The QT interval may be so prolonged in neonates
Exercise restriction, and avoiding drugs that are known to
that it may lead to second-degree or complete heart block.
cause prolongation of the QT interval (see Syncope p. 154)
are recommended. Advise parents to learn cardiopulmonary
Emergency Department resuscitation (as exercise restriction and drug therapy may
be ineffective for some children). An implantable cardio-
Treatment and Disposition
verter-defibrillator may be considered in patients with con-
Treatment of VT associated with LQTS includes support tinued episodes of syncope in spite of therapy, and in those
of airway, breathing, and circulation (ABCs) and providing with a history of cardiac arrest.

FIGURE 5.8 ■ Prolonged QT Interval. A 12-lead ECG demonstrates sinus rhythm with a rate of 95 bpm. There is 1:1 AV conduction. The
PR interval and QRS duration are normal. The QT interval is prolonged and the QTc measures 580 ms. This patient presented with syncope
during exercise and was found to have long QT syndrome. (Photo contributor: Shyam Sathanandam, MD.)

152
LONG QT SYNDROME (CONTINUED) ■ 153

FIGURE 5.9 ■ Long QT Syndrome (LQTS)


With 2:1 Block. A 12-lead ECG obtained from a
neonate with LQTS. The QTc is 600 ms. There
is 2:1 AV block with an atrial rate of 140 and
ventricular rate of 70 bpm. LQTS associated
with heart block leads to a worse prognosis and
pacemaker insertion is indicated. (Photo contrib-
utor: Shyam Sathanandam, MD.)

Pearls 5. Patients with LQTS may develop fatal ventricular arrhyth-


mias, especially if exposed to some medications such as
1. Patients with LQTS may have a normal ECG in the emer-
antihistamines, macrolide antibiotics, or phenothiazines.
gency department (ED).
6. All family members of a patient with LQTS should
2. Congenital LQTS results in a mortality rate in excess of
undergo a 12-lead ECG (and cardiac evaluation as
90% if not diagnosed and properly treated.
indicated).
3. LQTS in association with hypertrophic cardiomyopathy
7. Any patient presenting with VT (especially torsades de
accounts for up to half of the cases of sudden cardiac
pointes or a polymorphic type) should have a corrected
death.
QT interval determined while in sinus rhythm.
4. Children with LQTS are predisposed to episodic ven-
tricular arrhythmias, torsades de pointes, syncope, and
generalized seizures.

FIGURE 5.10 ■ Long QT Syndrome: Polymor-


phic Ventricular Tachycardia (VT). A 6-lead
ECG obtained on a 12-year-old girl who pre-
sented to the emergency department with pal-
pitation on and off and syncope. The ECG
demonstrates that the child has long QT interval,
which lead to polymorphic VT while the ECG
was being recorded. She went in and out of VT
leading to symptoms. An intravenous infusion
of magnesium sulfate or a b-blocker could stop
this from progressing to full blown VT. (Photo
contributor: Shyam Sathanandam, MD.)
154 ■ LONG QT SYNDROME (CONTINUED)

FIGURE 5.11 ■ Long QT Syndrome (LQTS) with Complete Heart Block. A 12-lead ECG showing LQTS in a neonate. The QTc is 600 ms.
There is a complete heart block with an atrial rate of 144 and ventricular rate of 40 bpm. There is no constant relationship between the P-waves
and the QRS complexes. LQTS associated with heart block leads to a worse prognosis and pacemaker insertion is indicated. (Photo contribu-
tor: Shyam Sathanandam, MD.)

TABLE 5.6 ■ THE QTC INTERVAL


• Formula (Bazett equation) for calculating the corrected QT interval:
QTm
QTc =
R− R
Where QTc = corrected QT interval in seconds; QTm = measured QT interval in seconds; and R – R = interval between
previous two consecutive R waves on the ECG in seconds
• Normally the QT interval is heart-rate dependent
• The above formula corrects the measured QT interval to a heart rate of 60 bpm, at which the normal R – R interval is
1 second. Because the square root of 1 = 1, the QTc equals the QTm at a heart rate of 60 bpm, with a normal QT interval
range of 0.35 to 0.44 second.
• Prolonged QT interval: any QTc >0.44 seconds
• The QT interval normally shortens with exercise
• In patients with LQTS, the QT interval does not shorten appropriately with exercise.
Etiology of Prolonged QT Interval
Congenital (about 50% of cases):
• Two syndromes with LQTS (absence of structural heart disease):
1. Romano-Ward syndrome (autosomal dominant inheritance without deafness)
2. Jervell and Lange-Nielsen syndrome (autosomal recessive inheritance with sensorineural deafness)
Acquired:
• Heart disease (eg, myocarditis)
• Electrolyte abnormalities (eg, hypokalemia, hypocalcemia, hypomagnesemia)
• Medications
1. Tricyclic antidepressants (eg, imipramine, amitriptyline)
2. Phenothiazines (eg, thioridazine, pimozide)
3. Other psychotropic drugs (eg, haloperidol, risperidone, lithium carbonate)
4. Gastrointestinal prokinetics (eg, cisapride)
5. Antihistamines (eg, diphenhydramine)
6. Antiarrhythmics (eg, quinidine, procainamide, amiodarone)
7. Antibiotics (eg, trimethoprim, erythromycin, clarithromycin, azithromycin, sulfamethoxazole)
8. Antifungals (eg, fluconazole, ketoconazole)
9. Epinephrine
SYNCOPE

Clinical Summary Breath-holding spells are commonly seen between 1 and


5 years of age (peaks around 2 years of age) and resolves
Syncope is a sudden and brief loss of consciousness accom-
spontaneously by school age. There are two major types:
panied by a loss of postural tone with spontaneous recovery.
cyanotic type (about 80%) and pallid type (about 20%).
It result from a sudden decrease or brief cessation of cerebral
Provoking events include pain, anger, frustration, vigorous
blood flow.
crying followed by forced expiration and apnea (breath-
Vasovagal syncope (neurocardiogenic syncope, fainting
holding spell). Unconsciousness occurs due to decreased
spell) is the most common and includes emotional fainting, sit-
cardiac output. Generalized clonic jerks, opisthotonus, and
uational syncope, syncope with panic, and syncope associated
bradycardia and incontinence may occur. Normal physical
with exercise in athletes (without heart disease). Vasovagal
examination (including cardiac and neurologic) is a hallmark.
syncope is usually seen in adolescents and most often occurs
after prolonged standing (eg, crowded, warm environment),
or with noxious stimuli, strong emotions, or fatigue. Emergency Department
Orthostatic syncope occurs while standing or during
a rapid change from supine or sitting position to standing.
Treatment and Disposition
Measure orthostatic blood pressure (BP) 3 minutes after Determine the degree of hemodynamic stability including
patient stands up following a supine period of 5 minutes orthostatic BP and HR measurements. Specific treatment
(orthostatic changes present if BP changes >15 mm Hg and of syncope depends on the underlying etiology (eg, LQTS).
HR changes >10 bpm). Predisposing events include dehydra- Routine basic laboratory tests (eg, CBC, electrolytes, glu-
tion, acute blood loss, or vasodilator drugs. cose) are rarely helpful. Obtain additional tests as guided by
Cardiac syncope results from hypoxemia due to cyanotic the history and examination (eg, toxicology screen, carboxy-
heart disease or decreased cardiac output secondary to arrhyth- hemoglobin). Exclude pregnancy in adolescent girls. Obtain
mia, obstructive lesion, or myocardial dysfunction. Syncope a 12-lead ECG (with rhythm strip) in all patients (in spite of
occurs during physical exertion (eg, LQTS). Syncope occurs its low yield) because findings can lead to specific therapy
suddenly without warning. (eg, pacemaker for complete heart block, beta-blocker for

FIGURE 5.12 ■ Paced Rhythm. A 12-lead ECG demonstrates electronic pacemaker activity. The patent is being paced VVI at a rate of 100.
Any patient with a pacemaker presenting with syncope should get a 12-lead ECG and interrogation of the pacemaker to rule out pacemaker
malfunction. (Photo contributor: Shyam Sathanandam, MD.)

155
156 ■ SYNCOPE (CONTINUED)

A B

FIGURE 5.13 ■ Cardiac Tumor Presenting with Recurrent Syncope. (A) Squatting position assumed by patient while “feeling tired”. This
previously healthy 10-year old presented with recurrent syncope and weakness, increasing tiredness, and shortness of breath of 2 weeks
duration. He had hypotension with blood pressure of 87/63 mm Hg (Remember: The median systolic BP for children older than 1 year is
90 + [2 × age in years], and the lower limit is 70 + [2 × age in years]), heart rate of 90 bpm and a normal cardiac examination. Hepatomegaly,
right atrial enlargement on ECG and mild cardiomegaly on chest radiograph were also noted. (B) Right atrial mass (arrow) obstructing right
ventricular inflow tract on echocardiography. This 6.5 × 5.5 × 4 cm mass was resected and was found to be precursor B lymphoblastic lym-
phoma/leukemia (intracardiac lymphoblastic lymphoma). (Photo contributors: Barry Hahn, MD and Sudha Rao, MD.)

LQTS). Arrhythmias that lead to syncope may not be pres- Consult neurology if seizures cannot be excluded based on
ent. Consider 24-hour Holter monitoring. Consult cardiology the history or with an abnormal neurologic examination.
if cardiac syncope is likely based on history/examination or Reassure and educate patients about the benign nature of
abnormal ECG findings or syncope with chest pain, arrhyth- vasovagal syncope, identifying and avoiding precipitating
mias, or palpitations, or a family history of sudden death. factors (eg, dehydration, ensure intake of salty foods during

A B

FIGURE 5.14 ■ Cardiac Tumor Presenting with Recurrent Syncope. (A) Four-chamber view of a 3D echo in a young patient with recur-
rent syncope. There is a large tumor (arrow) in the interventricular septum that probably caused syncope secondary to obstruction of the left
ventricular outflow tract. (B) A parasternal short axis view shows the large tumor in the interventricular septum (arrow). Primary intracardiac
tumor in infants is most likely benign rhabdomyoma that usually regresses and >50% of these patients have tuberous sclerosis. (Photo con-
tributor: Shyam Sathanandam, MD.)
SYNCOPE (CONTINUED) ■ 157

TABLE 5.7 ■ ETIOLOGY OF SYNCOPE


Cardiac syncope (cardiac lesions producing syncope):
• Tetralogy of Fallot
• Long QT syndrome
• Hypertrophic cardiomyopathy
• Valvular heart disease (critical aortic stenosis, tricuspid stenosis, mitral stenosis)
• Bradydysrhythmia (sick sinus syndrome, heart block [second- and third-degree], pacemaker malfunction)
• Tachydysrhythmia (ventricular tachycardia, supraventricular tachycardia, torsades de pointes)
• Pericardial disease
• Myocardial ischemia/infarction
• Myxoma, adenoma
Noncardiac syncope:
• Autonomic dysfunction (vasovagal, orthostatic, breath-holding spell, situational [cough, micturition, defecation])
• Metabolic (eg, hypoglycemia)
• Hypoxia
• Carbon monoxide poisoning
• Neuropsychiatric syncope (hyperventilation syndrome, atonic seizures (drop attacks), hysterical syncope)

intense physical activity). Advise patients to learn to recognize Pearls


prodromal symptoms and assume a sitting or supine position
1. Syncope is a symptom, not a disease.
with elevation of the feet. For orthostatic syncope, provide
2. A thorough history and physical examination are essential
fluid therapy to patients with volume depletion. Encourage
for the evaluation of syncope and may lead to or suggest
patients to get up slowly after lying or sitting and discontinue
a diagnosis that can be evaluated with directed testing.
or reduce the dose of any medication that may be responsible.
3. Vasovagal syncope is the most common cause of faint-
Admit patients presenting with recurrent syncope of unde-
ing in children and adolescents and accounts for >50% of
termined etiology, cardiac syncope (eg, LQTS tachyarrhyth-
cases of childhood syncope.
mia, or symptoms suggestive of arrhythmias [eg, syncope
4. Dizziness, vertigo, and presyncope do not result in loss of
associated with palpitations, exertional syncope], atrioven-
consciousness or postural tone.
tricular block, valvular or congenital heart disease, pacemaker
malfunction, congestive heart failure, cyanotic spells).

TABLE 5.8 ■ DIFFERENTIAL DIAGNOSIS: SYNCOPE VERSUS SEIZURES


Syncope Seizures

Precipitants of episodes:
Pain, exercise, stressful event, micturition, Usually present Absent
defecation
Symptoms before or during episode Sweating, nausea, feeling of “passing out” Aura may be present
Disorientation after event Absent Present
Slowness in regaining consciousness Absent Present
Confusion on awakening Absent or mild Marked
Period of unconsciousness Usually seconds Minutes or longer
Unconsciousness >5 minutes Absent May be present
Rhythmic movements:
Tonic-clonic movements, myoclonic jerks Occasionally seen Commonly seen
Incontinence during episode Absent May be present
Electroencephalogram Normal May be abnormal
SINUS TACHYCARDIA

Clinical Summary Emergency Department


Sinus tachycardia (ST) is a rate of sinus node discharge Treatment and Disposition
higher than normal for the patient’s age (see Table 5.1). The Use continuous cardiac and pulse oximetry monitoring for
usual upper limit in infants and young children (up to 5 years patients presenting with ST near the range of supraventricu-
old) is up to 200 bpm. The upper limit in older children is lar tachycardia (SVT) (eg, HR approaching 200 bpm), and
up to 180 bpm. Tachycardia is a physiological response to treat the underlying cause of ST: antipyretics for fever, oxy-
the body’s need for increased cardiac output (CO) or oxy- gen therapy for hypoxia, fluid therapy for hypovolemia, pain
gen delivery (tachycardia is the first sign of hypovolemia: management. Do not attempt to decrease heart rate with phar-
CO = SV × HR; thus to maintain cardiac output, a compensa- macologic or electrical intervention. ST usually resolves once
tory increase in HR occurs, as the child’s ability to increase the stressor ceases and the heart rate returns to normal levels
stroke volume [SV] is limited). ST is a nonspecific sign of with appropriate treatment of the underlying cause.
an underlying condition rather than a true arrhythmia. On
an ECG, all P waves are normal in configuration (upright in
lead II), all QRS complexes are preceded by a P wave. The Pearls
P-, QRS-, and T-wave morphology are normal. 1. ST is the most common rhythm disturbance in children.
2. Treatment of ST involves treatment of the underlying
cause.
3. Persistent ST without an apparent inciting cause may be
indicative of occult cardiac disease (eg, myocarditis) or
an unrecognized noncardiac condition (eg, hypovolemia).

FIGURE 5.15 ■ Sinus Tachycardia (ST). A heart rate of 150 bpm was seen in a 5-year-old child with fever and several episodes of
vomiting and diarrhea. In ST, even though there may be some variation in the R–R interval, there is a constant PR interval with a nor-
mal P-QRS-T–wave sequence. The P-wave axis is normal (ie, the P waves are normal in configuration) and the QRS duration is normal
(<0.08 second). His heart rate decreased to 100 bpm after reduction of fever and rehydration. (Photo contributor: Ryan Jones, MD.)

158
SINUS TACHYCARDIA (CONTINUED) ■ 159

FIGURE 5.17 ■ Differential Diagnosis of Sinus Tachycardia.


Severe anemia. Extreme pallor is seen in this adolescent girl
FIGURE 5.16 ■ Differential Diagnosis of Sinus Tachycardia. with dysfunctional uterine bleeding presenting to the emergency
Myocarditis. A 6-month-infant presented with HR of 190 bpm, agi- department with easy fatigability, weakness, and tachycardia (HR
tation, and cold extremities with poor peripheral pulses (cardiogenic 130 bpm) with a hemoglobin value of 3.5 g/dL. (Photo contributor:
shock) following an upper respiratory tract infection. Myocarditis Binita R. Shah, MD.)
was clinically suspected. Frontal view of the chest shows very prom-
inent cardiothymic silhouette. Echocardiography showed ejection
fraction of 20%. (Photo contributor: John Amodio, MD.)

TABLE 5.9 ■ SINUS TACHYCARDIA (ST)


• Common causes of ST: Fever, crying, anxiety, exercise, pain
• Serious causes of ST:
1. Hypovolemia, hypoxia
2. Sepsis
3. Anemia
4. Toxins and drugs (eg, amphetamines, antihistamines, atropine/anticholinergics, cocaine, phencyclidine,
tricyclic antidepressants, ephedrine/pseudoephedrine, iron, organophosphates, thyroxine, carbon monoxide
and cyanide poisoning)
5. Myocarditis
6. Hyperthyroidism/thyroid storm
7. Pericardial tamponade
8. Tension pneumothorax
Differential Diagnosis: ST versus Supraventricular Tachycardia (SVT)
• Sometimes difficult to differentiate between ST and SVT
• Beat-to-beat variation is seen in ST, but not in SVT (eg, heart rate [HR] varies with activity in ST,
but not in SVT).
• Infants
1. HR > 220 bpm = probable SVT
2. HR < 200 bpm = ST
• Children
1. HR > 180 bpm = probable SVT
2. HR < 180 bpm = ST
SUPRAVENTRICULAR TACHYCARDIA

Clinical Summary tiredness. Episodes of SVT are often paroxysmal. Older chil-
dren may give a history of episodes of a racing heartbeat that
Supraventricular tachycardia (SVT) is a heart rate in infants
starts and stops suddenly. Chest pain is not a usual presenting
and young children >220 bpm (range: 220–320 bpm) and
symptom of SVT. Clinically the heart rate may be too rapid
heart rate in older children >180 bpm (range: 180–250 bpm),
to count.
most commonly caused by a reentry mechanism involving
Most first episodes of childhood SVT occur in the first
atrioventricular [AV] nodal reentry, accessory pathways,
2 months of life. About 60% of cases happen in infants
or increased automaticity. SVT due to accessory pathway
<4 months of age and 80% of cases in infants <12 months of
conduction (eg, Wolff-Parkinson-White syndrome [WPW
age. ECG is required to confirm the diagnosis. P waves may
syndrome]) is the predominant mechanism in the fetus and
not be visible (obscured by the ST segment), which helps dis-
young infant. AV nodal reentry typically appears in 5- to
tinguish it from sinus tachycardia; narrow QRS complex is
10-year-old children, and is the predominant mechanism in
seen in 90% of cases and wide QRS complex is seen in 10% of
adults. Primary atrial tachycardia (eg, atrial flutter or fibrilla-
cases (aberrant SVT). With persistent tachycardia, ST- and
tion) accounts for 10% to 15% of SVT at all ages. Associated
T-wave changes consistent with myocardial ischemia may be
structural heart disease (eg, Ebstein anomaly, corrected trans-
seen. Features of WPW syndrome may be seen once the epi-
position of the aorta) is present in about 20% of cases. Other
sode of SVT terminates, and may include a short PR interval, a
etiologies include drugs (eg, cold medications containing
delta wave (slow upstroke of QRS complex), and a wide QRS
sympathomimetics), hyperthyroidism, myocarditis, cardio-
complex. WPW syndrome sometimes closely resembles bun-
myopathy, or infections.
dle branch block and must be distinguished.
Infants usually present with nonspecific symptoms of
A chest radiograph may show the presence of cardiomeg-
poor feeding, irritability, or restlessness. If SVT persists for
aly, suggesting CHF or underlying structural heart disease.
many hours at rapid rates, signs of congestive heart failure
(eg, tachypnea, tachycardia, hepatomegaly) or cardiogenic
shock/cardiovascular collapse (eg, an acutely ill infant with Emergency Department
prolonged capillary refill, thready pulses, poor tissue perfu-
sion, ashen color, and metabolic acidosis) develop. Infants
Treatment and Disposition
may be completely asymptomatic and SVT may be detected Evaluate the hemodynamic status and stabilize as indi-
during a routine examination. Older children may present cated providing high-flow oxygen, continuous cardiac,
with pounding or racing heartbeat, dizziness, diaphoresis, or blood pressure, and pulse oximetry monitoring. Consult

FIGURE 5.18 ■ Supraventricular Tachycardia (SVT). A 12-lead ECG demonstrates a narrow complex tachycardia with a heart rate of
270 bpm suggestive of SVT. The clues to the mechanism of the SVT are suggested by retrograde P-waves following the QRS complexes that
are >50 ms apart representing orthodromic-reciprocating tachycardia. (Photo contributor: Shyam Sathanandam, MD.)

160
SUPRAVENTRICULAR TACHYCARDIA (CONTINUED) ■ 161

FIGURE 5.19 ■ Supraventricular Tachycardia with Aberrancy. A 12-lead ECG demonstrates an apparent wide complex tachycardia in a
12-year-old adolescent presenting with palpitation. Except for a heart rate of 220 bpm, other vital signs were normal. This is SVT with aber-
rancy and it may be difficult to differentiate this from ventricular tachycardia (VT). Awaiting cardiology consultation, if required treat this
rhythm as VT. (Photo contributor: Shyam Sathanandam, MD.)

FIGURE 5.20 ■ Wolff-Parkinson-White (WPW) Syndrome. (A) A classic ECG of a patient with WPW syndrome showing short PR
interval and initial slurring of the QRS complexes leading to wide QRS complex (pathognomonic for WPW syndrome). This is second-
ary to conduction via an accessory pathway. Patients with WPW syndrome are at risk for orthodromic-reciprocating tachycardia. (B) A
12-lead ECG obtained in the same patient (Figure 5.19) whose tachycardia was terminated without any complication after administration
of adenosine. The ECG demonstrates a short PR interval with initial slurring of the QRS complexes leading to wide QRS complex. (Photo
contributor: Ryan Jones, MD.)
162 ■ SUPRAVENTRICULAR TACHYCARDIA (CONTINUED)

TABLE 5.10 ■ DIFFERENTIAL DIAGNOSIS: SINUS TACHYCARDIA VERSUS SUPRAVENTRICULAR TACHYCARDIA


Sinus Tachycardia Supraventricular Tachycardia
History of volume loss (vomiting, diarrhea, blood loss), History nonspecific (eg, irritability, poor feeding,
fever, hypoxia excessive crying)
Signs of dehydration or hypovolemic shock or sepsis Signs of cardiogenic shock (tachypnea, sweating, pallor,
(depending on underlying etiology) or hypothermia)
Rate > normal for age (usually <220 bpm) Rate >220 bpm in infants
Rate >180 bpm in older children
Regular rhythm Usually regular rhythm (associated atrioventricular
block extremely rare)
Beat-to-beat variation (some variation in RR interval No beat-to-beat variation; monotonous/fixed rate
and HR decreases with sleep or when quiet)
Normal P-wave axis P-wave axis usually abnormal
P wave may not be identifiable (with very high P wave may not be identifiable (with very high
ventricular rate) ventricular rate)
Normal QRS duration Normal QRS duration in >90% of cases
Heart rate slows gradually with treatment (eg, fluids Abrupt termination to sinus rhythm (either spontaneously
for dehydration) or with treatment)
Normal P-QRS-T–wave sequence

cardiology for echocardiography in patients with a first epi- or those receiving beta-blockers. For SVT with circulatory
sode of SVT to exclude structural heart disease and to start compromise or severe CHF (unstable patient with shock,
long-term maintenance therapy (eg, digoxin, propranolol, acidosis), adenosine can be tried first if immediate vas-
procainamide, or amiodarone) as indicated with either a cular access is available. If not, perform synchronized
first episode or recurrent episodes of SVT. Possible elec-
trophysiology testing and radiofrequency catheter ablation
is needed for patients with refractory SVT, those requiring
multiple medications, or those with undesirable side effects
from medications.
ED treatment for SVT without circulatory compromise
(stable patient) involves vagal maneuvers that heighten the
vagal tone to the AV node. Application of an ice bag to the
face in infants or submersion of the face in ice cold water in
older children can be tried first (Caution: Application of ice
to infants should be brief [10 to 20 seconds max], and a cloth
or plastic barrier should be used to avoid the occurrence of
fat necrosis. Avoid repeated applications of ice to the same
location.). Other maneuvers include Valsalva maneuver (ask-
ing patient to strain as if straining at stool), unilateral carotid FIGURE 5.21 ■ Two-Hand/Two-Syringe Technique for Adminis-
massage (massage at the junction of the carotid artery and the tration of Adenosine. Because of its extremely short half-life, ade-
mandible). Ocular pressure should not be used (because of the nosine must be given as a rapid IV bolus (inject in 1 to 3 seconds to
risk of retinal detachment). maximize the concentration that reaches the heart). While maintain-
ing pressure on the plunger of the syringe containing the adenosine,
Medical therapy includes adenosine and verapamil. Do
simultaneously inject a rapid bolus of 3 to 5 mL of normal saline to
not use verapamil in infants <1 year of age (life-threatening accelerate delivery to the heart. Injection should be made close to
side effects include profound bradycardia, hypotension, car- the hub of the catheter, so that it is done closest to the patient. (Photo
diac arrest), in children with CHF, myocardial depression, contributor: Binita R. Shah, MD.)
SUPRAVENTRICULAR TACHYCARDIA (CONTINUED) ■ 163

TABLE 5.11 ■ ADENOSINE AND SUPRAVENTRICULAR TACHYCARDIA


• Drug of choice in stable patients or acutely ill patients with readily available vascular access
• Relatively safe drug that can be given to infants and children of all ages, including full-term and preterm newborn infants
• May also be used in children with Wolff-Parkinson-White syndrome or other atrioventricular (AV) bypass tracts
• After its administration:
1. Transiently depresses sinus and AV nodes, leading to slowed conduction and interruption of the reentry pathway
2. Be prepared to expect brief periods of sinus arrest (asystole).
3. Be prepared to treat cardiac effects such as bradycardia, AV block, atrial fibrillation, atrial flutter, ventricular
tachycardia, or ventricular fibrillation.
• Untoward effects are brief (ultra-short half-life [<10 seconds]):
1. Dyspnea, flushing, chest pain/discomfort, headache, episodes of apnea
2. Bronchospasm (asthma is not a contraindication; be prepared to treat bronchospasm)
• Dose and route of administration:
1. Initial dose 0.1–0.3 mg/kg (maximum first dose: 6 mg)
2. If initial dose is unsuccessful, may double and repeat dose once (maximum second dose: 12 mg)
3. Maximum single dose: 12 mg
4. Use rapid IV bolus followed by normal saline flush (two-hand technique)
5. May be given intraosseously
6. Adolescents ≥50 kg: 6 mg rapid IV push; if no response after 1–2 min, give 12 mg rapid IV push. May repeat a second
12-mg dose after 1–2 min, if required.

cardioversion to terminate SVT quickly. Consider sedation Pearls


in older children (if the patient is conscious and time and
clinical condition allow; however, sedation must not delay 1. SVT is the most common dysrhythmia seen in the pediat-
cardioversion). Reconsider the diagnosis of SVT if conver- ric age group.
sion to sinus rhythm does not occur; patient may actually 2. Aberrant SVT presents with a wide QRS complex and
have sinus tachycardia. Admit any patient presenting with may resemble VT. If uncertain, all wide-complex tachy-
hemodynamic instability to the intensive care unit (ICU). cardias are assumed to be VT.
Admit patients with a first episode of SVT (eg, for parental 3. A diagnosis of sepsis may be mistakenly made in an
and patient education, beginning maintenance therapy, espe- infant with SVT presenting with poor feeding, irritability,
cially in neonates and infants with possible recurrences of rapid breathing, or shock.
SVT) for further management. 4. Do not delay cardioversion in severely compromised
Discharge patients with previously diagnosed SVT once patients while trying to establish vascular access.
converted to sinus rhythm with a follow-up appointment with 5. Do not prescribe sympathomimetics (common in over-
the cardiologist or primary care physician. the-counter decongestants) for the treatment of upper
respiratory infections in children with SVT, and advise
patients also to avoid caffeine.
ATRIAL FLUTTER

Clinical Summary in the atrium. Atrial flutter without circulatory compromise


requires digitalization provided it is not from digitalis tox-
Atrial flutter (AF) is an atrial rate >300 bpm (range: 240–
icity. Digitalis increases the AV block and thereby slows
450 bpm) with a ventricular response with varying degrees
the ventricular rate. Propranolol may be added to digoxin.
of block (eg, 2:1, 3:1, 4:1), and normal QRS complexes. It
Amiodarone is also effective in acute treatment. Atrial flut-
is most commonly caused by an ectopic pacemaker in the
ter with circulatory compromise or severe CHF may require
atrium leading to “circus movement” (reentry mechanism)
electric cardioversion. Patients who are on digoxin cannot
within the atrium. It is most commonly seen in newborns with
undergo cardioversion. Rapid atrial pacing with a catheter in
a structurally normal heart; however, it can happen at any age
the esophagus or in the right atrium can be effective when
following heart surgery involving the atria (eg, repair of atrial
cardioversion is contraindicated. Long-term maintenance
septal defect). Atrial flutter in the newborns behaves differ-
therapy (eg, digoxin, propranolol, or amiodarone) is usually
ently than in older children. Atrial flutter is characterized by a
indicated in patients with either a first episode or recurrent
F wave with saw tooth configuration on ECG. The ventricular
episodes of AF.
rate determines eventual cardiac output; a too rapid ventricu-
lar rate may decrease cardiac output.
Pearls
1. Atrial flutter is the second most common arrhythmia in
Emergency Department
neonates after supraventricular tachycardia.
Treatment and Disposition 2. Recurrence of AF is less likely in neonates and infants
Evaluate hemodynamic status and stabilize the patient as indi- after successful conversion.
cated including high-flow oxygen, continuous cardiac, blood 3. Suspect a significant cardiac pathology (eg, myocarditis)
pressure, and pulse oximetry monitoring. Consult cardiology with AF in an older child with a structurally normal heart.
for echocardiography in patients with a first episode of AF 4. Exclude thrombus in the atrium before cardioversion to
to exclude associated structural heart disease or a thrombus prevent embolization.

FIGURE 5.22 ■ Atrial Flutter. (A) 12-lead ECG demonstrating atrial flutter with variable conduction in a 10-day-old infant who presented
to the emergency department with poor feeding and irritability. The flutter rate is more than 300.

164
ATRIAL FLUTTER (CONTINUED) ■ 165

FIGURE 5.22 ■ (Continued) (B) A dose of adenosine is very helpful to differentiate atrial flutter from supraventricular tachycardia (SVT).
Adenosine reversibly blocks the AV node; hence, there will be no AV conduction. As seen in this patient, when adenosine is given, the flutter
waves are not conducted, thus confirming the diagnosis. Adenosine only aids to confirm the diagnosis of atrial flutter but it would not termi-
nate the flutter. Adenosine could terminate SVT. (Photo contributor: Shyam Sathanandam, MD.)

FIGURE 5.23 ■ Atrial Flutter With Block. A 12-lead ECG demonstrating atrial flutter with 2:1 block. The flutter rate is 300 with a ventricu-
lar rate of 150 bpm. (Photo contributor: Shyam Sathanandam, MD.)
PREMATURE VENTRICULAR CONTRACTIONS

Clinical Summary then it is described as nonsustained ventricular tachycardia


(VT). A 24-hour Holter monitor, exercise stress testing, and
Premature ventricular contractions (PVCs), also known as
echocardiography may be considered per consultation with
ventricular premature contraction and ventricular extrasysto-
the cardiologist.
les, are premature, wide, and bizarre-shaped QRS complexes
originating from ventricular tissue. PVCs are commonly
seen in asymptomatic healthy adolescents but can be seen Emergency Department
in any age group including newborns. Patients present with Treatment and Disposition
palpitations or “skipped beats,” chest discomfort, chest pain,
Patients with “benign” PVCs do not require any treatment.
syncope, dizziness, and difficulty breathing. Sometimes
Educate the patient/family about the benign nature and about
irregular heartbeat is detected during routine examination or
avoiding stimulants (eg, excess caffeine intake, sympathomi-
as an incidental finding in ECG. Frequent PVCs in the pres-
metic agents). Cardiology consultation and hospitalization is
ence of compromised cardiac function may produce signs
necessary for “serious” PVCs, for patients presenting with
of CHF. ECG findings include widened, bizarre QRS com-
cardiac symptoms (eg, syncope, chest pain) with PVCs, and
plexes that are not preceded by P waves. P wave may show
patients with new onset of PVCs with underlying heart dis-
AV dissociation or retrograde conduction or may be absent.
ease. Treatment may include lidocaine, procainamide, pro-
The T-wave polarity usually is opposite to the major QRS
pranolol, or amiodarone.
deflection. A compensatory pause often follows a PVC. A
rhythmic pattern of PVC with a fixed ratio with normal beats
is referred to as bigeminy (1:1), trigeminy (2:1), or quadri-
Pearl
geminy (3:1). PVCs could be unifocal or multifocal. If three 1. If a PVC falls on the T wave of the preceding normal
or more PVCs occur together and lasts for 10 seconds or less complex (R-on-T phenomenon), it may initiate VT.

FIGURE 5.24 ■ Unifocal Premature Ventricular Contraction. A 12-lead ECG obtained on a 14-year-old boy presenting to the emergency
department with symptoms of “skipped beats.” ECG demonstrates benign monomorphic ventricular ectopics arising from the right ventricu-
lar outflow tract. These ectopics were suppressed completely during exercise. (Photo contributor: Shyam Sathanandam, MD.)

166
PREMATURE VENTRICULAR CONTRACTIONS (CONTINUED) ■ 167

FIGURE 5.25 ■ Multifocal Premature Ventricular Contraction. Note the multiform morphology of PVCs seen in a child with cardiomyopa-
thy. (Photo contributor: Shyam Sathanandam, MD.)

TABLE 5.12 ■ PREMATURE VENTRICULAR CONTRACTIONS (PVCs)


Etiology of PVCs
• Drugs/ingestions: Sympathomimetics, tricyclic antidepressants, digoxin, caffeine, tobacco
• Electrolyte imbalance: Hypokalemia, hypocalcaemia
• Underlying heart disease: Mitral valve prolapse, myocarditis, Lyme myocarditis, cardiomyopathy, coronary artery
disease, cardiac tumors, hemochromatosis
Differential of Benign versus serious PVCs
Benign PVCs: Serious PVCs:
• Usually asymptomatic adolescents/children Symptomatic
• Without underlying heart disease • Associated with underlying heart disease
• Unifocal (uniform morphology) • Multifocal (different morphology)
• Consistent interval from the preceding QRS • Varying intervals from the preceding QRS
• Infrequent; usually single • Pairs of PVCs or runs of PVCs (three or more PVCs [VT])
• Not associated with R-on-T phenomenon • Associated with R-on-T phenomenon
• Corrected QT interval normal • Associated with prolonged QTc interval
• PVCs suppressed by exercise • Either no effect or increased frequency after exercise
HYPERCYANOTIC SPELL OF TETRALOGY OF FALLOT

Clinical Summary shunt resulting in an oxygen saturation of 75% to 85% is seen.


Cyanosis shows minimal improvement with O2 supplementa-
Tetralogy of Fallot (TOF) is most common cyanotic heart
tion. Other findings include increased RV impulse at the
disease and consists of a spectrum of anatomic abnormali-
lower left sternal border (due to RV hypertrophy), loud single
ties (“tetrad”) that includes a large unrestrictive malaligned
heart sound (pulmonary closure sound very soft) and digital
ventricular septal defect (VSD), right ventricular (RV) out-
clubbing. Cyanotic spells in infants and children with TOF
flow tract obstruction (infundibular pulmonary stenosis [PS]),
are referred as hypercyanotic spells (also known as Tetralogy
RV hypertrophy, and overriding of the aorta. The severity
spells or “Tet spells”. Increased RV outflow tract obstruction
of PS ranges from mild to severe PS or to pulmonary atre-
exaggerates a right-to-left shunt, resulting in a decrease in
sia. Clinical presentation of TOF depends on the nature and
pulmonary blood flow and a hypercyanotic spell (nearly all of
degree of infundibular PS. An ejection systolic murmur is
the blood in the RV crosses through the VSD and enters the
usually heard at the mid-upper left sternal border (murmur
aorta). Complete blood count shows polycythemia (compen-
of PS) and may radiate toward the back. The loudness of the
satory due to chronic hypoxemia). The ECG shows RV hyper-
murmur depends on the volume of blood flowing across the
trophy with right axis deviation. Radiography in infants may
outflow tract. Varying degrees of cyanosis due to right-to-left
be normal or may show only decreased pulmonary vascular
markings. In older children a “Boot-shaped heart” is seen.

Emergency Department
Treatment and Disposition
Admit patients with hypercyanotic spells requiring more than
O2 therapy and positional interventions to abate the spell and
patients with complications related to prolonged hypoxemia
(eg, seizures). Consult cardiology for further management of
the hypercyanotic spell. Maintenance medical therapy may
be required to control recurrent hypercyanotic spells (eg, pro-
pranolol) awaiting surgical intervention.
A

FIGURE 5.26 ■ Cyanotic Congenital Heart Disease. Cyanosis


of the lips (A) and cyanosis of the nail beds (B) are seen in this FIGURE 5.27 ■ Clubbing and Cyanotic Nail Beds; Cyanotic
4-month-old infant with TOF presenting with a hypercyanotic spell Congenital Heart Disease. This 12-year-old patient with TOF had a
(lethargy and extreme cyanosis with hyperpnea and notable absence hemoglobin value of 22.5 g/dL, a hematocrit of 69.2%, an O2 satura-
of a heart murmur). These photographs were taken after stabiliza- tion of 75%, and a Po2 of 45 mm Hg on room air. (Photo contributor:
tion. (Photo contributor: Binita R. Shah, MD.) Binita R. Shah, MD.)

168
HYPERCYANOTIC SPELL OF TETRALOGY OF FALLOT (CONTINUED) ■ 169

FIGURE 5.28 ■ “Boot-Shaped Heart” in Tetralogy of Fallot;


Cyanotic Congenital Heart Disease. A frontal view of the chest in a
5-month-old infant with unrepaired tetralogy of Fallot (TOF) shows
a “boot-shaped heart” (uplifted apex due to the RV enlargement asso-
ciated with concavity of the upper left heart border due to a small or
absent main pulmonary artery segment), diminished pulmonary vas-
cular markings and a right-sided aortic arch (seen in about 25% of A
patients with TOF). (Photo contributor: Shyam Sathanandam, MD.)

FIGURE 5.30 ■ Total Anomalous Pulmonary Venous Return


(TAPVR); Cyanotic Congenital Heart Disease. (A) A frontal view of
the chest in a 4-week-old infant presenting to the emergency depart-
ment (ED) with tachypnea shows a “snowman” or a “figure of 8”
appearance that is characteristic for supracardiac type of TAPVR.
This appearance is secondary to the anomalous pulmonary veins
draining into a dilated innominate vein. (B) A frontal view of the
FIGURE 5.29 ■ “Egg on String Appearance” in Transposition of
chest in a 6-day-old neonate presenting to the ED with severe cya-
Great Arteries; Cyanotic Congenital Heart Disease. A frontal view of
nosis, lethargy, and poor feeding shows a ground-glass appearance
the chest in a 1-week-old baby presenting to the emergency depart-
secondary to obstructed pulmonary veins and a right pleural effu-
ment with severe cyanosis shows an “egg on string appearance” with
sion. Echocardiogram demonstrated an infracardiac type of TAPVR.
increased vascularity suggesting a diagnosis of D-transposition of
(Photo contributor: Shyam Sathanandam, MD.)
great arteries. This appearance is secondary to a narrow mediasti-
num, which is secondary to superimposition of the great vessels.
(Photo contributor: Shyam Sathanandam, MD.)
170 ■ HYPERCYANOTIC SPELL OF TETRALOGY OF FALLOT (CONTINUED)

FIGURE 5.31 ■ Tricuspid Atresia; Cyanotic Congenital Heart Disease. Twelve-lead ECG obtained on a 3-day-old neonate presenting to
the ED with respiratory distress and mild cyanosis shows a left axis deviation that is virtually pathognomonic for tricuspid atresia. (Photo
contributor: Shyam Sathanandam, MD.)

Pearls TABLE 5.13 ■ CYANOTIC CONGENITAL HEART


1. Two hallmarks of TOF are a PS murmur and cyanosis. DISEASE
2. Hypercyanotic spells of TOF are very dramatic in their 5 T’s:
presentation. Failure to recognize without immediate • Tetralogy of Fallot (most common cause of cyanotic
intervention may result in hypoxic seizures, profound heart disease)
metabolic acidosis, cerebrovascular accident (due to • Truncus arteriosus
polycythemia/hyperviscosity), deterioration in cardiac • Transposition of the great arteries
rhythm, and death. • Tricuspid atresia
• Total anomalous pulmonary venous connection
3. Increasing cyanosis in a child with a history of heart dis-
ease or history of squatting with exertion is an important
clue indicating a potential hypercyanotic spell.
4. A notable absence or lessening of a previously heard
heart murmur in a cyanotic child is the most important
clue suggesting a hypercyanotic spell.
HYPERCYANOTIC SPELL OF TETRALOGY OF FALLOT (CONTINUED) ■ 171

TABLE 5.14 ■ HYPERCYANOTIC SPELL OF TETRALOGY OF FALLOT


Presenting features:
• Spells usually occur in the morning shortly after awakening.
• Spells are self-limited; and usually last <15 to 30 minutes.
• Spells may occur spontaneously or be precipitated by stress, dehydration, injury, sudden fright, or anything that
makes the infant cry.
• Profound cyanosis (or history of increasing cyanosis) in a child with history of heart disease
• History of squatting or knee-chest position with exertion
During the spell, the infant or child may present with any of the following:
1. Irritability/crying/agitation (signs of hypoxemia)
2. Lethargy or unconsciousness or syncope (recurrent or prolonged)
3. Profound cyanosis
4. Seizures, cerebrovascular accidents (due to polycythemia)
5. Notable absence or decrease in intensity of a previously heard heart murmur (due to decreased antegrade flow
into the pulmonary arteries)
6. Increased rate and depth of respiration (hyperpnea, an important feature)
Management (intervene in the following order, as indicated):
Standard initial therapeutic interventions that result in prompt abatement of the spell:
• Give 100% O2 via a nonrebreathing facemask.
• Try to calm the child.
• Have the child assume the knee-chest position (knees flexed and drawn up close to the child’s chest and pressing
on the abdomen); this increases SVR in the lower extremities, promoting increased venous return to the heart and
increased pulmonary blood flow.
• Morphine sulfate given IM, SC, or IV. IM Ketamine is helpful to calm the child and raise systemic vascular resistance
(SVR), and is currently preferred over morphine.
• Maintenance rate of IV fluids or correct hypovolemia if present
• Correct hypoglycemia if present (hypoglycemia may contribute to a spell).
Additional interventions if above measures fail:
• Sodium bicarbonate (corrects metabolic acidosis seen after prolonged hypoxemia)
• Propranolol (decreases dynamic contraction of the infundibulum)
• Phenylephrine (increases SVR)
• Endotracheal intubation, mechanical ventilation, neuromuscular blockade to reduce O2 consumption due to
increased work of breathing
• Important: Do not use epinephrine or norepinephrine to abate the spell.
DUCTAL-DEPENDENT CARDIAC
LESIONS/HYPOPLASTIC LEFT HEART SYNDROME

Clinical Summary dependent on patency of the ductus arteriosus) include


pulmonary atresia with intact ventricular septum, tricus-
Hypoplastic left heart syndrome (HLHS) refers to a spec-
pid atresia and critical pulmonary stenosis; HLHS, a rep-
trum of CHDs that have very small LV and other associated
resentative of ductal-dependent lesions, is one of the most
anatomic abnormalities. There is inadequate anterograde
serious cardiac anomalies presenting in infancy. A neonate
flow to support the systemic circulation because of the
born with HLHS appears normal with normal Apgar scores
hypoplasia of the left heart. Because systemic circula-
because systemic perfusion and oxygenation are normal in
tion is supported by the right side of the heart through the
utero. Typically, on day 2 or 3 of life, signs of poor sys-
ductus arteriosus, these lesions are referred to as ductal-
temic perfusion occur as the ductus arteriosus closes. All
dependent lesions. Examples of ductal-dependent systemic
patients with HLHS have a single, loud second heart sound
blood flow lesions include HLHS, critical coarctation of
and variable heart murmurs that are usually not diagnostic.
the aorta and aortic arch interruption. Examples of right-
The ECG may show right atrial enlargement (∼30%–40%),
sided ductal-dependent lesions (pulmonary circulation
right ventricular hypertrophy (∼80%–90%) and diminished
left ventricular forces (∼30%–40%). Radiograph typically
shows marked cardiomegaly with normal to increased pul-
monary markings.

Left ventricle

FIGURE 5.32 ■ Cardiomegaly: Critical Coarctation of the Aorta


(Left-sided Ductal-Dependent Cardiac Lesion). A frontal view of
the chest shows marked cardiomegaly and increased vascularity in a
21-day-old neonate presenting with cyanosis, severe respiratory dis-
tress, tachycardia, wheezing, and shock with marked acidosis (arte-
rial pH 6.86, Pco2 47 mm Hg, Po2 35.7 mm Hg, and base deficit of FIGURE 5.33 ■ Hypoplastic Left Heart Syndrome. An echocar-
−22.4). His wheezing and respiratory distress initially were thought diographic image of a 3-day-old baby presenting to the ED in shock
to be due to bronchiolitis but with findings on the chest radiograph demonstrates a hypoplastic left ventricle (arrow) and restrictive duc-
a possibility of a ductal-dependent cardiac lesion with cardiogenic tus arteriosus. This patient’s hypoplastic left heart syndrome was
shock was entertained and PGE1 infusion was begun. A diagnosis undiagnosed at birth. Once the PDA started to constrict, baby devel-
of critical coarctation of the aorta was confirmed by ECHO. (Photo oped shock secondary to poor cardiac output. (Photo contributor:
contributor: Binita R. Shah, MD.) Shyam Sathanandam, MD.)

172
DUCTAL-DEPENDENT CARDIAC LESIONS/HYPOPLASTIC LEFT HEART SYNDROME (CONTINUED) ■ 173

two-dimensional echocardiography and subsequent referral


for surgical management.

Pearls
1. A ductal-dependent cardiac lesion must be considered
in any neonate (up to 28 days old) with sudden onset of
shock, severe hypoxemia, acidosis, or intense cyanosis
(systemic circulatory collapse).
2. Neonates presenting with shock should be treated as
having ductal-dependent lesions until proved otherwise;
PGE1 infusion, by maintaining patency of the ductus arte-
riosus, is life-saving in such infants.

FIGURE 5.34 ■ Ebstein Anomaly; Right-sided Duct-Dependent TABLE 5.15 ■ DUCTAL-DEPENDENT CARDIAC
Lesion. A frontal view of the chest in a 3-day-old baby present- LESIONS/HYPOPLASTIC LEFT HEART SYNDROME
ing to the ED with severe cyanosis shows impressive cardiomegaly
with decreased vascularity (difficult to assess here due to massive
Characteristic features:
cardiomegaly). This degree of cardiomegaly is seen in very few
• Typical age at presentation: usually within the first few
conditions; Ebstein’s anomaly is associated with some of the larg-
days of life (when the ductus closes)
est hearts seen by the chest radiographs. (Photo contributor: Shyam
(neonates may present as late as 1 or 2 weeks of life and
Sathanandam, MD.)
sometimes even older)
• Signs of shock
1. Skin cyanotic or mottled, cool extremities, prolonged
capillary refill
2. Peripheral pulses rapid, thready, weak, or absent
Emergency Department 3. Discrepancy in volume between peripheral and cen-
tral pulses
Treatment and Disposition 4. Irritability, lethargy, or unresponsiveness
5. Respiratory distress (compensation for severe meta-
All ductal-dependent cardiac lesions present in a similar fash-
bolic acidosis and increased pulmonary blood flow)
ion (like HLHS), and the initial medical management is iden- 6. Hypotension (decompensated shock)
tical in all. Support the ABCs and provide continuous cardiac 7. Metabolic acidosis due to poor tissue perfusion
and pulse oximetry. Maintain ductal patency with an infusion 8. Decreased or no urine output
of prostaglandin E1 (PGE1). Treat metabolic acidosis by fluid • Multisystem organ failure (eg, acute renal failure,
therapy and sodium bicarbonate (acidosis leads to increased seizures)
systemic vascular resistance, and in turn increases pulmonary Treatment of Ductal-Dependent Cardiac Lesions:
blood flow). Avoid any maneuvers that decrease pulmonary PGE1 infusion
vascular resistance and pulmonary pressure as these will • Vasodilator
steal blood flow from the systemic circulation (eg, supple- • Maintains or reopens the ductus arteriosus
mental O2 therapy [O2 acts as pulmonary vasodilator] and • Contraindications: almost none except total anomalous
pulmonary venous connection
hyperventilation [low PCO2 acts as pulmonary vasodilator]).
• Given as a continuous infusion (very short half-life)
Aim at the “ideal arterial blood gas with 7.4/40/40” (pH 7.4, • Side effects: vasodilator effects (flushing and periph-
PO2 40 mm Hg, PCO2 40 mm Hg). This is usually achieved eral edema), hyperpyrexia, jitteriness or convulsions,
with low (or no) mechanical ventilatory support on room metabolic (hypoglycemia, hypocalcemia), renal failure,
air. After initial stabilization, all patients require admission coagulopathies
to the pediatric ICU for subsequent management. Consult Life-threatening side effects: apnea, bradycardia, and
hypotension
cardiology (after stabilization) to confirm the diagnosis by
INFECTIVE ENDOCARDITIS

Clinical Summary C-reactive protein (CRP), and urinalysis (microscopic hema-


turia, proteinuria). Antibiotic therapy (2–6 weeks in duration)
Infective endocarditis (IE), may be classified as acute or
should be guided by the identification of the pathogen and
subacute or as native valve endocarditis, prosthetic valve
sensitivity and it is best not to administer antibiotics in the
endocarditis, or endocarditis in intravenous drug abusers.
ED while awaiting culture results. Duration of therapy varies
Gram-positive cocci account for 90% of culture-positive
from 2 to 6 weeks.
endocarditis; about 5% to 7% of cases are culture-negative.
Etiologic agents include Streptococcus viridans (most com-
mon in all age groups), Staphylococcus aureus (second most Pearls
common) followed by coagulase-negative S epidermidis,
1. Suspect endocarditis in any patient with a heart murmur
Enterococcus species, Streptococcus pneumoniae, HACEK
and persistent unexplained high fever, especially with
group (Haemophilus species, Actinobacillus actinomy-
underlying CHD or condition that increases the risk of
cetemcomitans, Cardiobacterium hominis, Eikenella cor-
bacteremia, or in any febrile IV drug abuser (even in the
rodens, and Kingella kingae) and Fungi (Candida species,
absence of a heart murmur).
Aspergillus species). Endocarditis in childhood is seen with
2. Patients with CHD presenting to the ED with evidence of
preexisting CHD and includes patients with complex cya-
soft tissue infections (eg, abscess) must receive appropri-
notic congenital heart disease (eg, tetralogy of Fallot), post–
ate antibiotic coverage prior to débridement or incision
cardiac surgery (eg, palliative shunt procedures) and patients
and drainage.
with a previous history of endocarditis. Endocarditis can also
occur without underlying CHD; predisposing factors include
infected indwelling central venous catheters. Intravenous
drug abuse is not a common predisposing factor in younger
children. Bacteremia may occur during severe burns, any
bacterial infection (eg, cellulitis, pneumonia, urinary tract
infection), or following any dental or surgical procedures, any
instrumentation of mucosal surfaces, or spontaneously fol-
lowing activities such as brushing teeth. Clinical features of
IE are mentioned in Table 5.16. The Duke criteria (refer to
American Heart Association website) are a combination of
major and minor criteria that help in arriving at the diagnosis
of IE. Differential diagnosis of IE includes malignancy, colla-
gen vascular diseases, and other infections affecting the heart Vegetation
(eg, acute rheumatic fever).

Emergency Department
Treatment and Disposition
Admit all patients with suspected IE for confirmation of the
diagnosis, IV antibiotic therapy, and continuous cardiac mon- FIGURE 5.35 ■ Vegetation on Prosthetic Mitral Valve. An echo-
itoring and critical care in those who are acutely ill. Blood cardiographic image showing a vegetation on the prosthetic mitral
cultures is the gold standard for diagnosis and is positive valve in a 9-year-old boy presenting to the emergency department
in shock. He had a blowing systolic murmur at the apex radiat-
in >95% of cases. Take at least three sets of blood cultures
ing to the axilla, splenomegaly, and signs of severe vasodilation.
from separate venipuncture sites with as large a quantity of With a prosthetic mitral valve replacement recently, an infective
blood as possible. Obtain CBC (normocytic normochromic endocarditis was clinically suspected. (Photo contributor: Shyam
anemia, leukocytosis), erythrocyte sedimentation rate (ESR), Sathanandam, MD.)

174
INFECTIVE ENDOCARDITIS (CONTINUED) ■ 175

FIGURE 5.36 ■ Osler Nodes. Subcutaneous, purplish, tender nod- FIGURE 5.37 ■ Splinter Hemorrhages. Note splinter hemorrhages
ules in the pulp of fingers known as Osler nodes. (Photo contributor: along the distal aspect of the nail plate due to emboli from subacute
the Armed Forces Institute of Pathology, Bethesda, MD.) bacterial endocarditis. (Courtesy of the Armed Forces Institute of
Pathology, Bethesda, Maryland.)

TABLE 5.16 ■ CLINICAL FEATURES OF INFECTIVE ENDOCARDITIS IN CHILDREN


• Fever (most common symptom), prolonged low-grade fever, rigors, diaphoresis
• Fatigue, nausea, vomiting, abdominal pain, anorexia, weight loss, malaise
• Splenomegaly (less common than in adults)
• Arthralgias or arthritis
• Chest pain
• Congestive heart failure (valve destruction, regurgitation)
• Valvulitis (new or changing murmurs)
• Cardiac arrhythmias
• Roth spots: small, pale, retinal lesions with areas of hemorrhage near the optic disc
• Subungual splinter hemorrhages
• Embolic events (CNS, kidneys, spleen, skin, lungs)
• Cerebral infarction signs/symptoms: seizures, changing mental status, ataxia, aphasia, acute hemiplegia, focal
neurologic deficits
Dermatological Manifestations
• Skin lesions occur in 15% to 50% of cases
• Types of skin lesions: petechiae, Osler nodes, and Janeway lesions
A. Petechiae
1. Most common skin manifestation, occur in small crops, usually transient
2. Sites: mucous membranes of the mouth, conjunctiva, upper part of the chest, extremities
B. Osler nodes
1. Pea-sized, erythematous indurated nodules with pale centers, few in number
2. Tender (pain may be elicited by palpating the tips of the digits)
3. Transient; resolves without necrosis or suppuration
4. Sites: pads of fingers and toes (most common), thenar and hypothenar eminences, sides of the fingers, arms
C. Janeway lesions (peripheral embolization)
1. Painless; small erythematous macules or small nodular hemorrhages
2. Site: palms or soles
ACUTE PERICARDITIS

Clinical Summary (eg, rheumatoid arthritis), and uremia (uremic pericarditis).


Pericardial effusion may be sero-fibrinous, hemorrhagic, or
Acute pericarditis, an acute inflammation of the parietal and
purulent. Effusion may be completely absorbed or may result
visceral surfaces of the pericardium, may be infectious or
in pericardial thickening or chronic constriction (constric-
noninfectious and can be associated with pericardial effusion.
tive pericarditis). A rapid accumulation of a large amount
Viral infections are the most common etiology, particularly
of fluid produces cardiac tamponade that is classically asso-
in infancy (eg, Coxsackie B virus, Adenovirus, enteroviruses,
ciated with a triad of low arterial blood pressure, jugular
echoviruses, cytomegalovirus, HIV, influenza virus). Other
venous distention, and distant, muffled heart sounds (Beck
etiologies include but are not limited to acute rheumatic
triad). Narrowed pulse pressure and pulsus paradoxus may be
fever, purulent pericarditis (eg, S aureus, S pneumoniae,
observed. Cardiac tamponade occurs more commonly with
Haemophilus influenzae, Neisseria meningitides, and strep-
purulent pericarditis.
tococci), tuberculosis (constrictive pericarditis), heart surgery
History may reveal preceding upper respiratory tract infec-
(postpericardiotomy syndrome), collagen vascular diseases
tion. Fever and chest pain may be present. Chest pain may

FIGURE 5.38 ■ Acute Pericarditis. A 12-lead ECG obtained in a 4-year-old girl presenting to the emergency department with chest dis-
comfort and difficulty breathing shows diffuse ST-T wave abnormalities in all limb and chest leads. This is a classic ECG finding of acute
pericarditis. (Photo contributor: Nancy Chase, MD.)

176
ACUTE PERICARDITIS (CONTINUED) ■ 177

Pericardial
effusion

FIGURE 5.39 ■ Pericardial Effusion; Acute Pericarditis. A


2-dimensional echocardiography in the same patient demonstrates
clear serous fluid in the pericardial space, suggesting pericardial
effusion (arrow). (Photo contributor: Shyam Sathanandam, MD.)

be relieved by leaning forward and may be made worse by FIGURE 5.40 ■ Acute Pericarditis. Seventeen-month-old girl pre-
supine position or deep inspiration. Pericardial friction rub sented to the emergency department with difficulty breathing that
(a grating to-and-fro sound in phase with the heart sounds) has progressed over the last 3 days. She had a recent viral upper
is the cardinal physical sign. Heart murmur is usually absent. respiratory tract illness. Chest x-ray reveals cardiomegaly and pul-
monary edema. This could be viral pericarditis with pericardial
Presence of a cardiac murmur should alert the consideration
effusion or viral myocarditis. Sometimes it is impossible to differ-
of acute rheumatic fever or endocarditis. entiate the two without getting a biopsy. (Photo contributor: Shyam
Sathanandam, MD.)

Emergency Department
Treatment and Disposition Corticosteroid therapy may be indicated in severe rheumatic
Obtain ECG, chest radiograph, and cardiology consult. ECG carditis or postpericardiotomy syndrome. For viral pericardi-
reveals low-voltage QRS complex (caused by pericardial tis there is no specific treatment; pericardiocentesis or surgi-
effusion) and diffuse ST-segment elevation in all precordial cal drainage is mandatory to identify the cause of pericarditis,
and limb leads. Chest radiograph will show varying degrees especially when purulent or tuberculous pericarditis is sus-
of cardiomegaly and increased pulmonary vascular markings pected. A drainage catheter may be left in place.
(with cardiac tamponade). Echocardiography is most useful
to diagnose pericardial effusion.
Stabilize patients with hemodynamic compromise. For
Pearls
cardiac tamponade, perform urgent decompression by surgi- 1. Viral infection is the most common cause of acute peri-
cal drainage or pericardiocentesis. Give fluid boluses with carditis. A history of upper respiratory tract infection
Plasmanate to increase central venous pressure and improve along with a symptom of chest pain that is relieved on
cardiac filling, which can provide temporary emergency sta- leaning forward is helpful in making a diagnosis.
bilization. Admit patients with hemodynamic compromise 2. Most serious complication of pericarditis is pericardial
or expected to have progressive disease to the ICU. Give effusion and cardiac tamponade, the presence of which is
salicylates for precordial pain and rheumatic pericarditis. an indication for urgent pericardiocentesis.
ACUTE MYOCARDITIS

Clinical Summary rate is as high as 75% in symptomatic neonates with acute


viral myocarditis although the majority of patients with mild
Acute myocarditis is caused by acute inflammation of the
disease recover completely. Some patients develop subacute
myocardium and may be infectious or noninfectious in origin.
or chronic myocarditis with persistent cardiomegaly and con-
Viral infection is the most common cause, and pathogens
gestive heart failure.
include Coxsackie virus B, Adenovirus, enteroviruses, echo-
viruses, cytomegalovirus, HIV, and influenza. Rarely, bacte-
ria, rickettsia, fungi, protozoa, and parasites are the causative Emergency Department
agents. Other etiologies include immune-mediated diseases
(eg, acute rheumatic fever, Kawasaki disease, collagen vascu-
Treatment and Disposition
lar diseases) and toxic myocarditis (eg, drug ingestion, diph- Obtain ECG, chest radiography, and consult cardiology.
theria exotoxin). Cardiomegaly of varying degrees is seen on chest x-ray.
Older children may have a history of preceding upper ECG reveals low-voltage QRS complex, ST-T changes, QT
respiratory tract or gastrointestinal infection. The illness prolongation, and arrhythmias, especially premature ven-
may have a sudden onset in newborns and small infants with tricular contractions. Echocardiography reveals cardiac
anorexia, vomiting, lethargy, respiratory distress, and circula- chamber enlargement and impaired left ventricle function.
tory shock. Arrhythmias including supraventricular tachycar- Occasionally, a thrombus in the left ventricle can be found.
dia or ventricular ectopic beats may be audible. The mortality Cardiac troponin levels and MB isoenzyme of creatine kinase
may be elevated. Endomyocardial biopsy is the best confir-
matory test though not practical in every situation.
Admit hemodynamically unstable patients and those with
aggressive arrhythmias to ICU. Attempt virus identification
by viral cultures from the blood, stool, or throat washing.
Bed rest and limitation of activity is recommended during the
acute phase. Treat arrhythmias aggressively. When the clini-
cal diagnosis of myocarditis is suspected, it is now recom-
mended to treat with high-dose IV γ-globulin in the hospital.

Pearls
1. Myocarditis severe enough to be recognized clinically is
rare; mild and subclinical cases are more prevalent.
2. Maintain a high index of suspicion as there is a high mor-
tality rate, especially in childhood.
FIGURE 5.41 ■ Acute Myocarditis. Frontal view of the chest shows
3. Acute myocarditis results in aggressive arrhythmias that
marked cardiomegaly with pulmonary edema in a 4-year-old in
severe respiratory distress with cyanosis and stupor requiring intuba-
may be hard to treat.
tion. Severe metabolic and respiratory acidosis, poor cardiac func-
tion noted on Echo and a rapid progression from sinus tachycardia
to ventricular tachycardia were seen (see Figure 5.46). Suspect acute
fulminant myocarditis in such scenario. (Photo contributor: Shyam
Sathanandam, MD.)

178
ACUTE MYOCARDITIS (CONTINUED) ■ 179

A B

C D

FIGURE 5.42 ■ ECG Changes; Acute Myocarditis. (A–D) Progression of 12-Lead ECG obtained from the same patient (Figure 5.41) over
a 15-minute period. (A) ECG showing sinus tachycardia at a rate of 150 bpm and diffuse ST-T wave abnormalities. There is hyper-acute ST
segment elevation suggesting myocardial injury or ischemia. (B) ECG obtained 5 minutes later demonstrates sinus tachycardia with ST eleva-
tion and ventricular bigeminy. (C) ECG obtained 5 minutes later shows wide complex tachycardia, possibly an accelerated idioventricular
rhythm with ST elevation. (D) ECG showing ventricular tachycardia (after about 15 minutes from the initial presentation) associated with loss
of consciousness. Even though the rate of the VT is slow, it is nevertheless an ominous sign. Patients with acute fulminant myocarditis can
progress rapidly and the condition can be fatal if not appropriately managed. (Photo contributor: Shyam Sathanandam, MD.)
ACUTE RHEUMATIC FEVER

Clinical Summary proximal portions of the extremities but never on the face.
Subcutaneous nodules are hard, painless, nonpruritic, freely
Acute rheumatic fever (ARF) is an immunogenic multisys-
mobile swellings of 0.2 to 2 cm in diameter found symmetri-
tem disease that occurs as a delayed sequel of group A strep-
cally, singly or in clusters, on the extensor surfaces of both
tococcal infection of the pharynx but not the skin. Important
large and small joints, over the scalp, or along the spine.
predisposing factors include family history of ARF, low
Sydenham chorea (St. Vitus dance) is more common in
socioeconomic status, and age between 6 and 15 years. ARF
prepubertal girls and can be the only manifestation of ARF.
is diagnosed by the use of revised Jones criteria. History of
It is a neuropsychiatric disorder consisting of both neurologic
streptococcal pharyngitis is commonly present 1 to 5 weeks
signs (choreic movement and hypotonia) and psychiatric
before the onset of symptoms or may be as long as 2 to
signs (emotional lability, hyperactivity, separation anxiety,
6 months in case of isolated chorea.
obsessions, and compulsions).
Arthritis is the most common manifestation usually involv-
Laboratory evidence of elevated ESR and CRP are objec-
ing large joints either simultaneously or in succession with a
tive evidence of an inflammatory process. Positive throat
characteristic migratory nature. Signs of carditis include (in
culture or rapid streptococcal antigen tests are less reliable
increasing order of severity) tachycardia out of proportion
than antibody tests. Antibody tests include ASO titer, anti-
to the degree of fever, a heart murmur of valvulitis caused
DNAase B, and the Streptozyme test. Nonspecific symptoms
by mitral or aortic valve regurgitation, pericarditis (friction
include abdominal pain, rapid sleeping heart rate, malaise,
rub, pericardial effusion, ECG changes), or signs of conges-
anemia, epistaxis, and precordial pain. The differential
tive heart failure (severe carditis). Erythema marginatum
includes juvenile rheumatoid arthritis, systemic lupus erythe-
is characterized by nonpruritic serpiginous or annular ery-
matosus, mixed connective tissue disorder, reactive arthritis,
thematous rashes most prominent on the trunk and the inner

TABLE 5.17 ■ GUIDELINES FOR DIAGNOSIS OF


RHEUMATIC FEVER
Five Major Manifestation
• Migratory polyarthritis (70%)
• Carditis (50%)
• Sydenham chorea (15%)
• Erythema marginatum (<10%)
• Subcutaneous nodules (10%)
Four Minor Manifestation
Clinical findings:
• Arthralgia
• Fever
Laboratory findings:
• Elevated acute phase reactants (ESR, CRP)
• Prolonged PR interval
Supporting Evidence of Antecedent
FIGURE 5.43 ■ Rheumatic Carditis. A portable chest radiograph
Group A Streptococcal Infection
shows severe cardiomegaly and pulmonary edema, suggesting a
• Positive throat culture or rapid streptococcal antigen test
diagnosis of CHF in a 16-year-old boy, a recent emigrant from Haiti,
• Elevated or rising streptococcal antibody titer
presenting with dyspnea, mild cyanosis, tachycardia, and bilateral
rales. He had arthritis of the knees. A pansystolic murmur radiating If supported by evidence of preceding group A streptococ-
to the axilla was noted. Additional history included fever and a recent cal infection, the presence of two major manifestations
sore throat. ECHO showed severe mitral regurgitation, pericardial or of one major and two minor manifestations indicates
effusion, and poor cardiac function, suggesting the involvement of a high probability of ARF
all layers of the heart. A clinical diagnosis of ARF was made. (Photo
Abbreviations: ARF, Acute rheumatic fever; CRP, C-reactive protein; ESR,
contributor: Shyam Sathanandam, MD.)
erythrocyte sedimentation rate.

180
ACUTE RHEUMATIC FEVER (CONTINUED) ■ 181

A B

FIGURE 5.44 ■ Erythema Marginatum; Acute Rheumatic Fever. (A) Annular plaques (complete rings) of varying sizes with flat, pale cen-
ters and fairly distinct, raised erythematous margins are seen in an adolescent patient who also had migratory polyarthritis involving knees
and elbows. (B) Note the serpiginous borders formed by a coalescence of several partial rings. (Reproduced with permission from: Shah BR,
Laude TL: Atlas of Pediatric Clinical Diagnosis. WB Saunders, Philadelphia, 2000, p. 418.)

serum sickness, infectious arthritis, and hematologic disor- the severity of carditis. Antibiotics to eradicate streptococci
ders such as leukemia. Only carditis causes permanent car- (eg, benzathine penicillin G given IM to patients not allergic
diac damage. to penicillin) and anti-inflammatory therapies (eg, salicylates
and steroids) are the mainstay of therapy but should not be
started until a definite diagnosis is made.
Emergency Department
Treatment and Disposition
Evaluate hemodynamic status and stabilize as indicated
Pearls
including the use of high-flow oxygen, continuous cardiac, 1. The revised Jones criteria are used for the diagnosis
blood pressure, and pulse oximetry monitoring. Admit of ARF.
patients for further workup and management when a diagno- 2. Maintain a high index of clinical suspicion for ARF as it
sis of ARF is made or clinically suspected. Consult cardiology is uncommon in the United States and it must be differ-
for echocardiography in patients with a murmur to diagnose entiated from other rheumatologic conditions.
CHEST PAIN

Clinical Summary musculoskeletal and nonorganic causes of chest pain can be


treated with rest, acetaminophen, or nonsteroidal anti-inflam-
Acute or chronic pain in the precordial region that could
matory agents. If respiratory causes of chest pain are found,
radiate to the neck, arm, or jaw associated with nausea and
treatment is directed at those causes.
typically precipitated by exercise is a common presenting
symptom in the ED. Relative frequency and causes in chil-
dren are listed in Table 5.18. Pearls
The initial history should be directed at determining the
1. Chest pain is often benign in children. No cause can be
nature of pain, associated symptoms, and concurrent or pre-
found in a majority of patients, even after a moderately
cipitating events that may help clarify the noncardiac origin
extensive investigation.
of the pain, including focusing on important past and fam-
2. History is the most important; most cases of chest pain in
ily histories. Before turning the focus to the chest, perform
children originate in organ systems other than the cardio-
a through physical examination, including observations
vascular system.
whether the child is in severe distress from pain, in emotional
3. Chest pain in children <12 years of age usually has a car-
stress, or hyperventilating. A typical angina pain is located
diorespiratory cause; psychogenic causes are more likely
in the precordial or substernal area and radiates to the neck,
in children >12 years of age.
jaw, arms, back, or abdomen. Patient describes pain as a deep,
4. ED physicians should make every effort to find a specific
heavy pressure; the feeling of choking; or a squeezing sensa-
cause before making a referral to a specialist or reassur-
tion. Exercise, cold stress, emotional upset, or a large meal
ing the child and the parents of the benign nature of the
typically precipitates angina pain.
complaint.

Emergency Department
Treatment and Disposition
A three-step diagnostic approach is recommended. First, look
for the three most common causes of chest pain: costochon-
dritis, musculoskeletal causes, and respiratory diseases. Next,
check for cardiac causes, with cardiac exam, chest x-ray, and
ECG, if clinically indicated. Last, if a cardiac cause is not
found, look for disease in other systems, including psycho-
genic and idiopathic causes.
Direct treatment at correcting or improving the cause once
a specific cause of chest pain is identified. Consult cardiology
when chest pain is anginal or there are any abnormal find-
ings in chest x-ray or ECG, with a positive family history for FIGURE 5.45 ■ Differential Diagnosis of Chest Pain. Bacterial
Pneumonia. Frontal view of the chest shows right upper lobe consol-
cardiomyopathy, LQTS, or another hereditary disease associ-
idation with a slight bulge in the minor fissure in a 7-year-old child
ated with cardiac abnormalities. Cardiac evaluation requires
presenting to the emergency department with chest pain and diffi-
further studies such as echo, exercise stress test, Holter culty breathing. Fever, tachypnea, tachycardia, and hypoxemia were
monitoring, or even cardiac catheterization and, depending additional findings. Her blood culture was positive for Streptococcus
on the cause, treatment will be medical or surgical. Most pneumoniae. (Photo contributor: Binita R. Shah, MD.)

182
CHEST PAIN (CONTINUED) ■ 183

FIGURE 5.46 ■ Differential Diagnosis of Chest Pain. Pneumothorax and Pneumomediastinum. Single frontal view of the chest demonstrates
right lower lobe atelectasis and a moderate right sided pneumothorax as well as pneumomediastinum in an adolescent patient presenting with
chest pain. Patient was noted to have tachypnea and wheezing with exacerbation of asthma. (Photo/legend contributors: Vikas S. Shah, MD
and Rachelle Goldfisher, MD.)

FIGURE 5.47 ■ Differential Diagnosis of Chest Pain. Ventricular Tachycardia (VT). A 16-year-old boy presented to the emergency depart-
ment with precordial chest pain following a football practice. The pain lasted for 2 hours and was dull aching without radiation. He had simi-
lar chest pain in the past following football practice and was diagnosed as exercise-induced asthma and prescribed albuterol before practice.
This 12-lead ECG demonstrates a wide complex tachycardia at a rate of 140 bpm with retrograde P-waves best seen in leads V3R and V4R
suggesting that this is VT. There is left bundle branch block and inferior axis (positive QRS in lead II, III, and aVF) suggesting the origin of
the VT from the right ventricular outflow tract. This is a benign form of VT commonly precipitated by exercise or β-agonists like albuterol
in young men. (Photo contributor: Shyam Sathanandam, MD.)
184 ■ CHEST PAIN (CONTINUED)

TABLE 5.18 ■ RELATIVE FREQUENCY OF CAUSES OF CHEST PAIN IN CHILDREN


• Idiopathic factors 12%–45%
• Costochondritis 9%–22%
• Musculoskeletal trauma 21%
• Cough, asthma, pneumonia 15%–21%
• Psychogenic factors 5%–9%
• Gastrointestinal disorders 4%–7%
• Cardiac disorders 1%–4%
• Sickle cell crisis 2%
• Miscellaneous 9%–21%
Noncardiac causes:
A. Thoracic cage:
1. Costochondritis (preceded by physical activity, exaggerated by breathing, may be chronic, tenderness on palpation
over chondrosternal junction)
2. Trauma or muscle strain (history of vigorous exercise or trauma), abnormalities of the ribcage or thoracic spine
and breast tenderness (mastalgia)
B. Respiratory (severe cough or bronchitis, pleural effusion, lobar pneumonia, exercise-induced asthma, spontaneous
pneumothorax, pulmonary embolism)
C. Gastrointestinal (esophagitis [burning substernal pain that worsens with reclining posture], peptic ulcer [usually a
burning substernal pain that worsens after certain foods], ingestion of foreign body.)
D. Psychogenic factors (hyperventilation, conversion disorder, somatization, and depression)
E. Miscellaneous (Texidor’s twinge-precordial catch or stitch in the side [lasts for seconds on minutes associated with
bending or slouching], Herpes Zoster, pleurodynia [Devil’s grip; coxsackie virus infection, characterized by sudden
episodes of sharp chest pain])
Cardiac Causes:
A. Structural abnormalities of the heart (eg, severe aortic or pulmonary stenosis, hypertrophic cardiomyopathy),
mitral valve prolapse
B. Coronary artery abnormalities (eg, Kawasaki disease, congenital coronary anomaly, coronary heart disease,
hypertension, sickle cell disease), cocaine abuse
C. Aortic dissection and aortic aneurysm (Turner, Marfan, and Noonan syndrome).
D. Inflammatory conditions (pericarditis [viral, bacterial or rheumatic]), postpericordiotomy syndrome, myocarditis
(acute or chronic), Kawasaki disease.
E. Arrhythmias (supraventricular tachycardia, frequent premature ventricular contractions, or ventricular tachycardia).
CONGESTIVE HEART FAILURE

Clinical Summary Tachycardia, gallop rhythm, and a weak, thready pulse are
common findings. Distended neck veins and ankle edema
In congestive heart failure (CHF), the heart is unable to pump
may be seen in older children, but are not seen in infants.
enough blood to the body to meet its needs. CHF may result
Splenomegaly is not indicative of chronic CHF.
from congenital or acquired heart diseases with volume or
pressure overload or from myocardial insufficiency. Volume
overload lesions like a ventricular septal defect (VSD); pat-
ent ductus arteriosus (PDA); atrioventricular septal defects
Emergency Department
(AV canal), and systemic arteriovenous fistula are the most Treatment and Disposition
common causes of CHF in the first 6 months of life. Large Obtain ECG and chest radiograph and consult a cardiologist.
left-to-right shunts do not cause CHF before 6 to 8 weeks Cardiomegaly is almost always present on chest x-ray, often
of age because the pulmonary vascular resistance does not with increased pulmonary vascular markings. ECG may help
fall low enough to cause a large shunt until this age. Atrial determine the type of defect but is not helpful in deciding
septal defect rarely causes CHF, and children with cyanotic whether CHF is present. Echocardiogram reveals cardiac
heart diseases do not develop CHF unless they have a large chamber enlargement and impaired left ventricle func-
systemic-to-pulmonary artery shunt like a VSD, PDA, or a tion and may help determine the cause of CHF. Hospitalize
surgical shunt. Pulmonary venous congestion in left-sided patients presenting in acute or chronic decompensated heart
heart failure leads to tachypnea, dyspnea with exertion, poor failure with hemodynamic compromise for inpatient therapy
feeding, orthopnea in older children, and wheezing and pul- to bring CHF back to a compensated state.
monary crackles. Systemic venous congestion in right-sided Management of CHF consists of elimination of underlying
heart failure results in hepatomegaly and periorbital edema. causes (eg, VSD), elimination of predisposing or contribut-
ing causes (eg, anemia, arrhythmia, infection) and control of
heart failure. In the ED, a heart failure state can be controlled
by the use of multiple drugs, usually inotropes, diuretics, and
afterload-reducing agents along with general supportive mea-
sures. A cardiac chair or infant seat is used to relieve respira-
tory distress. Oxygen with humidity is used in infants with
respiratory distress but care must be taken if a congenital
heart disease is suspected, as oxygen may increase the shunt
and make CHF worse.

Pearls
1. CHF may result from congenital or acquired heart dis-
eases; knowing the etiologies at various age groups may
provide clues for making the proper diagnosis.
2. Signs and symptoms of CHF are different in infants
and older children. Infants present with poor feeding of
recent onset, tachypnea that worsens during feeding, poor
FIGURE 5.48 ■ Ventriculoseptal Defect with Congestive Heart weight gain, and cold sweat on the forehead. Older chil-
Failure. Frontal view of chest shows cardiomegaly and pulmonary dren may complain of shortness of breath, especially with
venous congestion in a 4-month-old with tachypnea, poor feeding,
activity, easy fatigability, puffy eyelids, or swollen feet.
and decreased number of wet diapers over past 2 weeks. A loud
holo-systolic murmur over the left sternal border and scattered rales 3. Cardiomegaly on chest radiograph is nearly a prerequi-
over both lung bases were noted. Echo demonstrated a large peri- site sign of CHF.
membranous VSD. (Photo contributor: Shyam Sathanandam, MD.)

185
186 ■ CONGESTIVE HEART FAILURE (CONTINUED)

FIGURE 5.49 ■ Vein of Galen Malformation with Congestive Heart Failure. (A) Frontal view of the chest demonstrates marked cardio-
megaly and vascular congestion (congestive heart failure) in an infant presenting with difficulty breathing. (B) Arteriogram demonstrates
arteriovenous malformation of the vein of Galen. (Photo contributor: John Amodio, MD.)

FIGURE 5.50 ■ Vein of Galen Malformation with Congestive


Heart Failure. Head CT with contrast shows a vein of Galen mal-
formation (arteriovenous malformation [AVM]). This 2-month-old
presented with respiratory distress and poor feeding. A very active
precordium, gallop rhythm, crackles over both lung fields, distended
neck veins, and a bruit over the fontanel suggested a cerebral AVM.
Systemic AVMs act as left-to-right shunt lesions and lead to CHF in
the newborn period. (Photo contributor: Shyam Sathanandam, MD.)
CONGESTIVE HEART FAILURE (CONTINUED) ■ 187

TABLE 5.19 ■ CAUSES OF CONGESTIVE HEART FAILURE

From Congenital Heart Disease


Age of Onset Cause
At birth Hypoplastic left heart syndrome
Large systemic arteriovenous fistula
1st week Transposition of great arteries
PDA (premature infants)
Total anomalous pulmonary veins
Critical aortic or pulmonary stenosis
1–4 weeks Coarctation of aorta
Large left-to-right shunts in prematurity
4–6 weeks AV canal defect
6 weeks-4 months Large VSD, PDA
Anomalous left coronary artery from pulmonary artery.
Other Etiologies of CHF
• Unrecognized SVT or frequent PVCs (tachycardia induced cardiomyopathy; at any age)
• Metabolic abnormalities (neonates; eg, severe hypoxia and acidosis, hypoglycemia, hypocalcemia)
• Complete heart block (neonates and early infancy)
• Viral myocarditis (infants)
• Severe anemia (any age)
• Endocardial fibroelastosis (infancy)
• Idiopathic dilated cardiomyopathy (childhood or adolescence)
• Hydrops fetalis (neonates)
• Acute rheumatic carditis (school-age children)
• Rheumatic valvular heart disease (adolescence)
• Acute hypertension (eg, acute postinfectious glomerulonephritis; school-age children)
• Acute cor-pulmonale (acute airway obstruction; any age)
• Cardiomyopathy associated with other diseases (eg, muscular dystrophy; adolescents)
• Drug-induced cardiomyopathy (eg, doxorubicin)
• Bronchopulmonary dysplasia (premature infants; right heart failure in early infancy)
Abbreviations: AV, atrioventricular; CHF, congestive heart failure; PDA, patent ductus arteriosus; PVC, premature ventricular contraction; VSD, ventricular
septal defect; SVT, supraventricular tachycardia.
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RESPIR ATORY
Chapter 6

RESPIR ATORY
DISORDERS
Nooruddin R. Tejani
Diana E. Weaver
Ari J. Goldsmith
Haidy Marzouk
Jessica Stetz

Massive aspiration

The authors acknowledge the special contributions of Binita R. Shah, MD, to prior edition.

189
TRACHEOBRONCHIAL FOREIGN BODIES

Clinical Features Aspirated FBs can be difficult to diagnose as clinical


symptoms may mimic asthma, recurrent pneumonia, or
Foreign body (FB) aspiration, though uncommon, accounts
URI. Sudden choking and gagging with dyspnea are the
for 7% of deaths in children under 4 years of age. Most aspi-
first signs of aspiration. However, in up to 50% of cases, the
rated FBs become lodged in the bronchi because their size
choking episode is not witnessed. After the initial phase of
allows for passage through the larynx and glottis. Large FBs
choking and paroxysms of cough, children often enter into
may become impacted in the larynx or trachea, potentially
an asymptomatic phase that lasts for hours or even weeks
causing complete obstruction, a true emergency. Nuts and
as the FB becomes lodged. In cases of FBs of the larynx or
seeds are the most commonly aspirated objects. Beans and
trachea, children may present with hoarseness, stridor, and
seeds absorb water and can swell in the airway over time.
possibly cyanosis. Children with bronchial FB often pres-
Organic FBs can cause a surrounding tissue reaction leading
ent with a triad of cough, wheezing, and decreased breath
to severe inflammation; nuts and seeds release linolic acid,
sounds. However, only about two-thirds of children have
which can cause unilateral or bilateral wheezing.

FIGURE 6.1 ■ Aspiration/Asphyxiation by Food Products. (A) The


most commonly aspirated food products by infants and children
include peanuts, chunky peanut butter, hot dogs, popcorn, seeds,
grapes, raisins, carrots, meat, and hard candies. Children too young
to chew and swallow carefully (usually <5 years of age) should not
be given these foods. (B) A piece of hot dog is seen lodged in the
trachea of a 2.5-year-old child presenting in cardiopulmonary arrest.
For children <5 years, hot dogs should be cut longitudinally and not
into round pieces. (Photo contributor: Binita R. Shah, MD [A] and
B
Charles Catanese, MD [B].)

190
TRACHEOBRONCHIAL FOREIGN BODIES (CONTINUED) ■ 191

all components of this triad. When these symptoms are Computed tomographic scan of the chest may also be useful
prolonged or atypical, FB should be suspected. Unilateral in detection of an FB but is not obtained initially unless the
decreased air entry on chest auscultation is only present in patient presents with complications or plain radiographs are
one-third of cases. Untreated, patients may enter into the nondiagnostic.
third phase of the disease course, resulting in complications
from atelectasis to pneumonia. Emergency Department
Most FBs are not radiopaque and small FBs may cause
symptoms but no radiologic changes. Frontal view of chest
Treatment and Disposition
may show air trapping secondary to obstructive emphy- The criterion standard for the diagnosis and management of
sema. Bronchial FB results in obstruction during expiration, tracheobronchial FB is bronchoscopy and removal. Patients
where air entry is possible during inspiration due to a partial with suspected airway FB must have specialty evaluation
obstruction (“ball-valve”) or can result in complete obstruc- early. Consult otolaryngology and pediatric pulmonary ser-
tion with poor pneumatization and atelectasis. The sensitiv- vices. Children with laryngotracheal FBs may present in acute
ity of plain chest radiograph is increased when inspiratory and severe airway obstruction. Attempt standard back blows
and expiratory films are taken. Mediastinal shift to the or abdominal thrusts first and if unsuccessful in dislodging
opposite side on the expiratory phase is diagnostic. Patients the FB, attempt laryngoscopy and removal. If the FB cannot
who are not old enough to obtain inspiratory and expira- be removed by direct laryngoscopy in the emergency depart-
tory films should have right and left lateral decubitus radi- ment (ED), use intubation to advance the FB into a bronchus.
ographies performed. The lung positioned in the dependent Bronchial FBs rarely require emergent intervention. Give
position will deflate under the weight of the heart unless it supportive care with oxygen or heliox and monitor closely,
is obstructed. Fluoroscopy is also an option, if available, including continuous pulse oximetry. Give steroids to reduce
and may demonstrate air trapping or mediastinal shift. airway inflammation as needed.

A B

FIGURE 6.2 ■ Radiolucent Foreign Body Aspiration. (A) Single frontal view of the chest shows hyperinflation of the left lung. This
18-month-old toddler presented with persistent wheezing of several days duration. (B) Shell of a sunflower seed that was removed from the
right mainstem bronchus. It is conceivable that initially FB was in the left mainstem bronchus and subsequently got dislodged during cough-
ing and moved to the right mainstem bronchus. (Photo contributor: Johnathan Cohen, MD.)
192 ■ TRACHEOBRONCHIAL FOREIGN BODIES (CONTINUED)

A C

FIGURE 6.3 ■ Radiolucent Foreign Body Aspiration. (A) Frontal


view of the chest shows hyperinflation of the left lung. (B, C)
Bilateral decubitus views of the chest show persistent hyperinfla-
tion of the left lung with decubitus positioning, consistent with air
trapping. (D) CT scan shows overinflated left lung with air trap-
ping. (E) CT scan showing impacted FB in left mainstem bronchus
E
(arrow). (Photo contributor: Rafael Rivera, MD.)
TRACHEOBRONCHIAL FOREIGN BODIES (CONTINUED) ■ 193

FIGURE 6.4 ■ Radiopaque Foreign Body Aspiration. Frontal and lateral views (A, B) of the chest shows a screw in the left mainstem bron-
chus with atelectasis of the left lung. (Photo contributor: John Amodio, MD.)

Pearls 2. The presence of an airway FB should be considered for


all patients with new onset of noisy breathing or wheez-
1. Lack of witnessed choking episode and/or normal chest
ing (especially in toddlers) or unexplained persistent or
films are the most important factors contributing to
recurrent pulmonary findings (eg, pneumonia) regardless
delayed diagnosis.
of the history of an aspiration event.
PERTUSSIS

Clinical Summary tolerate macrolides. Macrolides given as soon as possible to


an exposed person (preferably during the incubation period)
Pertussis, or whooping cough, is infection of the respiratory
will prevent or modify the course of the disease. Pertussis
tract by Bordetella pertussis, a gram-negative pleomorphic
vaccine (follow standard immunization schedule) should
bacillus. Humans are the only known hosts and transmission
also be given to unimmunized or incompletely immunized
is by respiratory droplets.
exposed children who are under 7 years of age. Admit patients
A viral prodrome and mild cough (catarrhal stage) is fol-
under 1 year of age and those with apnea or cyanosis during
lowed by a paroxysmal stage of progressive, repetitive, and
episodes, pneumonia, or respiratory distress. Initiate respira-
severe episodes of coughing that lead to a forceful inspiration,
tory droplet precautions for 5 days after initiation of antibiotic
producing the characteristic whoop. Symptoms take weeks
therapy or until 3 weeks after the onset of paroxysms without
to months to resolve (convalescent stage). Pertussis is most
antibiotic therapy.
severe in the very young, presenting as gagging, gasping, or
apnea without characteristic whoop. Complications among
infants include bronchopneumonia, seizures, encephalopathy, Pearls
cerebral anoxia, and sudden unexpected death. Submucosal 1. Pertussis is a life-threatening infection and is preventable
bleeding, diaphragmatic rupture, umbilical and inguinal her- by universal immunization.
nias, and rectal prolapse have been reported. Complications 2. Neither infection nor immunization provides lifelong
among adolescents include syncope, sleep disturbance, immunity to pertussis.
incontinence, rib fractures, and pneumonia. A number of
other pathogens can cause pertussis-like symptoms (eg, ade-
noviruses, RSV, para influenza viruses, influenza viruses,
mycoplasma). Leukocytosis with absolute lymphocytosis
(total count >50,000 cells/mm3) may be seen at the beginning
of the paroxysmal stage. Chest radiograph may show perihi-
lar, patchy, or diffuse infiltrates. Diagnosis is confirmed by
culturing a nasopharyngeal sample on special media (Regan-
Lowe; a negative culture does not exclude the diagnosis)
or polymerase chain reaction assay using a nasopharyngeal
sample for detection of B pertussis. In the absence of immu-
nization within 2 years, an increasing titer or a single IgG
anti-Pertussis toxin value 100 EU/mL or greater can be used
for diagnosis.

Emergency Department
Treatment and Disposition
Provide supportive care, including suctioning of secretions,
humidified oxygen for hypoxia, and maintenance of adequate
hydration and nutrition, and treat both the patient and his FIGURE 6.5 ■ Bilateral Subconjunctival Hemorrhages and
or her contacts with macrolide antibiotics (azithromycin, Periorbital Ecchymosis; Pertussis. Violent episodes of coughing
erythromycin, or clarithromycin). Use azithromycin in leading to hemorrhages (subconjunctival and periorbital) were the
presenting complaints of pertussis in this unimmunized adolescent
patients under a month old as there is an association between
boy from El Salvador. His nasopharyngeal aspirate that was posi-
orally administered erythromycin and infantile hypertro- tive for Bordetella pertussis. (Important: These findings of subcon-
phic pyloric stenosis. Trimethoprim-sulfamethoxazole is an junctival hemorrhages and ecchymoses can be mistaken for inflicted
alternative for patients more than 2 months old who cannot injuries from child abuse.). (Photo contributor: Binita R. Shah, MD.)

194
CROUP

Clinical Summary syncytial virus (RSV), influenza viruses A and B, and ade-
novirus. Differential diagnosis includes epiglottitis, bac-
Croup is an acute viral inflammation of the larynx and struc-
terial tracheitis, foreign body aspiration, retropharyngeal
tures inferior to the larynx classically described as acute
abscess (RPA), peritonsillar abscess, angioneurotic edema,
laryngotracheitis or laryngotracheobronchitis. A distinction
is made from spasmodic croup, which is thought to have
an allergic component and presents with noninflammatory
edema in the subglottic region.
Croup affects children aged 6 months to 6 years and
occurs in early fall and winter. Patients present with 1 to
3 days of upper respiratory tract symptoms and fever with
progression to the characteristic barking cough, hoarseness,
stridor, and respiratory distress. Tachypnea, retractions,
hypoxia, and altered mental state are often ominous signs
of worsening obstruction. The commonest etiologic agents
include Para influenza viruses (two-thirds cases), respiratory

FIGURE 6.7 ■ Foreign Body Ingestions Presenting With Stridor;


Differential Diagnosis of Croup. (A) Frontal view of the chest
shows impacted coin in a child with “noisy breathing” and drooling.
FIGURE 6.6 ■ Croup. Anteroposterior view of the subglottic air- (B) Frontal view of the neck in a different child shows a jack in the
way demonstrates symmetrical narrowing producing the classic region of the esophagus. This child also presented with a stridor and
“steeple” shape. (Photo contributor: John Amodio, MD.) difficulty swallowing. (Photo contributor: John Amodio, MD.)

195
196 ■ CROUP (CONTINUED)

A B

FIGURE 6.8 ■ Laryngeal Papillomatosis Presenting With Stridor; Differential Diagnosis of Croup. (A) Respiratory papillomatosis of the
left half of the larynx, obstructing the glottic inlet in a patient presenting with hoarseness, stridor, and airway obstruction. (B) Papillomatous
lesions involving both vocal cords in a different patient with stridor and difficulty breathing. (Photo contributor: Bhuvanesh Singh, MD.)

congenital anomaly, mediastinal mass (eg, lymphoma), and the child appears well, he or she may be discharged home.
diphtheria. Routine laboratory studies are not helpful. Plain Hospitalize any child who does not improve after adrena-
radiographs of the neck are not routinely indicated and line administration. Intubate and admit patients with signs
should be used only when diagnosis is unclear to exclude of respiratory failure who do not respond to racemic epi-
other conditions such as retropharyngeal abscess or foreign nephrine and steroids. Helium oxygen mixture (Heliox) may
body aspiration. be useful in the treatment of severe croup as it reduces work
of breathing enough to prevent intubation and allow other
medications to reach therapeutic peak. There is no evidence
Emergency Department
for the use of humidified or cold air, prophylactic antibiot-
Treatment and Disposition ics, or antitussive agents.
Management of croup depends on the severity of upper
airway obstruction. For mild to moderate croup, the treat-
ment is oral or parenteral or nebulized steroids, including
Pearls
dexamethasone, prednisolone, or nebulized budesonide. 1. Croup is the most common form of acute upper airway
For severe croup, nebulized racemic epinephrine should be obstruction in infants and children.
given urgently as 2.25% solution diluted in 3 mL of nor- 2. Regardless of the level of illness acuity, corticosteroid
mal saline given over 15 minutes. If racemic epinephrine therapy (dexamethasone either orally or intramuscularly)
is not available then nebulized l-epinephrine 1:1000 can is now the standard of care.
be administered. Improvement of symptoms should occur 3. Drooling, dysphagia, high fever, and toxic appearance
within 10 to 30 minutes of administration. If after 2 hours are notably absent in viral croup suggesting another
of observation, there is no stridor or chest retractions and diagnosis.
CROUP (CONTINUED) ■ 197

FIGURE 6.9 ■ Laryngeal Hemangioma Presenting with Stridor; Differential Diagnosis of Croup. Subglottic hemangioma causing airway
narrowing. This patient had a beard-distribution cutaneous hemangioma (see Figure 7.94)) and presented with biphasic stridor and respiratory
distress. (Photo contributor: Sydney C. Butts, MD.)

TABLE 6.1 ■ ASSESSMENT OF THE SEVERITY OF CROUP

Level of Severity Characteristics


Mild Occasional barky cough, no audible stridor at rest, and no to mild
suprasternal and/or intercostal indrawing
Moderate Frequent barky cough, easily audible stridor at rest, and suprasternal and
sternal wall retraction at rest, without agitation
Severe Frequent barky cough, prominent inspiratory and—occasionally—
expiratory stridor, marked sternal wall retractions, and significant
distress and agitation
Impending Barky cough (often not prominent), audible stridor at rest (occasionally
respiratory hard to hear), sternal wall retractions (may not be marked), lethargy or
failure decreased level of consciousness, and often dusky appearance without
supplemental oxygen
Adapted with permission from Toward Optimized Practice (TOP), http://www.topalbertadoctors.org (CPGs/Emergency
Medicine/Croup 2008).
BACTERIAL TRACHEITIS

Clinical Summary respiratory distress, which can lead to airway obstruction


and respiratory arrest. Patients with bacterial tracheitis are
Bacterial tracheitis is a potentially fatal acute infectious upper
toxic appearing and show little response to inhaled steroids
airway obstruction, occurring in children of age 3 months
or adrenaline. Visualization of the airways reveals subglot-
to 5 years, predominantly in fall and winter. A prodrome
tic inflammation, edema of the tracheal mucosa, and copious
of coryza, sore throat, cough, and pyrexia of 1 to 3 days is
purulent endotracheal secretions. Staphylococcus aureus is the
followed by acute onset of stridor and rapidly worsening
most common bacterial pathogen followed by Streptococcus
pneumoniae, Streptococcus pyogenes, Haemophilus influen-
zae nontypeable, Moraxella catarrhalis, and Pseudomonas
aeruginosa. Viral coinfection is common with Influenza A,
Parainfluenza, RSV, and adenovirus. Differential includes
viral croup, epiglottitis, foreign body aspiration, and retro-
pharyngeal abscess. Complications include cardiorespiratory
arrest, acute respiratory distress syndrome, hypotension, toxic
shock syndrome, renal failure, pneumothorax, pulmonary
edema, and subglottic stenosis. White blood cell count is usu-
ally elevated or abnormally low. Chest and lateral neck radio-
graphs show subglottic narrowing on posterior-anterior view,
a hazy tracheal air column and irregular soft-tissue densities
in trachea (indicating purulent exudate), pneumonia, and
pulmonary edema.

FIGURE 6.10 ■ Bacterial Tracheitis. Lateral neck view of patient


with bacterial tracheitis. Note presence of irregular tracheal mar- FIGURE 6.11 ■ Retropharyngeal Abscess; Differential Diagnosis
gins (arrows). (Courtesy W. McAlister, MD, Washington University of Bacterial Tracheitis. Lateral view of the neck shows collection of
School of Medicine, St. Louis, MO.) (Reproduced with permission air in retropharyngeal soft tissues compatible with abscess. Stridor,
from Tintinalli JE, Stapczynski JS, Ma OJ, et al. (eds): Tintinalli’s drooling, and respiratory distress in a highly febrile child were the
Emergency Medicine: A Comprehensive Study Guide, 7th ed. presenting signs mimicking bacterial tracheitis. (Photo contributor:
McGraw-Hill, New York.) John Amodio, MD.)

198
BACTERIAL TRACHEITIS (CONTINUED) ■ 199

FIGURE 6.12 ■ Foreign Body Ingestion; Differential Diagnosis


of Bacterial Tracheitis. Frontal and lateral views (A, B) of the chest
show a button battery impacted in the esophagus at the level of the
aortic arch in a child presenting with stridor and respiratory dis-
tress. (Photo/legend contributors: Nooruddin R. Tejani, MD/John
B
Amodio, MD.)

Emergency Department antimicrobials and aggressive supportive care, rapid improve-


ment and extubation within 72 to 96 hours is possible.
Treatment and Disposition
Adequate airway protection with intubation is the single most
important therapeutic intervention. Obtain tracheal bacterial
Pearls
cultures at intubation and send secretions for direct immuno- 1. The clinical hallmark of bacterial tracheitis is toxic
fluorescent identification of viral agents. Treat hypotension appearance and worsening stridor 1 to 3 days after a viral
aggressively with normal saline fluid boluses and inotropic prodrome.
support. Begin empirical therapy with anti-staphylococcal 2. In contrast to viral croup, patients with bacterial tracheitis
agent (eg, vancomycin) and a third-generation cephalo- show little response to inhaled steroids or adrenaline.
sporin (eg, ceftriaxone or cefotaxime). Admit all intubated 3. Visualization of airway shows presence of purulent tra-
patients to the intensive care unit (ICU). With appropriate cheal secretion.
EPIGLOTTITIS

Clinical Summary
Epiglottitis is an acute life-threatening inflammation of supra-
glottic structures involving the epiglottis, aryepiglottic folds
(false vocal cords), arytenoids, and vallecula. It is usually seen
in children aged 2 to 7 years and has become rare since the
introduction of H influenzae type b (Hib) conjugate vaccines
in 1991. The most common organisms include S pyogenes,
S aureus, S pneumoniae, Moraxella, and Candida species.
H influenzae type b is still seen in nonimmunized children.
Non infectious causes include direct trauma and thermal
injuries (eg, scalding burns of face, drinking hot liquids).
Symptoms occur with rapid progression and include fever, V A
irritability, and toxic appearance, difficulty swallowing, and
drooling. Croupy cough is absent and stridor is a late finding.
The child usually sits upright with the chin pushed forward
(tripod position) to open the airway. Gentle visualization of A
the oropharynx without the use of a tongue depressor may
reveal an erythematous epiglottis protruding at the base of the
tongue. Use radiographs when the diagnosis of epiglottitis is
unlikely and to exclude retropharyngeal abscess and foreign
bodies. Lateral neck radiograph is the image of choice, and
the classic finding of epiglottitis is the “thumbprint sign.”
Contrasted CT scan of the neck may also indicate the pres-
ence of epiglottic edema and possibly phlegmon or abscess in
the epiglottis or the base of tongue.

Emergency Department
Treatment and Disposition B

Assemble a multidisciplinary team including pediatric inten- FIGURE 6.13 ■ Epiglottitis. (A) Lateral view of the airway shows
sive care, anesthesiology, and otolaryngology. Bring the enlargement of the epiglottis (arrow), thickening of the aryepiglot-
patient to the operating room emergently to confirm diagno- tic folds (A), and amputation of the vallecula (V) leading to the
“thumbprint sign”. (B) Endoscopic view of almost complete airway
sis by direct visualization. Edema with intense erythema of
obstruction secondary to epiglottitis in a different patient. Note the
epiglottis (often described as cherry-red appearance) and slit-like opening of the airway. (Photo contributors: John Amodio,
surrounding structures including arytenoids and aryepiglottic MD [A] and Department of Otolaryngology, Cincinnati Children’s
folds and vocal cords are seen. Perform intubation or trache- Hospital Medical Center, Cincinnati, OH [B].) (Reproduced with
ostomy as needed. Admit the patient to the ICU for continu- permission from Knoop K, Stack L, Storrow A, Thurman RJ: Atlas
ous monitoring. Postpone diagnostic tests and placement of of Emergency Medicine, 3rd ed. McGraw-Hill, New York, 2010.)
IV lines until the airway is secure. Obtain CBC with differen-
tial and cultures of the blood and epiglottal surface for precise
microbiologic diagnosis. Treat empirically with a second-
or third-generation cephalosporin or ampicillin/sulbactam.
Children usually require intubation for 24 to 72 hours, until
reduction in airway edema occurs.

200
EPIGLOTTITIS (CONTINUED) ■ 201

Vallecula

Swollen
epiglottis

Swollen
glottic area

Purulent material
on lingual tonsils

A B

FIGURE 6.14 ■ Epiglottitis. (A) Lateral view of the neck shows marked enlargement of the epiglottis, amputation of the vallecula (black
arrow) and thickening of the aryepiglottic folds (white arrow) in a 21-year-old patient presenting with history of sore throat, fever, and diffi-
culty handling secretions. His epiglottal culture was positive for Streptococcus pyogenes. (B) Fiberoptic laryngoscopy showing an edematous
epiglottis and glottic area with marked airway compromised in an adult with epiglottitis. (Photo contributors: Michael Secko, MD [A] and
Edward C. Jauch, MD [B].) (Reproduced with permission from Knoop K, Stack L, Storrow A, Thurman RJ: Atlas of Emergency Medicine,
3rd ed. McGraw-Hill, New York, 2010.)

Pearls 3. Confirm epiglottitis with direct visualization rather than


radiography as patients may develop life-threatening
1. Do not force the patient to lie in a supine position as a laryngospasm when attempting to hyperextend the neck
gravity-induced change in the position of the epiglottis for the radiograph.
may lead to total obstruction.
2. Do not use a tongue blade to examine the pharynx as it
may induce life-threatening laryngospasm.
BRONCHIOLITIS

Clinical Summary gastroesophageal reflux, congestive heart failure, vascular


ring, cystic fibrosis, mediastinal mass, bronchogenic cyst,
Bronchiolitis is defined as a constellation of clinical symp-
and tracheoesophageal fistula.
toms and signs including a viral upper respiratory prodrome
followed by increased respiratory effort and wheezing. It
occurs in children under 2 years old, primarily between late Emergency Department
fall and spring. RSV account for 50% to 80% of cases fol-
lowed by para influenza viruses, influenza, and human meta-
Treatment and Disposition
pneumovirus. An upper respiratory tract infection (URI) Measure O2 saturation because a pulse oximetry level of
prodrome is followed by cough, tachypnea, tachycardia, <94% is associated with a more than 5-fold increase in like-
grunting, flaring, supraclavicular and intercostal retractions, lihood of hospitalization. Chest radiograph is not recom-
and head bobbing. Nonspecific symptoms include poor feed- mended routinely and classically shows hyperinflation. Rapid
ing and irritability. Examination may reveal chest hyperin- viral antigen tests, in outpatient cases, have little impact on
flation with costal retraction, and fine inspiratory crackles management (in hospital setting, it is used to reduce nosoco-
and expiratory wheeze on auscultation. Hypoxemia is pri- mial infection). In a neonate with a fever, a positive RSV test
marily due to ventilation/perfusion mismatch, and hypercap- may be all that is needed as concomitant bacterial infection is
nia is a late phenomenon. Progression to respiratory failure rare (0.2%).
may occur. Factors associated with severe disease include Provide supportive therapy including O2 therapy and fluid
age <3 months, gestational age <34 weeks, toxic appear- replacement. Admit to the hospital or ICU if warranted by
ance, respiratory rate >70 breaths per minute, chronic lung age, prematurity, chronic lung disease, or significant cardiac
disease, congenital cardiac abnormalities, neuromuscu- disease. There is no proven pharmacologic treatment. Do not
lar disease, or immune deficiencies. Differential diagnosis routinely use α- or β-agonists, ipratropium bromide, inhaled or
includes pneumonia, laryngotracheomalacia, foreign body, systemic corticosteroids, ribavirin, antileukotriene antagonists,

A B

FIGURE 6.15 ■ Bronchiolitis. (A) An infant presenting with new onset of wheezing associated with subcostal and intercostal retractions and
pulling inward of the sternum with exacerbation of the pectus excavatum deformity. (B) Frontal view of chest shows hyperinflation, peribron-
chial cuffing, and increased pulmonary markings compatible with bronchiolitis. (Photo contributor: Binita R. Shah, MD.)

202
BRONCHIOLITIS (CONTINUED) ■ 203

A B

FIGURE 6.16 ■ Foreign Body Aspiration; Differential Diagnosis of Wheezing. (A) Frontal view of chest showing unilateral hyperinfla-
tion of the right lung, flattening of the right hemidiaphragm with a slight mediastinal shift to the left. About 20 hours following a choking
episode while eating peanuts, this toddler presented with coughing and first episode of wheezing. In bronchiolitis, hyperinflation (a hallmark
finding) is present bilaterally unlike unilateral hyperinflation seen in foreign body aspiration. (B) Unilateral hyperinflation is no longer seen
after extraction of the peanut fragment from the right lower lobe bronchus by rigid bronchoscopy. (Photo contributor: Binita R. Shah, MD.)

and antibiotics. A trial of albuterol may be given and should


be continued only after a documented beneficial response.
Epinephrine may provide short-term benefits in the outpatient
setting. Use antibiotics only in neonates with fever and sus-
pected bronchiolitis pending cultures or if chest radiograph
demonstrates pneumonia or superinfection. Nebulized hyper-
tonic saline has been associated with improvement in clinical
score and duration of hospitalization.
Consider using ribavirin if there is documented RSV
bronchiolitis with severe disease or in immunocompromised
and/or hemodynamically compromised patients. Intravenous
RSV immune globulin is ineffective in the acute treatment of
RSV lower respiratory tract infection.

Pearls
1. Bronchiolitis is the most common wheezing-associated
respiratory illness in infants <2 years of age.
2. Apnea may be the presenting manifestation of RSV bron- FIGURE 6.17 ■ Foreign Body Aspiration; Differential Diagnosis
chiolitis in the neonate. of Wheezing. Frontal view of chest showing a foreign body (rock)
3. Preventive strategies such as RSV immunoglobulin or within the left mainstem bronchus. New onset of wheezing and
the anti-RSV monoclonal antibody, palivizumab, can coughing were the presenting complaints of this 9-month-old infant
seen in the ED during the RSV season. However, wheezing was uni-
decrease disease severity and need for hospitalization.
lateral (unlike bronchiolitis), necessitating a radiograph. (Photo con-
tributor: Kanwal Chaudhry, MD.)
204 ■ BRONCHIOLITIS (CONTINUED)

FIGURE 6.18 ■ Acute Asthma; Differential Diagnosis of Wheezing. It is impossible to clinically differentiate bronchiolitis from asthma
in a patient with a first episode of wheezing. Reversible bronchospasm, recurrent wheezing, eosinophilia, history of eczema, and family his-
tory of asthma or atopy suggest a diagnosis of asthma. This infant presented with a new onset of wheezing, and had severe atopic dermatitis
with lichenified plaques with exudation over extremities. He responded to bronchodilator therapy. (Photo contributor: Binita R. Shah, MD.)

FIGURE 6.19 ■ Congestive Heart Failure; Differential Diagnosis of Wheezing. Frontal view of the chest shows cardiomegaly and increased
markings emanating from the hila, compatible with pulmonary venous congestion (CHF). There are also increased markings extending to
the periphery of the lungs, representing pulmonary arterial hypertension secondary to a ventriculoseptal defect. This 3-month-old infant
presented with wheezing and tachypnea. Signs and symptoms of CHF (eg, tachypnea, tachycardia, wheezing, rales, respiratory distress, and
hepatomegaly) can be easily mistaken for bronchiolitis. (Photo contributor: Shyam Sathanandam, MD.)
BACTERIAL PNEUMONIA

Clinical Summary radiograph. However, clinical findings may precede radio-


logic evidence; thus, a negative chest radiograph does not
Pneumonia is an infection of the lung parenchyma, and 10%
exclude pneumonia (eg, sickle cell disease with acute chest
to 40% of cases are bacterial in origin. Bacterial pneumonia
syndrome). Outpatient management requires no laboratory
occurs throughout the year with no seasonal peaks.
evaluation. In admitted patients, CBC, electrolytes, and
Group B Streptococcus and gram-negative bacilli are com-
blood culture are usually obtained. Perform tuberculin skin
mon neonatal bacterial pathogens. Chlamydia trachomatis
testing especially in areas with a high incidence of tuberculo-
is seen at about 6 weeks of age, presenting with tachypnea,
sis. In patients with empyema, pleural fluid typically reveals
staccato cough, and absence of fever. S pneumoniae is the
an exudate with polymorphonuclear cells, elevated protein,
most common pathogen followed by S aureus and Group A
and a low glucose concentration; Gram stain and cultures will
streptococcus beyond 3 months of age. Community-acquired
identify the pathogen. A chest tube drain or VATS (video-
multiresistant S aureus resulting in a necrotizing fulminant
assisted thoracoscopic surgery) is usually required if pleural
pneumonia has emerged. Chlamydophila pneumoniae infec-
fluid is exudative (empyema).
tions occasionally are seen in preschool-age children and once
children reach school age, Mycoplasma pneumoniae becomes
an important pathogen. Symptoms of fever and cough or diffi- Emergency Department
culty breathing are hallmarks; tachypnea (breathing >50/min
for infants aged 2 months up to 1 year; breathing >40/min for
Treatment and Disposition
children aged 1 to 5 years) is the most sensitive and specific Admit patients who are in respiratory distress with or with-
sign. Severity is based on presence of retractions, grunting, out hypoxia, have a toxic appearance, are age <3 months,
hypoxia, inability to drink, vomiting, or altered mental sta- are immunocompromised, are not able to maintain adequate
tus. Lung examination demonstrates dullness to percussion, hydration, or have pneumonia with pleural effusion/empyema.
crackles, decreased breath sounds, and bronchial breathing. Administer oxygen, IV fluids, and antibiotics.
Diagnosis is suspected clinically and confirmed by chest

A B

FIGURE 6.20 ■ Round Pneumonia. (A) Frontal chest radiograph shows left paraspinal density in a rounded configuration, mistaken for a
mass. (B) A repeat radiograph after antibiotics show resolution of the “mass”, compatible with a “round” pneumonia. (Photo contributor:
John Amodio, MD.)

205
206 ■ BACTERIAL PNEUMONIA (CONTINUED)

to children with aspiration pneumonia. In some patients, for


example, SCD with pneumonitis, therapy with 2 agents may
be necessary (macrolide combined with a third-generation
cephalosporin). If the initial response to IV antibiotics is good,
institute oral treatment to complete a 10- to 14-day course.
Children not requiring hospitalization can be treated as
outpatients. The first-line agent is high dose amoxicillin. A
dose of ceftriaxone may be given parenterally on day 1 to
achieve rapidly effective serum concentrations. For children
who have non–type 1 allergy to amoxicillin, a cephalosporin
(eg, cefdinir or cefpodoxime) may be used. For those with a
history of type 1 reactions, a macrolide may be chosen. Use
macrolide antibiotics (eg, azithromycin or clarithromycin)
in older children for clinically suspected M pneumoniae. If
FIGURE 6.21 ■ Lobar Consolidation in Pneumococcal Pneumonia.
the child’s condition deteriorates or does not improve in 2 or
Frontal chest radiograph shows consolidation of the right upper lobe.
Blood culture was positive for S pneumoniae. Bacteremia is present 3 days, consider other pathogens, and obtain a chest radio-
in 10% to 30% of patients with pneumococcal pneumonia. (Photo graph to exclude parapneumonic effusion or empyema. For
contributor: Binita R. Shah, MD.) children who improve with treatment, a repeat chest radio-
graph is not recommended.

Choice of antibiotics depends on the organism suspected.


Pearls
Treat neonates with ampicillin and cefotaxime. A macrolide
will cover C trachomatis pneumonitis (first 3 months of life). 1. The sine qua non of infectious pneumonia is fever and
For older infants and children, give ceftriaxone or ampicillin tachypnea.
to cover pneumococcus. If Staphylococcus is a concern 2. Most children with cough, runny nose, and fever without
(including methicillin-resistant S aureus), give clindamycin the presence of “fast breathing” do not need antibiotics.
or vancomycin. Give ampicillin-sulbactam or clindamycin 3. Lower lobe pneumonia can present with signs of paralytic
ileus and/or referred pain to the right lower quadrant (with
right lower lobe pneumonia), mimicking acute abdomen.

A B

FIGURE 6.22 ■ Pneumococcal Pneumonia With Empyema. (A) Frontal chest radiograph shows large soft tissue density in right hemithorax,
with mediastinal shift suggesting large pleural collection. (B) CT scan shows a large loculated empyema (arrow) with surrounding consoli-
dated lung. Blood and empyema cultures were positive for S pneumoniae (serotype A19). (Photo contributor: John Amodio, MD.)
BACTERIAL PNEUMONIA (CONTINUED) ■ 207

A B

FIGURE 6.23 ■ Staphylococcal Pneumonia. (A) A highly febrile infant with respiratory distress. An abrupt onset and rapid progression of
pneumonia in very young infants should be considered due to staphylococci until proved otherwise. (B) CT slice through upper lobes shows
consolidation of the right upper lobe with areas of necrosis (arrows). (Photo contributors: Binita R. Shah, MD [A] and John Amodio, MD [B].)

A B

FIGURE 6.24 ■ Staphylococcal Pneumonia. (A, B) Frontal and lateral chest radiographs show involvement of entire right upper lobe with
multiple pneumatoceles, some of which have air-fluid levels. The minor fissure is displaced inferiorly (“bulging fissure”). Cystic fibrosis
was diagnosed after this pneumonia due to Staphylococcus aureus. Clinical deterioration with rapid progression from bronchopneumonia to
effusion or pyopneumothorax with or without pneumatoceles are highly suggestive of staphylococcal pneumonia. Other pathogens may also
cause empyema or pneumatoceles (eg, S pneumoniae, group A streptococci). (Photo contributor: Binita R. Shah, MD.)
VIRAL PNEUMONIA

Clinical Summary and bronchodilator therapy if indicated. Infants and young


children who are only mildly ill and afebrile do not need anti-
Pneumonia is an infection of the lung parenchyma, most com-
biotics. Admit those who are unable to tolerate oral fluids,
monly caused by viruses in patients 3 months to 5 years of age.
dehydrated, have moderate to severe tachypnea or oxygen
Incidence peaks in cold months, and transmission includes
saturation of <94%. Evaluate ill-appearing children or have
droplet spread of respiratory pathogens directly or indirectly
severe influenza for possible bacterial coinfection and poten-
by fomites. RSV and parainfluenza viruses predominate.
tial need for antimicrobial therapy. Use antiviral medications,
Other agents include human metapneumovirus, bocavirus,
oseltamivir or zanamivir, for treatment of influenza A and
rhinovirus, influenza, and adenovirus. Signs and symptoms
B. Most patients with viral pneumonia recover uneventfully
include a well-appearing infant or child with low-grade
without any sequelae. Rare complications include chronic
fever, a viral prodrome including sore throat, nasal conges-
lung disease leading to bronchiolitis obliterans (eg, adenovi-
tion, rhinitis, prominent cough, and mild respiratory distress.
ral infection).
Wheezing and/or diffuse crackles or rhonchi are typical aus-
Respiratory tract secretions are infectious and good hand
cultatory findings. Presence of fever of ≥39°C, retractions,
hygiene is important. Because viral infections usually are
and grunting may suggest a complicated pneumonia or bacte-
either the direct cause of pneumonia or predispose children to
rial coinfection. Specimens for viral culture and rapid antigen
infection with bacterial or atypical pathogens, immunization
identification can be used to detect the viruses in nasopha-
against influenza is particularly helpful.
ryngeal secretions obtained by swab, aspirate, or wash. CBC
and chemistry are not required. Afebrile children normally do
not require chest radiography. Those who are ill appearing or Pearls
highly febrile may benefit with both a blood culture and chest 1. Respiratory viruses are the most common etiology of
radiograph, for detection of a complicated pneumonia. pneumonia in the first few years of life.
2. Children with severe influenza should be evaluated care-
Emergency Department fully for possible coinfection with bacterial pathogens
and may require antimicrobial therapy.
Treatment and Disposition 3. Children and adolescents with influenza should not receive
Management of viral pneumonitis is supportive and aspirin or any salicylate-containing products because of
includes monitoring for cardiac and oxygen status, provid- the resulting increased risk of developing Reye syndrome.
ing humidified O2 if indicated, maintaining good hydration,

FIGURE 6.25 ■ Viral Pneumonia. Frontal view of the chest demonstrates hyperinflation with marked increase in bronchovascular markings
emanating from hilum and areas of atelectasis at both bases. (Photo contributor: John Amodio, MD.)

208
VIRAL PNEUMONIA (CONTINUED) ■ 209

portable

LEFT

D
Liver

A
Lung

FIGURE 6.26 ■ Influenza A Infection With Secondary Bacterial


Infection. (A) Frontal view of the chest shows opacification of the
right hemithorax with no mediastinal shift, compatible with pleu-
ral effusion. This toddler presented with severe respiratory distress
associated with fever, cough, and rhinorrhea during flu epidemic.
Her nasopharyngeal aspirate was positive for influenza A virus
and blood and empyema cultures were positive for S pyogenes.
(B) Longitudinal sonogram of the right hemithorax shows large
complex effusion (E), which was found to be an empyema. (Photo
B
contributor: John Amodio, MD.)

FIGURE 6.27 ■ Foreign Body Aspiration; Differential Diagnosis


of Wheezing. Frontal view of the chest shows foreign body (rubber
cap) in right mainstem bronchus in a child presenting with coughing
and wheezing (which are also common symptoms of viral pneumo-
nia). (Photo contributor: Rafael Rivera, MD.)
PULMONARY TUBERCULOSIS

Clinical Summary
Tuberculosis (TB) is infection caused by Mycobacterium
tuberculosis (MTB) and may be latent or active. Latent tuber-
culosis infection (LTBI) is a positive tuberculin skin test (TST)
without physical findings of disease, and a chest radiograph
that is either normal or shows granulomas or calcification in
the lungs and/or regional lymph nodes. Active MTB is the
presence of clinical and/or radiographic findings consistent
with active pulmonary and/or extrapulmonary MTB disease.
Active MTB may cause fever, weight loss, and night sweats.
Clinical manifestations of pulmonary TB (PTB) may
include cough, localized wheezing (endobronchial involve-
ment), hemoptysis (cavitary lesions), ausculatory findings
consistent with consolidation or atelectasis, or dullness to per-
cussion with pleuritic chest pain (pleural effusion). However,
many children with PTB and intrathoracic adenopathy and/ A
or infiltrates will be asymptomatic. Extrapulmonary mani-
festations are more common in children younger than age
3 years and may include meningitis, osteomyelitis (Pott’s
disease of the spine; see Figure 19-91), TB arthritis, infection
of the middle ear, and mastoiditis. Infants and toddlers may
also have miliary tuberculosis with dissemination involv-
ing lungs, central nervous system (CNS), liver, spleen, and
kidneys. The differential diagnosis of PTB includes acute
pneumonia, intrathoracic adenopathy from another etiology
(eg, lymphoma, sarcoidosis), parapneumonic effusion from

FIGURE 6.28 ■ Positive Tuberculin Skin Test. A 5-TU PPD skin B


test result is assessed at 48 to 72 hours after administration. The
diameter of induration (and not erythema) is measured perpendicu- FIGURE 6.29 ■ Active Pulmonary Tuberculosis. (A, B) Frontal and
larly to the long axis of the forearm. With an intense reaction, a bul- lateral projections of the chest demonstrate bilateral hilar adenopathy
lous formation may also occur. (Refer to local DOH guidelines for and right perihilar/upper lobe infiltrate in an asymptomatic 6-month-
interpretation). (Photo contributor: Binita R. Shah, MD.) old infant with positive PPD. (Photo contributor: John Amodio, MD.)

210
PULMONARY TUBERCULOSIS (CONTINUED) ■ 211

FIGURE 6.30 ■ Pulmonary Tuberculosis With Lymph Node


Calcifications. The hallmark of childhood pulmonary TB is Ghon
primary complex (hilar lymphadenitis and parenchymal focus with
or without lymph node calcification). Hilar adenopathy is inevitably
present with childhood TB. However it may not be detected on a
plain x-ray in the absence of calcification. A CT scan detects both
calcified and noncalcified lymph nodes. (Photo contributor: Binita
R. Shah, MD.)

A B

FIGURE 6.31 ■ Active Pulmonary Tuberculosis. CT scan of the chest showing right upper lobe consolidation (A, arrow) and bilateral hilar
adenopathy (B, arrow). Patient also had subcarinal adenopathy. (Photo contributor: John Amodio, MD.)
212 ■ PULMONARY TUBERCULOSIS (CONTINUED)

A B

FIGURE 6.32 ■ Cavitary Pulmonary Tuberculosis. (A) Frontal view of the chest demonstrates a large cavity in the left upper lobe (arrow)
with reticulonodular disease (endobronchial spread) of active Tuberculosis. (B) CT slice of the lower lobe region in a different patient shows
air-filled cavity (arrow) compatible with cavitary tuberculosis. (Photo contributor: John Amodio, MD.)

FIGURE 6.33 ■ Reactivation Pulmonary Tuberculosis. Frontal view of chest showing a large cavitary lesion with an air-fluid level in the
right upper lobe and a smaller cavitary focus (multiloculated; arrow) in the left upper lobe apical segment in an immunocompromised patient.
The air-fluid level on the right represents a secondary infection with development of lung abscess. The commonest form of reactivation TB
is an infiltrate or cavity in the apex of the upper lobes. This patient’s sputum was positive for AFB, and culture positive for MTB. (Photo
contributor: Binita R. Shah, MD.)
PULMONARY TUBERCULOSIS (CONTINUED) ■ 213

Emergency Department
Treatment and Disposition
Admit all patients with active MTB for collection of appro-
priate diagnostic specimens, initiation of anti-TB drugs (usu-
ally isoniazid, rifampin, and pyrazinamide with addition of
ethambutol if drug resistance is suspected), investigation of
the source case(s), and any further diagnostic workup (eg,
bronchoscopy, CT scan, lumbar puncture). Sputum is helpful
in patients with cavitary disease; however, pediatric patients
are often sputum acid-fast bacillus (AFB) negative and gastric
aspirates are more helpful. Respiratory isolation is required
for children with cavitary disease, possible laryngeal involve-
ment, immunosuppression, or an AFB positive smear from
a pulmonary source. Children younger than 12 years of age
FIGURE 6.34 ■ Pulmonary Tuberculosis With Pleural Effusion. with primary pulmonary TB (noncavitary pulmonary lesions,
Frontal chest radiograph shows opacification of left middle and absent or minimal cough with no sputum production and no
lower zones with obliteration of the left cardiac margin, left hemidia- expectoration of bacilli) are not contagious and do not require
phragm, and costophrenic angle associated with a mediastinal shift respiratory isolation. Patients with LTBI do not need hospi-
to the right in patient with a positive PPD test. The pleural fluid was talization; begin INH therapy and refer patient to their PCP
transudate with a negative AFB smear but positive culture for MTB.
for ongoing follow-up. Notify the local public health agency
(Photo contributor: Binita R. Shah, MD.)
especially if the child has a recent exposure to a known case
of PTB (in many hospitals, the infection control department
can assist with this process).

another etiology (bacterial, viral, autoimmune), foreign body


aspiration, and infection with nontuberculous Mycobacteria.
Pearls
After initial infection, TST may become positive in 2 to 1. Diagnosis of active or latent TB infection in children
12 weeks; however, in some cases, the TST may remain indicates recent transmission in the community and man-
negative (eg, malnutrition, HIV infection, immunosuppres- dates a thorough health department investigation to find
sion, lymphoma). A Mantoux test containing 5 tuberculin the source.
units of purified protein derivative (5 TU PPD) is used for 2. Children usually have paucibacillary pulmonary disease
the TST. Blood-based tests such as the QuantiFERON-TB but develop extrapulmonary disease more frequently than
Gold, an interferon gamma release assay, are more reliable adults, especially children aged <3 years.
but not as readily available. Chest radiograph is recom- 3. Reactivation TB or adult-type TB (ie, cavitary disease) is
mended for all children with a positive TST. Radiographic quite rare in children (less than 6% of cases in children
findings may include none (LTBI), intrathoracic lymphade- aged <13 years) but can occasionally occur in adolescents.
nopathy, infiltrate(s) and/or atelectasis, pleural effusion, 4. Localized wheezing and collapse/consolidation findings
miliary disease, cavitary lesion(s), or residual calcification seen on radiograph in endobronchial TB are similar to
of the primary focus with local lymphadenopathy. Isolation those seen in foreign body aspiration.
of MTB by culture of sputum, gastric aspirate, pleural fluid, 5. Although most young children with PTB are not conta-
or intrathoracic lymph node biopsy is confirmatory for PTB. gious, the source case is often a parent. Rapid screening
With associated extrathoracic disease, isolation of MTB from of all close household contacts, especially adolescents
other culture sites (CSF, bone, joints, or lymph nodes) is also and adults, is essential. Send parents for chest radiograph
diagnostic. immediately to prevent the potential risk of exposing the
pediatric ER/ward to a case of active TB.
MILLIARY TUBERCULOSIS

Clinical Summary poor appetite, headache, cough, and myalgia. Generalized


lymphadenopathy, hepatosplenomegaly, adrenal insufficiency,
Miliary tuberculosis (TB) results from widespread hematog-
and meningeal and/or focal neurologic signs may be present.
enous dissemination of MTB and is characterized by a high
Some patients may present with fulminant disease manifested
load of TB bacilli. It can be fatal if misdiagnosed. Risk factors
by multiorgan failure, septic shock, and ARDS. Differential
include age (<5 and >65 years), poor nutritional status, and
diagnosis includes pyogenic bacterial infection (eg, S aureus,
immunocompromise.
Haemophilus influenzae), endemic fungal infection (eg, histo-
The term miliary refers to numerous small, scattered pul-
plasmosis, blastomycosis), Histiocytosis X, sarcoidosis, pneu-
monary nodules (usually 1 to 2 mm in size) resembling mil-
moconiosis, hypersensitivity pneumonitis, lymphoma, and
let seeds seen on chest radiograph; this pattern of scattered
lymphangitic spread of malignancy (such as hepatoblastoma).
nodules can also be seen in other affected organs including
spleen, liver, and brain. Approximately 25% of patients have
CNS involvement, and this commonly occurs in children
Emergency Department
<5 years of age. Children may present with nonspecific symp-
toms including fever, weight loss, weakness/lethargy, nausea, Treatment and Disposition
Perform a tuberculin skin test in all suspected cases, noting
that a negative TST does not exclude the diagnosis. Blood-
based tests such as QuantiFERON-TB Gold can also be used
but are not as readily available as the TST. Obtain a chest radio-
graph, which may show the classic miliary pattern, as well as
other findings of pulmonary TB (lymphadenopathy, pleural
effusion, infiltrates, cavities). Use high-resolution chest CT
scanning when a chest radiograph is inconclusive as this is
very sensitive and specific and may detect nodules. Obtain
a head CT with contrast and/or magnetic resonance imaging
(MRI) of the brain to exclude CNS involvement. Use abdomi-
nal sonography to detect diffuse liver disease, hepatospleno-
megaly, or para-aortic adenopathy and echocardiography to

FIGURE 6.35 ■ Miliary Tuberculosis. (A) Frontal view of the chest


shows multiple tiny nodules bilaterally with more confluent lesions FIGURE 6.36 ■ Miliary Tuberculosis. Frontal chest radiograph
in the left upper lobe. Note the absence of adenopathy. (B) CT image showing innumerable bilateral lung parenchymal opacities in an
through the upper lobes confirms multiple tiny nodules bilaterally. infant who presented with fever, hepatosplenomegaly, and failure to
(Photo contributor: John Amodio, MD.) thrive. (Photo contributor: Binita R. Shah, MD.)

214
MILLIARY TUBERCULOSIS (CONTINUED) ■ 215

FIGURE 6.37 ■ Tuberculomas. An axial CT image of the brain FIGURE 6.38 ■ Tuberculomas. MRI of brain, T-1 weighted images
with contrast shows multiple ring-enhancing lesions bilaterally with postgadolinium show multiple enhancing parenchymal nodules
surrounding edema in a 5-year-old girl presenting with afebrile sei- compatible with TB granulomas in a patient who had confirmed TB
zures. She had a positive PPD with a negative chest radiograph. The meningitis. (Photo contributor: John Amodio, MD.)
most common location of tuberculoma is infratentorial at the base of
the brain near the cerebellum and they can be singular or multiple.
(Photo contributor: Binita R. Shah, MD.)

test for pericardial involvement and effusion. Obtain cultures Pearls


from any site (eg, sputum, gastric aspirate, urine, blood,
1. Miliary TB may cause disturbed regulation of antidiuretic
lymph node, CSF, other body fluids) for diagnosis and sen-
hormone and present with hyponatremia (most common
sitivity testing.
electrolyte disturbance). Less commonly, hypercalcemia
All patients with suspected miliary TB require pulmonary
may be present (like other granulomatous diseases).
consultation and hospitalization (see also page 213). Early
2. Untreated, miliary TB is almost always fatal, and most
treatment greatly reduces mortality and improves long-term
deaths occur within the first 2 weeks in hospitalized
outcome. Stabilize patients presenting with septic shock and/
patients. Early treatment of suspected cases is essential.
or ARDS and manage accordingly. Initiate antituberculous
3. Patients with major CNS involvement may have very
therapy even if all diagnostic specimens have not yet been
mild or nonspecific findings. Post-contrast head CT and/
collected. Notify the hospital infection control department
or MRI followed by lumbar puncture (without evidence
and local health department immediately as a TB-infected
of mass lesion or hydrocephalus on imaging) should be
child represents recent transmission in the community.
routinely performed.
OBSTRUCTIVE SLEEP APNEA SYNDROME

Clinical Summary episodes described as snoring followed by “choking” or


“gasping,” restless sleep, enuresis, behavioral issues, school
Obstructive sleep apnea syndrome (OSAS) is disordered
performance, daytime somnolence or fatigue, and morn-
breathing during sleep characterized by intermittent com-
ing headaches. Physical examination while awake may be
plete airway obstruction and/or prolonged partial obstruc-
normal or reveal findings related to adenotonsillar hyper-
tion. OSAS disrupts normal ventilation during sleep and
trophy such as adenoidal facies, mouth breathing, enlarged
normal sleep patterns. Predisposing conditions for OSAS
tonsils, nasal obstruction during wakefulness, and hypona-
include adenotonsillar hyperplasia (most common cause),
sal speech. Children may show failure to thrive (inadequate
obesity, and genetic syndromes with associated craniofacial
caloric intake during the day and hypermetabolic state at
anomalies such as Pierre-Robin syndrome and Down syn-
night) or obesity. In longstanding OSAS, pectus excava-
drome. Peak incidence occurs in preschool-aged children
tum, systemic hypertension, pulmonary hypertension and
between 2 and 6 years when the tonsils and adenoids are
cor pulmonale with pulmonary edema (severe cases) may
largest in relation to airway size. Nasal polyps (from severe
be seen. The main differential diagnosis for OSAS is pri-
allergic rhinitis or cystic fibrosis), nasal septal deviation,
mary snoring, which is a clinically benign condition (seen
and choanal stenosis are less common causes of OSAS.
in 7%–10% of children) and represents snoring without
Once a history of nightly snoring or worsening of snoring
obstructions, disrupted sleep, or gas exchange abnormali-
with URI is elicited, a more detailed history needs to be
ties. Primary snoring is usually exacerbated by URI but does
obtained. Inquire about repeated URIs and/or recurrent oti-
not lead to obstructive apnea.
tis media, labored breathing during sleep, observed apnea

FIGURE 6.39 ■ Obstructive Sleep Apnea Syndrome. (A) A child


presented with worsening of snoring during infection with EBV
(infectious mononucleosis). In ED, he had numerous episodes
of OSA during his sleep (cessation of airflow at nose and mouth
appreciated by listening with a stethoscope despite apparent, vigor-
ous, inspiratory efforts). His pulse oximetry was 96% while awake
and 85% during sleep. Supraclavicular, suprasternal, subcostal, and
intercostal retractions are seen. A nasopharyngeal airway was used
to relieve his obstruction. (B) Lateral view of the neck shows adenoi-
dal enlargement (A) with encroachment on nasopharynx and tonsil-
B
lar (T) enlargement. (Photo contributor: Binita R. Shah, MD.)

216
OBSTRUCTIVE SLEEP APNEA SYNDROME (CONTINUED) ■ 217

Pearls
1. OSAS is a becoming more common in childhood as rates
of childhood obesity increase.
2. The 2 most common presenting symptoms of OSAS are
snoring and sleep disturbances; however, many children
will have behavioral manifestations. Ask parents about
sleep-disordered breathing in any child with poor school
performance or behavioral problems.
3. A child with suspected OSAS must be examined during
sleep including observation of pulse oximetry readings
as physical signs of obstruction may be absent or subtle
when the child is awake.
4. Children with OSAS may have an impaired swallowing
mechanism or gastroesophageal reflux disease. Both of
these entities can lead to aspiration, especially in neuro-
FIGURE 6.40 ■ Nasopharyngeal Airway; Obstructive Sleep logically impaired children who are also at a higher risk
Apnea Syndrome. Obstructive sleep apnea resulting in significant for OSAS.
oxygen desaturation occurred in this child who had a long history
of snoring. A nasopharyngeal airway (well tolerated in children)
was required to acutely relieve the obstruction. (Photo contributor:
Binita R. Shah, MD.)
TABLE 6.2 ■ USEFUL LABORATORY TESTS IN
SUSPECTED OSAS

Hemoglobin/hematocrit:
Emergency Department • May show polycythemia from chronic nocturnal hypoxia
Treatment and Disposition Serum electrolytes:
Admit any patient presenting with cardiorespiratory failure or • Serum CO2 may be elevated from compensatory meta-
worsening of airway obstruction (eg, during URI) to the ICU bolic alkalosis (secondary to nocturnal hypoventilation
and respiratory acidosis)
for continuous cardiac and pulse oximetry monitoring. Use a
nasopharyngeal airway to bypass the obstruction, or continu- Chest radiograph:
ous positive airway pressure (CPAP) therapy to establish a • Pulmonary edema
temporary airway until a more definitive procedure such as • Cardiomegaly
an adenoidectomy and/or tonsillectomy can be performed. A • Aspiration pneumonia
short course of oral or parenteral corticosteroids can be given Neck soft tissue radiograph:
to reduce nasopharyngeal lymphoid tissue that may worsen • Nasopharyngeal airway narrowing
OSAS (eg, in patients with infectious mononucleosis). Refer • Adenotonsillar hypertrophy
patients not requiring hospitalization to their PCP to arrange
Electrocardiogram and echocardiogram:
for a polysomnogram.
• Cardiomegaly, cor pulmonale, pulmonary hypertension

Arterial blood gases:


• Awake: normal Po2 and Pco2
• Asleep: elevated Pco2 and decreased Po2
• Elevated bicarbonate from compensatory metabolic
alkalosis
ASTHMA

Clinical Features Provide supplemental oxygen to maintain O2 saturation


at or above 95%, and give inhaled short-acting beta agonists
Asthma is reversible airway hyperresponsiveness to external
(SABA) in the form of albuterol (by nebulizer or metered dose
stimuli (eg, viral infections, allergens, tobacco smoke, pol-
inhaler and valved holding chamber) for 3 doses 20 minutes
lutants, strong odors, emotions, exercise) resulting in bron-
apart in the first hour of ED management. Administer sys-
chial inflammation, edema, excess mucous production and
temic corticosteroids (oral or IV depending on the child’s
bronchoconstriction of airway smooth muscle. This leads
overall clinical status). If the exacerbation is severe, add
to airflow limitation, prolonged expiration, air trapping, and
inhaled ipratropium bromide to the initial doses of SABA.
hyperinflation. Wheezing (result of airflow limitation) is the
In life-threatening attacks, use adjunct treatments such as IV
characteristic physical finding of high-pitched, polyphonic
magnesium sulfate, continuous nebulized albuterol, and IV or
“whistling” sounds noted during an acute attack of asthma.
subcutaneous terbutaline to avoid intubation if possible.
Acute asthma is one of the most common entities treated in
Discharge children with mild to moderate attacks who
the pediatric ED. Asthma exacerbations are acute or subacute
have good response to ED management (ie, no respira-
episodes of progressively worsening shortness of breath,
tory distress, normal physical exam, response is sustained
cough, wheezing, and chest tightness, or some combination
>60 minutes after last SABA, oxygen saturation is ≥95%, and
of these symptoms. Status asthmaticus occurs when a child
peak expiratory flow [PEF] is ≥70%). Provide inhaled SABA,
continues to have significant respiratory distress and wheez-
a short course of oral corticosteroids, and close medical fol-
ing despite adequate treatment of the acute attack with appro-
low-up. Admit patients with residual mild to moderate symp-
priate bronchodilators. The differential for asthma includes
toms despite adequate ED management (ie, mild to moderate
any entity that can cause large airway obstruction (eg, foreign
wheeze and distress, oxygen saturation <95%, and PEF 40%
body aspiration, tracheomalacia, bronchomalacia, endobron-
to 69%) to the pediatric ward for continued management.
chial masses, extrabronchial obstruction from an extrabron-
chial mass or vascular ring/sling, subglottic stenosis, vocal
cord dysfunction) or small airway obstruction (eg, acute
viral bronchiolitis, bronchopulmonary dysplasia, chronic
aspiration).

Emergency Department
Treatment and Disposition
First, quickly assess the severity of the current exacerbation
with physical exam and a brief focused history including med-
ications used at home especially bronchodilators, past history
of hospitalizations, intubation, and known asthma triggers.
Next determine possible cause(s) of the current exacerbation,
and obtain a history of any known allergies. The physical
exam should include evaluation of mental status, respiratory
rate and effort, heart rate, blood pressure (including assess-
ment for pulsus paradoxus in severe exacerbations), oxygen
saturation, lung auscultation (focusing on severity and loca-
tion of wheezing), and use of accessory muscles. In children
older than 6 years, obtain peak expiratory flow with a peak
flow meter. This value can help to provide an objective mea-
FIGURE 6.41 ■ Asthma. Frontal view of the chest shows hyper-
surement of the degree of expiratory flow impairment. Order inflation, increased bronchovascular markings and peribronchial
diagnostic tests as indicated (eg, ABG, chest radiograph, cuffing in a child presenting with an acute exacerbation of asthma.
CBC, serum electrolytes). (Photo contributor: John Amodio, MD.)

218
ASTHMA (CONTINUED) ■ 219

A B

FIGURE 6.42 ■ Acute Asthma with Air-Leak Syndrome. (A, B) Frontal and lateral views of chest show extensive subcutaneous emphysema,
pneumomediastinum, right pneumothorax, and pneumopericardium. (Photo/legend contributors: Miriam Krinsky, MD/John Amodio, MD.)

A B

FIGURE 6.43 ■ Tension Pneumothorax; Asthma with Respiratory Failure. (A) Frontal view of the chest shows a large tension pneumothorax
on the left, a small pneumothorax on the right and right-sided subcutaneous emphysema in a child with asthma presenting with respiratory
failure requiring intubation. (B) Frontal view of the chest shows a left chest tube with subsequent diminution of the left tension pneumothorax
and persistent right subcutaneous emphysema in the neck. (Photo contributor: Vikas Shah, MD.)
220 ■ ASTHMA (CONTINUED)

A B

FIGURE 6.44 ■ Foreign Body Aspiration; Differential Diagnosis


of Wheezing. (A) A round radiopaque density is seen in the area
of right main stem bronchus without a mediastinal shift, air trap-
ping or distal atelectasis in a 4-year-old patient presenting with
worsening cough of 6 days’ duration and first episode of wheez-
ing. (B) Close-up of the FB in anteoposterior view. (C) Close-up
of brown metal button measuring 1 × 1 × 0.5 cm that was removed
from the right bronchus by direct bronchoscopy. (Photo contribu-
C
tor: Binita R. Shah, MD.)

Admit those with severe symptoms, PCO2 >42 mm Hg, or collateral channels of ventilation, softer rib cage, and
intubation in the ED to the ICU. All medication dosages as tendency of respiratory muscles for easy fatigability.
well as detailed ED treatment strategies are contained in the 3. Current guidelines do not advocate methylxanthines,
“Summary Report of the Expert Panel 3 Guidelines for the aggressive hydration, chest physiotherapy or mucolytics
Diagnosis and Management of Asthma” (pages 53 to 58). It is for status asthmaticus. However, adequate hydration is
strongly recommended to download this document from the required when using IV magnesium sulfate as dehydra-
following link: www.nhlbi.nih.gov/guidelines/asthma. tion can potentiate adverse drug effects such as hypoten-
sion and hypotonia.
4. Do not forget to consider aspirated foreign bodies just
Pearls because your patient has asthma. Consider this possibil-
1. Chronic severity classification of asthma (intermittent, ity in any asthmatic child who is not responding to ther-
mild persistent, moderate persistent, or severe persistent) apy and note that in such cases, chest radiograph can be
does not predict the severity of an individual exacerbation misleading as many asthmatics have atelectasis or asym-
as intermittent asthmatics can have fatal exacerbations. metric hyperinflation due to mucous plugging.
2. Infants and young children are more at risk because of 5. Monitor serum potassium as frequent dosing of albuterol
their smaller airways, limited lung elasticity, limited can lead to hypokalemia.
ASTHMA (CONTINUED) ■ 221

FIGURE 6.45 ■ Foreign Body Aspiration; Differential Diagnosis of Wheezing. Frontal chest radiograph shows unilateral hyperinflation of
the right lung with a mediastinal shift to the left. This 13-month-old infant presented with a new onset of wheezing and coughing after choking
while eating a chunky peanut butter sandwich. (Photo contributor: Binita R. Shah, MD.)

FIGURE 6.46 ■ Endobronchial Tuberculosis; Differential Diagnosis of Wheezing. Frontal chest radiograph shows right upper lobe opacity
with an elevation of the right fissure suggesting right upper lobe collapse. This infant presented with unilateral wheezing and coughing. He
was cared by a babysitter who had cavitary pulmonary disease. (Photo contributor: Binita R. Shah, MD.)

TABLE 6.3 ■ INDICATIONS FOR INTUBATION IN SEVERE STATUS ASTHMATICUS

Absolute Indications
• Cardiac/respiratory arrest
• Circulatory collapse

Relative Indications
• Respiratory muscle fatigue (paradoxical respirations; rising Pco2 (>45 mm Hg); disappearance of pulsus paradoxus)
• Falling pH (<7.25)
• Respiratory rate >40 breaths/min in a child >3 years
• Altered mental status (confused or unresponsive)
• Po2 <60 mm Hg
• “Silent chest” (obstruction is so severe that there is minimal air exchange)
PRIMARY SPONTANEOUS PNEUMOTHORAX

Clinical Summary with acute onset of chest pain (usually pleuritic in nature),
shortness of breath, and sometimes cough. Chest pain will
Pneumothorax is the presence of air or gas between the vis-
often improve after the first 2 hours even without medical
ceral and parietal pleura and occurs when air enters the pleural
intervention. Tachycardia, tachypnea, and hypoxia may be
space through the chest wall in penetrating trauma or across
present. Lung auscultation of the affected side may reveal
the lung parenchyma via the visceral pleura. Pneumothoraces
diminished or absent breath sounds and/or hyperresonance
may occur in diseased lung (secondary spontaneous pneumo-
to percussion. Tension pneumothorax is rare in PSP. The dif-
thorax), as a result of medical interventions (iatrogenic), or
ferential diagnosis is mainly the differentiation of PSP from
without any prior lung pathology or trauma (primary sponta-
secondary or iatrogenic pneumothorax.
neous pneumothorax [PSP]).
PSP is most commonly seen in adolescent males and is
Emergency Department
usually caused by rupture of asymptomatic blebs or bullae
that were not clinically evident until they resulted in PSP. Treatment and Disposition
Although Valsalva maneuvers and increased intrathoracic Take a thorough history focusing on preexisting lung diseases
pressures are described as triggering factors, PSP most com- such as asthma, tuberculosis, cystic fibrosis, interstitial lung
monly occurs at rest. Additional risk factors include a tall, disease, or congenital pulmonary airway malformation. After
lanky body habitus, smoking, and a prior history of PSP. When initial evaluation and supportive care such as oxygen, obtain
air enters the pleural space from a ruptured bleb, the lung will a chest radiograph. Use of inspiratory and expiratory chest
collapse until there is equilibrium or the air leak is sealed; films may help to better visualize the pneumothorax. CT is
therefore, as the pneumothorax enlarges, the lung volume is not generally indicated in the ED.
diminished. Young, healthy patients can tolerate the resulting Manage stable, asymptomatic patients with a small PSP
reduction in partial pressure of oxygen and vital capacity with (<3 cm from the apex of the lung to the top margin of the vis-
minimal to no respiratory distress even though the PSP may ceral pleura) in a monitored bed and provide oxygen. Perform
have been present for several days. Patients generally present needle aspiration in those that are symptomatic, but stable, with

A B

FIGURE 6.47 ■ Primary Spontaneous Pneumothorax. (A) Frontal view of chest shows large pneumothorax on left without mediastinal shift
in an adolescent male without any underlying lung pathology. (B) Follow-up study showed resolution of pneumothorax with linear atelectasis
in left upper lobe. In addition, multiple small blebs can be seen at left upper lobe apex (arrow). (Photo contributor: John Amodio, MD.)

222
PRIMARY SPONTANEOUS PNEUMOTHORAX (CONTINUED) ■ 223

A
B

FIGURE 6.48 ■ Primary Spontaneous Pneumothorax. (A) Frontal


view of the chest shows very large left pneumothorax without evi-
dence of tension in an adolescent male without any underlying lung
pathology. (B) Frontal view of the chest shows small catheter in
left hemithorax with partial re-expansion of the left lung and small
residual pneumothorax (arrow). (C) CT scan at level of lung apices
show residual pneumothorax on left with small blebs at left lung
C
apex (arrow). (Photo contributor: John Amodio, MD.)

small (<3 cm) PSP and observe for reaccumulation. Admit 2. Patients with PSP should avoid activities that cause sud-
patients with a large (>3 cm) PSP to the ICU for aspiration by den changes in intrathoracic pressure for a minimum of
pigtail catheter placement to low suction or water seal. Treat 4 to 6 weeks.
patients with recurrent PSP (defined as ipsilateral relapse after 3. Preventive intervention is not required unless patients
more than 1 month) with a chest tube, and consult the surgeon had a chest tube and thoracoscopy (as part of initial
for thoracoscopy (preferably video-assisted thoracoscopic management of PSP), failure of air leak to resolve after
surgery [VATS]) with pleurodesis and possible bullectomy/ 5 to 7 days, or a vocation that puts them at high risk for
blebectomy. Consult surgery for the first contralateral PSP. recurrence and puts themselves or others at risk (eg, deep
sea diver or airplane pilot).
4. Patients with a second ipsilateral PSP require interven-
Pearls tion to prevent recurrence.
1. All patients with prior PSP must avoid exposure to
cigarette smoke. Offer smoking cessation counseling to
patients and close family members.
PULMONARY EMBOLISM

Clinical Summary of adults with PE. A negative result makes PE unlikely; how-
ever, a positive result is not specific. Obtain an ECG, which
Pulmonary embolism (PE) is difficult to diagnose in children,
may demonstrate tachycardia or right heart strain. Obtain a
as it is rare and the classic presentation does not occur in the
chest radiograph, which is abnormal in two-thirds of cases,
majority of pediatric patients. PE is more prevalent in sickle
usually with nonspecific findings, and also can be used to
cell patients and is precipitated by dehydration and infection.
exclude pneumonia or pneumothorax. The most commonly
Other predisposing factors include nephrotic syndrome, can-
noted abnormal radiographic findings of PE are atelectasis,
cer, chemotherapy, inherited coagulable states (eg, protein C
effusions, and focal infiltrates. Two characteristic findings are
or S deficiency, lupus anticoagulant, factor V Leiden), cath-
uncommon, a localized area of hypoperfusion (Westermark
eters, surgery, trauma, pregnancy, oral contraceptive use, pro-
sign), and a peripheral wedge-shaped density above the dia-
longed immobilization, and cardiomyopathy.
phragm (Hampton hump). Use ultrasound to evaluate for DVT;
Classic presentations include pleuritic chest pain, short-
in the appropriate clinical setting a positive duplex indirectly
ness of breath, cough and hemoptysis, but as noted, these
corroborates with the presence of a PE. Echocardiography
complaints may be absent. Tachypnea is a common physical
may be used to evaluate signs of right heart strain but is a
finding; tachycardia and respiratory distress may also be pres-
late finding. CT angiography is rapidly becoming the test of
ent. Lung exam may be significant for rales but most often
choice and criterion standard for diagnosis of PE, because
is normal. Signs and symptoms of deep venous thrombo-
of its relative ease and availability, and has replaced conven-
sis may or may not be present. The classic Virchow’s triad
tional angiography and nuclear V̇/Q̇ scan. Of concern, CT
predisposing to thromboembolism consists of any state of
angiography confers significant radiation, though less than
relative stasis, prothrombogenic tendency, and injury to the
V̇/Q̇ scanning.
vascular wall. Consideration of disease processes that lead to
Provide respiratory support, definitive airway management
these factors may aid in diagnosis. Differential diagnosis of
including endotracheal intubation and ventilation as needed
PE includes more common diagnoses like pneumonia, acute
for respiratory compromise. Patients with vital sign instabil-
chest syndrome in sickle cell patients, pneumothorax, myo-
ity and hypotension refractory to fluid therapy may require
carditis, pericarditis, pleural effusion, asthma, and anxiety.
pressor support. In the setting of cardiopulmonary instability,
thrombolytic therapy (eg, tissue plasminogen activator) may
Emergency Department be considered, though it has not been specifically evaluated in
pediatric patients. Optimize precipitating factors like infec-
Treatment and Disposition tion, trauma, and heart failure. Admit patients with PE to the
Laboratory tests cannot definitively diagnose or exclude PE ICU for oxygen therapy for hypoxemia and anticoagulation
(often normal or nonspecific). ABG may demonstrate an ele- therapy like unfractionated or low-molecular-weight heparin.
vated alveolar arterial gradient. Respiratory alkalosis may be
present reflecting dyspnea and tachypnea. Conversely respira-
tory acidosis may be present reflecting ventilation-perfusion
Pearls
(V̇/Q̇) mismatch. Metabolic acidosis is an ominous sign and 1. Physical exam and laboratory testing may be nonspe-
reflects poor perfusion. D dimer testing and enzyme-linked cific, and definitive diagnosis is usually made by CT
immunosorbent assay have not been specifically evaluated in angiography.
the pediatric population. A positive D dimer is seen in 90% 2. Massive PEs may benefit from thrombolytic therapy.

224
PULMONARY EMBOLISM (CONTINUED) ■ 225

A
B

C D

FIGURE 6.49 ■ Pulmonary Embolism. (A, B, C) CT scans of the chest show multiple filling defects in the pulmonary arteries bilaterally
(arrows). (D) Doppler sonogram of the lower extremity demonstrates non compressibility of the popliteal vein (arrow), compatible with
thrombus. (Photo contributor: Johm Amodio, MD.)
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DERMATOLOGY
Chapter 7

DERMATOLOGY
Sharon A. Glick
Jeannette Jakus
Falguni Asrani
Kunal M. Shah

Linear IgA Bullous Dermatosis

The authors acknowledge the special contributions of Binita R. Shah, MD, to prior edition.

227
URTICARIA

Clinical Features and referral to an allergist can often help with management
and investigation for the trigger.
Urticaria, or hives, are pruritic raised erythematous super-
ficial skin wheals that arise in response to histamine, leu-
kotrienes, prostaglandins, and other substances released by Pearls
stimulated mast cells. Lesions develop and resolve quickly;
1. Urticaria is characterized by erythematosus, edematous
individual lesions should resolve in < 24 hours. Urticaria is
lesions that are pruritic and evanescent.
often associated with hypersensitivity reactions, including
2. If the lesions last longer than 24 hours, leave a mark, or
anaphylaxis. Symptoms are characterized as acute if they
are painful rather than pruritic, refer the patient for skin
occur daily for < 6 weeks, and chronic if they occur for
biopsy to rule out urticarial vasculitis.
longer. Triggers can include allergic reactions to drugs,
3. The underlying cause for chronic urticaria is rarely found.
foods, insect stings, and rarely aeroallergens. Urticaria can
also be caused by physical stimuli including cold, heat or
exercise (cholinergic), pressure, vibration, and sun expo-
sure. Infections including hepatitis, EBV, helminthes,
and fungi have been associated with urticaria. Patients
with collagen vascular diseases often develop urticaria.
Differential diagnosis includes mastocytosis/urticaria pig-
mentosa, erythema multiforme, bullous skin disorders, or
dermatitis herpetiformis. Obtaining a detailed history can
help find an etiology. Urticaria that has been occurring for
< 6 weeks usually does not require any laboratory workup.
Laboratory tests may be done as indicated for chronic urti-
caria. Helminth infections would be associated with very
high eosinophilia.
A

Emergency Department
Treatment and Disposition
Attempt to identify the trigger based on a thorough history.
Advise patients to discontinue or avoid the offending agent,
if known. The main form of treatment includes H1 blockers
(eg, diphenhydramine) given intravenously (IV) or orally.
Second-generation antihistamines (eg, cetirizine) can be used
once or twice a day until symptoms resolve. H2 blockers (eg,
cimetidine, ranitidine) may be combined with H1 blockers.
For patients with severe urticaria, interventions may include
epinephrine given subcutaneously, corticosteroids given IV
as hydrocortisone or methylprednisolone, or oral prednisone B
given first bolus followed by once-daily dose. Suspected
FIGURE 7.1 ■ Acute Urticaria with Chemosis; Anaphylaxis.
anaphylaxis should be treated accordingly (see page 643). Erythematous, serpiginous lesions with well-demarcated edges (A)
Patients should be educated and sent home with injectable and conjunctival edema (B) associated with wheezing were seen in
epinephrine. Urticaria can be very frustrating for the patient, this patient. (Photo contributor: Ee Tay, MD.)

228
URTICARIA (CONTINUED) ■ 229

A B

FIGURE 7.2 ■ Acute Urticaria. (A, B). An infant with acute urticaria. Etiology of lesions often is undetermined. (Photo contributor: Dawn
Davis, MD.)

FIGURE 7.3 ■ Dermographism. This “rash” was produced within 3 minutes of stroking the skin with a tongue blade. Dermographism (abil-
ity to write on the skin) is an example of physical urticaria. Triggering factors may include contact with clothing, towels, or sheets. It can also
occur as an isolated disorder. Linear pruritic wheals appear on skin within 2 to 5 minutes of stroking and usually resolve within 30 minutes
to 3 hours. Most patients are without any systemic symptoms. (Photo contributor: Binita R. Shah, MD.)
ANGIOEDEMA

Clinical Features angioedema often triggered by trauma. There are 3 genetic


types, the most common is inherited in an autosomal domi-
Angioedema is a swelling of the deeper dermal subcutane-
nant fashion; however, many patients have spontaneous
ous tissue. Commonly affected areas include the extremities
mutations. This disorder is characterized by abnormal levels
and face, specifically lips and tongue. Airway involvement
or function of C1 esterase inhibitor. Patients often present
may occur and is life-threatening. It is often painful rather
with initial attacks around puberty and many have isolated
than pruritic. Angioedema occurs secondary to release of
visceral angioedema that mimics an acute abdomen. Patients
various mediators including histamine, leukotrienes, and
often report episodes of swelling worsening over a period of
prostaglandins from stimulated mast cells or abnormalities in
12 to 24 hours, usually with resolution within 3 to 5 days.
the complement or arachidonic acid pathways. Angioedema,
The swelling may migrate to different areas. The edema is
like urticaria is often associated with hypersensitivity reac-
usually unresponsive to antihistamines. Attacks are usually
tions, including anaphylaxis. Chronic angioedema describes
periodic and are commonly followed by weeks of remission.
symptoms that last longer than 6 weeks. Triggers may include
Laboratory screening is accomplished by obtaining a C4 level
allergic reactions to drugs, foods, and insect stings. Certain
that is low during attacks. Further testing with C1 esterase
medications including ACE inhibitors often cause a non–IgE-
inhibitor level or functional C1 esterase inhibitor assays can
mediated reaction manifesting as angioedema. Hereditary
be used for confirmatory diagnosis if initial C4 screen is low.
angioedema presents as spontaneous recurrent attacks of

A C

FIGURE 7.4 ■ Angioedema. Swelling of the ear (A), periorbital area (B) following mosquito bites and swelling of the lips and face with an
urticarial rash (C), following ingestion of shellfish were the presenting complaints. Angioedema usually involves the loose connective tissues
of the ear or the periorbital or perioral areas, but may involve the oropharynx or extremities. The edema is nonpitting, well-circumscribed,
and usually nonpruritic (unless coexisting with urticaria). (Photo contributor: Binita R. Shah, MD.)

230
ANGIOEDEMA (CONTINUED) ■ 231

Emergency Department Pearls


Treatment and Disposition 1. Patients with hereditary angioedema usually do not
Anaphylaxis should be treated emergently (see page 643). For develop urticaria.
hereditary angioedema, treatment includes supportive care 2. Family history is important when considering hereditary
and pain management. Patients presenting with breathing dif- angioedema.
ficulty due to laryngeal swelling may need elective intuba- 3. Children with hereditary angioedema may present with
tion. Epinephrine or corticosteroids may help some patients. severe abdominal pain mimicking acute abdomen and
Refer the patient to an allergist for continuity of care and often undergo unnecessary abdominal surgeries.
management. Long-term treatments include synthetic andro-
gen steroids, for example, danazol and stanozolol, which may
be considered in older children after careful consideration of
the side effects.

FIGURE 7.5 ■ Angioedema. Swelling of the upper and lower eyelids with facial swelling occurred following ingestion of peanuts in this
child. (Photo contributor: Binita R. Shah, MD.)
SERUM SICKNESS/SERUM SICKNESS–LIKE REACTIONS

Clinical Summary
Serum sickness is an immune complex–meditated type III
hypersensitivity reaction, produced by exposure to a variety
of agents. It is characterized by fever, joint involvement, skin
rash, lymphadenopathy, splenomegaly, arthralgia, and pro-
teinuria. The classic serum sickness is rarely seen nowadays
and can be caused by blood products (eg, human γ-globulin)
and animal-derived serum (eg, antitoxins for treatment of
spider and snake envenomations [eg, Crotalidae antivenin],
antitoxins for clostridial intoxication [eg, botulism, gas gan-
grene], and antirabies serum). Diagnosis is suspected based
on history of an exposure to a foreign antigen.
A
Serum sickness–like reaction is a drug reaction that can
be seen 1 to 3 weeks, after exposure to the etiologic agent.
The mechanism of this reaction is not well understood. The
most commonly associated medication in children is cefaclor.
Other medications have been implicated, including penicil-
lins, cephalosporins, sulfa drugs, minocycline, propranolol,
bupropion, and griseofulvin. Cutaneous morphology can be
urticarial, morbiliform, or erythema multiforme-like. Other
features are joint swellings, arthralgia, fever, and lymph-
adenopathy. Unlike classic serum sickness, frank arthritis,
hepatic, renal and CNS involvement are rarely seen. Skin
biopsy is not necessary to make the diagnosis. Differential
diagnosis includes urticaria, erythema multiforme, vasculitis,
and drug reactions.

Emergency Department
Treatment and Disposition
The majority of serum sickness–like reactions are self-limited
and resolve in 2 to 3 weeks without sequelae in most cases.
Patients can be managed by discontinuation of the offending
agent (if still receiving it), antihistamines, and nonsteroidal
anti-inflammatory drugs as indicated. A short course of sys-
temic corticosteroids may be used in severe cases of arthral- B
gias and myalgias.
FIGURE 7.6 ■ Serum Sickness–Like Reaction. Erythematous mac-
ulopapular and urticarial edematous plaques are seen on the thighs
Pearls (A). (B) in a patient 2 weeks after receiving Bactrim. Fever, arthral-
gia, and generalized lymphadenopathy (arrow) were other findings.
1. Drugs are the most common cause of serum sickness–like Patient improved with stoppage of Bactrim and supportive care.
reaction especially the cephalosporin Cefaclor. (Photo contributor: Erin Gilbert, MD, PhD.)
2. Typical cutaneous reactions seen in serum sickness–like
reactions are urticarial, morbilliform, or erythema multi-
forme–like eruptions.

232
SERUM SICKNESS/SERUM SICKNESS–LIKE REACTIONS (CONTINUED) ■ 233

FIGURE 7.7 ■ Serum Sickness–like Reaction. Morbilliform eruption, swollen, tender knees, elevated ESR, thrombocytopenia, and mild
proteinuria were the findings in this patient while receiving cefaclor for 8 days for a sinus infection. Urticarial wheals are the most common
type of rash seen with serum sickness–like reaction followed by erythema multiforme–like lesions. (Photo contributor: Binita R. Shah, MD.)
ERYTHEMA MULTIFORME

Clinical Summary Systemic symptoms are mild or absent. Active herpes


simplex lesions or a past history of herpes may be positive.
Erythema multiforme (EM) is an acute hypersensitivity
Differential diagnosis of EM includes urticaria (lesions not
reaction that occurs in response to various etiologic agents
target-like and transient), urticaria multiforme, drug erup-
such as infections (especially Herpes simplex 1 & 2) and
tions, subacute lupus erythematosus, and vasculitis. If diag-
rarely drugs. It is self-limited but potentially recurring.
nosis is unclear, dermatology consult followed by skin biopsy
Herpes simplex virus is the most common cause; others
is indicated.
include mycoplasma, parapoxvirus, and histoplasma. EM
is classified into minor and major types, which can be dis-
tinguished by the presence or absence of mucosal lesions. Emergency Department
EM minor has no or mild mucosal involvement versus EM
major, which has severe involvement of one mucosal sur-
Treatment and Disposition
face. The classic EM lesion is a target “iris” lesion charac- No specific therapy is required for EM minor, which is a self-
terized by 3 zones. The innermost zone consists of dusky limited disorder. Acyclovir may be given if active herpes sim-
purpura, hemorrhagic crust, or blistering; the middle zone, plex lesions are present. Antihistamines, antipyretics, topical
the largest, is pale pink and edematous, whereas the outer- steroids (eg, desonide cream bid) can be given for symptom-
most zone is composed of a ring of erythema. Occasionally, atic relief. Typical patients with EM minor are usually not
atypical targetoid (composed of 2 zones only) papular severely ill and can be managed as outpatients with follow-up
lesions can be present. The classic distribution of EM with a primary care provider. Systemic steroids may be consid-
lesions is extremities (especially elbow, knees, wrists, and ered for severe cases of EM major (eg, prednisone 1-2 mg/kg/d
hands), palms and soles, with few truncal lesions. The skin divided once or twice daily for 5-14 days). Prophylactic oral
lesions are symmetrically distributed and tend to be fixed acyclovir can be used for recurrent HSV-induced EM. Prompt
for more than 5 days. treatment of HSV infections in such patients will prevent EM.

FIGURE 7.8 ■ Herpes Simplex Virus Infection and Erythema


Multiforme. (A) Primary HSV infection of the lips in a patient
who had classic target lesions of EM on the face, palms, and soles.
No other mucosal surfaces were involved. (B) Classic target “iris”
lesions of EM in a different patient following HSV infection. (Photo
contributor: Sharon A. Glick, MD [A] and Reproduced with per-
mission from Shah BR: Atlas of Pediatric Clinical Diagnosis. WB
B
Saunders, Philadelphia, 2000, p. 181. [B])

234
ERYTHEMA MULTIFORME (CONTINUED) ■ 235

A B

FIGURE 7.9 ■ Herpes Simplex Virus Infection and Erythema Multiforme. (A) A 15-year-old female with culture proven HSV labialis.
Her lips have coalescing vesicles with overlying crusts. (B) Her palm showing classic iris or target lesions. Note the 3 concentric zones with
central necrosis surrounded by a zone of edema and a third surrounding ring of erythema. She also had similar lesions on her soles. (Photo
contributor: Sharon A. Glick, MD.)

Pearls 4. Target or “iris” (with 3 concentric zones) lesions are


pathognomonic of EM; however, not all patients with
1. EM is a separate entity from Stevens-Johnson syndrome
EM will have such lesions. Some of these lesions may be
and toxic epidermal necrolysis (TEN). EM does not prog-
targetoid (with 2 zones) or papular lesions.
ress to Stevens-Johnson syndrome or TEN.
5. EM lesions are distributed mainly on the extremities
2. EM is referred to as multiforme because the morphology
including palms and soles with either none or only one
of lesions is so variable.
mucosal surface involvement.
3. EM lesions are fixed unlike urticaria, and EM lesions
6. Patients with HSV-associated EM may develop target
may expand, but they remain on the same body part for 4
lesions on the lips associated with lip necrosis that can
to 7 days.
mimic Stevens-Johnson syndrome.

A B

FIGURE 7.10 ■ Bullous Erythema Multiforme. (A, B). Bullous EM (a variant of EM) showing classic target “iris” lesions on the hand and
palm. (Photo contributor: Jeanine Daly, MD.)
STEVENS-JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS

Clinical Summary tract symptoms, skin pain, and mucocutaneous lesions. The
cutaneous lesions are targetoid with dusky purpura, truncal
Stevens-Johnson syndrome (SJS) and toxic epidermal necrol-
and central in distribution, and with a tendency to coalesce.
ysis (TEN) are part of a spectrum of hypersensitivity disor-
There may be areas of epidermal detachment and a positive
ders with similar etiologies (most commonly drugs) and can
Nikolsky sign. Mucosal surfaces of the eyes, oral cavity, and
be severe and life-threatening. SJS is the milder form and
upper gastrointestinal tract, respiratory tract, and genitals
TEN more severe. In SJS, skin and at least 2 mucosal surfaces
may show blisters, hemorrhagic crusts, painful erosions, and
are involved. SJS has severe mucosal surface involvement as
ulcerations.
compared to TEN, whereas epidermal detachment is much
Diagnosis is usually made clinically. Leukocytosis, ele-
more in TEN. Depending on area of epidermal denudation,
vated ESR, and liver enzyme abnormalities may be present.
a 3-grade classification has been proposed: SJS (<10% body
Frozen-section biopsy of the skin may be performed for con-
surface area [BSA]), overlap SJS-TEN (10%-30% BSA), and
firmation of the diagnosis. Hematoxylin-eosin (H&E) stain-
TEN (>30% BSA). The most commonly implicated drugs are
ing of the frozen section of the exfoliating skin will show a
NSAIDS, antibiotics (penicillins, sulphonamides), and anti-
full-thickness epidermal necrosis.
epileptics (phenytoin, carbamazepine).
Complications include secondary bacterial infection, fluid
The incubation period is usually 1 to 21 days and pro-
and electrolyte imbalances, and scarring and strictures of the
dromal symptoms and mucosal surface involvement may
skin and mucosal surfaces. Ocular sequelae include sym-
occur before rash and epidermal detachment begins.
blepharon, corneal ulceration, and blindness. The course is
Clinical features include fever, malaise, upper respiratory

A B

FIGURE 7.11 ■ Stevens-Johnson Syndrome. (A) A 7-year-old


girl with this rash while receiving penicillin (eighth day) for phar-
yngitis. Nikolsky sign was positive with peeling of the epidermis
(seen on the face) with minimal pressure. (B) Close-up of erosive
stomatitis with hemorrhagic-crusted erosions of lips. Erosions were
also present on the buccal mucosa, palate, posterior pharynx, and
conjunctiva. (C) Erythematous and purpuric macules are seen on
the back with blistering leading to erosions on the buttocks. Less
than 10% of the body surface area was involved. (Photo contributor:
C
Binita R. Shah, MD.)

236
STEVENS-JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS (CONTINUED) ■ 237

protracted over 3 to 6 weeks with a mortality rate ranging is controversial (risk of increased morbidity and mortality;
from 1% to 5% for SJS and 15% to 35% for TEN. hence routine use is discouraged). The current and newly rec-
ommended therapy is IV IG given as 1 g/kg/d daily for 3 days.
It helps to reduce development of new blisters and helps to
Emergency Department arrest the progression of the disease. (IV IG should be free of
Treatment and Disposition any IgA to prevent hypersensitivity reaction in IgA-deficient
individuals.)
Hospitalize all patients with suspected diagnosis of SJS or
TEN preferably to a burn unit or ICU. Discontinue all drugs
introduced within the past month, and do not introduce drugs Pearls
of a similar class unless the risk of abrupt discontinuation is 1. Most common etiology for SJS and TEN are drugs,
too high. For example, phenobarbital, carbamazepine, and while most common etiology for EM is herpes simplex
phenytoin share a common metabolic pathway (cytochrome virus.
P450) and may induce a cross-reaction; thus, all 3 drugs are 2. In children, SJS is more commonly seen than TEN.
contraindicated after SJS/TEN resulting from any one of Incidence of TEN is more common in human immunode-
them. Obtain urgent dermatology, ophthalmology, urology, ficiency virus (HIV) patients.
and ENT consultations. Supportive care includes manage- 3. Differential diagnosis of TEN includes staphylococcal
ment of fluid and electrolytes, pain, thermoregulation, nutri- scalded skin syndrome (SSSS). TEN is full-thickness
tional support, and meticulous skin care (gentle saline soaks epidermal necrosis and denudation, whereas SSSS is a
and petrolatum gauze to provide barrier over denuded areas). superficial peeling of skin (within the epidermis, below
Do not use silver sulfadiazine in patients with sulfonamide- the stratum corneum). Hence, complications are much
induced SJS or TEN. Provide reverse isolation to prevent more grave and severe in TEN.
secondary bacterial infection. Prophylactic use of systemic 4. Both SJS and TEN are potentially life-threatening dis-
antibiotics is not recommended. Systemic corticosteroid use eases because of multisystem involvement.

FIGURE 7.12 ■ Stevens Johnson Syndrome. Targetoid lesions with 2 zones consisting of central purpura and outer red/pink zone of ery-
thema and edema on the trunk of a patient following exposure to penicillin. This was followed by evidence of epidermal denudation. (Photo
contributor: Eve Lowenstein, MD, PhD.)
238 ■ STEVENS-JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS (CONTINUED)

A B

C D

FIGURE 7.13 ■ Toxic Epidermal Necrolysis. A 3-year-old boy with TEN (70% BSA involvement) with this rash a week after taking phe-
nobarbital for recurrent febrile seizures. (photographs taken between second and third day of illness). (A) Bilateral eye involvement with
mucopurulent discharge. (B) Erosive stomatitis and hemorrhagic crusted erosions of the lips. (C, D). Abrupt onset and worsening within
24 hours with dark necrotic skin peeling off, leaving a raw dermis. (Photo contributor: Binita R. Shah, MD.)
STEVENS-JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS (CONTINUED) ■ 239

A B

FIGURE 7.14 ■ Toxic Epidermal Necrolysis. (A) Five-year-old girl with this rash (100% BSA involvement) while receiving trimethoprim-
sulfamethoxazole (10th day) for a urinary tract infection (photographs taken on seventh day of illness). (A) Extensive involvement of the face
with necrotic skin is seen. (B) Close-up of mucosal involvement of the lips showing painful hemorrhagic erosions. (Photo contributor: Binita
R. Shah, MD.)

FIGURE 7.15 ■ Toxic Epidermal Necrolysis. Large tense bullae with erythema and skin tenderness were seen in a patient following expo-
sure to a cephalosporin. Nikolsky sign was positive and the bullae progressed to epidermal denudation in the next few hours. (Photo contribu-
tor: Deeptej Singh, MD.)
DRUG-INDUCED HYPERSENSITIVITY SYNDROME

Clinical Summary is considered an adverse eruption to medications or vaccine


administration. The closest clinical differential diagnosis is a
Drug-induced hypersensitivity syndrome (DIHS) is a subset
viral exanthem. Eosinophilia is more common in drug erup-
of adverse drug eruptions, which occur 1 to 21 days after
tion than viral exanthems.
starting a new medication, whereas it takes only a few days
for the reaction to occur following second exposure to the
same class of medication. The most commonly implicated Emergency Department
drugs are antibiotics (penicillins, sulfonamides), antiepilep-
tics (phenytoin), NSAIDS, antihypertensives, and anti-HIV
Treatment and Disposition
medications, though any drug can cause an adverse reaction. The diagnosis is clinical, and a detailed current and previ-
Cross-reactivity is common among different drugs of same ous drug history with time charting is mandatory to pin-
class of medications. Drug reactions are more common in point the offending drug. If clinical suspicion of an adverse
HIV-positive individuals. Exanthematous (maculopapular or drug reaction is high, discontinue the suspected offending
urticarial) reaction is seen most commonly, and has a wide- drug. Laboratory tests include CBC, liver enzymes, and
spread, bilateral and symmetric distribution that may involve urinalysis. Hospitalize children with severe skin findings,
the palms and soles. Fever, malaise, and mild mucosal sur- evidence of systemic involvement, or inability to maintain
face involvement may be the presenting symptoms. In a vari- hydration (eg, during erythrodermic phase). A dermatology
ant known as drug reaction with eosinophilia and systemic consultation would assist in such decisions. Biopsy of the
symptoms (DRESS), the exanthematous drug rash is associ- skin reveals a dense infiltrate of the papillary dermis, which
ated with fever, facial edema, and involvement of at least one is composed mostly of lymphocytes and occasionally of
internal organ (liver commonest) with eosinophilia. The incu- eosinophils. Treatment is mostly supportive and includes
bation period of DRESS is usually 2 to 6 weeks and it takes skin care (eg, topical steroids to alleviate symptoms), pre-
weeks to subside, even after drug withdrawal. Another vari- vention of infection, and treatment of any complications.
ant is acute generalized exanthematous pustulosis (AGEP), Oral or IV steroids are sometimes used in patients with
which is an acute eruption characterized by multiple tiny severe systemic involvement and in DRESS, to prevent
pustules that heal in 4 to 10 days with desquamation. This complications.

A B

FIGURE 7.16 ■ Drug-Induced Hypersensitivity Syndrome. (A, B). Morbilliform maculopapular drug hypersensitivity reaction seen over the
trunk and leg in a child following exposure to azithromycin. The rash improved after stoppage of the drug and supportive management. No
mucosa was involved. (Photo contributor: Julie Cantatore, MD.)

240
DRUG-INDUCED HYPERSENSITIVITY SYNDROME (CONTINUED) ■ 241

A B

FIGURE 7.17 ■ Drug-Induced Hypersensitivity Syndrome/DRESS.


This child presented with an extensive papular rash occurred 35 days
after starting phenytoin for seizure disorder. Papular lesions are seen
on both legs (A), and arms (B), close-up shows indurated nature of
C
rash (C). (Photo contributor: Binita R. Shah, MD.)

A B

FIGURE 7.18 ■ Drug-Induced Hypersensitivity Syndrome/DRESS. (A) Patient presented with fever, facial edema, lymphadenopathy, a
monomorphous rash all over the body, and elevated liver enzymes requiring hospitalization. He was treated with a slow taper of oral steroids.
(B) Close-up of the monomorphous rash. (Photo contributor: Sharon A. Glick, MD.)
242 ■ DRUG-INDUCED HYPERSENSITIVITY SYNDROME (CONTINUED)

FIGURE 7.19 ■ Drug-induced Hypersensitivity Syndrome. Acute generalized exanthematous pustulosis (AGEP). Patient presented with
fever and an acute eruption of multiple sterile pustules all over the face, extremities, and trunk following exposure to an antibiotic 2 weeks
prior. The lesions healed with desquamation following stoppage of the antibiotic. (Photo contributor: Sharon A. Glick, MD.)

FIGURE 7.20 ■ Drug-Induced Hypersensitivity Syndrome (DIHS)


versus Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis.
(A) Mucous membrane involvement in patients with DIHS is either
minimal or absent. (B) Extensive mucous membrane involvement
is seen in patients with Stevens-Johnson syndrome/toxic epidermal
A
necrolysis. (Photo contributor: Binita R. Shah, MD.)
DRUG-INDUCED HYPERSENSITIVITY SYNDROME (CONTINUED) ■ 243

FIGURE 7.21 ■ Drug-Induced Hypersensitivity Syndrome (DIHS)


versus Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis.
(A) Absence of necrolysis in spite of very extensive skin involve-
ment is seen in DIHS. (B) Vesicles, bullae, or purpuric lesions and
necrolysis are typical findings in Stevens-Johnson Syndrome/toxic
A
epidermal necrolysis. (Photo contributor: Binita R. Shah, MD.)

Pearls 2. AGEP is an acute exanthematous drug eruption, char-


acterized by fever, multiple sterile pustules that subside
1. DIHS is often misdiagnosed as a viral infection. Prompt
spontaneously with desquamation. In children, it occurs
recognition and discontinuation of the drug is of the
following a viral exanthem, vaccine administration, or
utmost importance in preventing or minimizing the sever-
rarely drugs.
ity and duration of internal organ involvement.
HENOCH-SCHÖNLEIN PURPURA

Clinical Summary evaluate scrotal problems. In doubtful cases, skin biopsy


helps to confirm leukocytoclastic vasculitis.
Henoch-Schönlein purpura (HSP) is a small vessel vasculitis
Complications include bowel obstruction or perforation,
characterized by palpable purpura, arthritis, gastrointestinal
intussusceptions, nephrotic syndrome, azotemia, oliguria,
(abdominal pain), and renal manifestations (glomerulonephri-
chronic glomerulonephritis, hypertensive encephalopathy,
tis) and seen in children age 2 to 11 years. About 50% of
central nervous system (seizures, coma, paresis), and acute
patients have preceding respiratory infection; mainly group
scrotum (mimicking acute testicular torsion).
A beta hemolytic streptococcal infection. Other implicated
pathogens include hepatitis B virus, adenovirus, mycoplasma,
herpes simplex virus, parvovirus, and HIV. Certain foods, Emergency Department
drugs, exposure to cold weather, and insect bites are other
predisposing factors. Palpable purpura on lower extremities
Treatment and Disposition
bilaterally is the presenting sign, and new lesions may develop There is no specific treatment. The majority of cases are
in crops. This may be associated with fever, joint swelling and self-limiting, and symptoms resolve in a few weeks with-
arthralgia, abdominal pain, bloody stools, and hematuria. out permanent residua. Supportive, symptom-directed treat-
Diagnosis is clinical. CBC may show leukocytosis, ment includes bed rest, bland diet, and NSAIDs for relief
thrombocytosis, and anemia. ESR may be elevated and of arthritis and fever. Elevation of the scrotum helps scrotal
serum complement levels depressed. Antistreptolysin anti- edema. Hospitalize any patient presenting with complica-
bodies are positive in about 50% of cases. Perform urinaly- tions (eg, hypertension, oliguria, intestinal obstruction, or
sis (may show gross or microscopic hematuria, proteinuria, gastrointestinal bleeding). Remove the offending antigen,
white blood cells, and casts), and stool guaiac to screen for if identified (eg, drugs). Treat the infection if identified
renal and gastrointestinal involvement. Use ultrasound to (eg, group A B-hemolytic streptococcus). Systemic corti-
evaluate for intussusception and Doppler flow studies to costeroids are indicated for systemic involvement such as

A B C

FIGURE 7.22 ■ Henoch-Schönlein Purpura (HSP). (A) A 6-year-old boy with crops of palpable purpura on lower extremities and arthritis.
(B) Shown here is sparing of the trunk. Lesions in HSP can also appear on the arms, face, and ears. (C) Close-up of purpura and swollen right
ankle. (Photo contributor: Binita R. Shah, MD.)

244
HENOCH-SCHÖNLEIN PURPURA (CONTINUED) ■ 245

severe arthritis, renal involvement, gastrointestinal tract


complications, and acute scrotum or CNS complications.
Corticosteroid therapy does not prevent recurrences, which
are usually seen in up to 50% of cases within the first few
months (milder and more common in patients with nephri-
tis). Long-term morbidity and mortality is attributed almost
exclusively to renal disease. Serial urinalyses and follow-up
of renal functions are recommended, if the initial tests show
any abnormalities.

FIGURE 7.24 ■ Henoch-Schönlein Purpura. A teenager with crops


Pearls of palpable purpura on the lower extremities. Patient also had mal-
1. HSP is the most common cause of nonthrombocytopenic aise, fever, and arthralgia. (Photo contributor: Sharon A. Glick, MD.)
purpura.
2. The classic finding is palpable purpura seen most promi-
nently on the lower extremities.
3. Long-term morbidity and mortality is attributed almost
exclusively to renal involvement.
4. Arthritis or gastrointestinal symptoms may precede the
appearance of rash by up to 2 weeks.
5. Testicular involvement occurs in up to 35% of patients,
and acute scrotal swelling may present prior to purpura
and can mimic acute testicular torsion or incarcerated
inguinal hernia.

FIGURE 7.25 ■ Henoch-Schönlein Purpura with Vasculitis.


FIGURE 7.23 ■ Henoch-Schönlein Purpura. Typical distributions Purpuric lesion with edema on ear can be mistaken for child abuse,
of palpable purpuric lesions. Angioedema on the right foot is also especially when ear involvement precedes purpura. (Photo contribu-
seen. (Photo contributor: Binita R. Shah, MD.) tor: Binita R. Shah, MD.)
SEBORRHEIC DERMATITIS

Clinical Summary mineral or olive oil compresses followed by washing several


hours later and lifting off the scale with a fine-toothed comb
Seborrheic dermatitis is an inflammatory disorder commonly
may be helpful for scales that are dense, thick, and adherent.
seen in infancy and adolescence. Affected areas include the
For older children and adolescents, antiseborrheic shampoos
scalp, face, retroauricular areas, mid-chest, axillae, groins,
containing selenium sulfide (eg, Selsun blue), zinc pyrithione
and other intertriginous areas where sebaceous glands are
(eg, Head & Shoulders), coal tar (eg, Neutrogena T/Gel), or
prominent. It is characterized by an erythematous, greasy
ketoconazole (eg, Nizoral) can be used intermittently (eg,
scaly scalp (cradle cap). Lesions may be present in the diaper
2-3 times per week). Instruct the patient/parent to lather the
area including folds (which is spared in atopic dermatitis).
shampoo and let it sit on the scalp for 5 to 10 minutes before
Petaloid erythematous noneczematous scaly oval plaques may
rinsing. If there is evidence of inflammation, application of a
be present on the trunk, back, and flexures. Secondary monil-
topical low- to mid-potency corticosteroid solution to the scalp
ial infection (due to colonization with Candida albicans) and
and cream to nonhairy inflamed areas, is indicated. Caution
secondary bacterial infections are common, especially in the
patients that topical steroids should not be used as mainte-
diaper area. Peak occurrence in infancy is at 3 months of age
nance therapy. Topical antifungal agents are also effective.
and usually starts to resolve around 8 to 12 months of age.
Differential diagnosis includes atopic dermatitis, Langerhans
cell histiocytosis, and immunodeficiency disorders. Overlap
Pearls
of atopic dermatitis and seborrheic dermatitis is quite com- 1. Seborrheic dermatitis is the most common rash in the first
mon in infants. Lack of response to topical steroids in sus- few months of life.
pected atopic dermatitis in an infant should raise the suspicion 2. Suspect coexisting atopic dermatitis when lesions are
of seborrheic dermatitis. weeping and associated with pruritus.
3. With pronounced localized scaling, seborrheic dermatitis
Emergency Department may resemble psoriasis.
4. Seborrheic dermatitis is one of the most common cutane-
Treatment and Disposition ous manifestations of AIDS in older children and adoles-
For infants, minor amounts of scale are easily removed by fre- cents, and onset usually occurs before AIDS symptoms.
quent shampooing with products containing sulfur, salicylic 5. Seborrheic dermatitis is a self-limiting disorder and does
acid (eg, Sebulex shampoo), or ketoconazole shampoo. Warm not cause permanent or scarring alopecia of the scalp.

FIGURE 7.26 ■ Seborrheic Dermatitis. Greasy scaling present in


the scalp of child with seborrheic dermatitis. Tinea amiantacea refers FIGURE 7.27 ■ Seborrheic Dermatitis. Erythematous scaly plaques
to seborrheic dermatitis of the scalp with dense patches of yellow- seen classically distributed in the folds like axillae, antecubital fos-
white scale firmly adherent to the hair shafts. (Photo contributor: sae in an infant. Greasy scaling was present in the scalp. (Photo con-
Jeannette Jakus, MD.) tributor: Jeannette Jakus, MD.)

246
SEBORRHEIC DERMATITIS (CONTINUED) ■ 247

A B

FIGURE 7.28 ■ Seborrheic Dermatitis. (A) Greasy papular eruption seen on the face, around the ears, and scalp. (B) Close-up of scalp show-
ing scaly lesions. (Photo contributor: Binita R. Shah, MD.)

FIGURE 7.29 ■ Seborrheic Dermatitis. Well-defined scaly, greasy plaque seen within the nasolabial folds of a 9-year-old boy. This is an
early manifestation of adolescent seborrheic dermatitis. (Photo contributor: Sharon A. Glick, MD.)
ALLERGIC CONTACT DERMATITIS

Clinical Summary Emergency Department


Allergic contact dermatitis results from exposure to a topical Treatment and Disposition
allergen by a sensitized individual. Percutaneous absorption
Once allergic contact dermatitis has been diagnosed, iden-
of an allergen leads to specific T lymphocytes carrying the
tification and elimination of the allergen (eg, removal of
immune memory to recognize the antigen as a foreign mate-
nickel jewelry), and avoidance of further exposure is neces-
rial. Re-exposure to the same allergen results in prolifera-
sary. Antihistamines are used (eg, hydroxyzine) to control
tion of sensitized T lymphocytes that release inflammatory
the pruritus. Avoid use of topical diphenhydramine, as it is
mediators, leading to a localized eczematous dermatitis. The
a sensitizer (with prolonged use, the patient may develop
most common contact allergens include plants (eg, urushiol
sensitization). Topical high- or mid-potency corticosteroid
in poison ivy, poison sumac, poison oak), nickel (eg, jewelry,
cream applied twice a day for 2 to 3 weeks, with avoidance
metal wrist band, snap buttons of trouser jeans), shoe materi-
of allergen, treats the condition. Rarely, systemic steroids
als (eg, rubber and leather [potassium dichromate used for
may be needed (eg, in cases caused by poison ivy, predni-
tanning leather]), thimerosal (a preservative in eye drops and
sone 1-2 mg/kg/d tapered over 2 to 3 weeks, helps to control
vaccines), fragrances, cosmetics, and topical medications (eg,
the acute inflammation). For poison ivy, if possible provide/
preparations containing neomycin). Allergic contact derma-
titis is seen in children and first exposure to an allergen may
occur as early as in infancy.
Diagnosis is based on distribution and morphology of the
lesions (eczematous, vesiculation, oozing, and in chronic
lesions lichenification) and history of exposure. Sharp demar-
cation between normal and affected skin, with linearity,
suggests allergic contact diagnosis. A patch test, done by a
dermatologist (if etiology not apparent), can help with a diag-
nosis. A positive test shows erythema and papules confined
to the test site.

FIGURE 7.30 ■ Allergic Contact Dermatitis. Contact blisters FIGURE 7.31 ■ Allergic Contact Dermatitis. Multiple itchy ery-
with erythema present on bilateral dorsum of feet, in a patient thematous papules developed on the areas of contact with neomycin,
with allergy to shoe material (most likely rubber). Avoidance of which was used to treat the wound infection. Avoidance of neomycin
the specific material cures the condition. (Photo contributor: Julie and topical moderate potency steroids was advised. (Photo contribu-
Cantatore, MD.) tor: Jeannette Jakus, MD.)

248
ALLERGIC CONTACT DERMATITIS (CONTINUED) ■ 249

show a picture of the offending plant for patients and family 3. A thorough history is the most important step in man-
members so they can avoid further exposure. When outdoors, agement and includes inquiring about animal exposure.
advise using a barrier cream (eg, Ivy Shield), which blocks Poison ivy dermatitis can result from touching an animal
the resin from touching the skin and washing the exposed exposed to the plant.
area immediately, if accidental exposure occurs. Recurrences 4. Autosensitization dermatitis (Id eruption) occurs as acute
are common if the suspected allergen is not eliminated com- papulovesicular pruritic symmetric eruptions on the
pletely; thus all the ingredients (even in the minutest of pro- trunk, forearms, extensor surfaces, and rarely on the face
portions) in the suspected allergen should be avoided. and is a hypersensitive reaction to acute dermatitis and
bacterial or fungal infections.
5. Koebner phenomenon (isomorphic phenomenon), in
Pearls
which trauma elicits similar lesions, is also seen in an Id
1. Diagnosis is based on the history of exposure and the pat- eruption. The diagnosis is clinical and treatment of the
tern of the rash (eg, a symmetrical erythematous rash on primary dermatitis is crucial. Open wet compresses, anti-
both ear lobes in a patient wearing nickel earrings). histamines, and topical steroids may also help.
2. One of the most common contact allergens in chidren is
urushiol, the sensitizing antigen found in poison ivy.

A B

FIGURE 7.32 ■ Allergic Contact Dermatitis. (A) Adolescent with


recurrence of poison ivy dermatitis seen on face and neck. Premature
termination of oral corticosteroids (patient treated for 3 days only),
as occurred in this case, may result in rebound dermatitis. In severe
cases, it is important to treat with a steroid taper over 2 to 3 weeks.
(B) Erythematous linear streaks of urticaria on the abdomen due to
spread of urushiol oil. (C) Linear array of vesicles of the fingers.
C
(Photo contributor: Sharon A. Glick, MD.)
ATOPIC DERMATITIS

Clinical Summary Emergency Department


Atopic dermatitis (AD) is an eczematous dermatitis mani- Treatment and Disposition
fested as a result of persistent inflammation of the skin. It
The majority of patients with AD are treated as outpatients.
is often associated with allergic diseases like asthma and
Management consists of establishing realistic parental expec-
allergic rhinitis and manifests as acute, subacute, and chronic
tations that AD is a chronic disorder that involves remissions
dermatitis. Diagnosis is based on classic morphology and
and exacerbations. Goals of therapy are to control the flare-
distribution of dermatitis depending on the age group. The
ups, prevent secondary infections, and keep the skin barrier
infantile phase is from 2 months to 2 years, characterized by
function intact by constant moisturizing. Advise patient to
severe pruritus, oozing, vesiculation, eczematous papules,
avoid trigger factors like hot water, heat, allergens like dust
plaques seen on the face, scalp, extensors of extremities, and
mites, pollens, molds, foods like eggs and peanuts, materi-
trunk. During infancy, the sparing of groin and diaper area
als like wool and fur, exposures to animals (eg, dogs, cats)
helps to differentiate infantile atopic dermatitis from sebor-
or items filled with feathers or down (eg, pillows), stress,
rheic dermatitis. The childhood phase lasts from 2 years of
and infections (eg, Staphylococcus aureus, herpes simplex,
age to puberty and is characterized by dry scaly, lichenified
dermatophytes). Advice on constant and regular care of the
pruritic plaques, seen on the antecubital fossae, popliteal fos-
dry and xerotic skin includes daily moisturizing, brief baths
sae, periorbital and perioral areas, wrists, hands, and feet. The
or showers in lukewarm water lasting not more than 5 to
adult phase begins at puberty and continues into adulthood
10 minutes, use of a mild unscented soap or liquid gentle
and is characterized by chronic pruritic lichenified plaques
skin cleanser (eg, Dove fragrance-free soap, Aveeno), patting
present on the flexors, neck, face, trunk, hands, and feet.
(don’t rub) the skin dry, leaving some moisture. Moisturizers
Nummular eczema is a variant of AD characterized by dis-
(eg, Vaseline, Aquaphor) are applied all over the skin, as soon
coid, well-circumscribed, annular plaques, commonly seen
as the child gets out of the bath or shower to trap moisture in
on the extremities. Associated features of AD include pity-
the skin. Moisturizers should be applied to the entire body
riasis alba, periorbital darkening, ichthyosis vulgaris, white
2 to 3 times a day. Mid- to high-potency topical steroids are
dermographism, keratosis pilaris, and hyperlinear palms.
used for body areas (except groin, face, axillae). Mild topical
Elevated serum IgE with peripheral eosinophilia may be seen.
steroids are used for face, groins, and axillae but not more
Secondary bacterial and viral infections are common.
than 2 to 3 weeks at a stretch. Topical antibacterial agents (eg,
mupirocin) can be used if there is any evidence of excoria-
tions or open wounds. If the infection is severe, oral antibiot-
ics for 7 to 10 days can be used. Topical immunomodulators
like pimecrolimus 1% or tacrolimus 0.03% or 0.1% ointment
can be used as steroid-sparing agents. Antihistamine (eg,
hydroxyzine) is used for control of the severe itch that causes
the rash. Patient should be referred to a primary care physi-
cian or dermatologist for ongoing care.

Pearls
1. Atopic dermatitis is the “itch that rashes.” Pruritus, xero-
sis, and eczema are prominent features of AD.
2. Hospitalization is indicated for eczema herpeticum, gen-
FIGURE 7.33 ■ Nickel Contact Dermatitis. Nickel sulfate found in eralized erythroderma, severe AD with compromised
a belt buckle was responsible for this rash that was treated with ste- skin barrier causing failure to thrive or severe flare-ups
roid cream without success because patient continued to wear belt.
unresponsive to conventional treatment.
Contact dermatitis from nickel snaps on denim and from belt buckles
is often mistaken for eczema (like in this patient). This patient’s rash 3. Moisturizers form the mainstay of therapy. Topical ste-
improved only after discontinuation of wearing the belt. (Photo con- roids, antibacterials, and avoidance of trigger factors are
tributor: Binita R. Shah, MD.) the other pillars of the therapy.

250
ATOPIC DERMATITIS (CONTINUED) ■ 251

A B

FIGURE 7.34 ■ Infantile Atopic Dermatitis. Involvement of forehead, scalp, and both cheeks with sparing of the nose (“headlight sign”) (A),
and distribution of lesions in more extensor than flexor surfaces (B) are seen. (Photo contributor: Binita R. Shah, MD.)

FIGURE 7.35 ■ Atopic Dermatitis. Well-defined erythematous plaques, topped with thick scales, seen on the cheeks of an infant. Cheeks
are a common site of atopic dermatitis in children under 2 years of age. (Photo contributor: Morgan Rabach, MD.)
252 ■ ATOPIC DERMATITIS (CONTINUED)

FIGURE 7.37 ■ Atopic Dermatitis (AD). Pruritic hyperpigmented,


lichenified scaly plaques on the popliteal fossae in a patient with
chronic AD. The rubbing caused the lichenification. (Photo contrib-
utor: Sharon A. Glick, MD.)

FIGURE 7.36 ■ Atopic Dermatitis. Well-defined hyperpigmented,


lichenified, pruritic plaques of eczema are seen in the antecubital
fossae (a classic site). Typical morphology of chronic atopic derma-
titis is seen due to constant itching and rubbing. (Photo contributor:
Sharon A. Glick, MD.)

FIGURE 7.38 ■ Atopic Dermatitis (AD). Acute on chronic dermatitis is commonly seen in long-standing AD. A wet lichenified plaque with
hyperpigmentation, scaling and fissuring is seen on the forehead. Secondary bacterial infection is common in these plaques. (Photo contribu-
tor: Falguni Asrani, MD.)
ATOPIC DERMATITIS (CONTINUED) ■ 253

FIGURE 7.39 ■ Generalized Erythroderma. An infant with AD


who developed generalized erythroderma. The upper layer of the
skin is exfoliating in pieces on a background of generalized ery-
thema. (Photo contributor: Binita R. Shah, MD.)

FIGURE 7.41 ■ Atopic Dermatitis With Secondary Bacterial


Infection. A lichenified plaque with hyperpigmentation, scaling,
and fissuring is seen on the lower extremity. Fissuring and exudation
suggest secondary bacterial infection. (Photo contributor: Binita R.
Shah, MD.)

FIGURE 7.40 ■ Atopic Dermatitis. Nickel dermatitis. Well-defined


hyperpigmented lichenified plaque seen on the waist, below the
umbilicus (a classic site for nickel dermatitis), in an atopic patient
due to constant contact with nickel in the buttons or belts touching
the skin. (Photo contributor: Sharon A. Glick, MD.)
ECZEMA HERPETICUM

Clinical Summary fluorescent antibody assays, Tzanck smear, viral culture, and
viral DNA polymerase chain reaction for HSV. Complications
Eczema herpeticum (EH) is a disseminated cutaneous herpes
include secondary bacterial infection (Streptococcus pyogenes
simplex virus (HSV) infection superimposed on a preexisting
and S aureus), keratoconjunctivitis, viremia, and scarring.
active skin condition. A likely cause is the impaired skin bar-
rier in active atopic dermatitis, which allows viral particles to
incubate and proliferate quickly, leading to severe dissemina- Emergency Department
tion. EH is most commonly caused by HSV-1, and less com-
monly by HSV-2. Though most commonly seen in patients
Treatment and Disposition
with atopic dermatitis, it can also occur with other primary der- High index of suspicion for EH in patients presenting with a
matologic disorders (eg, seborrheic dermatitis, irritant contact history of “worsening” atopic dermatitis is an important step
dermatitis, pemphigus, burns, ichthyosis vulgaris, skin grafts). necessitating DFA or Tzanck smear (DFA preferred over
A history of contact with a family member with recurrent oral/ Tzanck because of superior sensitivity, rapid turnover) and viral
facial HSV infection is often found. Clinically, monomorphous culture, if diagnosis is uncertain. Dermatology consultation
vesicles, pustules, and erosions may be seen on a preexisting will be valuable in such cases. Ophthalmology consultation is
dermatitis. There may be multiple punctate erosions, some mandatory for involvement of or near the eyes. Hospitalization
coalescing to form larger polycyclic erosions. EH is more com- of infants and young children with EH is indicated in severe
monly seen on the face, neck, and chest areas and can lead to cases to prevent complications with prompt administration
fatality in infants and young children, if not treated promptly. of IV acyclovir till lesions crust over. Maintaining hydration
The diagnosis is usually clinical but can be confirmed by direct and skin care are also important. Bland emollients may be

A B

FIGURE 7.42 ■ Eczema Herpeticum. (A) Acute eruption of multiple erythematous vesiculopapular lesions with central crusting is seen on
the right cheek of a young boy with atopic dermatitis. As lesions involve the face and are close to the eye, an urgent ophthalmology consult
is indicated to exclude corneal involvement. (B) Erythematous vesiculopapular lesions around the eyes and mouth with crusting are seen in a
different infant. (Photo contributors: Falguni Asrani, MD. [A] and Christy Riley, MD [B].)

254
ECZEMA HERPETICUM (CONTINUED) ■ 255

applied to the affected areas. Mild cases of EH can be man-


aged as outpatients with acyclovir given orally with a very
close follow-up by a primary care physician or a dermatologist.
Antistaphylococcal antibiotics should be given along with acy-
clovir either IV (severe EH requiring hospitalization) or oral or
topical mupirocin may be used with early localized mild cases
not requiring hospitalization.

Pearls
1. “Punched out” erosions superimposed on a preexisting
active dermatitis-like atopic dermatitis are important clues.
2. Suspect EH when infected-appearing eczema does not
respond to appropriate antibiotic therapy; this may indi-
FIGURE 7.43 ■ Eczema Herpeticum. Acute eruption of multiple
punctate erosions on the face and neck of an adolescent girl with cate HSV infection.
recalcitrant atopic dermatitis. These punctate erosions also coalesced 3. Appropriate acyclovir dosing and skin care is key to
to form large polycyclic erosions. Viral culture was positive for HSV, prompt recovery from EH.
type 1. (Photo contributor: Haamid Chamdawala, MD.)

A B

FIGURE 7.44 ■ Eczema Herpeticum. (A) Widespread “punched-out” lesions on upper trunk, neck, and extremities in a highly febrile
4-month-old infant with atopic dermatitis. The mother suffered from recurrent cold sores (herpes simplex labialis), and had cold sores 5 days
prior to the appearance of this infant’s rash. (B) Close-up of the “punched-out” lesions. (Photo contributor: Binita R. Shah, MD.)
PSORIASIS

Clinical Summary steroid creams on other body parts (not more than 2 weeks).
High-potency steroid creams (not for more than 2 weeks)
Psoriasis is a chronic polygenic skin disorder characterized
are reserved for older children and adolescents with severely
by well-circumscribed, erythematous plaques with an over-
affected areas. Consider antibiotics (eg, penicillin, cepha-
lying silvery-white scale affecting about 2% of the general
lexin) when a pharyngitis or perianal cellulitis is suspected
population. The most common clinical variant is plaque pso-
as precipitating a guttate flare (see Figure 7-51) and obtain
riasis. Patients often experience a waxing and waning course.
cultures for confirmation. Refer all patients to dermatology.
Precipitating factors include minor trauma (Koebner phe-
Other therapeutic options in refractory cases include topical
nomenon) infection, stress, and drugs. In the severe form of
vitamin D analogue creams or retinoids, phototherapy, or
generalized pustular psoriasis, patients are often ill-appearing
oral therapies that include, retinoids, methotrexate, or cyclo-
with fever, generalized erythema, and pustules. Onset is acute
sporine. Biologic agents (ie, etanercept, infliximab) are the
and most patients have an antecedent nonpustular psoriasis
newest class of medications being used for treatment.
or a genetic predisposition. Episodes may be precipitated by
pregnancy, medications (ie, coal tar, lithium), hypocalcemia,
or withdrawal of both topical and systemic steroids. Relapses Pearls
are common.
1. Koebner phenomenon (new lesions at the site of trauma)
Diagnosis is usually clinical and skin biopsy confirms
is an important feature of psoriasis.
the diagnosis. Complications include generalized exfoliative
2. Psoriasis may be seen as early as the first months of life.
dermatitis (erythroderma) and psoriatic arthritis. Differential
Diaper area is commonly involved.
diagnosis includes nummular eczema, tinea corporis, pity-
3. Pinpoint bleeding where scale is removed (Auspitz sign)
riasis rosea, pityriasis rubra pilaris, contact dermatitis, lichen
is highly characteristic.
simplex chronicus, Reiter syndrome, psoriasiform drug erup-
4. Involvement of nails is also an important feature and
tions, and other conditions of the scalp including seborrheic
includes nail pitting, oil spots, subungual hyperkeratosis,
dermatitis, tinea capitis, and atopic dermatitis.
and onycholysis (detachment of the distal nail plate).

Emergency Department
Treatment and Disposition
Admit patients with severe forms (eg, generalized pustular
psoriasis, a serious and at times a fatal disease). Educate par-
ents about the chronic nature of the condition and relapses
and remissions. The goal of therapy is to control flare-ups.
Advise patients on gentle skin care including lukewarm
brief baths (with bath oil or coal tar), gentle soaps, avoiding
trauma or scratching the lesions, and use of emollients for
skin hydration (eg, Vaseline, petrolatum, mineral oil). For
thick scalp lesions, use shampoo 2 or 3 times per week (coal
tar, selenium sulfide, or zinc pyrithione). Sunlight expo-
sure helps psoriasis; however, patients should be cautioned
to avoid sunburn as this may aggravate their condition.
Consider topical corticosteroids creams for management of
acute flairs. Low-potency steroid creams may be used on the FIGURE 7.45 ■ Psoriasis. Deep pitting of the nail due is a common
face and groin (not more than 2 weeks) and medium-potency feature. (Photo contributor: Falguni Asrani, MD.)

256
PSORIASIS (CONTINUED) ■ 257

FIGURE 7.47 ■ Psoriasis. Bilateral, symmetric plaques with over-


lying white micaceous scale. (Photo contributor: Erin Gilbert, MD.)

FIGURE 7.46 ■ Palmar-Plantar Psoriasis. This variant affects the


palms (A) and soles (B). Note thick, micaceous scale with underly-
ing erythema. (Photo contributor: Falguni Asrani, MD.)

A B

FIGURE 7.48 ■ Generalized Pustular Psoriasis. (A) Discrete annular erythematous plaques with a border of pustules. This young girl
developed this generalized pustular rash after being treated with oral steroids for presumed atopic dermatitis (steroid withdrawal can trigger
pustular psoriasis). The patient was started on methotrexate. (B) Close-up of pustular psoriasis. Numerous tiny, sterile pustules evolve from
an erythematous base and coalesce into lakes of pus. Pustules are superficial and are easily ruptured. (Photo contributor: Binita R. Shah, MD.)
GUTTATE PSORIASIS

Clinical Summary the patient to a dermatologist for ongoing care. Guttate


psoriasis may resolve spontaneously in a few weeks or
Guttate psoriasis is a variant characterized by an abrupt onset
months, especially with antibiotic therapy. Tonsillectomy or
of profuse oval or round “guttate” or drop-like lesions, most
monthly injections of benzathine penicillin may be helpful
commonly following group A β-hemolytic streptococcal
if flares are precipitated by recurrent streptococcal infec-
infections (ie, streptococcal pharyngitis, perianal streptococ-
tions. Exposure to sunlight and broadband or narrowband
cal dermatitis, otitis, or sinusitis). Streptococcal pharyngitis
UVB phototherapy also helps in resolution of lesions. For
or a viral URI precedes the eruption of guttate psoriasis by 1
persistent lesions, the same therapy as that for psoriasis is
to 3 weeks.
recommended (see page 256).

Emergency Department Pearls


Treatment and Disposition 1. Guttate psoriasis is a variant seen more commonly in
Laboratory tests should include a throat culture and serolog- children and adolescents and may herald the development
ical titer (eg, antistreptolysin O titer) to confirm streptococ- of generalized plaque psoriasis.
cal infection, and if infection is confirmed, give antibiotics. 2. Guttate psoriasis is characterized by an abrupt onset of
The drug of choice is penicillin. Erythromycin or clinda- drop-like lesions, mainly on the trunk, buttocks, hips, and
mycin are good alternatives for penicillin allergy. Refer proximal extremities.

FIGURE 7.49 ■ Guttate Psoriasis. An abrupt onset of diffuse erythematous, scaly, drop-like plaques in a 15-month-old infant. Note intense
erythema of the diaper area. Perianal culture was positive for group A β-hemolytic streptococci. She was treated with penicillin with a com-
plete resolution of the diaper and guttate rash. (Photo contributor: Jeannette Jakus, MD.)

258
GUTTATE PSORIASIS (CONTINUED) ■ 259

A B

FIGURE 7.50 ■ Guttate Psoriasis. (A) A 6-year-old boy with sudden onset of a diffuse papulosquamous rash of guttate psoriasis. (B) One
week later, the same child with more classic appearing drop-like scaly papules and plaques of guttate psoriasis. (Photo contributor: Falguni
Asrani, MD.)

FIGURE 7.51 ■ Guttate Psoriasis. An abrupt onset of erythema-


tous, scaly, drop-like plaques developed on the trunk of this girl
1 week after scarlet fever (throat culture positive for group A
β-hemolytic streptococci). Intense erythema and desquamation are
also seen on the palm (A) and both feet (B) as part of scarlet fever.
A
(Photo contributor: Binita R. Shah, MD.)
PITYRIASIS ROSEA

Clinical Summary with florid PR). Atypical clinical variants of PR are seen in
about 25% of cases and include
Pityriasis rosea (PR) is a benign eruption of unknown etiol-
ogy (presumed viral) with a distinct presentation of a soli- 1. Papular PR (especially in African American children)
tary “herald patch” lesion followed by an exanthema. About 2. PR with intensely irritated or inflamed edematous lesions
50% of cases occur before age 20 with highest incidence 3. “Inverse form of PR,” which includes lesions limited to
among adolescents. It is rare in children aged < 5 years. It face, scalp, distal extremities, groin, and axillae
is commonly seen during the fall, winter, and spring. About 4. PR with vesicular, pustular, urticarial, or hemorrhagic (pur-
5% of patients have a prodrome of fever, malaise, arthral- puric) or large annular erythema multiforme-like lesions
gia, and pharyngitis preceding the PR eruption (usually seen 5. PR with oral lesions.

A B

FIGURE 7.52 ■ Pityriasis Rosea. (A) Herald patch. This


lesion preceded the PR eruption by about 10 days in this
child. (B) Oval-shaped papulosquamous lesions of PR on
the trunk. The name PR is derived from Greek (pityriasis
= scaly) and Latin (rosea = pink). (C) Close-up shows the
long axis of the papulosquamous oval-shaped plaques is
oriented along the skin lines. A fine, wrinkled, tissue-like
scale remains attached within the border of the plaque, giv-
ing the characteristic ring of scale, called collarette scale.
C
(Photo contributor: Binita R. Shah, MD.)

260
PITYRIASIS ROSEA (CONTINUED) ■ 261

The diagnosis is made clinically and rarely a skin biopsy


(to exclude other diseases) may be required for atypical cases.
Recurrences of PR are rare (about 2% of cases).

Emergency Department
Treatment and Disposition
Most patients are asymptomatic and do not need any specific
treatment except reassurance about the benign nature and the
total duration of the eruption (about 6-8 weeks). Patients are
NOT contagious and do not need any isolation. Symptomatic
relief from pruritus can be treated by soothing nonprescrip-
tion lotions like calamine, menthol, or pramoxine. Lukewarm
colloidal oatmeal bath (not hot, as this may intensify itch-
ing) may be soothing. Nonfluorinated topical corticosteroids
(eg, hydrocortisone lotion 1% or 2%) and antihistamines
may be given for more intense pruritus, especially at night
when pruritus is more troublesome. A rare patient with
extensive disease with intense itching may respond to a short
course of oral prednisone (dose: 0.5-1 mg/kg/d given orally
for 7 days). This is rarely indicated in children and is best
reserved for patients with intractable pruritus with severe
FIGURE 7.53 ■ Pityriasis Rosea. An adolescent boy with scaly
disease. Refer such patients to a dermatologist. Direct sun
oval papules on his back. Numerous lesions on the back, oriented
along the skin lines (parallel to the ribs), give the appearance of exposure may hasten the resolution of individual lesions;
drooping pine-tree branches or Christmas-tree distribution. (Photo however, burning should be avoided as this may exacerbate
contributor: Erin Gilbert MD.)

FIGURE 7.54 ■ Pityriasis Rosea. Oval-shaped, salmon colored lesions with overlying scale in a young girl. A herald patch can be seen on
the neck. (Photo contributor: Deeptej Singh, MD.)
262 ■ PITYRIASIS ROSEA (CONTINUED)

the rash. Alternatively, ultraviolet B (UVB) administered 2. Lesions run parallel to the lines of skin cleavage, forming
in 5 consecutive daily erythemogenic exposures, results in a “Christmas tree” appearance on the back.
decreased pruritus and hastens the involution of lesions. 3. A herald patch is seen in 70% of cases and heralds the
Therapy is most beneficial within the first week of eruption onset of PR; however, its absence does not preclude the
and is rarely indicated in children. diagnosis of PR.
4. Secondary syphilis may be indistinguishable from PR,
especially if the herald patch is absent; obtain serology
Pearls for syphilis in all sexually active patients presenting with
1. PR is a self-limiting eruption lasting about 6 to 8 weeks a rash of PR.
and is characterized by pink oval papules or plaques with
a collarette of scale affecting the trunk and proximal
extremities.

FIGURE 7.55 ■ Pityriasis Rosea. (A) The classic “collarette” of


scale seen in this infant with extensive generalized PR. (B) Lesions
on the back follow the lines of skin cleavage displaying a “Christmas
tree” pattern. (C) Lesions extending to diaper area. (Photo contribu-
C
tor: Falguni Asrani, MD.)
GRANULOMA ANNULARE

Clinical Summary clinical but can be confirmed on biopsy. The histopathology


shows palisading granulomas in the dermis with mucin and
Granuloma annulare (GA) is a granulomatous skin disorder
necrobiotic degenerated collagen.
of unknown etiology occurring in any age group. Multiple
associations have been hypothesized including postvacci- Emergency Department
nation (injectables), trauma, insect bites, and sun exposure.
A historic association with diabetes has most recently been
Treatment and Disposition
refuted. The classic and subcutaneous types are common in GA is a benign chronic condition and usually followed by a
children. The characteristic morphology is a nonscaly indu- dermatologist. It is known to resolve spontaneously in several
rated annular plaque with a papular border and central clear- months to years. Treatment is mainly in form of topical mod-
ing. In the classic type, multiple or solitary painless lesions erate to high potency steroids or injectable steroids (expected
may be seen distributed over the wrists and dorsum of hands to work better).
and feet but can involve any part of the body. In subcutaneous
GA, patients present with multiple or solitary deep nodules Pearls
(similar distribution as classic form). The differential diag- 1. GA can be confused with tinea corporis because of its
nosis includes tinea corporis and dermal processes such as annular configuration and raised papular border. However,
sarcoidosis. The subcutaneous type may often be confused unlike tinea corporis, GA lacks superficial scale.
with rheumatoid nodules or other tumors. The diagnosis is 2. GA sometimes resolves spontaneously after a biopsy.

FIGURE 7.56 ■ Granuloma Annulare. (A) Well-defined asymp-


tomatic annular plaques with a raised papular indurated border and
central clearing, seen on the dorsum of foot, dorsum of hands and
feet (B) and on