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Table of Contents!

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~Otorhinolaryngology~!

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ENT Study Guide! Audiological Testing! Physiology of Hearing! ! Vertigo & the Physiology of Balance! Otitis Media & Otitis Externa! ! Epistaxis! Sinusitis! Hoarseness! ! ! ! ! ! ! ! ! ! ! ! ! ! ! Nasopharyngeal Carcinoma!

Laryngeal Carcinoma! ! Salivary Gland Tumours! Dysphagia! Achalasia! ! ! ! !

Swellings of the Thyroid Gland! ! Penetrating Injuries to the Neck!! Tonsils and Adenoids! ! Stridor! ! ! ! ! ! ! Tracheostomy! ! Facial Trauma! ! ! ! ! ! !

! Dermatology!! ! !

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ENT Clerkship Guide!

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Overview!
Ear, Nose, and Throat Surgery (ENT) is better understood as Otolaryngology- Head and Neck Surgery. Surgery above the level of the clavicles, that does not involve the brain or the eyes, is predominantly performed by the Otolaryngologist. Subspecialties in this field include head and neck cancer surgery, endoscopic sinus surgery, facial plastic and reconstructive surgery, neurotology, paediatric otolaryngology, and maxillofacial surgery. The specialty deals with clinical issues as diverse as neonatal hearing screening, medical management of vertigo and allergic rhinitis, surgical management of the difficult airway, surgical resection of tumours of the larynx and cervical oesophagus, removal of foreign bodies from the bronchus, to microscopic surgery of the middle ear and mastoid. !

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The flexible endoscope, the rigid telescope and the microscope play important roles in the diagnostic workup and in the postoperative follow-up of the ENT surgical patient. These procedures are often performed by doctors on the ward and allow the medical student magnified views of the ear, nose and throat via monitors. !

The ENT clerkship is a five-week rotation, which is shared jointly with dermatology and radiology. The ENT clerkship provides a broad exposure to otolaryngologic pathology and will familiarise the student with inpatient care, operating room procedures and outpatient clinic management. !

Aims !
This rotation is designed to allow the student to develop:! An introductory knowledge in otolaryngology, including the subspecialties of head & neck cancer, laryngology, otology, endoscopic sinus surgery, maxillofacial surgery, and paediatric otolaryngology. ! The ability to take a pertinent otolaryngologic history. ! The ability to perform a thorough head and neck physical examination.! An understanding of outpatient management of common ENT disorders. ! An understanding of peri-operative management in otolaryngology patients through direct involvement with inpatient care.! An understanding of head and neck anatomy through observing and participating in operative and outpatient procedures.! Recognise the ENT emergency, initiate management and refer appropriately.! Recognise treatment failures or complex conditions that require timely referral. ! Observe endoscopy of the upper aerodigestive tract and audiological testing.!

Students will get the opportunity to: !

! Learning Objectives! ! ! Hearing loss/ Ear Discharge/ Imbalance ! ! ! Discuss the basic physiology of hearing and of balance.! ! a. b. List the functions of the eustachian tube.! !
Upon completion of this clerkship, the student should be able to: !

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c. Differentiate between conductive and sensorineural hearing loss. List common causes of each, and identify treatable disorders. ! d. Describe common methods of hearing assessment in adults and in children.! e. List the indications for, and limitations of hearing aids.! f. Discuss the clinical features and management of otitis media with effusion.!

g. Discuss the clinical features and management of cholesteatoma.! h. List complications of chronic otitis media. ! i. List the treatment options for otosclerosis.!

! j. Define vertigo. Differentiate peripheral from central causes of vertigo.! Upper Airway Obstruction/ Hoarseness/ Dysphagia! ! ! Define stridor and discuss its significance.! ! a. List the differential diagnoses for stridor in children and in adults.! ! b. c. Discuss the investigation of the patient with stridor.! ! d. List the indications for tracheostomy. Identify complications of this procedure. ! ! e. Define obstructive sleep apnoea. List the treatment options.! ! f. List the indications for adenoidectomy and tonsillectomy.! ! List the causes and describe the investigations of hoarseness.! ! g. h. Describe the clinical features and management of carcinoma of the larynx.!
i. j. List the risk factors for head and neck cancer.! Discuss the differential diagnosis and management of dysphagia.!

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Nasal obstruction/ Nasal discharge/ Epistaxis!

a. Describe the normal drainage pattern of the paranasal sinuses.! b. Outline the concept of the ostiomeatal complex.! c. Discuss the aetiology, pathogenesis and management of sinusitis.! d. List the complications of sinusitis.! e. List the differential diagnosis for nasal obstruction.! f. Describe the clinical features of carcinoma of the maxillary sinus. !

g. Discuss the aetiology and describe the clinical features of nasopharyngeal carcinoma.!

h. Discuss the management of epistaxis.!

! Lumps in the Neck / Thyroid Nodules/ Salivary Glands! ! ! ! ! ! ! ! ! ! ! ! ! Skills Objectives! ! !

a. b. c. Classify thyroid masses.! Discuss the investigation of thyroid nodules.! d. e. f. List the indications for and the complications of thyroidectomy.! Describe the anatomy of the thyroid. ! Describe the clinical features of carcinoma of the thyroid.! g. h. i. Outline the anatomy of the parotid and the submandibular glands.! Upon completion of this clerkship, the student should be able to: ! 1. 2. 3. 4. 5. 6. 7. 8. 9. Take an ENT history! Demonstrate competence in the physical examination of the neck! Syringe the ear! Interpret basic pure tone audiograms! Interpret basic tympanometry! Participate in the care of the patient with a tracheostomy! Exhibit proficiency in the use of the nasal speculum! Interpret lateral soft tissue X-rays of the neck!

i.

Discuss fractures of the midface and mandible.!

Discuss the differential diagnosis of lumps in the neck, based on the site of the lesion and the age of the patient.!

Describe the pathology and clinical features of common benign and malignant salivary gland tumours.! Discuss complications of surgery of the parotid and submandibular glands.!

Demonstrate competence in the use of the otoscope, including pneumatic otoscopy.!

! ! LEARNING METHODS! !
1. 2. 3. 4. 5. 6.

Interactive tutorials ! Demonstration of and participation in practical procedures! Ward Rounds (including presentations by students)! Clinic Teaching ! Observing and assisting in operating theatre! Attending postgraduate seminars !

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LEARNING RESOURCES!

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A) Required Reading (Latest Editions)! 1. 2.

Lecture Notes by H. Ashman and C. Lyn (available on CD in ENT Dept.)! Lecture Notes on Diseases of the Ear, Nose, and Throat by P.D. Bull ! or! Hall and Colman's Diseases of the Ear, Nose and Throat "

by Suzanna Leighton, Andrew Robson, John Russell, Martin Burton!

B) Recommended Internet Sites! a) eMedicine at! www.emedicine.com/ent/contents.htm! b) Martindale's Otolaryngology at www.martindalecenter.com/MedicalAudio_2_C.html!

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Student Assessment!
MCQ paper (70 % of final clerkship grade) ! ! !

Oral Examination (30% of final clerkship grade)

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A satisfactory activity card is essential for successful completion of this clerkship. Unsatisfactory completion of the card may require repeating the clerkship. !

Clerkship Evaluation!
Students may be asked to evaluate the clerkship. This evaluation will be held at the end of the clerkship.! TUTORIALS ! A) Physiology of hearing ! B) Common causes of hearing loss! 2. 3. 4. 5. Otitis media with effusion and acute suppurative otitis media! Chronic suppurative otitis media and its complications! Delayed speech development, congenital hearing loss and hearing screening! A) Physiology of balance !

1.

B) Clinical assessment of the patient with vertigo! 6. 7. 8. 9. Otitis externa / malignant otitis externa! Epistaxis! Nasal obstruction and neoplasms of the nose and sinuses! Acute and chronic sinusitis!

10. Nasopharyngeal carcinoma! 11. Fractures of the mid-face and mandible! 12. Tonsils and adenoids ! 13. Hoarseness and carcinoma of the larynx! 14. Dysphagia! 15. Salivary gland neoplasms and sialadenitis! 16. Thyroid nodules and thyroid carcinoma! 17. Stridor in children/ indications for and complications of tracheostomies! 18. Congenital and acquired neck masses ! 19. Foreign bodies!

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Sample Questions!
A seventeen year old boy presents to the ENT Clinic with a history of hearing loss in the left ear, bloodstained postnasal discharge and bilateral upper cervical lymphadenopathy for 3 months.!

! 1. Which of the following is the best investigation?! ! 2. Likely finding on tuning fork tests!
a. b. c. d. e. a. b. c. d. e. Plain X-rays of the nose and sinuses! CT scan of the base of the skull and neck! CT scan of the nose and sinuses! Lateral soft tissue X-rays of the neck! Erythrocyte sedimentation rate (ESR)!

! 3. Audiological investigation of choice!

a. Impedance tympanometry!

Webers test lateralize to the right! False negative Rinnes test on the left! Webers test lateralize to the left! False positive Rinnes test on the left! Features of a left sensorineural hearing loss!

! 4. The most likely diagnosis is!

a. b. c. d. e.

b. c. d. e.

Otoacoustic emissions! Behavioral audiometry! Auditory brainstem response (ABR) ! Electronystagmography (ENG)! Angiofibroma! Nasopharyngeal carcinoma! Nasal Polyps! Chronic sinusitis! Hodgkins Lymphoma!

5) Hearing loss in this patient is typically managed by! a. Hearing aid! b. Cochlear implant! c. Myringoplasty! d. Grommet insertion! e. Stapedectomy!

! !

Answers

1: b!

2: c!

3:a ! 4: b!

5: d!

The most likely diagnosis is nasopharyngeal carcinoma. The best investigation will assess the extent of the lesion in the nasopharynx, skull base involvement and cervical metastases. These patients usually have a mild conductive hearing loss due to otitis media with effusion.!

OTOLOGY!

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The Physiology of Hearing!

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Sound is a waveform of mechanical energy, which exerts pressure throughout the medium in which it is transmitted. Hearing (the perception of sound) is essential for speech and language development, and is thus a

prime prerequisite for communication.!

The pinna functions as a funnel that collects sound and transmits it to the external auditory canal. We can tell the direction of sound by the fact that sound will tend to be louder in the ipsilateral ear and will also arrive earlier at that ear. The external auditory canal amplifies sound in the frequency range 3-4 kHz. This is due to the resonating properties of the external auditory canal.! ! For hearing to occur, sound needs to pass from air in the external canal into the fluid medium of the cochlea, wherein lie the pressure sensors the hair cells. If sound should pass directly into the cochlea, over 99% of sound energy would be reflected at the interface. To match the impedance (resistance to sound transmission) of air to that of the cochlear fluid, we need an intervening mechanism for sound transmission. The tympanic membrane and the ossicular chain (malleus, incus and stapes) are responsible for "impedance matching.! A much greater pressure is required to transmit sound through liquid as compared to transmission by air. Pressure equals force divided by area. By the hydraulic principle, the same force that is exerted on the tympanic membrane, when exerted on the footplate of the stapes, will exert a pressure at the footplate that is 13 times greater than that at the tympanic membrane. This occurs because the ratio of the functional area of the tympanic membrane to that of the footplate of the stapes is 13 to 1.! The pressure at the footplate of the stapes is further increased by the mechanical advantage gained by the lever system of the malleus and the incus. The relative lengths of the handle of the malleus to that of the long process of the incus is 1.3 to 1.0.! The tympanic membrane and ossicular chain therefore increase the pressure at the footplate of the stapes 17 fold (1.3 times 13).! A requirement for maximum sensitivity of the tympanic membrane to sound pressure is equality of pressure in the external auditory canal to that in the tympanic cavity. The eustachian tube is responsible for maintaining the middle ear pressure close to atmospheric pressure. This ensures that energy is not wasted in overcoming any pressure differential across the tympanic membrane.! The middle ear is a balanced system i.e. it moves about a fulcrum and the moments about that fulcrum are equal. The ossicles are suspended in the tympanic cavity by very tenuous ligaments. These provide minimal frictional force and resistance to movement.! Sound pressure that reaches the inner ear produces travelling waves in the liquid of the cochlear duct and eventual movement of the basilar membrane. The sensitive receptor hair cells lie in the organ of Corti on the basilar membrane. We can identify the frequency of sound heard by the place on the basilar membrane that best responds (resonates) to that sound (Place Theory). High frequencies mainly stimulate the basal turn of the cochlea.!

The intensity of sound is predominantly detected by the rate of production of action potentials in the auditory nerve.!

Hearing loss! Hearing loss can be broadly classified into conductive and sensorineural hearing loss. Conductive hearing loss occurs when conditions of the external ear or middle ear prevent efficient transfer of sound pressure to the inner ear. Conditions affecting the inner ear or the auditory nerve will lead to sensorineural hearing loss.!

! Common causes of conductive hearing loss include! !

b. Wax impaction--- common in all age groups! c. Acute and chronic otitis media!

a. Otitis media with effusion--- commonest cause in children!

d. Ossicular chain discontinuity (post trauma or secondary to infection)! e. Otosclerosis--- Second most common cause in Caucasian adults (after wax impaction). Family history in 67%, autosomal dominant. Woven bone results in fixation of stapes. Treated by stapedectomy or hearing aid (occasionally sodium fluoride)!

Common causes of sensorineural hearing loss include! a. Presbycusis---usually secondary to ischaemia of the Organ of Corti! b. Noise induced hearing loss! c. Ototoxicity (aminoglycosides, loop diuretics, cytotoxic agents e.g. Cisplatin, quinolones)! d. Meningitis! e. Viral infections (Measles, Mumps, Herpes Zoster etc)!

Sensorineural Hearing Loss in Childhood! a) 50 % thought to be of genetic origin ---majority autosomal recessive! e.g 1. Ushers Syndrome ---Sensorineural hearing loss (SNHL) and retinitis pigmentosa! 2. Pendreds Syndrome (SNHL and hypothyroidism)!

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b) 25-30%---Environmental factors (Rubella and other members of TORCHES group, Meningitis, Kernicterus, Hypoxia perinatally)! c) Idiopathic (Unknown cause)! Audiological Testing!

! a) Free field testing using whispered voice, conversational voice and loud voice !
b) Tuning fork tests Rinnes, Weber and Absolute Bone Conduction Test!

50 % of patients with a conductive hearing loss of 20 dB will have a negative Rinnes (bone conduction better than air conduction). 90 % of patients with a 40 dB conductive hearing loss will have a negative Rinnes. A negative Rinnes test therefore usually indicates a substantial conductive hearing loss. A false negative Rinnes test is associated with profound hearing loss. In such cases, no significant perception of sound occurs when the tuning fork is placed beside the ipsilateral ear. When the tuning fork is placed on the ipsilateral mastoid, bone conduction allows detection of the sound by the opposite cochlea. !

! ! !

In the Webers test, sound typically lateralize to the ear with a conductive hearing loss. In a patient with a false negative Rinnes test, sound lateralizes to the good ear.! The Absolute Bone Conduction Test is useful in the bilateral, equal sensorineural loss, when the Rinne would be positive and the Weber not lateralized. Here a doctor (who has normal hearing) compares his bone conduction with the patients.! c) Pure Tone Audiometry ! Here the patients Air and Bone Conduction are tested for different frequencies in a sound proof environment. The threshold for each frequency is charted. One can detect and quantify both conductive and sensorineural hearing loss. Children 2-4 years will not be suitable for this test. Instead Behavioural Audiometry is used. In the latter test, sound is introduced while the child is occupied playing and one expects alteration of behaviour if the sound is heard. !

d) Impedance Audiometry used to elucidate:! Middle Ear Pressure! Compliance of Conductive system! Acoustic Reflexes, indirectly giving an idea of the threshold of hearing! Give an indication of type of lesion in sensorineural loss.!

Patients with middle ear effusions usually have Type B tympanograms (flat tracings) while those with negative middle ear pressures will have Type C curves (peak in the negative range)!

e) Otoacoustic emissions test! This is a very important objective tool in neonatal hearing screening. The test is quickly administered and it does not require highly trained personnel to operate and interpret the test. Sound is introduced into the ear and sound emanating from the cochlea is detected in patients with hearing better than 30 dB.!

f) Auditory Brain Stem Response or Evoked Response Audiometry! This is another objective test that is useful in neonates, in infants and in people who cannot or will not cooperate for hearing tests (malingerers) .An auditory stimulus is introduced into the ear and this will result in electrical activity sequentially in the cochlear nerve, the cochlear nucleus, the superior olivary nucleus, the lateral lemniscus and the inferior colliculus. The test is repeated while a computer subtracts the background EEG activity leaving behind the response to the sound stimulus. Thus one can objectively follow the stimulus throughout the auditory system, and can detect problems therein.!

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VERTIGO AND THE PHYSIOLOGY OF BALANCE!

The maintenance of equilibrium at rest and in motion is achieved by a complex interaction of peripheral receptors and the central coordinating areas in the brain. The whole balance mechanism serves to orient the individual in space.!

The peripheral sensory systems are! 1. Vestibular system (semicircular canals, utricle and saccule)! 2. Visual Pathway! 3. Proprioceptive system! There are also important inputs from the

Central coordination occurs in the cerebellum and the vestibular nuclei.

cerebral cortex, limbic system, red nucleus and substantia nigra that modulate these centres. Usually a positive activity in one side of the body is balanced by a negative activity on the contralateral side. Dysequilibrium occurs when the inputs from one system does not match with those from the other systems. ! ! The cerebellum has a mainly dampening effect on Vestibular Nuclear activity, inhibiting excessive electrical activity of the Vestibular Nuclei. The Midbrain Nuclei have a less negative effect and can be regarded as a finer adjuster of Vestibular Nuclear activity. The Cerebrum has a positive or negative effect on Vestibular Nuclear activity and maybe under voluntary control.! Vertigo is an abnormal sensation of rotation relative to one's environment or a hallucination of movement. Vertigo is specifically associated with the disorders of vestibular system.! The vestibular receptors for the detection of angular acceleration are located in the semicircular canals. The semicircular canals lie at right angles to each other, occupying the three planes of space. Thus, in the semicircular canals, we have sensors to monitor circular movement in the three planes of space. The utricle and the saccule contain receptors oriented at right angles to each other for the detection of linear acceleration and tilt. A gelatinous matrix called the otoconial membrane covers these receptors. Embedded in the otoconial membrane are dense calcific bodies called, otoconia, which exert pressure on the hair cells. Since the otoconia and hair cells have different inertia, they will move differently if there is accelerating or decelerating force acting on them. This gives us the ability to sense linear movement with gravity or against gravity. ! The vestibular nerve carries information from these receptors to the vestibular nuclei. There are important connections between the vestibular system and the visual system via the medial longitudinal fasciculus. This accounts for the vestibulo- ocular reflex and for nystagmus in vestibular disorders. Patients who have disorders of the peripheral vestibular system are initially vertiginous, nauseous (and often vomit) and sweaty. In three to four weeks significant compensation occurs predominantly in the cerebellum so that the patient becomes much less symptomatic.!

! ! Diagnostic pointers in the assessment of the dizzy patient!

Patients should be initially classified into one of the following groups:! a. Patients with true vertigo (problem lies in the peripheral or central vestibular system)! b. Patients with syncopal sensation or actual syncope (inadequate cerebral blood flow, oxygenation, or nutrition)!

c. Miscellaneous group of patients who experience imbalance, but no syncope or vertigo. This may be secondary to

almost any medical disorder.!

Peripheral vestibular disorder Nausea, vomiting, sweating with initial attack

Central vestibular disorder Nausea and vomiting occurs late (often with raised intracranial pressure)

Nystagmus only present when patients have Nystagmus often noted in the absence of significant systemic symptoms Nystagmus is usually in the horizontal plane systemic symptoms Nystagmus may occur in any plane

Nystagmus worsened by the loss of visual Nystagmus not influenced by visual fixation fixation Compensation occurs in three to four weeks As the lesion is central, compensation may not occur

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If one has determined by the symptoms and signs that this is a peripheral vestibular disorder, one will next need to decide whether it is the left or right vestibular system that has been affected.!

The vestibular system and the hearing mechanism are closely related. If the patient has unilateral hearing loss it is reasonable to assume that the vestibular problem originates from the ipsilateral side. The caloric test is a good method of testing each vestibular system individually. The mnemonic COWS is useful in remembering the responses. Infusion of Cold water causes nystagmus to the Opposite side!

Infusion of Warm water causes nystagmus to the Same side.!

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The commonest cause of vertigo in elderly patients is vertebrobasilar ischaemia. Some of the conditions that result in vertebrobasilar ischaemia are atherosclerosis, cervical spondylosis and cardiac dysfunction including arrhythmias. Benign paroxysmal positional vertigo, labyrinthitis, vestibular neuronitis, eustachian tube dysfunction are important in all the age groups. ! Menire's Disease! This disorder has stimulated much interest. It is however an uncommon cause of vertigo. It is characterized by recurrent episodes of vertigo, sensorineural hearing loss and tinnitus. The attacks last 1/2 hour to 12 hours and are often preceded by a sensation of aural fullness. This is a peripheral vestibular disorder and patients usually experience significant nausea and vomiting.!

The aetiology is unknown but the salient histopathologic finding is endolymphatic hydrops with marked increase in the volume of endolymphatic fluid. It is believed that symptoms occur when the endolymphatic compartment is so distended that Reissner's membrane ruptures. This results in the mixing of endolymphatic and perilymphatic fluid.!

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In severe attacks, patients are admitted to hospital for intravenous hydration, antiemetics and sedation. Most patients will never need surgical intervention. Patients who have frequent, disabling vertigo and very poor hearing in the affected ear are best managed by labyrinthectomy. This procedure is followed by compensation within three to four weeks. If the hearing is good, vestibular neurectomy is one useful surgical option.!

Benign paroxysmal positional vertigo! This condition is thought to be due to cupulolithiasis. Calcium deposits from the otoconial membrane become dislodged and travel in the endolymphatic fluid to the posterior semicircular canal. Transient vertigo is induced by certain head positions that place the posterior semicircular canal in the dependent position.! After appropriate head positioning (Dix-Hallpike test), there is a latent period of 5-10 seconds. This is followed by vertigo and nystagmus that lasts 15-30 seconds. Nystagmus is rotary with the fast component in the direction of the dependent ear.! Most cases of benign paroxysmal positional vertigo will resolve in 18 to 24 months. Many cases respond well to vestibular exercises. Occasionally surgery is necessary and this entails transection of the nerve to the posterior semicircular canal (the singular nerve).!

! ! Definitions! !
Acute suppurative otitis media:!

! ! ! ! ! ! !
Otitis Media and Otitis Externa!

Acute inflammation of the mucosal lining of the middle ear cleft [tympanic cavity, eustachian tube and the mastoid air cell system]. The features of acute inflammation are present. Acute suppurative otitis media may be complicated by perforation of the tympanic membrane. These perforations will generally heal within four weeks.!

Otitis media with effusion is the presence of liquid behind an intact tympanic membrane, in the absence of the features of acute inflammation.!

Chronic suppurative otitis media:! This condition is characterized by the presence of a persistent (> 4 weeks) perforation of the !

tympanic membrane.! ! Otorrhoea!

! !

Otorrhoea may be secondary to a primary pathology in the external auditory canal or in the middle ear. Base of skull fractures involving the temporal bone may present with CSF otorrhoea.! In some cases, visualization of the tympanic membrane is difficult because of exudate in the external auditory canal. Without seeing the tympanic membrane one can often still distinguish between acute otitis externa and acute otitis media. (Unfortunately the two sometimes co-exist)!

Otitis Externa
Otalgia often severe. Onset of otorrhoea not associated with diminution of otalgia.

Otitis Media Severity of otalgia varies. Otalgia markedly reduced at onset of otorrhoea Discharge is mucopurulent Mild to moderate hearing loss Pain unrelated to movement of pinna

No mucus in otorrhoea Very mild hearing loss

Pressure on the tragus and movement of the pinna cause significant pain

Pathogenesis of middle ear infections!

! !

The pathogenesis of middle ear infections is strongly linked to disorders of the eustachian tube. The eustachian tube has the following functions:! Ventilation of the middle ear! Equalization of the pressure in the middle ear with that of atmospheric pressure! Drainage of middle ear secretions into the nasopharynx! Protection of the middle ear from nasopharyngeal secretions and organisms !

Dysfunction of the eustachian tube is generally secondary to obstruction and or infection.!

Acute suppurative otitis media and otitis media with effusion are most commonly associated with children. Adenoidal hyperplasia is the commonest source of obstruction to the eustachian tube in this age group. The adenoids are an important source of ascending infection into the middle ear via the eustachian tube. Congenital cysts such as Thornwaldts cysts can also obstruct the ostium of the eustachian tube. In male teenagers angiofibroma is an important cause of otitis media with effusion and severe epistaxis.!

One must always be on the alert for cases of nasopharyngeal carcinoma since this tumor frequently presents with otologic complaints. The nasopharyngeal carcinoma directly obstructs the ostium of the eustachian tube but also has the effect of obstructing the lymphatic drainage of the eustachian tube, further perpetuating the otitis media with effusion.! Infections of the nose, paranasal sinuses and the nasopharynx result in edema of the ostium of the eustachian tube and subsequent obstruction. These infections are more common in patients who have obstructive lesions in the nose such as nasal polyps, hypertrophied turbinates and marked septal deviation. Allergic rhinitis and vasomotor rhinitis produce obstruction of the nose and of the of the paranasal sinuses with subsequent frequent infections. ! Children who attend nurseries have a high incidence of upper respiratory tract infections and, consequently, frequent otitis media with effusion or acute suppurative otitis media.! Passive smoking impairs mucociliary function by damaging the cilia. Otitis media with effusion occurs more frequently and is more difficult to eradicate in children who are exposed to smoking.!

Patients with cleft palate, those with cystic fibrosis and patients who are immunocompromised will

obviously get frequent ear infections.!

!
Chronic Suppurative Otitis Media! This condition is characterized by the presence of a persistent (> 4 weeks) perforation of the tympanic membrane. ! Chronic suppurative otitis media can be divided into:! a. Cholesteatoma or squamous disease ---- presence of keratinising squamous epithelium in the middle ear cleft.! b. Tubotympanic or mucosal disease.!

Genesis of cholesteatoma! 1. Retraction pocket---- commonest mechanism! The external auditory canal is lined by squamous epithelium, which extends onto the tympanic membrane to form the external layer of the drum. Long-standing eustachian tube dysfunction can produce persistent negative pressure in the tympanic cavity. This can result in the weakest parts of the tympanic membrane being pulled medially to form retraction pockets. Classically this affects the pars flaccida (which lacks a fibrous layer) or the posterosuperior aspect of the pars tensa. These retraction pockets are lined by keratinising squamous epithelium and an accumulation of keratin occurs if the opening of the retraction pocket is relatively small. Secondary infection in the retraction pocket results in the production of collagenases and phosphatases that ultimately erodes surrounding bone and ligaments.!

2. Squamous epithelial growth at the edge of a perforation! In cases of long-standing perforation of the tympanic membrane, squamous epithelium can overgrow the edge of the perforation and extend into the middle ear. This is especially likely to occur if, in the region of the perforation, there is loss of mucosa from the medial aspect of the tympanic membrane.!

3. Implantation cholesteatoma! Squamous epithelium can be implanted by a penetrating injury to the tympanic membrane, or this can occur during surgery.!

! 4. Squamous Metaplasia of the middle ear mucosa! !

5. Congenital cholesteatoma! In this case congenital squamous epithelial rests are trapped in the middle ear.!

! !

Chronic suppurative otitis media can be active or inactive. Cholesteatoma, by its very nature, is always considered active.! Cholesteatoma is important because of the complications associated with this disorder.! These include:! 1. Hearing loss secondary to adhesions, granulation tissue, perforation of the tympanic membrane, exudate in the middle ear, and ossicular chain discontinuity! 2. Facial nerve palsy! 3. Acute and chronic mastoiditis! 4. Sub periosteal abscess! 5. Bezold's abscess-- along the sternomastoid! 6. Citellis Abscess-- along the digastric muscle! 7. Perilabyrinthitis and labyrinthitis! 8. Gradenigo's syndrome-- lateral rectus palsy, pain in the distribution of the fifth cranial nerve and a discharging ear. These features are noted when chronic suppurative otitis media involves the apex of the petrous temporal bone (petrous apicitis)! 9. Sigmoid sinus thrombosis which can extend into the internal jugular vein! 10. Meningitis! 11. Extradural abscess! 12. Subdural abscess! 13. Temporal lobe or cerebellar abscesses are usually secondary to chronic suppurative otitis media! 14. Benign intracranial hypertension!

! !

It is clear that cholesteatoma is a serious disorder with the risk of life-threatening complications. Cholesteatoma is a surgical disease and all patients who are medically fit to undergo surgery should be given this option. ! Prior to surgery, an ear swab should be sent for culture and sensitivity and the condition of the ear optimized by using topical ear drops, aural suctioning plus or minus systemic antibiotics. The commonest organisms grown are Gramnegative organisms such as Pseudomonas and Proteus. Often the infection is a mixed infection involving anaerobes and gram negatives. Failure to control the ear discharge with medications should hasten rather than delay the surgical procedure.!

The actual surgery is usually a form of a modified radical mastoidectomy. In this procedure, the squamous lining in the mastoid cavity and middle ear is removed, the posterior wall of the external auditory canal is usually removed and the

external auditory canal is widened (meatoplasty). At the end of the procedure, the mastoid cavity and middle ear can easily be visualised and cleaned through the external auditory canal. The ear would have been converted from an "unsafe ear" to a "safe ear". Once the cholesteatoma is controlled, the hearing mechanism can be reconstructed or a hearing aid can be utilised.!

! OTITIS MEDIA WITH EFFUSION: AETIOLOGY AND MANAGEMENT IN CHILDREN! !

INTRODUCTION! Otitis media with effusion is defined as the presence of liquid behind an intact tympanic membrane, without any of the symptoms or signs of acute inflammation. It is thought to be the commonest cause of hearing loss in children. Otitis media with effusion is predominantly a disease of children 1-9 years old. The peak incidence is found in children 6 months to 2 years old but as the diagnosis in this age group is difficult and the condition often asymptomatic, the peak incidence in children attending otolaryngology clinics is in the age group 3-6 years. In many developed countries, otitis media with effusion is the commonest indication in children for admission to hospital for elective surgery. In these countries, sixty percent of children will experience otitis media with effusion by the age of 2 years and 80% prior to school entry. The prevalence falls from 38 % at age 2 years to 1 % at 11 years. In Jamaica, the prevalence rate in children 5-7 years is 2 %! ! There is a high incidence in North American Indians and Eskimos. OME is generally thought to be more common in Caucasians than in Blacks, even though most studies do not define the racial composition of the study group. Males are more frequently affected than females. There is a seasonal variation with the highest incidence in January, February and March and the lowest incidence in August, September and October.!

AETIOLOGY! The major underlying factors are related to eustachian tube dysfunction and infection. The classic example of eustachian tube dysfunction occurs in cleft palate where the tensor palati muscle is abnormal and thus active opening of the eustachian tube is impaired. Patients with cleft palate also have a direct pathway for the passage of vomitus, sputum and drinks to gain access to the pharyngeal opening of the eustachian tube. The result is secondary infection. Otitis media with effusion is also very common in patients with a submucous cleft palate.! Obstruction to the eustachian tube can either be intrinsic or extrinsic. In the intrinsic type, oedema and secretions associated with inflammation cause obstruction of the eustachian tube. This occurs in upper respiratory infections. The nose, sinuses and the adenoids can act as reservoirs for infection producing repeated intrinsic obstruction of the eustachian tube. A distinct possibility is that the lymphatic drainage of the eustachian tube becomes obstructed by peritubal inflammation and this causes otitis media with effusion. In the child who is less than 7 years old, abnormal compliance of the eustachian tube, due to a relative reduction in the amount and stiffness of the cartilage, can cause collapse of the eustachian tube with resultant obstruction. Extrinsic obstruction of the eustachian tube is seen in patients with adenoidal hyperplasia or nasopharyngeal tumours that compress the eustachian tube. In patients with craniofacial abnormalities such as Downs, Hunters and Hurlers syndromes, there is a relative disproportion of the adenoidal size to the nasopharyngeal space which predisposes to obstruction of the eustachian tube.!

When obstruction to the eustachian tube occurs, the air in the middle ear is gradually utilised or absorbed, leaving a vacuum. The result is transudation of fluid from the mucosa to the tympanic cavity. ! ! The situation is much more complex than mere obstruction to the eustachian tube. Infection plays a significant role. Cultures of aspirates from middle ear effusions have grown organisms in 30-60% of cases. The organisms that have been found are the same as those associated with acute suppurative otitis media. The most frequent organisms found are Streptococcus pneumoniae, Haemophilus influenzae, and Moraxella (Branhamella) catarrhalis. Although the adenoids can cause obstruction of the eustachian tube, it appears that the main role of the adenoids is as a source of sepsis. Ascending infection up the eustachian tube causes oedema and exudate with eventual obstruction. ! It is likely that otitis media with effusion and acute suppurative otitis media are different ends of a spectrum of manifestations of obstruction and inflammation of the eustachian tube. In patients with otitis media with effusion, the host mechanisms and the organisms have created a balance so that a latent infection is maintained. In acute suppurative otitis media, the host mechanisms have been temporarily overwhelmed. It is known that otitis media with effusion can be a sequel to acute suppurative otitis media. ! ! ! In chronic otitis media with effusion, there is hyperplasia of the goblet cells and an increased number of Some patients have an inefficient mucociliary system, which predisposes them to otitis media with effusion. mucous glands in the middle ear cleft. This will tend to perpetuate the effusion. ! These include patients with the immotile cilia syndrome, Kartageners syndrome and cystic fibrosis. Viral and bacterial infections can adversely alter the mucociliary system.! ! The role of allergy in otitis media with effusion is uncertain. A true causal relationship has not been established. Some studies have found an increased incidence of atopic disease in children with otitis media with effusion. The adenoids have been shown to have a large number of mast cells and these can bind IgE with the resulting release of histamine. However, antihistamines and decongestants have not altered the course of otitis media with effusion. This casts doubts on the significance of allergy in these patients. ! ! ! ! Otitis media with effusion is usually asymptomatic or presents with symptoms related to hearing loss. In the CLINICAL FEATURES! infants and young children, parents may complain that the child has delayed or impaired speech development. In the older child, his teacher may report that his academic performance is poor, that the child does not pay attention or that he has general behavioural problems. Excessive shouting, and increasing the volume of the television may be subtle signs of hearing loss.! ! Otalgia is uncommon in a pure otitis media with effusion. However some patients get recurrent acute suppurative otitis media and otalgia often heralds the onset of this complication. Disorders of balance may also occur.!

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DIAGNOSIS! The diagnosis is based on the clinical findings and audiological investigations. If the tympanic cavity is filled with fluid, the colour of the drum may suggest underlying liquid. Many patients have evidence of retraction of the tympanic membrane (horizontal handle of the malleus, fore-shortened malleus, prominent lateral process of the malleus). If the fluid is opaque, the middle ear structures will be obscured. When the eustachian tube is partially patent or functioned recently, air bubbles or air-liquid levels may be identified. The most useful clinical signs are the reduction in the mobility of the

tympanic membrane that is found on pneumatic otoscopy. The tuning fork tests may show features of a conductive hearing loss depending on the severity of deafness.! ! Impedance tympanometry and pure tone audiogram are then performed. The impedance tympanometry is an objective assessment. It is the most sensitive audiological test for the presence of middle ear effusions. There is a high correlation between a Type B tympanogram and the presence of otitis media with effusion but this is not specific and clinical correlation is necessary.! ! The pure tone audiogram can be performed in children older than 5 years. It assesses the severity of the hearing loss and is thus useful in the management of this disorder. !

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TREATMENT! Treatment can be divided into the following categories! a. Management of predisposing factors! b. Medical treatment of otitis media with effusion! c. Surgical management of otitis media with effusion! ! It is important that identifiable predisposing factors are adequately addressed. Parental smoking has been linked to otitis media with effusion. The control of recurrent acute suppurative otitis media is important, as otitis media is frequently a sequel. Nasal or sinus infections must be treated. Although considerable controversy exists, in patients who are known to have allergies to inhaled or ingested allergens, appropriate measures should be taken in managing these allergens. Frequent hand washing and wearing an extra layer of clothing in winter can reduce the incidence of viral infections that may predispose the patient to secondary bacterial infection and otitis media with effusion.! Medical management of otitis media with effusion! ! Since 90% of the cases of otitis media with effusion will resolve within three months surgery is not indicated in this period. Medical therapy should be undertaken while the patient is being observed. Unfortunately, no medications have given long lasting results.! Antibiotics! In 30-60% of cases, cultures of middle ear effusions have grown organisms similar to those that cause acute suppurative otitis media. Antibiotic therapy should incorporate drugs that are effective against these bacteria. A !-lactamase-stable antibiotic should be prescribed for patients who have persistent otitis media with effusion despite therapy with one of the medications above. !

Other medications! Antihistamines, decongestants and non-steroidal anti-inflammatory drugs have efficacy comparable to placebo and are therefore not recommended. Systemic steroids have been used alone or in combination with antibiotics in the treatment of otitis media with effusion. The results have been inconsistent and, with the risk of significant complications, they should not be recommended.!

Surgical management of otitis media with effusion! Surgery is indicated in cases that fulfil all of the following criteria:! a. Hearing loss secondary to otitis media with effusion !

b. Persistence of the effusion for greater than 3 months! c. Failed medical therapy! Surgical management aims to! 1. Reverse hearing loss and the associated disability! 2. Prevent recurrent suppurative otitis media! 3. Prevent complications such as cholesteatoma!

! Surgery entails the insertion of grommets and adenoidectomy. ! !

Surgery may be required to eliminate potential causes of recurrent otitis media with effusion. This may include repair of cleft palate, antral washouts, turbinectomies and polypectomies. !

OTITIS EXTERNA!

The external ear consists of the external auditory canal and the pinna. The squamous lining of the external canal extends unto the tympanic membrane to form the lateral surface of the drum. Inflammation of the external canal is therefore commonly associated with hyperaemia of the tympanic membrane. The skin of the external auditory canal is firmly adherent to the perichondrium laterally and to the periosteum medially. Infection involving the lining of the external auditory canal is therefore often extremely painful. !

Infection of the external canal occurs in three forms:! a. Diffuse otitis externa! b. Localised otitis externa (furuncle or carbuncle)!

c) Malignant otitis externa!

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Diffuse otitis externa ! Normal skin is a good protective organ. Otitis externa is usually initiated by secondary infection of skin that has been traumatized by scratching, macerated in persistently moist conditions or affected by particular skin disorders.!

Factors commonly associated with diffuse otitis externa include:! -Seborrhoeic dermatitis! -Frequent swimming! -Hot, humid climates! -Repeated trauma to the external auditory canal (frequently produced by excess cleaning ! of the external auditory canal).! -Hypersensitivity reaction to otologic topical preparations!

The typical features of acute otitis externa include otalgia, pruritus, scant exudate, oedema and hyperaemia of the external auditory canal wall. In the more severe cases, cellulitis of the peri- auricular soft tissue occurs and this is usually accompanied by pyrexia.! The management of acute otitis externa aims to remove debris and exudate from the external auditory canal, eradicate the infective organism, remove predisposing conditions and return the skin of the external auditory canal to normal.! Ear swabs should be sent for culture and sensitivity prior to initiating antibiotic therapy. The commonest organisms associated with acute otitis externa are Pseudomonas, Proteus and Staphylococcus aureus. In severe cases the external canal can be virtually occluded by oedema. In such a case, a wick soaked in glycerine and ichthammol should be inserted. This can be changed 48 hours later. After the oedema has lessened significantly, treatment can be continued with topical drops containing a fluoroquinolone or gentamycin and a corticosteroid. Systemic antibiotics are generally unnecessary except in cases with cellulitis. Analgesic therapy is an important part of the treatment of this painful condition. Non-steroidal anti-inflammatory drugs are usually effective. Regular aural toilet, preferably by suctioning, is the most important treatment modality in these patients.! In chronic otitis externa, Pseudomonas and Fungi are most commonly isolated. Treatment consists of aural toilet, topical gentamycin/steroid drops or antifungals (eg clotrimazole drops and ketoconazole orally). One should always search for predisposing conditions and identify cases of immnuosuppression in recurrent acute or chronic otitis externa.!

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Furuncles are most commonly located in the superior aspect of the cartilaginous canal. They are treated with topical and systemic antibiotics. It is usually unnecessary to incise and drain these. Incision and drainage can be complicated by perichondritis of the pinna.! MALIGNANT OTITIS EXTERNA!

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Malignant otitis externa is a progressive, necrotising infection of the external auditory canal, which is most

commonly found in elderly diabetics. Most of the morbidity associated with malignant otitis externa is secondary to osteomyelitis of the temporal bone and the skull base which, when inadequately treated, result in cranial nerve palsies and intracranial complications. Chandler coined the term malignant external otitis in 1968 when he reported his experience with his first thirteen cases. Six of these cases died from this disease or underlying medical problems.! Malignant otitis externa is an aggressive form of infection of the external auditory canal, which is most frequently detected in immuno-suppressed patients. Classically, it is found in elderly diabetics. Pseudomonas aeruginosa is the responsible organism in most cases. Males are affected twice as frequently as females. Malignant otitis externa has now been diagnosed in patients on cytotoxic therapy, acquired immune deficiency syndrome and in malnourished infants.! ! ! The pathogenesis is an infection by a virulent organism in an immunosuppressed host. The organisms produce exotoxins that cause tissue necrosis at the advancing edge of the infection or produce substances that encapsulate the infection from host defences and from systemic antibiotics. Of importance in the diabetic is the microangiopathy, which makes the tissues ischaemic and impairs the effect of systemic therapy.! The infection spreads in the base of skull predominantly as an osteomyelitis and periosteitis. It involves the cranial nerves as they exit the skull via their various foramina. The facial nerve is most frequently affected in an abscess cavity close to the stylomastoid foramen. The

ninth, tenth and eleventh cranial nerves are affected close to the jugular foramen, while the hypoglossal nerve is affected at the hypoglossal canal. The osteitis and periosteitis spreads along the petrous temporal bone to involve the sixth cranial nerve close to the petrous apex.! ! !

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CLINICAL FEATURES! Typically, the patient is an elderly diabetic who has purulent otorrhoea with otalgia disproportionate to the clinical signs. The otalgia is worse at nights and is associated with hearing loss. The discharge is usually yellow or green. Characteristically, granulation tissue is noted in the external auditory canal at the osseo-cartilaginous junction. The granulation tissue marks the site of abscess formation beneath the tympanic plate.! ! Conductive hearing loss is found in almost fifty percent of patients. This hearing loss is secondary to obstruction to the ear canal or to otitis media with effusion. The inflammation at the skull base causes obstruction to the lymphatic and venous drainage of the eustachian tube and this will predispose the patient to otitis media with effusion.! ! Marked tenderness is invariably present on palpation between the mastoid process and the ascending ramus of the mandible, just below the floor of the external auditory canal. In advanced cases, there may be trismus and tenderness over the temporo-mandibular joint.! ! ! Blood:! ! A complete blood count and blood film should be requested. Neutrophilia represents the host response to bacterial infection but neutropaenia or abnormal white cells can be important clues to underlying immunosuppression, leukaemia or lymphoma.! ! ESR: The ESR is usually markedly elevated. The ESR can be used to monitor the response to therapy.! Urea and electrolytes must be monitored as patients may be placed on nephrotoxic drugs or diabetic nephropathy may be present.! ! Glucose profile --- as most cases are found in diabetics.! Pure tone audiogram --- as a baseline study, since the patient may be treated with ototoxic drugs.! Cranial nerve palsies are common in advanced lesions. ! !

Investigations!

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Ear Swab! Microscopy and culture and sensitivity tests are done to guide antibiotic therapy. Cultures for aerobic, anaerobic and acid fast bacteria should be done.!

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Histology! Multiple biopsies are sent to rule out malignancy. The histopathologist should look for any evidence of fungal infection.!

Nuclear Scanning:!

Nuclear scanning of the temporal bone and the base of skull will show increased uptake. Technetium 99 (Tc 99) is

commonly used. Technetium is absorbed by osteoblasts and osteocytes. Unfortunately, the Technetium scan may be positive for an indefinite period. The Technetium scan is therefore not useful in the follow-up of the patient. Gallium 67 citrate scan reverts to normal when active inflammation ceases and this scan can be done six-weekly to assess the patients response to therapy!

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CT Scans! CT scans are less sensitive than nuclear scans in assessing the involvement of the temporal bone. CT however, is far more sensitive than plain x-rays!

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TREATMENT ! The treatment of malignant otitis externa has undergone considerable changes as the pathogenesis of this disorder Surgical management is limited to regular suction clearance of the ear, biopsy of the granulation tissue, drainage became clearer.! ! of abscesses and debridement of necrotic bone and cartilage. Patients who are deteriorating despite adequate medical therapy should be considered for debridement. ! ! Medical management is the mainstay of therapy. It is important to control underlying medical problems. The diabetes should be controlled. Anaemia and malnutrition should be treated. The patients fluid and electrolyte status must be monitored.! ! Intravenous aminoglycosides used to be the main antibiotic therapy. ! Ceftazidime is a useful parenteral cephalosporin that can be used empirically with oral fluoroquinolones in the initial management of the patient. This combination avoids the nephrotoxicity and ototoxicity of the aminoglycosides. The tissue necrosis seen in malignant otitis externa provides a good medium for the growth of anaerobic organisms. Metronidazole should therefore be included in the antibiotic regime.! Daily suction toilet of the ear is performed and a gentamycin wick used or gentamycin drops administered.! ! Treatment should continue for six to sixteen weeks. Treatment is discontinued when the external canal has returned to normal, the white blood cell count and ESR are normal, and the Gallium scan (if available) shows no evidence of active inflammation.!

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THE MANAGEMENT OF EPISTAXIS! Epistaxis can be defined as bleeding emanating from the internal nose. Epistaxis is a frightening experience for

the patient. Most cases are easily controlled and can be managed on an outpatient basis. On the other hand, epistaxis can be a life-threatening problem. It is important that the clinician has a logical approach to the management of epistaxis. Management will include the establishment of haemostasis, identification and treatment of hypovolaemia or circulatory shock (when present) and the specific management of any aetiologic factor.!

BLOOD SUPPLY OF THE INTERNAL NOSE! The nose is supplied by both the external carotid and internal carotid arterial systems and there are multiple anastomoses between these systems in the nose. The major blood supply is via the maxillary branch of the external carotid artery. !

Venous drainage! The venous drainage of the central parts of the lateral nasal wall is to the pterygoid venous plexus. The anterior areas drain to the facial vein while posteriorly the drainage is to the pharyngeal venous plexus.!

! The specific blood supply to the septum must be reviewed, as this is the commonest source of epistaxis.! !
The blood supply of the nasal septum ! The major source of arterial blood to the nasal septum is the sphenopalatine branch of the maxillary artery. The ascending branch of the greater palatine artery passes through the incisive canal to anastomose with the sphenopalatine, the superior labial and the anterior ethmoidal arteries at Littles area on the antero-inferior portion of the septum. The plexus of arteries at this site is called Kiesselbachs plexus. The commonest site for epistaxis is Littles area. The posterior ethmoidal artery gives a small contribution to the antero-superior aspect of the septum.! There are important anastomoses across the midline between the two anterior ethmoidal vessels and also occurring in the nasopharynx. This can explain some of the cases in which arterial ligation fails to control epistaxis.! ! Venous drainage! The septum drains anteriorly to the facial vein, posteriorly to the pterygoid venous plexus and superiorly via ethmoidal veins to the ophthalmic veins. The retrocumellar vein is an important source of venous bleeding in children and young adults. In 1% of individuals, the nasal veins are connected to the superior sagittal sinus by a vein that passes through the foramen caecum in front of the crista galli. This facilitates intracranial spread of nasal sepsis.! ! !

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Causes of epistaxis! Examples of local causes!

a) Trauma: trivial or major injuries, nasal fracture, surgical trauma, foreign body, excessive sneezing or coughing (with increased pressure in the nasal vessels).! b) Inflammation, infection and allergy: e.g. acute and chronic, specific and non-specific rhinitis.! c) Neoplasms (and tumour- like masses) of the nose, nasopharynx and sinuses: ! 1) Benign - angiofibroma, haemangioma, inverted papilloma, pyogenic granuloma (tumour-like), other granulomas.! 2) Malignant - squamous cell carcinoma, adenocarcinoma, salivary gland tumours, melanocarcinoma, midline lethal granuloma (generally considered a lymphoma).! d) Drugs - cocaine abuse.! e) Congenital vascular malformation.! In many cases of epistaxis the actual aetiology is never elucidated. These cases are often labelled as spontaneous epistaxis but a careful clinical assessment will often reveal contributory factors such as minor trauma and inflammation. !

Examples of general causes! Abnormal bleeding may result from a defective coagulation pathway, thrombocytopaenia, platelet function defects and vascular abnormalities.! A) Congenital coagulopathies e.g. haemophilia, Christmas disease, von Willebrands disease.!

! B) Acquired defects in the coagulation pathway: heparin or warfarin therapy; hepatic or obstructive biliary diseases.! !
C ) Thrombocytopaenia! 1) Platelet production failure! i) Selective depression of the megakaryocytes by drug toxicity (cotrimoxazole, phenylbutazone, thiazides, tolbutamide) or viral infections. ! ii) Generalised bone marrow failure: plastic anaemia, leukaemia, marrow infiltration or myelosclerosis. ! iii) Ineffective platelet production: megaloblastic anaemia.! 2) Increased platelet destruction: ! i) Idiopathic thrombocytopaenic purpura ! ii) Secondary immune thrombocytopaenia ( post-infection, SLE, chronic lymphocytic leukaemia) ! iii) Disseminated intravascular coagulation! iv) Drug- induced immune thrombocytopaenia (sulphonamides, quinine, PAS, rifampicin digitoxin). ! 3) Splenic pooling in patients with splenomegaly.! 4) Massive transfusion of old blood: dilutional thrombocytopaenia!

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D) Abnormal platelet function! Aspirin therapy, hepatic and renal disease, multiple myeloma and Waldenstroms macroglobulinaemia are all acquired causes of platelet dysfunction .!

E) Vascular abnormalities: Hereditary haemorrhagic telangiectasia, steroid-induced vascular weakness, scurvy, Monckebergs sclerosis.!

Hypertension and anxiety tend to cause prolongation of epistaxis but the incidence of epistaxis is not increased in these patients. Hypertension is associated with vascular changes, which inhibit vasoconstriction thus prolonging epistaxis. The increased arterial pressure will also increase the severity of the epistaxis. Thus hypertensive patients are more frequently admitted to hospital for the control of epistaxis. The control of hypertension is an essential part of the management of epistaxis in the hypertensive patient.! ! Management of active epistaxis!

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History! A brief history is obtained from the patient or a relative while preparations are being made to control the epistaxis. It is useful to inquire whether the epistaxis is predominantly an anterior or posterior bleed and whether the epistaxis is unilateral. If the bleeding is reported as bilateral, one should still inquire as to the side that initially bled (often blood is entering the opposite nasal passage at the posterior choanae by passing around the border of the septum). It is important to ask about any syncopal sensation, which may be indicative of cardiovascular decompensation. The patient should be asked to give an estimate of the blood loss in terms of common utensils such as cups or tablespoonfuls. Patients tend to exaggerate the blood loss, but often have containers with them that they have used to collect the blood and these can be very helpful in estimating the blood loss. One should ask about nasal trauma( including nose-picking), upper respiratory tract infections, recent medications, history of bleeding diathesis and any significant medical illness.! ! It should be clear that the thoroughness of the initial history would depend on the clinical status of the patient. The patient who is in hypovolaemic shock will require emergency resuscitation while attempts are made to control the bleeding. The patient whose cardiovascular system is stable but who is bleeding heavily is best managed by controlling the bleeding and then completing the history.!

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Examination! The patients pulse and blood pressure should be taken. If the cardiovascular system is stable the patient should be examined sitting up or in the semi-recumbent position. Strong suction and a good headlight are prerequisites for an adequate assessment of the patient with epistaxis. It is useful to attempt a preliminary assessment of the nose without vasoconstriction because this can sometimes stop the bleeding and prevent identification of the bleeding point. After suctioning the clots, local anaesthetic and vasoconstrictive nasal drops or sprays will facilitate examination of the nose and assist with haemostasis in patients who are not bleeding profusely. Cocaine is a very potent vasoconstrictor and local anaesthetic and is therefore frequently used in this setting. The maximum dose should be 1.5mg/kg. An alternative preparation is a mixture of 0.1% xylometazoline and lignocaine. ! ! While local measures are being undertaken, it is important that resuscitation of the circulatory volume with intravenous fluid and blood transfusion (if necessary) is carried out. Sedation should be prescribed as this relieves the patients anxiety, reduces the blood pressure and facilitates nasal cautery and packing. ! Anterior epistaxis!

Effective treatment for anterior epistaxis includes tamponade of the nasal vessels between the fingers, nasal

cautery, nasal packing, and surgery with ligation of the anterior ethmoidal artery. Most patients who are being assessed by an otolaryngologist will have already attempted to tamponade the nasal vessels between the fingers, but may not have applied pressure properly and so the otolaryngologist should give the relevant instructions. The patient can attempt to tamponade the nasal vessels while preparations are being made to examine the nose.! ! If the source of an anterior epistaxis is identified, the treatment of choice is nasal cautery. This treatment is usually successful unless the bleeding is profuse. The commonest site of epistaxis is Littles area on the antero-inferior nasal septum. The immediate area around the focus of bleeding should first be cauterised before cautery to the actual bleeding site is attempted, otherwise there would be a significant risk of aggravating the epistaxis. Successful nasal cautery has the advantage of avoiding nasal packs. Patients who have not had major bleeds with cardiovascular decompensation can often go home after a short period of 20- 30 minutes of observation for recurrent bleeding. Silver nitrate, trichloro-acetic acid and electrocautery are highly effective. Electrocautery is the more effective than chemical cautery in the presence of active bleeding, but would require general anaesthesia in children ! There is a risk of septal perforation when unilateral cautery is overzealously performed. Bilateral nasal cautery at equivalent sites on the nasal septum should be avoided, as this is associated with a high incidence of perforations. ! ! Nasal packs will be required in cases of profuse anterior epistaxis and in cases where the focal bleeding point cannot be identified. A half- inch petrolatum gauze-strip soaked in antibiotic ointment or bismuth iodoform paraffin paste (BIPP) packing is commonly used. The nasal packing should be applied meticulously, in loops, from the nasal floor to the nasal roof and extending as far posteriorly as is feasible. Patients often find the insertion and the removal of these packs very uncomfortable. Adequate local anaesthesia and analgesia should be administered. Merocel sponges can be inserted easily and are better tolerated. These are especially useful in children. Experience with Merocel sponges suggest that they are less effective than BIPP or gauze strip packs. They are most useful in mild epistaxis. Gauze strip packs, BIPP and Merocel all adhere to the nasal mucosa to varying degrees and epistaxis may resume on removal of the nasal packs. ! ! Nasal balloons are easy to insert and to remove, but are slightly less effective than traditional nasal packs in Anterior nasal packs should be left in situ for 24-48 hours. During this period the patient should be on antibiotics and should be nursed propped up so that that the head is elevated. ! .! ! ! Nasal endoscopy has increased the frequency with which the actual bleeding point is identified. This has Nasal endoscopy! facilitated both chemical and electro-cautery. Nasal endoscopy has been particularly helpful in epistaxis that is relatively posterior in location. The 0 and the 30 degrees rigid telescopes or the flexible fibreoptic scopes can be used. The flexible fibreoptic scope is particularly useful in identifying bleeding points that are posterior to septal deviations. ! Occasionally septoplasty(operation to straighten the septum) must be performed to facilitate access to a bleeding point so that cautery or effective nasal packing can be accomplished. ! controlling epistaxis because they do not adapt very well to the irregularity of the lateral nasal wall. !

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Treatment options for posterior epistaxis! Posterior epistaxis can be very difficult to manage. Identification of the bleeding point is sometimes impossible and one has to rely on post- nasal packs or balloon tamponade initially if significant active bleeding is occurring.

Sometimes the patient presents with mild posterior epistaxis. In these cases the nose should be decongested and topical anaesthesia applied to facilitate examination endoscopically or microscopically. It is worthwhile to attempt a preliminary nasal examination without the use of vasoconstriction as the latter occasionally stops the bleeding and prevents identification of the bleeding point. If the bleeding point is identified, the site should be cauterised. In cases where heavy posterior bleeding is occurring, the initial management should be the insertion of a double-lumen balloon to apply nasal as well as postnasal tamponade since one would not be certain of the exact source of the bleeding. This method also increases the efficiency of the tamponade. Under local anaesthesia, nasal balloons can be inserted quickly and easily with minimal discomfort to the patient. Nasal balloons are tolerated much well than the traditional anterior and posterior nasal packs. The nasal balloons should remain in situ for 48 - 72 hrs.! ! If bleeding persists despite the insertion, or on the removal, of the nasal balloons, endosopic or microscopic examination of the nose should be performed. If the epistaxis is not controlled by posterior endoscopic or microscopic cautery, then a Foleys catheter should be inserted and the balloon inflated with 15 ml of air or saline. Saline does carry with it the tiny theoretical risk of aspiration but has the advantage that it does not leak as easily as air. Bilateral anterior nasal packs (petrolatum gauze strips) should also be inserted to maximise the tamponade effect.! ! When posterior endoscopic cautery (under local anaesthesia) and nasal packs fail to control epistaxis, or epistaxis recurs on removal of the packs, the nasal passages and the postnasal space should be examined under general anaesthesia. Any bleeding point that is identified should be cauterised. Identification of the bleeding point may require fracturing the turbinates to allow access to the meati. If the bleeding site cannot be identified, the maxillary artery should be ligated. The alternative is to insert firm anterior and posterior nasal packs under general anaesthesia, leave these in situ for 72- 96 hrs and to proceed to ligate the maxillary artery if bleeding recurs on removal of the packs. It is perhaps a better choice to avoid two general anaesthesias and to proceed with ligation of the maxillary artery at the first general anaesthesia.! ! Ligation of the maxillary artery is effective in 95% of cases. Embolization of the maxillary artery using gelfoam, has been successful in controlling epistaxis, but has a higher failure rate than ligation of the maxillary artery and its sphenopalatine and greater palatine branches. It is rarely necessary to ligate the anterior ethmoidal vessels when adequate ligation of the maxillary arteries and its main branches has been performed.! Special conditions! ! There are many possible causes for epistaxis. In the management of epistaxis one should be careful not to miss a malignancy of the nose, sinuses or the nasopharynx. Careful clinical assessment and radiologic studies will identify these malignancies. The male teenager may have an angiofibroma requiring angiography and CT scanning to plan the surgical approach to be utilised after embolization of the major feeding vessels. Patients who have hereditary haemorrhagic telangiectasia are difficult to manage. Septodermoplasty has produced transient success but telangiectactic vessels tend to grow into the split skin graft with resultant recurrent epistaxis. Oestrogens and local radiotherapy have been useful in some cases. Recurrent bleeding from the edges of a septal perforation can be controlled with cautery.!

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NASOPHARYNGEAL CARCINOMA!

INTRODUCTION!

Nasopharyngeal carcinoma is a tumour of epidermoid origin that is endemic in certain ethnic groups. It constitutes

90% of all malignancies found in the nasopharynx. Nasopharyngeal carcinoma is prevalent in the Cantonese Chinese in Southern China, Hong Kong and Singapore. The age-adjusted incidence in males in Hong Kong is 26: 100,000. In Jamaica it is found in 1.4: 100,000 males and 0.5: 100,000 females. This cancer is uncommon among Caucasians. In North America the incidence is 1: 100,000. In Northern China nasopharyngeal carcinoma has an incidence of only 3 per 100,000. There is a moderately high incidence in the Malays, Indonesians, Thais and Filipinos. In North African countries such as Tunisia, Algeria and Sudan the incidence is less than that in South-east Asia but still appreciably higher than in North America and Europe. The Eskimos in Alaska have an incidence that is 15 times that of the general USA population. The aetiology is multifactorial and includes genetic, viral and environmental factors.! ! ! Although this carcinoma has its highest incidence in particular racial groups, it occurs universally. Clinicians must Nasopharyngeal carcinoma is more commonly found in males. The male to female ratio is 2-3: 1. In high risk have a high index of suspicion in order not to miss this lesion since the presentation can be subtle and varied.! regions the incidence of nasopharyngeal carcinoma rises from the end of the second decade to reach a peak in the fourth decade and then remains at a plateau. In low risk countries such as Jamaica, a high proportion of cases have their onset in the second decade of life with a second peak occurring in the sixth decade.!

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Aetiology! Nasopharyngeal carcinoma is an interesting tumour that demonstrates the interaction of several factors in carcinogenesis. The important aetiologic factors are viral, genetic and environmental. Exciting data has been collected from the continued research into the aetiology of this carcinoma.!

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The role of the Epstein-Barr virus! The Epstein-Barr virus has been associated with Burkitts lymphoma, infectious mononucleosis, non-Hodgkins lymphoma in immunosuppressed individuals, some cases of Hodgkins lymphoma and nasopharyngeal carcinoma. Antibodies to the Epstein-Barr virus have been strongly associated with nasopharyngeal carcinoma. In high risk regions there is a rise in the titres of certain antibodies to the Epstein-Barr virus prior to the onset of the malignancy.! that the DNA of this virus (EBV DNA) was present in biopsy material from nasopharyngeal carcinoma ! In situ hybridisation is considerably more sensitive for the detection of EBV-encoded RNA than it is for EBV DNA because of the high levels of the former in specimens from nasopharyngeal tumours. ! ! ! ! The high incidence of nasopharyngeal carcinoma among certain ethnic groups strongly suggests a genetic link, The major histocompatibility gene complex on the short arm of chromosome six comprises six recognised loci called HLA-A, -B, -C, -DR, -DQ, -DS. There are now well-established associations between certain HLA haplotypes (human leukocyte antigen) and nasopharyngeal carcinoma. ! Genetic factors! which possibly interacts with environmental factors. ! ! The association of the Epstein-Barr virus and nasopharyngeal carcinoma was subsequently confirmed by demonstrating

Deletion of the short arm of chromosome 3 (3p) has been noted in these cancers and this may reflect the loss of a tumour suppressor gene.!

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Environmental factors! Ingestion of Cantonese- style salted fish, especially in childhood, has been linked to nasopharyngeal carcinoma. It is known that carcinogenic nitrosamines are present in such salted fish. !

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Histopathology! The World Health Organisation Classification recognises three histologic types ! endemic areas.! b. Non- keratinising squamous cell carcinoma (WHO-2)! c. Undifferentiated carcinomas that have abundant non-neoplastic, lymphocytic infiltrate (WHO-3). This latter histologic type has often been called lymphoepithelioma.! a. Keratinising squamous cell carcinoma (WHO-1) -30-50 % of the cases in non-endemic regions but only 5 % in

Anatomy! The nasopharynx has dimensions of 4 cm x 4 cm x 3 cm. The anterior wall is really the posterior choanae separated by the posterior border of the nasal septum. The floor consists of the superior surface of the soft palate and the nasopharyngeal isthmus. The roof and the posterior wall form one continuous sloping surface formed by the body of the sphenoid, the basiocciput and the first two cervical vertebrae. The prevertebral fascia and muscles separate the adenoids from the vertebrae. The lateral wall contains the pharyngeal end of the eustachian tube, the tubal tonsils and the Fossa of Rosenmuller.! ! Most cases of nasopharyngeal carcinoma seem to arise in the region of the Fossa of Rosenmuller. In the adult this cleft-like space may be as deep as 1.5 cm. Its base lies superomedial to the pharyngeal opening of the eustachian tube and it extends posterolaterally to its apex which reaches the petrous apex and the anterior margin of the carotid canal. The foramen ovale and the foramen spinosum lie lateral to the apex of the Fossa of Rosenmuller. Lateral to the fossa lie the mandibular division of the trigeminal nerve, the tensor palatini, the parapharyngeal space with the internal carotid artery, the internal jugular vein and the lower four cranial nerves. Above the superior border of the superior constrictor muscles (at the sinus of Morgagni), the nasopharynx is separated from the parapharyngeal space by the pharyngobasilar fascia and the pharyngeal mucosa only, allowing easy spread of tumour in the parapharyngeal space.!

Clinical features! Symptoms may be due to the local effects of the tumour mass in the nasopharynx, direct spread into contiguous structures (including erosion of the skull base), regional or distant metastases, cranial nerve involvement or paraneoplastic syndromes.! Sixty percent of patients present with palpably enlarged cervical nodal metastases. ! CT scans have detected nodal metastases in 75% to 90% of nasopharyngeal carcinomas of histologic types WHO 2 and WHO 3. The initial spread of nasopharyngeal carcinoma is to the retropharyngeal nodes (nodes of Rouviere) but the first palpable nodes are the jugulodigastric nodes followed by apical nodes deep to the superior aspect of the sternomastoid.

Metastases to submandibular and parotid nodes are relatively common. The tumour can directly invade the deep lobe of the parotid. Bilateral cervical nodal metastases frequently occur. Fifty percent of undifferentiated carcinoma of the nasopharynx will present with bilateral cervical lymphadenopathy.! Nasal symptoms are common but may be quite subtle. Nasal obstruction along with bloodstained postnasal discharge is common. Epistaxis may only occur after hawking. Secondary infection or obstruction to the drainage of the paranasal sinuses can cause the symptoms of sinusitis.! Aural symptoms are frequently present. The tumour obstructs the eustachian tube or infiltrates the tensor or levator palati muscle, interfering with the function of the eustachian tube. Otitis media with effusion is commonly the end result. Recurrent acute suppurative otitis media may also be found. One must always be suspicious of nasopharyngeal carcinoma in any teenager or adult who has unilateral otitis media with effusion (although this lesion can also cause bilateral otitis media with effusion). Other aural symptoms include otalgia and tinnitus.! Cranial nerve palsies are found in 15-20 % of patients. At the University Hospital of the West Indies, cranial nerves VI and XII are most commonly involved.! Nasopharyngeal carcinoma can easily invade the fifth cranial nerve since the foramen ovale lies lateral to the apex of the Fossa of Rosenmuller. Cranial nerves V and VI can both be affected at the petrous apex. Nasopharyngeal carcinoma can spread superiorly through the foramen lacerum and along the internal carotid artery to the cavernous sinus. In this way cranial nerves III, IV, Va, Vb and VI may be invaded or compressed. Spread to the parapharyngeal space, the jugular foramen and/or the hypoglossal canal can cause cranial nerve palsies involving the last four cranial nerves. The tumour can spread anteriorly to the pterygopalatine fossa and the maxillary sinus with subsequent involvement of the orbit and the optic nerve. It is conceivable that the olfactory nerve may rarely be affected by further spread into the roof of the nose or the anterior cranial fossa.! Other symptoms and signs include trismus due to invasion of the pterygoid muscles and severe headaches resulting from erosion of the skull base or secondary sinusitis.! Distant metastases can be detected in 20- 30 % of nasopharyngeal carcinomas. Skeletal metastases (especially to the thoracolumbar spine) account for 50 % of these lesions. Intracranial spread has been found in 3-12 % of cases. !

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Diagnosis! The nasopharynx can be assessed by nasopharyngeal mirrors or by endoscopes in the Outpatients Department. The diagnosis is confirmed by the histology of nasopharyngeal biopsies under local or general anaesthesia.! One must stress that the lesion may be almost entirely submucosal and deep biopsies may be required to obtain adequate samples. These cases are best biopsied under general anaesthesia. The CT scan or MRI can often alert one of the need for deep biopsies.!

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Assessment of the extent of the tumour! Clinical and radiological studies are the main methods of assessing the tumour extent. CT scans of the base of the skull and the neck should be the minimum investigations. Plain X-ray can demonstrate erosion of the skull base and the foramina but is too insensitive a method to accurately assess the extent of the lesion. Plain X-rays of the head and neck are

only indicated when CT or MRI is unavailable. Chest X-rays are used in the general evaluation of the patient and also to detect pulmonary metastases ( CT scan more accurate for the latter). ! ! ! ! Nasopharyngeal carcinoma is predominantly treated with radiotherapy. The use of Cobalt 60 and megavoltage TREATMENT! linear accelerator has decreased the incidence of complications while at the same time improving the results. Imaging studies have allowed better delineation of the tumour extent and therefore better planning of radiotherapy. Doses of 65-70 Gy are now given. Hyperfractionation is being utilised but there are no well-controlled, prospective studies assessing the efficacy of this technique, in nasopharyngeal carcinoma, when compared to that of traditional radiotherapy. Intracavitary and interstitial radiotherapy may be particularly useful in recurrent lesions.! ! Surgery is generally limited to biopsies and radical neck dissections for residual tumour following radiotherapy. In patients with massive lymphadenopathy, surgery to reduce the tumour mass, is sometimes advocated prior to radiotherapy. The rational for this is that radiotherapy is less effective in these cases since the centre of the lesion is likely to be hypoxic. When there is recurrent tumour in the nasopharynx, intracavitary radiotherapy or surgery may be initiated.! Grommets have been inserted to treat the otitis media with effusion that is often associated with nasopharyngeal carcinoma !

Prognosis! Major prognostic factors adversely influencing the outcome of treatment include large size of the tumour, higher T stage, and the presence of involved neck nodes (especially nodes > 8 cm diameter and supraclavicular nodes). Cranial nerve palsies, bone invasion and intracranial spread significantly affect survival. Small cancers of the nasopharynx are highly curable by radiotherapy with survival rates of 80%-90%. Moderately advanced lesions without clinical evidence of spread to cervical lymph nodes are often curable with survival rates of 50%-70%. The overall 5-year survival is 57 %. ! Patients with advanced lesions, especially those associated with clinically positive cervical lymph nodes, cranial nerve involvement, and bone destruction, are poorly controlled locally by radiotherapy with or without surgery and often develop distant metastases despite local control. !

MANAGEMENT OF SINUSITIS!

Acute sinusitis is defined as an acute inflammation of the paranasal sinuses, which resolves without residual mucosal change following appropriate medical therapy. Children who meet the following criteria are categorised as having chronic sinusitis: ! a)! Persistent symptoms and signs for twelve weeks ! or six episodes per year of recurrent acute sinusitis! b)! in association with persistent mucosal change on CT scan four weeks after medical therapy ! (International Conference on Sinus Disease: terminology, staging, therapy ---- 1993)! The paranasal sinuses develop as outgrowths from the lateral wall of the nose starting at the 40th gestational day. At birth, only the maxillary and the ethmoidal sinuses are readily identifiable. Most of the subsequent growth of the maxillary sinus depends on the eruption of the dentition. The frontal sinus usually develops from anterior ethmoidal air cells called the frontal recess. The majority of the paranasal sinuses drain into the middle meatus. Normal physiology of

the paranasal sinuses depends on the patency of the ostia of the sinuses, good mucociliary function and adequate immune function. ! Sinusitis is often initiated by obstruction of the ostiomeatal complex, which results in hypoxia of the sinus mucosa and ciliary dysfunction. This produces stasis of the sinonasal secretions and multiplication of bacteria. The inflammatory response perpetuates the obstruction of the ostia. Thus patients may have a primary lesion obstructing the ostia or inflammation may produce secondary obstruction of the ostia. Chronic sinusitis develops if the obstruction is not relieved. Persistent disease in the sinuses is usually secondary to disease in the anterior ethmoids and the ostiomeatal complex (middle meatus and adjoining areas). Medical and surgical management must attempt to correct the abnormalities in these key areas.! A thorough history and a full examination are essential in each patient. However it is often difficult to examine the middle meatus without the use of nasal endoscopes. When the middle meatus is not seen and symptoms and signs of purulent rhinorrhoea, postnasal discharge and nasal obstruction persist for more than ten days a presumptive diagnosis of rhinosinusitis should be made. It is also reasonable to make a diagnosis of rhinosinusitis in the patient who has nasal symptoms associated with pain in the region of the sinuses. ! In the past we relied heavily on plain X-rays in the investigation of the sinuses. Mucosal thickening, sinus opacification and fluid levels are often indicative of sinusitis. It is now possible to assess the sinuses with great precision. The state of the art techniques in the assessment of the sinuses are nasal endoscopy and high resolution computerised tomography. Nasal endoscopy has allowed us to examine areas that were previously inaccessible without general anaesthesia. Patients with suspected complications of sinus disease (e.g. orbital and intracranial complications) and those with persistent symptoms and signs despite adequate medical therapy require computerised tomography. This will allow precise planning of the surgical approach in each patient.!

! Complications of Sinusitis! !
Local ! 2. Mucocele!

1. Chronicity and irreversible mucosal change! 3. Osteomyelitis especially of the diploic anterior wall of frontal sinus!

! Orbital complications! ! 1. Dacrocystitis because of obstruction of the Naso-Lacrimal Duct.!

2. Conjunctivitis! 3. Subperiosteal abscess! 4. Periorbital cellulitis ! 5. Orbital Cellulitis! 6. Orbital abscess! 7. Ophthalmoplegia! 8. Blindness!

Intracranial Spread!

! Epidural Abscess!
Meningitis!

Subdural Abscess! Frontal lobe Abscess! Cavernous sinus thrombosis!

Note that the paranasal sinuses are the commonest source of frontal lobe abscess.!

Medical therapy of Sinusitis aims to control infection, establish patency of the ostia, relieve symptoms and treat predisposing conditions. Streptococcus pneumoniae and Haemophilus influenzae are responsible for 76% of cases of community-acquired sinusitis. Anaerobes, other streptococcal species, Moraxella catarrhalis and Staphylococcus aureus each account for 3-7% of cases. Amoxicillin plus Clauvulanic acid, and Cefuroxime are considered as good first line antibiotics. Augmentin gives anaerobic coverage as well and may therefore be a good choice in chronic infections. Metronidazole can be added for further anerobic coverage. Topical decongestants, steam inhalations and saline drops are generally useful. Nasal steroid drops and sprays are important agents in chronic sinusitis. Antihistamines are indicated in patients with a definite history of allergies or atopy. When standard medical therapy fails, anaerobic and fungal infections should be considered.! In children, adenoidectomy and antral washouts may be effective in the child who has recurrent rhinosinusitis associated with upper airway obstruction. When this treatment fails, traditional aggressive sinus surgery or functional endoscopic sinus surgery may be required. The latter technique has steadily gained popularity over the past two decades. It is based on the concept that limited surgery in the key areas of the ostiomeatal complex and the anterior ethmoids is usually all that is necessary to reverse pathological changes in the sinuses. The technique is generally successful except in patients whose mucociliary mechanism cannot return to normal (e.g. cystic fibrosis and Kartageners syndrome) and in those with irreversibly damaged mucosa.!

! HOARSENESS! !
Hoarseness is characterized by a change in the quality of voice. It is a symptom of disturbance of the physiology of the true vocal cords. Whilst hoarseness can easily be recognized by listening to the patient, it is important to realize that hoarseness is merely a symptom. Any patients who has hoarseness that has persisted for more than two weeks, requires examination of the larynx to rule out a malignant lesion or a benign laryngeal lesion that could potentially cause airway obstruction. !

Normal voice production involves! a) The generation of an expiratory airflow against a closed glottis resulting in an increase in the subglottic pressure.! b) Vibration of the true vocal cords! c) Resonation in the supraglottis, pharynx, nose and sinuses! d) Articulation!

! ! Vibration of the true vocal cords! !

During phonation the true vocal cords are adducted. When the expiratory respiratory muscles contract, the subglottic air pressure increases. The subglottic air pressure will eventually be high enough to force the true vocal cords apart allowing the egress of expiratory air. The resultant fall in the subglottic pressure, the elastic recoil of the tissue and the Bernoulli effect will restore the true vocal cords to their original adducted position. Once again, the cycle of increasing subglottic pressure and separation of the true vocal cords will begin again. Studies have demonstrated that throughout the cycle of phonation, the vocal cord ligaments remain in an adducted position. Adduction and separation predominantly involves the mucosa. Thus we can speak about a mucosal wave of vibration. This explains the marked deterioration of the voice that is associated with disease of the mucosa of the true vocal cords.! Normal vocal cord mobility requires intact recurrent laryngeal nerves, neuromuscular junction, glottic muscles and cricoarytenoid joints.!

! CAUSES OF HOARSENESS! ! Intrinsic lesions of larynx! !

Acquired lesions! Vocal cord nodules! Vocal cord polyps! Reinke's oedema (marked oedema in the submucosa of the true cords)! Acute and chronic laryngitis! Granulomas of the vocal cords: intubation granulomas, pyogenic granulomas, and tuberculosis!

Myxoedema!

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Benign tumours:! Laryngeal papillomatosis! Malignant tumours:! Squamous cell carcinoma (95%)! Trauma with haematoma and oedema! Foreign bodies at the glottis! Congenital lesions! Vocal cord palsy! Vocal cord webs! Laryngeal cysts! Laryngocoele! Haemangioma or lymphangiomas!

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Recurrent laryngeal nerve palsy! This may be secondary to a lesion of the vagus or a direct lesion of the recurrent laryngeal nerve.! Supranuclear lesions of the vagus! Cerebrovascular accident! Nuclear lesion! Bulbar poliomyelitis! Motor neuron disease! Lateral medullary syndrome!

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Posterior fossa lesions! Acoustic neuroma! Meningioma! Jugular foramen lesions! Glomus jugulare! Metastatic carcinoma of the nasopharynx! Cervical lesions! Penetrating neck injuries!

Carotid body surgery! Radical neck dissection!

Thoracic lesions! Carcinoma of esophagus! Tuberculous or metastatic nodes! Aortic aneurysm or aneurysmal surgery ! ! Lesions of the recurrent laryngeal nerve in the neck! Invasion by malignancies of the thyroid, cervical oesophagus, pyriform sinus. (Carcinoma of the larynx typically causes vocal cord palsy by infiltration of the intrinsic laryngeal muscles)! Thyroidectomy! Pharyngeal pouch surgery ! Repair of tracheal stenosis or tracheo-oesophageal fistula!

Viral infections and disorders such as vasculitis (e.g. associated with diabetes and autoimmune disorders) can affect either the vagus or the recurrent laryngeal nerve.!

! Hoarseness can also occur because of disorders at the neuromuscular junction: myasthena gravis! ! Muscular disorders: muscular dystrophies! !
Cricoarytenoid joint dysfuntion: ! Perichondritis, arthritis, post-traumatic adhesions!

The assessment of the patient would require a thorough history and examination, special radiologic studies and blood investigations. It is essential that the vocal cords be visualised if hoarseness persists beyond two weeks.!

! ! CARCINOMA OF THE LARYNX! !

Carcinoma of the larynx usually presents in the sixth or seventh decades of life. It is strongly associated with cigarette smoking and alcohol intake. Polycyclic aromatic hydrocarbons derived from cigarette smoking are converted by aryl hydrocarbon hydroxylase to epoxides, which bind DNA and are the true carcinogens. Other associated factors include human papilloma virus types 16 and 18, nickel and asbestos, mate tea in Latin America and prior radiation. Genetic factors may be important, as there is a higher incidence of laryngeal cancer in some families than the incidence found in the general population.! Men are affected five times as frequently as women are. In the United States of America 10,000 to 12,000 cases of laryngeal cancer are reported annually. In men, laryngeal carcinoma occurs in 9 per 100,000 while women have a

reported incidence of 1.5 per 100,000. In Jamaica, the incidence is 4 per 100,000 males annually. Worldwide, ethnic differences have been noted in the incidence of laryngeal cancer. Blacks are affected more often than Caucasians, while Japanese and the Chinese have lower incidences than both groups. There is a higher incidence in urban than in rural populations. This may be related to urban pollution. ! Ninety-five percent of cases are squamous cell carcinomas. Most are moderate to well differentiated. In Jamaica, United States, and Great Britain sixty percent of cases arise at the glottis, thirty-five percent at the supraglottis and the five percent in the subglottis. Wine producing countries such as Italy, Spain and France have a higher incidence of supraglottic than glottic cancers and patients tend to present in middle age at the time of diagnosis.! Glottic cancers tend to present early with persistent hoarseness, which becomes progressively worse. Any adult patient who has persistent hoarseness for more than two weeks must be assumed to have malignancy until proven otherwise. Examination of the larynx is mandatory. Early laryngeal cancer can be treated with minimal morbidity and good preservation of voice. Late diagnosis on the other hand may result in a relatively uncomfortable and unpleasant death.! Dyspnoea and stridor may be the presenting features in patients with advanced laryngeal cancer. These symptoms may be present without hoarseness in the patient with a supraglottic or subglottic tumour.! Cervical nodal metastases are common in supraglottic cancers but are uncommon in glottic cancers until the late stages. This is a reflection of the poor lymphatic drainage of the mucosa of the true vocal cords.!

The larynx can be examined by a number of methods in the clinic:! Indirect laryngoscopy using a mirror! Flexible fiber-optic nasolaryngoscopy! Rigid telescope (70 degree)!

The lesion usually arises in the anterior one-third of the vocal cords. Often it appears as warty excrescences on the true vocal cords. Areas of leukoplakia may be present. Vocal cord palsy may be present in the more advanced lesion. In cases where the diagnosis has been greatly delayed, tumour may extend from the supraglottis to extend across the glottis into the subglottis (transglottic tumour). These patients often present with stridor because of the compromised airway. Deep jugular chain cervical lymphadenopathy may be present. This is especially likely in supraglottic tumours where 30% of patients present with palpable nodes (often bilaterally). The lymphatic drainage tends to follow the arteries. (Superiorly along the superior laryngeal vessels to pass through the thyrohyoid membrane to mid deep jugular chain nodes and inferiorly along the inferior laryngeal and inferior thyroid vessels to jugulo-omohyoid nodes). There is also anterior lymphatic spread to prelaryngeal (Delphian node) and pretracheal nodes.!

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Once the diagnosis is suspected the patient should be assessed for his/her fitness for surgery and for the possibility of metastatic spread. Chest Xray and CT examination of the neck and chest should be done. If a CT scan cannot be done because of cost constraints, ultrasound examination of the neck can be substituted. ! The diagnosis is confirmed by biopsies at direct laryngoscopy or microlaryngoscopy. Prior to taking biopsies, the extent of the tumour must be assessed endoscopically and also by palpation of the base of the tongue. There is a 10% incidence of

an associated second primary tumour in the head and neck or bronchus. Panendoscopy (laryngoscopy, tracheobronchoscopy, oesophagoscopy and nasophayngoscopy) is often performed. Fine needle aspiration cytology should be perfomed if nodal enlargement is present. However, even if FNA is negative, the neck must still be treated in patients with cervical lymphadenopathy since there is a small false negative rate and all nodes may not have been adequately sampled.!

Treatment! Early laryngeal cancer is best treated with radiotherapy. ! When the lesion is confined to one vocal cord and there is no impairment of cord mobility, the ten-year survival is 90%. Partial laryngectomy is the second choice for early lesions but results in a poorer voice than when radiotherapy is the treatment modality. Advanced lesions may require total laryngectomy and radiotherapy.!

Metastases to cervical nodes are treated by neck dissection. The radical neck dissection entails resection of all the lymph nodes in the anterior and posterior triangles of the neck along with the sternomastoid muscle, the accessory nerve and the internal jugular vein. A functional neck dissection spares the non-lymphatic structures. Extranodal spread (into nonlymphoid tissue) is more common in patients with nodes> 3 cm diameter and in patients with multiple nodes. Functional neck dissection would not be advisable in these patients.!

! Chemotherapy is still experimental---- 30-50% respond but no increase in survival rate has been demonstrated.! !
Voice Rehabilitation! There are a number of methods of achieving speech after total laryngectomy:! a. Oesophageal speech b) Electronic larynx c) Tracheo-oesophageal puncture and insertion of Blom- Singer or Provox valve d) Neoglottic techniques!

The tracheo-oesophageal puncture with insertion of a one- way valve is now most popular. The valve allows air to go into the oesophagus while the patient is expiring but will not allow food or drinks to get into the trachea.!

! SALIVARY GLAND TUMOURS ! !

Relations!

The major salivary glands are the parotid, submandibular and sublingual glands. Hundreds of minor salivary glands are found in the palate, buccal mucosa, tongue, vallecula, larynx, trachea and paranasal sinuses. ! The parotid gland is the largest of the salivary glands. ! The parotid gland is a serous gland, which has three surfaces: anteromedial, posteromedial and lateral or superficial. The anteromedial surface embraces the ascending ramus of the mandible, which is sandwiched between the masseter externally and the medial pterygoid muscle medially.!

Posteromedial surface is related to the mastoid with the associated attachments of the sternomastoid and the posterior belly of the digastric. The external carotid artery grooves this surface before entering the gland. The styloid process and its associated muscles separate the internal carotid artery and the internal jugular vein from this surface of the gland. ! This superficial or lateral surface is covered by skin and superficial fascia. ! The gland extends superiorly to the level of the zygomatic arch. ! Inferiorly the gland extends into the neck, overlapping the superior aspect of sternomastoid. Anteriorly it extends to overlap the masseter muscle.! The facial nerve traverses the gland and the portion of the gland superficial to the nerve is called the superficial lobe while that lying deep to the nerve is called the deep lobe of parotid. The parotid gland has a true capsule formed by condensation of the investing layer of the deep cervical fascia. This explains why acute inflammatory conditions of the parotid are so painful. The greater auricular nerve supplies the skin overlying the parotid and its posterior branch supplies the ear lobe. This posterior branch can sometimes be saved during parotidectomy but the anterior branch has to be sacrificed.!

The submandibular gland is a mixed salivary gland; which is divided into superficial and deep lobes by the mylohyoid muscle. The marginal mandibular branch of the facial nerve is a lateral relationship to the gland. The lingual and the hypoglossal nerves are closely related to the medial aspect of the gland. The submandibular duct (Whartons duct) emerges from the deep lobe and courses anteriorly between the hyoglossus and mylohyoid muscles. While occupying this plane the duct is crossed twice by the lingual nerve, once on its lateral aspect near its origin and again on the medial aspect of the duct near its termination at the lingual frenulum. The facial artery and vein supplies the gland and are intimately linked to the submandibular gland.!

Tumours of the salivary glands! The diversity of histopathologic lesions that are expressed in the salivary glands make these tissues truly unique. Salivary gland tumours constitute 3-6 % of all head and neck tumours. Sixty-five to eighty-five percent of all salivary gland tumours occur in the parotid gland. The majority (80 %) of parotid tumours are benign; while 50% to 60% of submandibular tumours and 40% of sublingual and minor salivary gland tumours are benign. Parotid neoplasms are slightly more common in females. There is a slight racial predilection for African - Americans over Caucasians. In Kingston, Jamaica, the incidence of malignant tumours of the salivary gland is 1.2 per 100 000 males and 0.5 per 100 000 females. Salivary gland tumours have an annual incidence of 1 per 100 000 in the United States of America. The majority of patients present in the age range 30 to 70 years.! ! CLASSIFICATION! Parotid tumours can be classified as tumours of epithelial or non-epithelial origin. malignant. The malignancies may be primary or metastastatic parotid tumours.! The tumours may be benign or

! Tumours of epithelial origin! !

Benign: !

Mixed tumour (pleomorphic adenoma) ! Papillary cystadenoma lymphomatosum (Warthin's tumour) ! Oncocytoma ! Monomorphic tumours ! e.g. Basal cell adenoma and Myoepithelioma !

Primary Malignant Parotid Tumours: !

Mucoepidermoid carcinoma (low, intermediate and high grade): 15 % of all salivary gland tumours! Adenoid cystic carcinoma: 10 % of all salivary gland tumours ! Adenocarcinoma: 8% of all salivary gland tumours! Carcinoma ex pleomorphic adenoma (carcinoma arising in a mixed tumour) and Malignant mixed tumour (biphasic tumour): 6% of all salivary gland tumours ! Acinous cell (acinic cell) carcinoma: 3% of all salivary gland tumours ! Oncocytic carcinoma !

Secondary or Metastatic Parotid tumours!

Tumour-like swellings of the parotid! Sjogren's syndrome! Benign lympho-epithelial lesion! Sialosis!

! !

The malignant tumours can also be classified into low and high-grade carcinomas according to their aggressiveness. Acinic cell carcinoma is a good example of a low-grade carcinoma. The malignant mixed tumour is usually a high-grade carcinoma. There are also aggressive subtypes of mucoepidermoid and adenoid cystic carcinoma.! Pleomorphic adenoma! This is the commonest benign tumour of the parotid, submandibular and sublingual glands. It is responsible for 70 percent of parotid tumours. It occurs slightly more frequently in females (female to male ratio of 3: 2). It occurs most commonly in the 5th decade of life. The tumour is slow growing with occasional growth spurts. Facial nerve palsy is extremely rare and the presence of any facial weakness should suggest malignant transformation. Pleomorphic adenomas that have been present for greater than 10 years undergo malignant transformation in 2-10 percent of cases (carcinoma ex-pleomorphic adenoma).! Grossly the tumour is firm and grey-white with variegated myxoid and blue translucent areas of chondroid.! The tumour is pleomorphic in a number of ways.! The stroma may show chondroid, osteoid, myxomatous or fibrous features! the ratio of stroma to epithelial component varies from tumour to tumour and from site to site within any one tumour.! The epithelial cells may be polygonal, cuboidal or columnar and may form nests or cords of cells!

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The tumour has an incomplete capsule with pseudopodial projections extending through the capsule. This explains the high recurrence rate if lumpectomy is performed.!

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Most of the tumours arise in the superficial lobe and the treatment is superficial parotidectomy with preservation of the facial nerve. While paresis of the facial nerve branches occasionally occurs, permanent facial nerve palsy is rare in the hands of the otolaryngologist. The recurrence rate after superficial parotidectomy is approximately five percent. When lumpectomy is performed, the recurrence rate can vary from 20- 35 percent! Warthin's tumour! This is also called adenolymphoma or papillary cystadenoma lymphomatosum. It is the second most common benign neoplasm of the parotid. Warthins tumour comprises 4-8 percent of all salivary neoplasms. It is most commonly found in elderly males with a male to female ratio of 7:1. It is the commonest bilateral parotid tumour. Bilateral Warthins tumours are found in 10 percent of patients. The lesion has been associated with cigarette smoking and the rising incidence in women is thought to be to be related to increased smoking in this gender.! Most cases arise in the lower pole of the parotid and are soft or cystic. There is no involvement of the facial nerve. The lesions are (hot) on Technetium scanning.!

! !

The lesion is characterized histologically by cystic spaces with papillary projections of epithelial cells and marked lymphoid infiltrate. transformation.! Mucoepidermoid carcinoma! This is the commonest malignant parotid tumour. Adenoid cystic carcinoma occurs more frequently than any other malignancy in the other salivary glands. ! Mucoepidermoid carcinoma may behave as a low-grade or aggressive carcinoma. epidermoid to mucus-secreting cells tend to behave more aggressively.! Lesions with a higher ratio of Treatment is surgical: superficial parotidectomy. There is no association with malignant

Adenoid cystic carcinoma! Histologically the lesion can be classified as solid, cribiform or tubular. The worst prognosis is associated with the solid type. Characteristically, the tumour spreads by invasion of the perineural spaces ( especially common with the cribiform type). Lymphatic spread is unusual. Although the five-year survival rates is 60 percent, it falls to 15 percent at 15 years. Eventually fifty percent of the tumours disseminate widely to bone, liver and lungs.!

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The commonest malignant non-epithelial salivary tumours are malignant fibrous histiocytoma, malignant schwannoma, rhabdomyosarcoma and non-Hodgkins lymphomas. Many lymphomas are associated with Sjgrens syndrome or HIV.! Metastases to the parotid generally arise from primary head and neck squamous! cell carcinomas or melanomas of the skin of the head and neck.!

Hematogeneous metastases are rare and originate mainly from the lungs,! kidneys, or breasts.!

Aetiology! Malignant lesions! The only well-documented risk factor for salivary gland cancer is ionizing radiation. ! Benign lesions! ! Radiation-induced neoplasms in the salivary glands are usually benign.The commonest associated tumour is the pleomorphic adenoma. The increasing incidence of Warthins tumour, especially in women, has been linked to cigarette smoking . !

Presentation! Mass!

! The majority of patients with parotid neoplasms present with painless masses typically in front of or below the ear.! !
Pain! Pain is generally found with inflammatory conditions rather than neoplasms. Advanced malignant parotid lesions may however present with intractable pain. 12 to 24 percent of malignant tumours of the salivary glands present with pain. !

! ! !

Facial nerve palsy! A parotid lesions that is associated with facial nerve weakness should be assumed to be malignant until proven otherwise. However only 10 to 15 percent of malignant lesions present with facial nerve palsy.! Skin involvement and cervical lymphadenopathy! Skin ulceration occurs in 9 percent of malignant lesions. Cervical nodal metastases may be found in malignant parotid tumours of epithelial origin or primary lymphomas.! DIAGNOSIS! A thorough history and physical examination are essential components of the assessment but are most useful in the patient with bilateral parotid enlargement. Sjgrens syndrome, diabetes mellitus, alcoholism, malnutrition, AIDS and drug hypersensitivity are important causes of bilateral (and less frequently unilateral) parotid enlargement. !

Diagnostic Imaging! The imaging options available include plain X-rays, sialography, radiosialography, ultrasound, CT and MRI. Plain X-rays are only useful in the detection of radio-opaque calculi. They should not be recommended in the assessment of parotid masses as less than 20% of parotid duct calculi are radio-opaque. However, 80% of submandibular calculi are radiopaque.!

CT/ MRI are routine investigations in some centres. CT and MRI are unlikely to influence the management of welldefined, freely movable tumours. CT and MRI are valuable in the evaluation of patients presenting with palatal or tonsillar swellings in order to distinguish between deep lobe parotid tumours and other lesions of the parapharyngeal space. ! Radiosialography: The salivary gland striated ductal epithelium can extract Tc99m (pertechnetate) from the capillary network of the salivary glands. The presence of a hot mass is usually indicative of Warthin's tumour or oncocytoma. However, hot masses may occasionally be malignant and therefore radiosialography is unreliable and is not recommended.! ! Since the advent of contrast CT, sialography and ultrasonography are rarely indicated in the investigation of the patient who has parotid disease. Sialography is helpful in suspected cases of ductal stricture, calculi or sialectasia. !

The role of fine needle aspiration cytology! Fine needle aspiration (FNA) has revolutionised the management of many head and neck lesions. The thyroid gland has been a fruitful area for the cytopathologist. The results in the salivary glands have been far less impressive. While a diagnostic accuracy of greater than 90% is sometimes quoted, this has not been the experience in most centres. ! ! .! Patients who have clinically apparent cervical nodal metastases should undergo parotidectomy, radical neck dissection and radiotherapy. Patients who have aggressive histologic types such as high-grade mucoepidermoid carcinoma, malignant mixed tumour, high-grade adenocarcinoma and squamous cell carcinoma should have prophylactic neck dissections and radiotherapy. This also applies to T3 lesions and lesions associated with facial nerve palsy.! Neck Dissection and Radiotherapy!

! !

Complications of parotidectomy! Facial nerve palsy! Haemorrhage from retromandibular vein, postauricular artery, superficial temporal artery, maxillary artery ordirectly from the external carotid artery!

Parasthesia in the distribution of the greater auricular nerve! Neuroma of the greater auricular nerve ! Freys Syndrome : this is characterized by sweating over the parotid region when the patient thinks about food or eats. It is thought to be due to aberrant regrowth of parasympathetic secretomotor nerves which end up supplying the sweat glands.!

! ! !

DYSPHAGIA!

SURGICAL ANATOMY AND PHYSIOLOGY! The pathway for swallowing involves the oral cavity, the pharynx and oesophagus.! Three anatomical areas of narrowing occur in the oesophagus:! 1.! At the level of the cricoid cartilage (at the cricopharyngeus )! 2.! In the midthorax, from compression by the aortic arch and the left main stem bronchus! 3. At the level of the oesophageal hiatus of the diaphragm!

! Deglutition can be divided into three phases: oral, pharyngeal and oesophageal phases. ! !
The oral phase is a voluntary phase during which the bolus is formed and prepared for transfer into the pharynx. Tongue mobility and adequate salivary flow are very important in this phase. The pharyngeal phase is the most complex phase, as it requires the safe transfer of the bolus across a pathway that is also used for respiration. During the pharyngeal phase the nasopharynx is separated from the oropharynx by the actions of the tensor palatini and the levator palatini muscles. Respiration temporarily stops at the end of inspiration. The larynx is elevated and pulled anteriorly under the base of the tongue. The laryngeal inlet, the false cords, and true cords are closed as a three tiered protective mechanism. All of this activity requires a finely coordinated neurological system that is also responsible for relaxation of the cricopharyngeus muscle, allowing transfer of food from the pharynx to the esophagus. Neurological disorders will often manifest themselves with dysphagia associated with episodes of aspiration. During the oesophageal phase the bolus is transferred by a series of peristaltic waves that are preceded by waves of relaxation. Liquids are predominantly transferred by gravity when the patient is in the upright position.!

! ! CLASSIFICATION OF DYSPHAGIA! !

The discussion will predominantly focus on the oesophagus!

! Oral lesions! ! Malignant lesions such as carcinoma of the tongue and the floor of the mouth ! ! Inadequate salivation e.g. post radiotherapy and Sjogrens syndrome! ! Pharyngeal lesions! ! Malignant lesions of the oropharynx and hypopharynx! ! Pharyngeal webs and strictures! !
Oesophageal lesions! Lesions in the lumen of the oesophagus ! Foreign bodies (commonly coins in children; bones or meat bolus in adults)! Lesions in the wall of the oesophagus! 1.! Benign Strictures! a.Gastro-oesophageal reflux with reflux oesophagitis! b.Corrosives! c.Post-irradiation! d.Post-traumatic! ! 2. Tumours: malignant and benign! 3. Plummer-Vinson-Paterson-Kelly syndrome ! 4. Schatzki's ring with hiatus hernia! 5. Motility disorders! ! a.! Achalasia of the cardia! b. Cricopharyngeal spasm! c. Diffuse oesophageal spasm ! 6.Scleroderma! 7. Dermatomyositis! 8.Crohn's disease! 9.Congenital atresia (tracheo-oesophageal fistulae) or stenosis!

! Lesions outside the wall of the oesophagus! !

1.! Retrosternal goitre ! 2.! Para-oesophageal hiatus hernia!

3.! Pharyngeal pouch ( Zenkers diverticulum)! 4. Mediastinal tumours or mediastinal lymph nodes ! 5.! Aortic aneurysm! 6.! Dysphagia lusoria!

Neural and Neuromuscular disorders! 1. Bulbar Palsy! 2. Myasthena Gravis! 3. Tetanus!

! The commoner conditions are:! !

1. 2. 3. Carcinoma of the oesophagus! Reflux oesophagitis with stricture formation! Achalasia of the cardia!

! DIAGNOSIS AND MANAGEMENT! !

The diagnosis is made after a careful consideration of the history, examination and special tests.! These tests may include barium or gastrografin swallow, endoscopy, videofluoroscopy and manometry.!

History!

! !

Age! Achalasia occurs most frequently in patients 30 to 40 years old.! Patients 50-70 years old, with gradual, progressive dysphagia have oesophageal carcinoma until proven otherwise.!

Sex! Paterson-Kelly syndrome occurs almost exclusively in females. Postcricoid carcinoma is an important complication of Paterson- Kelly syndrome. Apart from this carcinoma, malignant lesions of the pharynx and oesophagus are predominantly founded in males.! ! Symptoms! Dysphagia.! 1.! A sudden onset may suggest foreign body obstruction. Occasionally foreign body impaction may be the first evidence of carcinoma of the oesophagus.! 2.! A slow onset is more likely with carcinoma of the oesophagus, achalasia and stricture formation.!

Pain! Retrosternal or back pain following oesophagoscopy or dilatation suggests oesophageal perforation.! Regurgitation. diverticulum. ! This is characteristic of long-standing achalasia, a sliding hiatus hernia and pharyngeal pouch formation. Regurgitation of offensive, undigested food, or a feeling of a bulge in the neck suggests Zenker's

! ! !

Signs! Malignancy may be evidenced by weight loss, palpable cervical lymph nodes, hepatomegaly or ascites. Glossitis and koilonychias may be indicatice of oesophageal webs.!

Special tests! Barium swallow, barium meal and oesophagoscopy are most frequently helpful.!

Barium swallow! 1.! Pharyngeal pouch may be visualized as a rounded pouch ! 2.Achalasia of the cardia produces! a.Dilatation of the oesophagus ! b.The distal oesophagus tapers to a smooth point. !

! ! !

3.! Carcinoma of the oesophagus will appear as an irregular filling defect. An oesophago-bronchial fistula may be demonstrated.! Barium meal! May demonstrate associated gastric disorders such as carcinoma of the cardia extending into the stomach, hiatus hernia or peptic ulceration.! Oesophagoscopy! Flexible fibreoptic endoscopy is the preferred method for diagnostic oesophagoscopy. Suspicious lesions should be biopsied.! Manometry! This is most beneficial in the investigation of motility disorders.!

! Curative treatment! !
1.!

Pharyngeal pouch. Cricopharyngeal myotomy is an important component of the treatment and may suffice for

small pouches. The gold stardard is excision of the pouch via an external approach and cricopharyngeal myotomy. Endoscopic transection of the partition wall between the pharyngeal pouch and the oesophagus is an alterative option (Dohlmans procedure).!

2.!

Corrosive stricture. Oesophagoscopy should be done within 36 hours to determine the extent of injury and to Initially broad-spectrum antibiotics and corticosteroids are prescribed. Subsequently

guide further management.

oesophageal strictures may require repeated dilatation but ultimately bypass, usually with gastic transposition or a gastric tube flap may be necessary. If the stomach is not suitable, colonic interposition is an alternative option.! 3.Achalasia. Tthe best treatment is division of all the muscle layers at the oesophagogastric junction (Heller's cardiomyotomy). !

!
!

Carcinoma of the oesophagus! This occurs particularly in males over 60 years. There is a higher incidence in females with Plummer-VinsonPatterson-Kelly Syndrome. Longstanding achalasia and corrosive strictures are predisposing factors. Southern Africa and China have high incidences. Iron brewing pots and nitrosamines have been linked to oesophageal carcinoma.! The lower third of the oesophagus is involved in 50% of cases and the middle third in 35%.! Most are non-keratinising squamous cell carcinomas. Metaplasia of the distal oesophagus (Barretts oesophagus.) may precede the development of an adenocarcinoma. ! ! The main modalities of spread are direct and via lymphatics.! Oesophageal mucosal skip lesions may also occur as the cancer spreads in the sumucosal plane.! The mortality from oesophagectomy is still of the order of 15% and the average survival time after operation is about 15 months. !

!
Achalasia!

Failure of relaxation of the lower oesophagus (achalasia) is considered to result from a neuromuscular disorder, which may be associated with degeneration of Auerbachs plexus. Similar changes have been noted in Trypanosoma cruzi infection (Chagas' disease). The condition usually occurs in middle-aged.! The best treatment is division of all the muscle layers at the oesophagogastric junction (Heller's cardiomyotomy). !

! !

Dysphagia lusoria! The oesophagus may be compressed by an anomalous right subclavian artey or a double aortic arch.!

!
!

SWELLINGS OF THE THYROID GLAND!

Identification: - The thyroid is surrounded by the pretracheal fascia and is firmly attached to the trachea by the ligaments of Berry and thus moves upwards on swallowing.! ! When a patient presents with a thyroid mass, the doctor will need to ascertain the following:!

1. The functional status of the thyroid ! 2. Whether or not there is a neoplastic process going on! 3. Whether or not the mass is causing a local functional problem, by compression or invasion! 4. Whether there is an inflammatory or infiltrative process going on.!

5. Whether or not the thyroid mass presents a cosmetic problem to the patient.!

! Patients with thyroid swellings can be classified clinically as having! !

Diffuse, non-toxic goitre e.g. physiologic goitre! Diffuse toxic goitre e.g. Graves! Nodular goitre ----solitary or multiple and toxic or non-toxic! Most thyroid neoplasms present as solitary, non-toxic goitres!

Most thyroid masses are multinodular goitres. If the thyroid is not producing adequate amounts of thyroxine (as occus when dietary iodine intake is poor), TSH levels rise and the gland becomes hyperplastic. When thyroxine production is sufficient, increased colloid stores are found. After several cycles, multinodular goitres develop with areas of hyperplasia and other areas with colloid stores. Cystic degeneration, calcification and haemorrhage may occur in these multinodular goitres.! ! In the early stage in the development of these goitres, thyroxine supplement should be given to reduce TSH secretion. This can cause regression of hyperplastic areas but would not be expected to affect areas of cystic change and large colloid nodules. Surgery is recommended for lesions causing significant compression of the trachea, oesophagus or recurrent layngeal nerves.!

! ! Thyroiditis! !

a) Graves Disease (accounts for 85% of cases of thyrotoxicosis)! This is an autoimmune disorder characterised by a diffuse goitre, hyperthyroidism and ophthalmic involvement. Thyroidstimulating immunoglobulins (e.g. LATS, an IgG antibody to TSH receptor) and thyroid growth-stimulating antibodies result in hyperplasia and overactivity of the thyroid. The patient presents with features of a hypermetabolic state as well as symptoms related to overactivity of the sympathetic system (thyroxine facilitates sympathetic activity). Palpitations, excess sweating, weight loss despite increased appetite; heat intolerance, nervousness, tremors and menstrual disturbances are common. Cardiac arrhymias are most common in the elderly. Exophthalmos, lid lag, lid retraction, ophthalmoplegia and chemosis are the ophthalmic features. Lid lag and lid retraction are the result of increased activity in the smooth muscle of the levator palpebrae superioris (supplied by sypathetics) Pretibial myxoedema and gynaecomastia may occur. The T4 and T3 levels are elevated and the TSH suppressed. Thyroid scan demonstrates diffuse increased uptake.!

Treatment ! a. Medical Therapy with antithyroid drugs( usually carbimazole plus B-Blocker)! Often used in young patients, pregnant women, patients with cardiac complications. The disadvantages include a 50% recurrence rate after 2 years of therapy, drug rashes and leucopaenia.! b. Radioiodine 131 !

Can be given as a simple drink. Avoids the risk of surgery and the complications of medical therapy. There are theoretical risks of carcinogenesis and teratogenicity. Therefore generally given to men and women over age 40 years. The onset of its effect is slow.! c. Surgery! Acts quickly, but 1% incidence of permanent hypoparathyroidism and recurrent laryngeal nerve palsy. Generally in thyrotoxicosis, surgery is usually done for large goitres, compression symptoms, failed medical therapy, toxic nodule, and retrosternal extension. Patients should be medically controlled prior to surgery to avoid a thyroid storm!

Hashimotos Disease! This is another form of autoimmune thyroiditis. Initially patients may be hyperthyroid as hormones are released from damaged follicles. With time many become hypothyroid. This condition is associated with an increased risk of a lymphoma of the thyroid!

Causes of Hyperthyroidism! Graves Disease! Toxic nodule in multinodular goiter! Toxic adenoma! Excess thyroxine therapy! Early thyroiditis!

DeQuervains thyroiditis! Often preceded by URTI. The patient develops painful enlargement of the thyroid that may last for several weeks. The ESR is characteristically very high. The thyroxine level is elevated, the TSH level is initially normal but then decreases by negative feedback. Unlike in Graves disease, the thyroid scan demonstrates decreased uptake. Some of these patients eventually become hypothyroid.! ! Thyroid neoplasms! Adenomasvariants of follicular adenoma (including the Hurthle cell adenoma)! Carcinoma: Papillary, follicular, medullary, anaplastic! Thyroid lympomas! Papillary carcinoma! Commonest malignancy of the thyroid. Presents in younger patients than follicular carcinoma does. Cervical nodal metastases are common. Usually good prognosis with 90% 10-year survival.!

! !

Follicular carcinoma! Vascular spread predominantly. Diagnosis made by demonstrating vascular and capsular spread on histological assessment of tissue (rather than cytology).! Medullary carcinoma!

!
Most cases occur sporadically but 10 % associated with MEN IIa and IIb and 10% more occur familially but without MEN syndromes.!

! !

Anaplastic carcinoma! This is an aggressive tumour with very poor prognosis. Widespread invasion of the airway, oesothagus and pharynx are usually present at diagnosis. ! Patients dont usually present with a diagnosis and so the management of the patient with a thyroid nodule must be clearly understood.!

! ! ! ! ! MANAGEMENT OF THE SOLITARY THYROID NODULE! !

INTRODUCTION! Thyroid nodules are common. Palpable thyroid nodules are present in 5% of the adult population. Autopsy studies have found thyroid nodules in 50% of patients dying from non-thyroidal disease. Of the nodules detected at autopsy, 75 % were multinodular goitres and 25 % solitary nodules. ! The surgeon must utilise his clinical skills along with the appropriate investigations to identify the nodules that are malignant so that they can be treated appropriately. Benign nodules may also require surgical management because of symptoms of compression, hyperthyroidism with failed medical therapy or for cosmetic reasons. !

! !

The major forms of thyroid cancer are the papillary, follicular, medullary and anaplastic carcinomas but lymphomas, sarcomas, carcinosarcomas and metastatic lesions can also occur in the thyroid.! HISTORY AND PHYSICAL EXAMINATION ! A careful history and a thorough physical examination are required before embarking on special investigations of the thyroid. There is no symptom or sign that is pathognomonic of carcinoma of the thyroid but some are more frequently associated with malignancy. If the clinical features suggest malignancy, lobectomy and histologic diagnosis are generally required even if the special investigations suggest a benign lesion.!

HISTORY! men are three times more likely to be malignant.!

! !

The patients age and sex are important aspects of the history. While 80% of thyroid nodules occur in women, nodules in A history of exposure to low dose gamma radiation (0.06 to 20 Gy) increases the incidence of both malignant and benign thyroid nodules for at least 30 years after the exposure (Harvey 1990). Most of these malignancies are papillary carcinomas.!

A family history of thyroid carcinoma suggests the possibility of medullary carcinoma of the thyroid although 80% of medullary carcinomas are sporadic cases. Papillary carcinoma of the thyroid can also be familial. Patients who have Gardners syndrome and those who have Cowdens disease have an increased risk of developing thyroid cancer. Cowdens disease is characterised by mucocutaneous lesions and internal malignancy. The disorder is autosomal dominant and is associated with breast cancer, cancer of the colon and benign and malignant thyroid nodules.! A history of surgery for a previous thyroid carcinoma is important as any recurrent thyroid nodule would have to be deemed malignant unless proven otherwise by histology. Recurrence may occur because the lesion was multifocal. Persistent carcinoma can remain in the thyroid when the original carcinoma is inadequately treated and then enlarge to form a palpable recurrent nodule.! Hoarseness, secondary to vocal cord palsy that occurs on the same side as a thyroid nodule, is highly suggestive of malignancy. Large, benign thyroid nodules can cause recurrent laryngeal nerve palsy but the presence of vocal cord palsy, makes histologic diagnosis mandatory.! Dysphagia and dyspnoea are non-specific symptoms that are found in both benign and malignant lesions. Pain is present in less than 10% of malignant thyroid nodules. In fact, pain suggests thyroiditis or haemorrhage in an adenoma or thyroid cyst.! The commonest cause of a rapid increase in the size of a thyroid nodule is haemorrhage in a benign nodule. If aspiration or ultrasound does not suggest that haemorrhage has occurred then the lesion should be considered malignant and histologic assessment is required. It is worthwhile remembering that benign thyroid lesions may undergo malignant degeneration. Anaplastic change may occur in a long-standing multinodular goitre. Patients with Hashimotos thyroiditis have a 70 times higher incidence of lymphoma than the general population.!

PHYSICAL EXAMINATION! Nodules that are hard, gritty or fixed to the surrounding structures are highly likely to be malignant. Malignancy is more common in solitary nodules than in multinodular lesions. The incidence of malignancy in multinodular goitres has been reported to be 1%. Forty to seventy-five percent of clinically solitary thyroid nodules are found to be multinodular on ultrasound examination. A dominant nodule in a multinodular goitre should therefore be managed as if it was a solitary nodule because malignant degeneration may occur in a multinodular goitre.! The presence of non-tender, cervical lymphadenopathy is a strong indicator of the probability of thyroid malignancy. Recurrent laryngeal nerve palsy has already been mentioned as evidence of malignancy until proven otherwise by histology. A solitary thyroid nodule associated with an ipsilateral Horners syndrome, though rare, can be a useful pointer to malignancy with extra-thyroidal spread.!

INVESTIGATION OF THE SOLITARY THYROID NODULE! When the history and physical examination findings are very suggestive of a malignancy, then diagnostic studies such as ultrasound, thyroid scan and fine needle aspirate are generally not indicated. Histologic diagnosis is mandatory and the most cost-effective method is to proceed with thyroidectomy after radiologic assessment of the extent of the lesion. However, ninety-five percent of all patients with malignant thyroid nodules will be euthyroid and asymptomatic Diagnostic screening methods are therefore usually required to select the nodules that are likely to be malignant.!

ULTRASOUND! Ultrasound examination of the thyroid can accurately assess the size of the thyroid gland, the number and dimensions of nodules present, and distinguish between solid and cystic lesions. Ultrasound is also useful in detecting cervical lymphadenopathy. Thyroid ultrasound is non-invasive, repeatable without any special preparation and does not expose the patient to radiation. It is especially useful in pregnant women and in children as it avoids the radiation of a scan and the mild discomfort of a fine needle aspirate. ! THYROID SCAN The most common isotopes used in radionuclide imaging are I131, I123 and Tc99 pertechnetate. Technetium-99m pertechnetate is trapped in the thyroid but unlike iodine it is not organified by the thyroid. Technetium-99m pertechnetate and radioactive iodine 123 (I-123) have replaced radioactive iodine 131 (I-131) in routine thyroid scanning because of the higher radiation dose with the latter isotope. I-131 is now mainly used therapeutically and in screening for metastases. Technetium-99m pertechnetate offers the lowest radiation dose and can be given intravenously with the test being performed on the same day and this is a distinct advantage over radioactive iodine.! Nodules can be classified according to their ability to take up the radioisotope. Non-functional nodules are labelled cold, normally functioning nodules are warm and hyper- functioning nodules are hot. In Ashcraft and Van Herles review of 22 series(1981) in which all patients underwent surgery regardless of the functional status of the nodules, 84% were cold, 10.5% were warm and 5.5 % were hot. Malignancy was found in 16% of the cold nodules, 9% of the warm nodules and 4% of the hot nodules. Thyroid scans cannot distinguish between benign and malignant thyroid nodules but rather give an indication of the probability of malignancy in a nodule. It is to be noted that even hot nodules can be malignant.!

FINE NEEDLE ASPIRATION CYTOLOGY! Fine-needle aspiration (FNA) cytology has revolutionised the assessment of the solitary thyroid nodule. ! The accuracy of the technique depends on adequate sampling of the thyroid and the experience of the cytopathologist. The best results are obtained when the cytopathologist takes the aspirate as well as interprets the sample. The sample is satisfactory for a diagnosis to be made in 85% to 90% of aspirates in experienced centres. Results can be classified as benign (75%), suspicious (20%) and malignant (5%). The main limitation of the fine-needle aspirate is its inability to distinguish follicular adenoma from the well-differentiated follicular carcinoma. Instead, a diagnosis of follicular neoplasm is offered and a lobectomy with histologic diagnosis is required. Vascular or capsular invasion is necessary for the histologic diagnosis of follicular carcinoma to be made. Follicular neoplasms are responsible for the majority of aspirates that are reported as suspicious. The reported incidence of false-negative results varies from 8% to 10% in most series while false-positive results occur in approximately 2.5 % of cases.!

Lesions diagnosed as malignant and those labelled suspicious should undergo at least a lobectomy unless the diagnosis is anaplastic carcinoma or the patient is unfit for the procedure. Alternatively, suspicious lesions can be followed up with ultrasonography and thyroid scan. Nodules labelled benign require follow-up with observation for clinical features suggesting malignancy and the fine needle aspirate can be repeated because of the incidence of falsenegative aspirates. Fine needle aspiration is useful in diagnosing and treating thyroid cysts. The cellular yield from aspiration of a cyst is poor and an attempt should be made to aspirate the wall of the cyst after removal of the fluid.

Ultrasound can be useful in guiding the aspiration. Large thyroid cysts (> 4 cm diameter) and cysts that recur three times after repeated aspiration require lobectomy and histology because they can represent cystic degeneration in a malignant nodule. ! X-RAYS, CT SCAN AND MRI! These radiologic studies are not generally used to differentiate benign and malignant thyroid lesions. Plain X-rays can demonstrate tracheal deviation and compression as well as any evidence of retrosternal extension. Pulmonary metastases are uncommon on initial presentation but these are often found on follow-up X-rays. The CT scan can accurately assess the extent of the primary lesion, cervical and pulmonary metastases. Unfortunately CT scans often involve the use of significant amounts of iodine in contrast reagents. This could interfere with subsequent radioactive iodine scan and therefore MRI is to be preferred if this is available.!

SPECIAL BLOOD INVESTIGATIONS! Patients with nodular goitres are almost always euthyroid. Free T4, free T3 and TSH levels can be obtained as baseline studies in patients who will undergo surgery. Thyrotoxicosis can usually be detected by a careful history and examination but one should confirm the clinical findings because there is a risk of a thyroid storm when surgery is performed on the thyrotoxic patient.! Serum calcitonin levels should be measured in patients with a family history of medullary carcinoma and in those with intractable diarrhoea. Occasionally, features of a multiple endocrine neoplasia syndrome (such as mucosal neuromas) may be discovered and calcitonin levels should be obtained in these cases.!

TOTAL VERSUS PARTIAL THYROIDECTOMY! Considerable debate exists on the surgical aspects of the treatment of thyroid cancer. There is no large prospective, randomised clinical trial comparing the efficacy of lobectomy and total thyroidectomy in the management of thyroid carcinoma.! ! With the exception of tumours localised to the isthmus, the minimum surgery that should be performed in a case of a thyroid nodule is an ipsilateral lobectomy and isthmusectomy. (Nodules confined to the isthmus can be excised with a 1 cm resection margin of normal tissue from the medial aspect of each lobe). Partial lobectomy should generally not be done because this ! a) increases the risk of entering and disseminating tumour! ! ! b) risks a recurrence on the ipsilateral side and the recurrent laryngeal nerve and the parathyroids would then be in considerable danger of injury at revision surgery! ! ! c) is associated with a higher recurrence rate and lower survival rate in the case of malignant nodules.! !

There is general agreement that total thyroidectomy should be performed when there is! 1) extrathyroidal or metastatic disease! ! 2) gross bilateral lobar involvement! ! 3) residual tumour after previous resection! ! ! !

4) history of significant radiation to the head and neck ! ! ! There is considerable controversy on the extent of surgical resection necessary for the unilateral and grossly intrathyroidal, differentiated carcinoma of the thyroid. The most important rationale for total thyroidectomy is that it facilitates the use of radioactive iodine in the detection and treatment of any local or metastatic lesions. Normal thyroid tissue has a 100-fold greater affinity for iodine than papillary or follicular carcinoma of the thyroid. The presence of residual thyroid tissue can therefore significantly impair the uptake of radioactive iodine by metastatic lesions. ! Histologic sections have demonstrated microscopic foci of carcinoma in the contralateral lobe in up to 85% of cases of papillary carcinoma. Total thyroidectomy would eliminate these foci, which are potential sources of recurrence. The lowest recurrence rate has been found in patients who have had a combination of total thyroidectomy, radioactive iodine and TSH suppression using exogenous thyroxine.! A true total thyroidectomy eliminates the 1% risk of anaplastic change that occurs in differentiated thyroid carcinomas. The use of thyroglobin levels as a tumour marker for recurrent thyroid carcinoma is also greatly facilitated by total thyroidectomy.! The arguments in favour of a lobectomy include the reduced morbidity when this operation is compared to total thyroidectomy. The incidence of recurrent laryngeal nerve palsy and that of permanent hypoparathyroidism is increased in total thyroidectomy compared to lobectomy.!

! Factors associated with a poor prognosis in differentiated carcinoma of the thyroid! !

1) Age and sex! Males greater than forty years old and females older than fifty years have a poor prognosis. !

! !

2) Size of primary tumour and direct extra- thyroidal extension! Primary cancers 5 cm or larger and evidence of extra-thyroidal spread are factors associated with a reduced survival.! 3) Regional nodal and distant metastases! Distant metastases have a significant effect on the mortality rate of differentiated carcinoma of the thyroid. In papillary carcinoma of the thyroid, cervical nodal metastases seem to increase the incidence of recurrence in the neck but do not significantly affect the mortality rate!

! !

4) Residual tumour after surgery! Incomplete resection of carcinoma of the thyroid is an independent prognostic factor that has a negative effect on survival.! 5) Histologic type and degree of differentiation. ! The following histologic variants of papillary carcinoma tend to run an aggressive clinical course:! a) diffuse sclerosing ! b) tall cell! c) columnar cell.! !! !! !

! The degree of capsular and especially vascular invasion has an inverse relationship to survival in follicular carcinoma of
the thyroid .! ! RECOMMENDATIONS! Total thyroidectomy is a relatively safe operation in the hands of experts with a 2% incidence of recurrent laryngeal nerve palsy and 3% permanent hypoparathyroidism (Lore,1988). Total thyroidectomy facilitates radioactive iodine therapy and also facilitates the use of thyroglobulin as a tumour marker for metastases, eliminates multifocal disease, reduces local recurrence and prevents anaplastic change in any tumour which would have remained in the thyroid had a lobectomy been performed. It is difficult to argue against total thyroidectomy when this operation is performed with minimal morbidity. ! All patients with follicular carcinoma or Hrthle cell carcinoma should have a total thyroidectomy unless the patient is unfit for surgery or the primary is not resectable. Follicular carcinoma and the Hrthle cell carcinoma tend to have worse prognoses than papillary carcinoma.. Papillary carcinoma that is associated with any of the factors listed above as indicators for a poor prognosis, should be managed by total thyroidectomy. All other cases of papillary carcinoma can be treated by a lobectomy because this reduces the surgical risks to the patient and gives equivalent recurrence and survival rates to total thyroidectomy in these patients.! !

When nodal metastases are present, a modified radical neck dissection should be performed and any paratrachael or superior mediastinal metastatic nodes resected. This approach will reduce the local recurrence rate but will not significantly alter the survival rate in papillary carcinoma.! Medullary carcinoma is best managed by total thyroidectomy and unilateral or bilateral neck dissections. Anaplastic carcinoma is usually not resectable at presentation and surgical management is often limited to incisional wedge biopsy of the isthmus and tracheostomy. Radiotherapy and chemotherapy can be added but survival beyond the first year is very unlikely.! Radioactive iodine (I-131) is a useful adjunct to total thyroidectomy for the management of microscopic metastases in differentiated carcinoma of the thyroid. High TSH levels and the absence of normal thyroid tissue enhance uptake of radioactive iodine by metastases. Thyroid suppressive therapy should not be given in the first six weeks postoperatively but rather, the patient should be made hypothyroid allowing the TSH level to rise to 30 - 60 mIU/ L. This will facilitate the uptake of radioactive iodine by functional metastases in the whole body diagnostic scan (5 mCi I-131)as well as therapeutic I-131 ( 50-150 mCi ). External beam radiotherapy should be added whenever there is gross focal metastatic disease or incomplete resection. Focal metastatic lesions that do not take up radioactive iodine require excision along with external beam radiotherapy. Chemotherapy utilizing doxorubicin and cisplatin has produced a response in only a small minority of cases of differentiated carcinoma of the thyroid.! !

! ! !

Follow-up! The patient is seen every 2 months in the first year, every 3 months for the next 2 years, every 6 months over the following 2 years; and then annually. Chest X-rays and thyroglobulin levels should be obtained annually and when indicated clinically. Diagnostic whole body scans are performed annually for the first 2 years and then every 3-5 years.!

PENETRATING INJURIES TO THE NECK! Five to ten percent of traumatic injuries involve the neck. A thorough knowledge of the anatomy of the neck is essential as, with the exception of the brain; there is no other region that has so many vital structures in such close proximity. When injuries involve the neck, the potential for mortality and major morbidity is great. The mortality rate from stab wounds is one to two percent and from gunshot wounds five to twelve percent.!

The neck can be divided into anterior and posterior triangles by the sternocleidomastoid muscle. Injuries that involve the anterior triangle of the neck have a greater potential for fatality. The neck can also be divided into the Zones I-III. ! Zone I: sternal notch to the cricoid cartilage! Zone II: cricoid cartilage to the angle of the mandible! Zone III: above the angle of the mandible! The platysma muscle covers the anterior triangle and a significant portion of the posterior triangle. Traditionally it has been said that injuries that penetrate the platysma require hospitalisation. Familiarity with the location of the platysma (just deep to the skin) will make one realize that this policy would result in virtually all patients being admitted to hospital.!

The initial management follows the ABCs of the resuscitation of the patient with multiple trauma. Of paramount importance is the establishment of adequate airway control. Stridor, air escape via the neck wound, hoarseness, surgical emphysema and haemoptysis are suggestive of injury to the airway. Adequate light and suctioning are essential. If flexible endoscopy is available this should be utilised to assess the upper airway and to facilitate intubation under direct vision. In patients with gunshot injuries to the neck, tracheostomy is usually the best option as endotracheal intubation can convert a partially transected trachea to complete transection. In many cases the site of the wound facilitates the initial insertion of the tracheostomy tube.!

! ! ! ! !

One should always protect the cervical spine until cervical spinal injury can be ruled out. It may therefore be necessary to perform tracheostomy with the neck in the neutral position.! The adequacy or ventilation must be ensured. One should check for features of a tension pneumothorax. Control of hemorrhage may be achieved by tamponade while blood is taken to group and cross match the patient. The patient who is in shock but has distended neck veins may have a tension pneumothorax or cardiac tamponade.! Clinical signs of significant injury in penetrating injuries to the neck! Airway injury! Stridor, dyspnea, hoarseness, subcutaneous emphysema! Vascular injury! Circulatory shock, expanding haematoma, active bleeding, thrill or bruit, absent or reduced pulses! Oesophageal injury!

Haemoptysis or haematemesis, dysphagia or odynophagia, subcutaneous emphysema!

Neurologic signs! Deteriorating consciousness level! Contralateral neurologic signs: suggest carotid injury on the ipsilateral side! Ipsilateral neurologic signs with sensory level: suggests spinal injury! Specific features of injury to the brachial plexus, vagus, recurrent laryngeal, hypoglossal, marginal mandibular or phrenic nerve may be present.!

Possible investigations include: ! CBC, urea and electrolytes, cross match ! Neck x-rays (including lateral view of cervical spine) and chest x-rays! Doppler ultrasound or angiography! Barium swallow and endoscopy!

Neck exploration! Patients in circulatory shock, who cannot be stabilised, require urgent neck exploration.! Patients, who are stable, can be investigated prior to surgery. This is especially important in patients with level I injury were the possibility of significant intra-thoracic vascular injury exist. Access to the major vessels may be difficult in patients with level III injuries and here; once again, investigations are helpful in locating the exact site of injury. In assessing the patient, one should always bear in mind the trajectory since an entry wound may be in level II but significant damage may still occur in level III or in the superior mediastinum.!

Tonsils and adenoids! The tonsils and adenoids are the largest members of Waldeyer's ring. They are strategically located posterior to the oral cavity and the nasal cavity respectively. They are able to sample foreign organisms and antigens and present the information to the rest of the immune system so that an adequate immune response can be mounted. Tonsillectomy and adenoidectomy may be necessary when the tonsils and adenoids are causing significant obstructive problems or when they repeatedly become infected. Tonsillectomy and adenoidectomy does not generally increase in the incidence of infections. This may be partially due to the fact that the other members of Waldeyer's ring can carry out the function of the tonsils and adenoids in their absence.!

Indications for tonsillectomy and adenoidectomy! A. Infections! 1. Recurrent acute tonsillitis of a frequency that significantly impairs the patients education or occupation. In children, 4 episodes of acute tonsillitis in one year would warrant tonsillectomy.! 2. Chronic tonsillitis---- characterized by persistent sore throats, small tonsils, and chronic cervical lymphadenopathy.! 3. Two episodes of peritonsillar abscesses (quinsy)!

4. Frequent tonsilloliths (usually occurring in patients with large intra-tonsillar recesses)! 5. Recurrent febrile convulsions due to tonsillitis! 6. Carriers of Diphtheria in the tonsils! 7. Recurrent attacks of acute rheumatic fever despite antibiotic prophylaxis! 8. Atypical Mycobacterium infection of tonsils with cervical lymphadenopathy!

! ! ! ! !

B. Obstruction! 1. Obstruction to the eustachian tube resulting in otitis media with effusion or recurrent acute otitis media! 2. Obstruction to the drainage of the nose and paranasal sinuses resulting in recurrent or chronic rhinosinusitis! 3. Obstruction to the airway which may result in snoring, mouthbreathing, obstructive sleep apnoea, cor pulmonale! 4. Obstruction to the oral pathway resulting in difficult eating!

C. Miscellaneous! 1. Unilateral enlargement of the tonsils (suggestive of malignancy)! 2. Metastatic cervical lymph nodes of unknown origin! 3. In the operation uvulopalatopharyngoplasty (used to treat severe snoring)! 4. In the surgical approach to the glossopharyngeal nerve (in patients with glossopharyngeal neuralgia)! 5. In the surgical approach to the styloid process! The main complication of tonsillectomy is primary or secondary hemorrhage. The blood supply to the tonsils consist of branches from! a. Tonsillar branch of the facial artery! b. Palatine branch of the facial artery! c. Lingual artery! d. Palatine branches of the maxillary artery! e. Ascending pharyngeal artery! STRIDOR!

!
pitched. ! Glottic obstruction-----Inspiratory stridor! Subglottis and trachea----Inspiratory and expiratory!

Stridor is the auditory manifestation of obstruction of the larynx or trachea. Often musical in character. High or low

! Always require prompt assessment, establishment of aetiology and if possible relief of the obstruction! !
Common causes in adults! Upper aerodigestive cancers: Cancers of the larynx, hypopharynx, oesophagus, trachea! Thyroid cancers! Bilateral vocal cord palsy: Post thyroidectomy, Bulbar palsy!

Blunt and penetrating cervical injuries!

! ! !

Common causes in children! Congenital: Laryngomalacia, subglottic stenosis, bilateral vocal cord palsy, subglottic haemangiomas, laryngeal cysts and webs! Acquired: Laryngotracheobronchitis and epiglottitis, Foreign body aspiration, Laryngeal papillomatosis, Caustic ingestion and steam inhalations. Diphtheria now an infrequent cause.! Croup Syndrome! Laryngotracheobronchitis 6 months-2 years Preceding URTI symptoms for 2-3 days Low grade fever Drooling absent Parainfluenza virus Acute Epiglottitis 3-7 years Develop over hours rather than days Marked pyrexia Marked dysphagia and drooling Haemophilus influenza

! ! ! ! !

TRACHEOSTOMY! Creation of an opening (window) in the anterior wall of the trachea. In children a slit is made and no cartilage resected (tracheotomy).!

!
! ! ! !

Indications! Upper airway obstruction----See causes of stridor! Protection of the tracheobronchial tree: ! Coma due to Drug Overdose, CVA! ! ! ! ! ! ! ! ! ! ! ! ! ! ! ! ! ! ! ! ! Bulbar Poliomyelitis! Guillain-Barre Syndrome! Trauma- surgical or accidental with bleeding! ! into upper airways!

! Respiratory Failure Requiring prolonged intubation, Reduction of dead space! ! Outline of Tracheostomy Technique! !
Tracheostomy should be performed, whenever possible as an elective procedure, with an endotracheal tube in place. Adequate suction, lighting and oxygen should be available. The operation is ideally performed in the Main Operating Theatre. Test the competence of the valve to the tracheostomy tubes bulb. In adult patients, size 8.5 or 9 mm Portex tube is generally adequate for males. While in females size 7.5 8 mm should be selected. Alternate sizes should readily be available should there be difficulty in inserting the tube.!

! Positioning: Supine with neck in extension (place sandbag under shoulders)! !

Inject with 1% lignocaine in adrenaline 1: 200,000! Incision with size 15 blade 2cm below the cricoid cartilage or midway between the sternal notch and the cricoid cartilage! Blunt dissection and retraction of strap muscles laterally! Elevate or transect thyroid isthmus! Control bleeding. Change sucker from rigid type to flexible suction tube.! Incise trachea and hold cartilage to be resected with Allis forceps. Suction trachea and insert tracheostomy tube. Inflate bulb with air. Secure tracheostomy tube with tapes or 2/0 silk sutures. !

! ! ! ! !

Complications of tracheostomy!

! Perioperative Complications! !
ima artery!

1. Haemorrhage from the anterior jugular veins, inferior thyroid veins, brachiocephalic veins and rarely thyroidea 2. Injury to the recurrent laryngeal nerve! 3. Injury to the oesophagus with the creation of a tracheo- oesophageal fistula! 4. Injury to thoracic duct leading to a chylous fistula! 5. Pneumothorax! 6. Massive surgical emphysema! 7. Apnoea in the patient who has had long-standing upper airway obstruction!

! Early Complications! ! ! !
Late Complications!

8. Obstruction to the tracheostomy tube! 9. Accidental decannulation!

10. Granulation tissue formation in the trachea! 11. Tracheal stenosis! 12. Erosion into the innominate artery! 13. Tracheo- cutaneous fistula! 14. Keloid formation at the tracheostomy site!

Facial Trauma!
-Mr. Ladi Doonquah!

Basic Approach to the trauma patient:! "! "! "! "! "! "! "! "! "! "! Primary and Secondary Surveys! Airway and Cervical spine precautions! Breathing! Circulation! Disabilities (functional and neurological)! Extremities (fractures, lacerations)! Airway (upper airway obstruction)! C-spine! CNS injury! Vascular injury (especially if there is penetrating injury to the neck)!

In the facial trauma patient, pay special attention to associated injuries:!

Facial Fractures! Fracture nomenclature:! Simple closed fracture! Compound open fracture (laceration, through periodontal ligament)! Complex open or closed, involving vital structure (example: neurovascular bundle, teeth)! Greenstick through one cortex (more common in children due to higher bone elasticity)!

! !

Frontal bone or frontal sinus fractures! Signs and symptoms to consider: epistaxis, CSF rhinorrhea, cosmetic deformity, periorbotal edema and emphysema, pneumocephalus (suggests posterior table fracture), CNS deficits! Work up: clinical exam, Neurosurgery consultation, plain film (Waters' view), CT scan (gold standard)! Treatment considerations:! Anterior +/- posterior table fractures! Frontal sinus reconstruction! Sinus obliteration (with pericranium, adipose tissue, cancellous bone) if ostium is obstructed!

Orbital fractures! Signs and symptoms to consider: periorbital edema, emphysema and ecchymosis, subconjunctival hemorrhage (suggests breach in periosteum/periorbita), dystopia, diplopia, enophthalmus, reduced range in extraocular muscle movement, epistaxis, infraorbital nerve (V2) paresthesia, opacification of ipsilateral maxillary sinus on CT or plain films! Work up: clinical exam, Ophthalmology consultation, CT scan!

Treatment considerations:! Reduction and fixation of fractured segments! Release of entrapped soft tissues! Reconstruction of avulsed orbital wall(s) (with autogenous cortical bone, alloplastic mesh)! Perioperative nasal precautions to prevent periorbital emphysema!

Notes on orbital blowout fractures:! Isolated orbital floor fracture! Commonly the inferior rectus muscle is entrapped between fractured bony segments causing limitation in Tear drop sign on Waters' view (inferior orbital fat herniation into maxillary sinus)!

upward gaze, and diplopia when looking up!

Zygoma (zygomaticomaxillary or ZMC) fractures! Signs and symptoms to consider: similar to orbital fractures, also look for facial asymmetry (depression of malar eminence), trismus (due to mechanical obstruction of coronoid process of mandible during opening), depressed zygomatic arch, step deformity of inferior orbital rim! Work up: clinical exam, Ophthalmology consultation, CT scan! Useful plain films include Waters', submentovertex views! Treatment considerations:! Reduction and fixation of fractured segments!

Nasal fractures! Signs and symptoms to consider: nasal deviation, depression of dorsum, nasal congestion or obstruction, epistaxis, septal deviation, septal hematoma (must look with nasal speculum), edema and ecchymosis! Workup: clinical exam, plain films (Waters', lateral nasal, anteroposterior views)! Treatment considerations:! Closed reduction or open reduction and fixation! Anterior nasal packings! External nasal splint! Septorhinoplasty (usually delayed and done secondarily)!

! ! !

Maxillary fractures! Classification based on fracture patterns:! Leforte I: horizontal! Leforte II: pyramidal! Leforte III: transverse (craniofacial dysjunction)! Combination of above (example: right Leforte I and left Leforte III)!

!
Signs and symptoms to consider: midfacial edema, mobility of maxilla, malocclusion (+/- open bite), and any signs and symptoms of orbital/ZMC fractures in patients with Leforte II or III fractures! Workup: clinical exam, plain films (Waters', AP, submentovertex views), CT scan (gold standard)! Treatment considerations:! Reduction and fixation of fractures segments!

Mandibular fractures! Fracture classification (based on location):! Symphyseal through mandibular incisor sockets! Parasymphyseal mesial or distal to canine! Body mesial to first premolar to distal of second molar! Angle mesial or distal to wisdom tooth through to gonial angle of mandible! Subcondylar/ramus inferior to condyle at or below level of sigmoid notch! Coronoid at or below level of sigmoid notch!

!
o o

Fracture classification (based on fracture segment favorability):! Horizontally favorable/unfavorable! Consider muscles with vertical vectors (temporalis, suprahyoids, medial pterygoid, masseter)! Vertically favorable/unfavorable! Consider muscles with horizontal vectors (mylohyoid)!

Signs and symptoms to consider: malocclusion, trismus, inferior alveolar nerve (V3) paresthesia, lingual or floor of mouth edema and ecchymosis, step deformity, segmental mobility of segments, facial asymmetry (often due to edema)!

! Workup: clinical exam, plain films (mandibular series), CT scan optional! ! ! !

Mandibular series:! Right and left lateral oblique views OR orthopantomogram, AND! AP view, AND! Reverse Towne's view (to assess for subcondylar fracture)!

! !

Treatment considerations:! Address upper airway obstruction, due to floor of mouth edema or bilateral parasymphyseal fractures! Closed reduction using maxillomandibular fixation ("mouth wired shut")!

Open reduction and fixation! Physiotherapy (jaw opening exercises) in condylar fractures to prevent TMJ ankylosis!

Approximate frequency of mandibular fractures based on anatomical location.!

Table of Contents!

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~Dermatology~!

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73! 80! 81! 84! 87! 89! 89! 95! 99! 100! 102! 103! 107! 108! 110! 112! 113! 114#

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Dermatology Study Guide! Introduction to Dermatology! Psoriasis! ! ! ! ! Structure & Function of the Skin! Lichen Planus! ! Pityriasis Rosea!!

Eczema & other types of Dermatitis! Fungal Infections of the Skin! Parasitic Infestations! ! Viral Infections of the Skin! Acne Vulgaris! ! Generalized Pruritus! Drug Eruptions!! ! ! !

Bacterial Infections of the Skin! ! Disorders of Pigmentation!

Skin Manifestations of Human Immunodeficiency Virus Infection! Skin Diseases Associated with Malignancies! Blistering Diseases! ! ! !

DERMATOLOGY STUDY GUIDE!

INTRODUCTION! Welcome to the Dermatology clerkship. Dermatology is the study of the science of the skin and its diseases. However, as the largest organ in the body the skin often reflects the health of the body and therefore often overlaps with other specialties.!

Dermatology evolved from general internal medicine during the course of the nineteenth century. Most diseases of the skin had fallen for many centuries within the province of the surgeon. Prior to the eighteenth century, physicians were not very concerned with the skin. However, during the last decades of the eighteenth century, many of the great physicians recorded their observations on diseases of the skin. In the past 50 years, the character of dermatology has changed and the work of the dermatologist now embraces every aspect of the biology of the skin, normal and abnormal. Although often overlooked, approximately 20% of consultations in family practice relate to the skin.!

! !

Prior to this clerkship, you should revise lecture notes received in Stage I in the module, Endocrinology and Skin. Lectures in Internal Medicine, Pathology and Microbiology may also be relevant.! AIMS AND OBJECTIVES! This 5-week clinical Dermatology clerkship aims to achieve the following objectives:! 1. 2. 3. To enable you to be able to conduct a proper examination of the skin! To enable you to understand and use the language of Dermatology! To enable you to diagnose and be familiar with the principles of management of common dermatological disorders in the Caribbean!

STRUCTURE OF THE CLERKSHIP! The Dermatology clerkship consists of a 5-week clerkship shared with Ear, Nose and Throat occurring in Year 4. Students should report to the Dermatology Clinic in the Medical Outpatients Department at 2.00pm on the first Tuesday of the clerkship, where an introduction to Dermatology will be given.!

! ! ! ! ! ! ! !

The weekly timetable is as follows:! Monday Tuesday Thursday ! Friday

Morning

9.30am! Ward Round!

!
Afternoon 2.00pm! Dermatology Clinic!

! ! !
11.00am! Lecture / tutorial

11.00am! Lecture / tutorial

2.00pm! Dermatology Clinic!

! !

! There may be variations in this timetable in some weeks and when this occurs you will be informed. ! !
Students will be divided into groups and rotated through the UHWI Clinics, Ward Rounds and the Paediatric Dermatology Clinics at the Bustamante Hospital for Children. Every attempt will be made to make the experience as uniform as possible throughout the groups in each clerkship. !

DRESS CODE AND MORAL / ETHICAL RESPONSIBILITIES! The dress code is as outlined for the Faculty of Medicine. Cellular telephones must be turned off during all clinical sessions, ward rounds, lectures and tutorials.!

! There should be no eating in the clinics and in the clinical areas on the ward.! !
LEARNING RESOURCES! Recommended Textbooks ! 1. 2. 3. Mackie R. Clinical Dermatology: An illustrated textbook; Oxford University press.! Graham-Brown R, Burns T. Lecture Notes in Dermatology; Blackwell publishing.! Marks R. Roxburghs Common Skin Diseases; Oxford University Press.!

! You only need to purchase one of the above recommended texts.! !

Reference Textbooks! 1.

Freedberg I et al. Fitzpatricks Dermatology in General Medicine; McGraw Hill.! Champion RH, et al. Rooks Textbook of Dermatology.!

2.

Journals:! 1. 2. 3. 4. Archives of Dermatology! Journal of the American Academy of Dermatology! Dermatology Clinics of North America! Journal of Investigative Dermatology!

! ! CORE TOPICS! ! 1. Clinical Approach to Skin Diseases! ! Refer back to the introductory lectures in Stage 1. You should be able to define a rash using basic terminology.! !
Symmetry! 3. Symmetrical lesions are usually endogenous! Asymmetrical lesions are usually exogenous! 4.

! !

Distribution! You should be able to discern dermatoses with predilection for certain sites e.g. seborrhoeic dermatitis, psoriasis.! Arrangement of lesions! 5. 6. 7. 8. 9. Grouped! Disseminated (widespread discrete lesions)! Generalized! Annular ! Linear!

Morphology of lesions! 10. Color! 11. Size! 12. Shape! 13. Border demarcation!

Types of lesions:! 14. Flat: macules, patches! 15. Solid elevated: papules, plaques, nodules! 16. Fluid filled: vesicles, bullae, pustules!

Involvement of mucosae, scalp or nails.!

!
How to approach skin rashes! 17. Take an appropriate history.! 18. Describe a patients rash in terms of distribution, morphology and associated findings.! 19. Suggest a differential diagnosis.! 20. Suggest relevant investigations.! 21. Formulate a treatment plan.!

! ! !
!

2. Chronic Leg Ulcers! Chronic leg ulcers are a common problem in the Caribbean and pose a major socio-economic problem.!

How to approach leg ulcers! 22. Definition! 23. Aetiology! 24. Clinical assessment! 25. Investigations! 26. Management! 27. Complications!

! ! ! 3. Common skin disorders encountered in clinical practice! !

! ! ! - ! Eczema! - ! Generalized pruritus! - ! Acne vulgaris! -! -! -! ! ! ! -! -! Psoriasis, Lichen planus, Pityriasis rosea! Blistering disorders and drug eruptions! Common infection and infestations! Changes in skin colour! Genetic diseases!

- ! Skin changes in systemic disease!

! ! !

4. Skin changes in systemic disease!

!
28. Genodermatoses: Neurofibromatosis, tuberous sclerosis! 29. Collagen vascular diseases: systemic lupus erythematosus, dermatomyositis, systemic sclerosis! 30. Skin markers of malignancy: Acanthosis nigricans, erythroderma, acquired ichthyosis.! 31. Skin manifestations of HIV and HTLV 1 infections!

! ! 1. !
2.

TUTORIALS! Formal tutorials will be done on the following topics:! 1st Wed, 11.00am: Structure & Function of the Skin.! 1st Fri, 11.00am: History, Examination & Investigation of Chronic Leg Ulcers. !

Management of Chronic Leg Ulcers. !

! ! 3. ! 4. ! ! 5. ! ! 6. ! ! 7. ! ! 8. ! ! 9. ! !

2nd Wed, 11.00am: Eczema, Generalized Pruritus! 2nd Fri, 11.00am: Acne vulgaris.!

3rd Wed, 11.00am: Psoriasis vulgaris, Lichen planus, Pityriasis rosea!

3rd Fri, 11.00am: Blistering disorders and drug eruptions.!

4th Wed, 11.00am: Common Infections & Infestations of the Skin.!

4th Fri, 11.00am: Changes in Skin Colour.!

5th Wed, 11.00am: Skin Changes in Systemic Disease. Genetic Diseases.!

ASSESSMENT! Twenty percent (20%) of marks will be from attendance and participation in the clerkship (See Clerkship Activity Record Sheet). Eighty percent (80%) of marks will be from the examination. Students will have a 1-hour examination consisting

of clinically orientated multiple choice questions at the end of the clerkship. This examination will take place on the last Friday of the clerkship unless changed by mutual agreement. !

! SAMPLE MULTIPLE CHOICE QUESTIONS! !

diagnosis is:! A. B. C. D. E. Acute tinea corporis! Seborrhoeic dermatitis! Pityriasis rosea! Atopic dermatitis! Psoriasis vulgaris!

1. An otherwise well 12-year old boy presents with a 2-week history of onset of a mildly pruritic rash involving his trunk and proximal limbs. The lesions are oval shaped or annular papules or plaques with peripheral scales. The most likely

Correct answer: C! The age of the boy makes pityriasis rosea likely. The relatively rapid onset and the distribution and types of lesion are all in keeping with pityriasis rosea. Generalized tinea corporis is quite uncommon and with such rapid onset would suggest an underlying illness. The distribution is not in keeping with seborrhoeic dermatitis, atopic dermatitis or psoriasis vulgaris.!

! !

2. The following statement is true regarding blistering disorders:! A. B. C. D. E. Pemphigus vulgaris responds promptly to moderate doses of oral corticosteroids.! Pemphigus foliaceus seldom presents with intact blisters.! Bullous pemphigoid has a predilection for the face and trunk.! Bullous pemphigoid affects the oral cavity in over 50% of cases.! Pemphigus foliaceus presents with oral ulceration in the majority of cases.!

Correct answer: B! Pemphigus vulgaris usually requires high doses of corticosteroids for control and even then usually responds slowly. Bullous pemphigoid has a predilection for the limbs and in severe cases involves the trunk. Involvement of the face is rare. Involvement of the oral cavity is also uncommon in both bullous pemphigoid and pemphigus foliaceus. Pemphigus foliaceus rarely presents with intact blisters as the split in the skin occurs superficially in the granular layer. Blisters, therefore, are flaccid and rupture easily.!

Written & edited by Dr. Althea East-Innis! Last revised: April 2011!

! !

! DEFINITIONS!

INTRODUCTION TO DERMATOLOGY!

Macule:! Flat lesion < 1 cm in diameter! Papule: ! Solid elevated < 1 cm in diameter! Vesicle:!Fluid-filled elevated lesion < 0.5 cm diameter! Patch:! ! Flat lesion > 1 cm in diameter! Nodule:! Solid circumscribed palpable lesion > 1 cm in diameter! Plaque:!! Solid elevated lesion > 1cm with a mesa-like or disc-like shape! Bulla:! ! Fluid-filled elevated lesion > 0.5 cm in diameter! Blisters:! Refers to both vesicles and bullae! Erosion:! Area of skin in which there is partial loss of epidermis! Ulcer:! ! Area of skin in which there is complete loss of epidermis!

! HISTORY! Commonest complaint is a rash.! ! History of presenting complaint:!

Duration?! What did it look like at the beginning?! Does it itch?! If it itches when is the itching worse?! Relieving or aggravating factors?! Is the rash intermittent or persistent?! What has the patient been putting on it and with what effect?! ! Past medical history:! Past history of skin disease! History of atopy! Other medical problems!

! Atopy is said to exist when there is a personal or family history of eczema of a particular distribution, asthma and hay
fever. It is a genetically determined disorder in which there is an increased liability to form IgE (reagin) antibodies. IgE plays no part in the aetiology of eczema but is important in asthma and hay fever.!! ! ! ! !

! Drug history:!

Cutaneous drug reactions are common.! Enquire about prescribed drugs and over the counter medication do not forget the oral contraceptive pill!! Remember topical medication.! Note any known drug allergies.!

! Social and family history:!

Intake of alcohol! Smoking specify cigarettes, marijuana etc.! Living conditions! Family history of skin disease and/or atopy! Do any other family members or members of the household have a skin problem?!

! Occupational history:! Note occupation and hobbies be specific.! ! EXAMINATION (2 main rules)!
1. Good light! Best light is daylight! Next best is fluorescent light!

! 2. Examine the skin in its entirety includes scalp, oral mucous membranes, external ear, finger and finger webs, !

external genitalia, toes and toe webs, nails.!

! Diagnosis is often made on the pattern of distribution. Therefore make a panoramic view first. Then look for primary and predominant lesion and morphology of each. ! ! a. Position the patient lying down undressed with only underwear or with a towel or sheet draped across the
genitalia.! b. Take a panoramic view from the foot of the bed. Comment on the distribution. You can comment briefly on the types of lesions but do attempt to describe the lesions in detail from that position.! c. ALWAYS EXAMINE FROM THE RIGHT SIDE OF THE PATIENT.! d. Examine anterior scalp, ears, forehead, eyelids conjunctiva, cheek, nose and chin, oral mucous membrane, neck.! e. Examine upper chest, anterior shoulders; examine right upper limb anterior and posterior aspect.! f. Examine the dorsum of the hands and REMEMBER TO EXAMINE THE NAILS.! g. ENQUIRE ABOUT TENDERNESS OF THE JOINTS AND PALPATE THE SMALL JOINTS OF THE HANDS.! h. Examine finger webs, palms, medial aspect of the right upper limb, axilla.! i. Repeat the process on the left side.! j. Continue examining from mid anterior chest, lower anterior chest, abdomen, genitalia, anterior lower limbs, dorsum of feet, toenails, and soles of feet.! k. DO NOT PALPATE THE FEET AT THIS POINT.! l. To examine the dorsal (posterior) aspect of the head, neck and body there are several positions which can be adopted:! i. If the patient is fit and health they can be asked to lie on the abdomen.! ii. The patient can be asked to roll away from you and lie on the left side.! iii. The patient can be asked to sit upright and the upper body examined, and then asked to roll on the side and lower body including buttocks examined (this is probably the most common position used in the clinic and exam setting).! m. Toes can be palpated at the end and toe webs examined.!

! This examination can be successfully completed in < 5 minutes.! ! Written & Edited by Dr. Althea East-Innis! ! ! STRUCTURE & FUNCTION OF THE SKIN! ! EPIDERMIS! ! Cornified layer! ! ! ! ! ! ! Stratum corneum! ! Granular layer! ! ! ! ! ! ! Stratum granulosum! ! Prickle cell layer! ! ! ! ! ! Stratum spinosum! ! Basal layer! ! ! ! ! ! ! Stratum basale! ! ! Malpighian layer = Prickle cell layer + Basal layer! ! Histologically, the epidermis consists of at least four cell types:!
-

! The basal layer is the germinative layer of the epidermis.!

keratinocytes! melanocytes! Merkel cells! Langerhans cells!

Keratinocytes are formed by division of cells in the basal layer.! Cells in the prickle cell layer are connected by the desmosome filament system.!

In the granular layer, cells contain keratohyalin and membrane coating granules.! Further maturation leads to loss of nuclei and flattening of the cellular outline to form squames in the stratum corneum.! The epidermal transit time or turnover time is approximately 28 days.!

! Melanocytes are dendritic cells derived from the neural crest.!

They are found only along the basal layer of the epidermis.! The ratio of melanocytes to basal cells varies considerably from 1:4 in the cheek to 1:10 in the arm.! The melanin granules are transferred along the dendritic processes to adjacent keratinocytes where they are actively phagocytosed.!

! The Merkel cell is a distinctive cell found in the epidermis of mammals.!

It is present in the basal layer of the epidermis, particularly on acral non-hairy skin (palms and soles), and also in association with hair follicles.! Merkel cells are also found within the dermis in association with Schwann cells of peripheral nerve endings.! These cells are thought to function as slowly adapting sensory touch receptors.!

! Langerhans cells are dendritic cells present in the suprabasal layers of the epidermis.! ! DERMIS!

They have also been found in the dermis.! It is now thought that the Langerhans cell is a bone-marrow-derived immunocompetent cell, which provides a trap for contact antigens and presents them to T cells.! The dermis is the supporting layer of the skin and consists of a fibrous component (collagen and elastin) together with the so-called ground substance.! Lying within it are the epidermal appendages and the neurovasculature, and a cellular component including fibroblasts and various inflammatory cells.!

! HAIR FOLLICLE!

Consists of the hair shaft surrounded by a downgrowth of epidermal cells into the dermis.! Supplied with blood vessels by an invagination of dermis known as the hair papilla.! Sebaceous glands open into the pilary (hair) canal, and some follicles in certain areas, notably in the axilla, have, in addition, an apocrine gland.!

! Hair undergoes cyclical periods of growth divided into three phases:! ! In children the hair covering the body is vellus hair. ! ! There are 2 types of sweat glands:!

Anagen, in which there is active hair growth approximately 3 to 5 years! Catagen, the slowing down phase (phase of involution) approximately 2 weeks! Telogen, the resting phase 3 months! The hair of the scalp, eyebrows and eyelashes is terminal hair, which has a medulla and is thicker and more pigmented than vellus hair.! At puberty terminal hair develops in the axillae and pubic area of both sexes, and in the male, also in the beard area and body.! 1.! Apocrine! - found in axillae and anogenital area! - function unknown but in animals responsible for scent production! 2.! Eccrine! - influenced by emotions and has thermoregulatory function!

! TREATMENT PREPARATIONS! ! Main topical preparations used are:!

lotions! creams! ointments!

! Lotions! ! Creams!

pastes! gels!

Almost pure water! Used in very wet lesions e.g. exuding lesions of eczema! Lotion commonly used is dilute potassium permanganate 1:10,000 soln, has antibacterial and drying effect! Calamine lotion also has a drying effect! Water and normal saline are examples of lotions! Semisolid emulsions containing both oil and water.! Oil in water (aqueous) creams are water miscible, cooling and soothing. They have a mild moisturizing and emollient effect e.g. aqueous cream.! Water in oil (oily) creams are immiscible with water and therefore more difficult to wash off. They are emollient, lubricant, moisturizing but less so than ointments.!

! Ointments! ! Pastes ! ! Gels!

Oil or grease based.! They are emollient and protective.! They restrict trans-epidermal water loss and are therefore hydrating and moisturizing.! Used on dry scaly lesions.! Powder in a greasy base.! Example is Dithranol in Lassars paste used in psoriasis.! Semisolid preparations with high molecular weight polymers e.g. methylcellulose and can be regarded as semiplastic aqueous lotions.! They are non-greasy, water miscible, easy to apply and wash off.! Especially suitable for treating hairy parts of the body. !

! Special preparations! Emulsifying ointment used as soap substitute and emollient! ! Hair pomade !
-

! TOPICAL STEROIDS! ! 2.! ! 3.! ! 4.!

-

used for scaling of the scalp! consists of 2% salicylic acid, 2% sulphur in an aqueous cream base!

Divided into:! 1.! Mild e.g. 1% hydrocortisone cream! - used on the face except in specific conditions! - used in babies and children! Moderately potent e.g. Clobetasone butyrate 0.05% (Eumovate)! Potent e.g. Betamethasone valerate 0.1% (Betnovate)! Very potent e.g. Clobetasol propionate 0.05% (Dermovate)! given under supervision for short periods of time (2 3 weeks)! !

! Side effects of topical steroids:!

1.! 2.

Atrophy or thinning of the skin! Hypopigmentation!

! Triamcinolone is an injectable steroid, which may be injected intralesionally.! ! FURTHER READING! Side effects of systemic steroids! !!
PSORIASIS! Definition:! A chronic, non-infectious, inflammatory disorder of the skin.!

3. 4. 5. 6.

Telangiectasia! Acne! Striae! Systemic absorption!

! Typical lesions:! Symmetrical, well-defined, erythematous (red) plaques covered by thick silvery scales.! ! The extent is variable and plaques may join to form irregular patterns.! Psoriasis is one of several disorders, which may develop in sites of injury KOEBNER PHENOMENON.! ! Aetiology:!
Affects both sexes equally.! No race is exempt but:! - most common in the Indian subcontinent! - least common in Afro-Caribbean, American black, Japanese and North American population.!

! 1/3 of patients recall a family history.! Strong association with human leucocyte antigens (HLA) B27 reported in pustular psoriasis and psoriatic arthropathy.! ! Pathology:!
Cells in the basal layer divide uncontrollably. ! The turnover time is decreased resulting in abnormal maturation.! Sometimes nuclei are retained in the horny layer this is called PARAKERATOSIS.! Accumulation of neutrophils in the stratum corneum may lead to Munro micro-abscess formation.! The rete ridges in the basal layer become more prominent.! Increased vascularity leads to erythema; if the skin is scratched, bleeding points can be seen AUSPITZS SIGN.!

! Precipitating factors:!

1) Streptococcal infections in guttate psoriasis! 2) Drugs may exacerbate the disease:! - Alcohol! - Antimalarials! - Lithium! - Beta-adrenergic blocking agents!

! Common sites:! ! Nails:!

Scalp! Extensor surfaces of elbows and knees! Lower back and sacrum! Anterior chest! Involvement of the nails occurs with or without psoriasis of the skin.! Nail changes include:! - Thimble pitting! - Onycholysis!

! ! GUTTATE PSORIASIS!

Subungual hyperkeratosis! Oil drop discolouration! Splinter haemorrhages!

Guttate means raindrops.! Occurs more commonly in adolescents or young adults.! ! ! Acute in onset.! Commences 2 to 3 weeks after streptococcal throat infection.! In most cases, resolves completely within 4 months.! In some patients psoriasis never recurs.! In others, it is followed by the insidious onset of psoriasis vulgaris.! Prognosis usually good.!

! FLEXURAL PSORIASIS (PSORIASIS INVERSUS)! ! Causes of skin lesions in the groin:!

1) Candidiasis! 2) Eczema! 3) Tinea cruris! 4) Psoriasis! 5) Erythrasma!

May occur in the major flexures as part of general cutaneous involvement but may specifically and predominantly affect flexures.!

! ERYTHRODERMIC PSORIASIS!

The term erythroderma implies that greater than 90% of the cutaneous surface is involved.! (Psoriasis is one of the most common causes but eczema, mycosis fungoides ! Usually areas of both erythema and scaling present.! 1) Patient often unwell with fever and leucocytosis.! 2) Generalised vasodilatation leads to excessive loss of body heat and central hypothermia.! 3) Increased cutaneous blood flow leads to high output cardiac failure.! 4) There is increased loss of water because the epidermal barrier to water loss is impaired.! 5) Increased loss of protein and iron through exfoliating scales leads to hypoproteinaemia and iron deficiency anaemia.!

! Factors, which may precipitate erythrodermic psoriasis:!

1) Infections! 2) Hypocalcaemia! 3) Antimalarial drugs! 4) Irritants and topical application to skin e.g. tar! 5) Corticosteroids ! - systemic! - potent topical!

! PUSTULAR PSORIASIS!

2 forms of the disease:! 1) Localised to the palms and/or soles! 2) Generalised!

! GENERALISED PUSTULAR PSORIASIS OF VON ZUMBUSCH!

The patient feels ill, has a fever and leucocytosis.!

Rare disorder but appreciable mortality.! ! Systemic steroids or large quantities of very potent topical steroids may precipitate disease if reduced or withdrawn too quickly.!

! SYSTEMIC STEROIDS SHOULD NEVER BE USED TO TREAT PSORIASIS.! !

Extensive sheets of small sterile yellow pustules cover skin, which is erythematous and sore.! Complications of the disease include:! 1) Septicaemia! 2) Dehydration! 3) Electrolyte imbalance! 4) Hypocalcaemia! 5) Hypoalbuminaemia! ! ! ! ! ! ! ! ! Methotrexate and Acitretin are effective drugs for treating pustular psoriasis.!

! PSORIATIC ARTHROPATHY! Predilection for distal interphalangeal joints and sacroiliac joint.! ! TREATMENT!

TOPICAL! 1) Tar products:! - Coal tar ointment! - Liquid coal tar! - Tar shampoo! 2) Emulsifying ointment as soap! 3) Hair pomade:! - 2% salicylic acid! - 2% sulphur! in aqueous cream base.! 4) Salicylic acid may be added to other products or to an ointment base keratolytic, aids removal of scales and improves penetration of keratolytics.! 5) Dithranol! - Dithrocream 0.1 to 2%! - Dithranol in Lassars paste 0.1 to 0.5% (occasionally 1% prescribed)! Lassars paste = 2% salicylic acid, 25% starch, 25% zinc oxide in soft paraffin!

!Tar or Dithranol should not be used in the flexures, as they are irritant.! ! 6) Topical corticosteroids!
-

! 7) Calcipotriol (Vitamin D analogue)! ! PUVA = PSORALENS + ULTRAVIOLET A!

Has a minor role! Used on delicate areas such as scalp, face and flexures.!

Psoralens are photosensitisers.! 8 methoxypsoralens given orally. Approximately 2 hours later, the skin is irradiated with UVA.! Topical psoralens either painted on to psoriasis or added to bath water followed by UVA may be used.!

! SYSTEMIC! If all have failed then systemic treatment should be used.! ! Methotrexate!
Indications for Methotrexate:! 1) Severe incapacitating disease! 2) No response to treatment! 3) Erythrodermic psoriasis! 4) Pustular psoriasis!

! Common side effects:!

-

Headache! Nausea and vomiting! Gastrointestinal haemorrhage (mucositis)!

Alopecia! Oligospermia! Teratogenesis in early pregnancy (so all female patients should take contraceptive precautions)! Hepatotoxicity!

Hydroxyurea! Less effective than Methotrexate!

! Acitretin (Vitamin A analogue)! Effective in all forms of psoriasis.! ! Common side effects:!
-

! Cyclosporin A!

Dryness of skin and lips! Increased liver enzymes! Increased serum triglyceride and cholesterol! Diffuse interosseous calcification! Teratogenesis!

Main side effects! - Hypertension! - Nephrotoxicity! - Hypertrichosis!

! LICHEN PLANUS! ! Definition: A self-limiting, pruritic, inflammatory disorder of the skin.! ! Typical lesions:!
-

! Aetiology:!
-

Flat-topped, polygonal, purple (violaceous) papules.! Fine white lines may be visible on the surface, so-called Wickhams striae.! Lesions may appear at sites of trauma to the skin Koebner Phenomenon!

Cause unknown.! ! ! ! ! ! ! ! ! ! The disease is seen in association with various autoimmune disorders such as primary biliary cirrhosis, myasthenia gravis, chronic active hepatitis and diabetes mellitus.!

! Some drugs may induce lichenoid eruptions e.g.!

! A lichenoid eruption also occasionally occurs on the hands in those who come in contact with colour developer in photography.! ! Clinical features:!
The eruption begins abruptly.! Usually very itchy.! ! Sites of predilection:! - Wrists! - Ankles! ! ! ! ! ! ! ! ! ! !

Thiazide diuretics! Methyldopa! Antimalarial drugs! Organic arsenicals! Anti-tuberculosis drugs e.g. PAS, streptomycin, isoniazid! Sulphonylureas! Penicillamine!

! The oral mucosa may be involved with:!

Shins! Lumbosacral region! Genitalia!

! Nails may be affected causing longitudinal ridging or permanent destruction.! The scalp may be involved leading to scarring alopecia.! ! New lesions continue to appear over 9 18 months; these then begin to subside leaving intense hyperpigmentation, which may remain for 2 3 years.! ! Varieties of lichen planus:!
1.! 2.! 3.! 4.! 5.! 6.! 7.! 8.! Predominantly papular! Atrophic! Bullous! Linear! Actinic (occurring in sun-exposed areas)! Hypertrophic (commonly occurs on the legs)! Annular (commonly occurs on the glans penis)! Guttate!

oral ulcers! lacy white patches on the buccal mucosa (pathognomonic)!

! Treatment:!
-

Mainly symptomatic.! 1.! Antihistamines for itching! 2.! Potent topical steroids!

! Systemic steroids may be indicated in:! ! !

hair involvement! nail involvement! widespread lesions with intense itching which interrupts normal activity! atrophic L.P! bullous L.P.! PITYRIASIS ROSEA!

Definition: Acute self-limiting disorder of the skin.!

! Viral aetiology suspected.! Occurs most commonly between ages 10 to 35 years.! ! Clinical features:!
neck! chest! abdomen! ! back! ! upper arms! upper thighs!

Starts with an initial lesion called a herald patch which is a pink (rosea) oval patch 3 6 cm in diameter with a collarette of scale towards the periphery.! It is followed in 10 days to 2 weeks by an eruption of similar but smaller lesions 1 3 cm.!

! The distribution of lesions is essentially that of a T-shirt and shorts i.e.!

! ! ! ! ! ! ! ! ! ! ! ! ! ! ! ! ! !

! ! !

! ! !

On the chest and back lesions tend to run along the skin lines giving a Christmas tree appearance.! New lesions continue to appear for 2 3 weeks, and then resolution occurs.! The condition lasts 6 10 weeks.! Second attacks are rare.! Treatment:! Purely symptomatic.! Antihistamines for itching.! Mild topical steroids may be used.!

! In atypical varieties rule out secondary syphilis, do VDRL, FTA.! ! ! ECZEMA! ! All eczema is dermatitis but not all dermatitis is eczema.! ! Definition:!

A pattern of inflammation of the skin characterized clinically in the acute stage by ill-defined groups of erythematous vesicles and/or papules and in the chronic stage by scaling and lichenification.!

! Lichenification is defined as a thickening of the skin with an exaggeration of normal skin markings. Sometimes there is hyperpigmentation but not necessarily so.! ! Clinical classification:!
-

! Histology:!

Acute! Subacute! Chronic!

Histological hallmark = spongiosis! Spongiosis means intercellular epidermal oedema this is most prominent in the acute stage.! In the subacute stage, spongiosis diminishes and acanthosis increases.! Acanthosis is hyperplasia and thickening of the prickle-cell layer.! In the chronic stage, acanthosis is the dominant feature.!

! Classification based on aetiology:!

1.! 2.!

! ENDOGENOUS! ! ATOPIC DERMATITIS!

-

Endogenous constitutional in origin! Exogenous!

! Infantile phase:!

Common, occurring in all races and affecting over one percent of children! Usually starts for 1st time at age 2 6 months INFANTILE ECZEMA. ! Often starts on the face and spreads to the trunk and limbs.! A.E. itches more than other eczemas because atopic patients have a lower threshold for itching. Stimuli, which do not cause itching normally affects them e.g. heat, woolly clothing, dry skin, possibly foods and anxiety.!

(age 2 months to 2 years)! Involvement primarily of chest, face, scalp, neck and extensor extremities with erythematous papulovesicles and oozing.! ! ! ! ! ! ! ! ! ! Childhood phase:! (age 4 to 10 years)! Lesions less acute and exudative, often localized in flexor folds of neck, elbows, wrists, and knees.!

! Adolescent and adult phase:!

(early teens to early twenties)! Lesions primarily dry, lichenified, hyperpigmented plaques in flexor areas and around the eyes. ! Persistent hand dermatitis may be the only remnant of an atopic diathesis.!

! Associated abnormalities:! ! 2. White dermographism.!

-

1. The atopic state.! - Asthma, hay fever and urticaria are commonly associated with atopic dermatitis, either in the patient or the immediate family! Abnormal tendency to vasoconstriction.! Pressure on skin leads to blanching.!

! 3. Increased susceptibility to viral infections.! ! 4. Xerosis or dry skin! ! Management:!

More susceptible to wart, vaccinia, herpes simplex and molluscum contagiosum viruses.! Infection with herpes simplex may lead to generalized herpes and ECZEMA HERPETICUM! Eczema herpeticum and eczema vaccinatum have similar eruptions known collectively as KAPOSIS VARICELLIFORM ERUPTIONS!

5. Alopecia areata! 6. Cataracts! 7. Conjunctival irritation! 8. Keratoconjunctivitis! 9. Keratoconus or conical cornea! General:! Confirm diagnosis if necessary with skin biopsy.! Patient education.!

! Management of the dryness:!

1. Soap substitutes e.g. emulsifying ointment! 2. Bath additives! Most based on liquid paraffin.! Increase lubrication of the skin.! ! ! ! ! ! ! 3. General purpose emollients e.g. aqueous cream! Should be used liberally.! 4. Vehicles! A vehicle is an inert carrier of the active ingredient.! For the treatment of eczema, creams in general are not satisfactory vehicles because they tend to have a drying effect.! Ointments are far better. Often impressive how the cream form of a steroid may be totally ineffective, yet a switch to the ointment form of the same steroid transforms the response.!

! Management of infection:!

1. Bacterial sepsis! 2. Molluscum contagiosum and warts! 3. Kaposis varicelliform eruption! If acute and vesicular, potassium permanganate 1:10,000 solution (pale pink) applied for 10 15 mins, 2 3 times per day for 2 3 days.! Potassium permanganate has both drying effect and antibacterial effect.!

! Other aspects of management:!

1. Antihistamines for itching! 2. Diet! 3. Hospitalization! 4. Psychological aspects!

! Specific!

Management of the dermatitis:! 1. Topical steroids! The choice of steroids depends on the site and stage of the dermatitis.! Site:! Hydrocortisone is usually the steroid used on the face.! ! 0.5% is usually ineffective, so in practice 1.0 is needed.! ! For body and limbs hydrocortisone often too weak except in children! ! and more powerful steroids required, either a moderately potent ! or a potent one.! Very potent steroids rarely required in atopic dermatitis.!

! ! The stage:!

Chronic lichenified dermatitis will usually require a potent steroid.! ! May benefit from occlusion.! ! ! ! Tar:! Before the advent of hydrocortisone in 1952, tar was mainstay of treatment.! Various ways of prescribing tar! - Coal tar ointment.! - Combined preparations with steroids.! - Bandages impregnated with tar.!

! SEBORRHOEIC DERMATITIS! ! Infantile seborrhoeic dermatitis:! ! Aetiology:!

Common self-limiting inflammatory condition occurring within the first few weeks of life, affecting primarily the scalp, face, axillae and napkin area.! Cause unknown.! Separate entity from adult seborrhoeic dermatitis and does not lead to adult form.!

! Clinical features:! ! Management:!

Eruption usually begins with yellow coarse scales in scalp so-called cradle cap.! Spreads onto face with yellow crusts particularly around eyebrows and ears.! Occurs under neck, in axillae and in some cases on the body.! Sometimes condition starts in napkin area rather than face! Secondary infection with bacteria or Candida may occur.! 1. Reassurance! Condition improves over ensuing few weeks.! Infant otherwise perfectly healthy.!

! 2. Treatment of the cradle cap!

Arachis oil useful.! Emollients - Emulsifying ointment and aqueous cream.! Topical steroids - Responds to mild topical steroids.!

! Adult seborrhoeic dermatitis! An eczematous process of varying degrees of severity with a propensity for the scalp, face, flexures and upper trunk.! ! Aetiology!
Cause unknown.! However role of Pityrosporum ovale suspected since last century.!

! Clinical features:!

Ill-defined roughness, redness and scaling of the skin.! Distribution:! 1. Face and scalp: ! Glabella, eyelids and ale of the nose, eyebrows, moustache area, sideburns, ears.! Often does not itch.! Scalp involvement either as dandruff sometimes coupled with erythema.!

! 2. Presternum and upper back:! ! 3. Flexural SE!

! ! Management:! Scalp:! Mild seborrhoeic dermatitis of scalp commonly called dandruff.! Responds well to frequent washing (often daily) with shampoos, which contain tar, selenium sulphide or ketaconazole.! If erythema present, topical steroids indicated, usually potent.! In severe cases may use salicylic acid products e.g. 2% sulphur, 2% sal acid in aqueous cream base.! ! Face:! Hydrocortisone as 1%.! Reasonable to use creams because more cosmetically acceptable than ointments on face.! Hydrocortisone may be mixed with sulphur or imidazole antifungal e.g. miconazole in Daktacort.!

! Presternum and upper back:! Potent steroids although tends to relapse very quickly.! ! STASIS DERMATITIS (Gravitational, Varicose)! !
-

Associated with venous stasis e.g. post-thrombotic, varicose veins.! Stasis can be demonstrated by pitting oedema.! Lesions start around the ankle and gradually spread up the leg.! ! ! ! Tends to become complicated by topically applied medication.! In addition to treatment of the eczema with topical steroids, stasis should be treated with bandaging.! EXOGENOUS!

! CONTACT DERMATITIS! ! Irritant contact dermatitis:!

Irritant! Allergic! Defn: Dermatitis, which results from overexposure of the skin to an irritant substance.! ! ! ! ! ! ! ! ! ! ! ! Aetiology:! An irritant dermatitis can occur in any individual, provided that the irritant agent is sufficiently concentrated and the exposure to it is long enough.! Certain individuals more prone to it particularly atopic subjects.! Results from repeated insults to the skin, which accumulate and eventually result in dermatitis.! Examples:! - Napkin dermatitis! - Dribbling, lip licking, thumb sucking in children.! - Irritant dermatitis of the hands: detergents, bleach, disinfectants.!

! !

Management:! The irritant substance should be removed from the skin and avoided.! Skin must be kept out of water as much as possible.! Emollients should be used liberally.! Oral antihistamines for itching.! Topical glucocorticosteroids: ! - 1% hydrocortisone cream anti-Candida agent may be used in napkin dermatitis.! - Potent steroids are usually necessary for irritant hand dermatitis.! Occupation change ?!

! Allergic contact dermatitis: !

Delayed hypersensitivity reaction, which results in dermatitis.! Once a predisposed individual has become sensitized, the potential to react persists indefinitely and the dermatitis will recur if patient re-exposed to allergen.! Reaction does not occur with the first exposure for the immune system requires priming.! There are many contact allergens so referral to a dermatologist is almost always necessary because patch tests required.! The most common non-industrial allergens:! - rubber! - perfumes! - nail varnish! - some plants! - metal! ! ! ! ! ! ! ! ! ! ! - dyes! - cosmetics! - medicaments! The most common industrial allergens! - chromates in cement! - resins in the plastic industry! - dyes! - rubber! - glues! Management:! The diagnosis of a suspected contact dermatitis should always be confirmed by patch testing.! There are 20 common allergens, which constitute a battery, to which other relevant potential allergens are added.! The chemicals are put into aluminium wells fitted into an adhesive tape and then stuck on to patients back.! Removed after 48 hrs, then skin inspected for dermatitis corresponding to application site of allergen.! Some reactions delayed so skin inspected after further 48hrs.! The patient will recover if antigen permanently removed.! Iatrogenic contact dermatitis can be avoided if doctors refrained from prescribing topical anaesthetics, topical antihistamines. ! ! SECONDARY DERMATITIS! Usually secondary to an infestation or infestation.! May be associated with scabies therefore patient should be examined carefully.! If scabies is treated with topical steroids it may lead to crusted scabies.! Sometimes seen in children with otitis externa or nasal discharge it is suspected that these children develop an allergy to a component of the bacteria.!

! HTLV 1 ASSOCIATED INFECTIVE DERMATITIS!

Characterized by a severe exudative dermatitis of the scalp, external ear and retroauricular areas, eyelid margins, paranasal skin, neck, axillae, and groin as well as a generalized fine papular rash.! There is also a chronic watery nasal discharge sometimes associated with crusting.! Staphylococcus aureus and /or !-haemolytic streptococci are commonly cultured from the anterior nares and skin.! The disease responds to antibiotics, but relapses if antibiotics are withdrawn.! The average age of disease onset is 2 years and 60% of patients are female.! The pathogenesis is as yet undefined.!

The skin manifestations usually become less severe with age perhaps with maturation of the immune system.!

! Other HTLV 1 associated disorders:!

Crusted scabies! Corneal opacities! Lymphocytic intersitial pneumonitis! Chronic bronchiectasis!

Adult T cell leukaemia/lymphoma! HTLV 1 associated myelopathy / Tropical spastic paraparesis (HAM/TSP)! Uveitis optic atrophy!

! Patients are more prone to developing:! ! ! 2 main groups:!

1.! 2.!

FUNGAL DISEASES OF THE SKIN!

! Superficial fungal infections:! ! DERMATOPHYTES!

3 main classes! 1. Trichophyton! 2. Epidermophyton! 3. Microsporum!

Superficial! Deep!

1. Dermatophytes: characterized by well-formed hyphae, which mat together to form ! mycelia! 2. Candida albicans and other species of Candida! 3. Pityriasis versicolor!

! Further divided into:!

Zoophilic originates in animals! Anthropophilic originates in man! Geophilic originates in the soil!

! Zoophilic and geophilic dermatophytes are extraneous fungi and therefore lead to increased inflammation and atypical
presentations! ! Tinea pedis: (dermatophyte infection of the foot)! 1. Interdigital type (Athletes foot)!

! 2. Vesicular type (Acute tinea pedis)! ! 3. Hyperkeratotic type (Chronic tinea pedis)!
-

! ! ! Ide reaction: Eczematous reaction on hands in response to dermatophyte infection of the feet.! ! Tinea manum: (dermatophyte infection of the hand)! ! Tinea unguium: (dermatophyte infection of the nails)! Features include:! - Discolouration! ! ! - thickening! - brittle nails with crumbling! ! ! ! ! ! ! ! ! ! ! ! ! !

Affects soles, heels and sides of feet! Sometimes referred to as moccasin-type!

! Tinea cruris: (dermatophyte infection of the groin)! ! Tinea corporis: (dermatophyte infection of the body)!
-

subungual hyperkeratosis! onycholysis!

! Tinea capitis: (dermatophyte infection of the scalp and hair) ! ! Identification!

Active advancing edge which is raised, scaly and sometimes vesicular.! Can form circinate irregular patterns.! Common in children, uncommon in adults.! Presents as round patch of alopecia, scaling of scalp with short broken hairs.! Most common organisms are: Microsporum audouini! Microsporum canis! Fluoresces green under Woods light if caused by the above organisms!

Scrapings can be taken from skin lesions:! - mounted in potassium hydroxide solution and viewed under the microscope for hyphae! - sent to Microbiology for culture.!

! Same can be done for nail and hair clippings. ! ! Treatment!

Topical:! Small areas can be treated topically.! The polyenes, Nystatin and Amphotericin are not effective against dermatophytes.! 1. Keratolytic drugs! - Whitfields ointment: benzoic acid + salicylic acid! - Tolnaftate (Tinactin) cream and powder!

! 2. Imidazoles:!

Eg miconazole (Daktarin)! clotrimazole (Canesten)!

! 3. Alkylamines:!

- ! Terbinafine (Lamisil) in topical preparation (cream), effective against dermatophytes, Candida and Pityriasis versicolor.! - Naftifine (Exoderil)!

! 4. Ciclopirox olamine (Batrafen)!

! Systemic:! Indications for systemic treatment are:! - Infection affecting large areas of the body! ! - Acute infections! - Nail infection! - Scalp and hair infection!

! Tinea capitis must be treated with a systemic drug for at least 4 weeks.! ! 1. Griseofulvin:! ! 2. Imidazoles:! !

In tinea capitis give Griseofulvin for 4 6 weeks + topical treatment (selenium sulphide or Ketaconazole shampoo to reduce infectivity).! Ketaconazole effective against dermatophytes, Candida and Pityriasis versicolor.!

! 3. Triazoles:!

Itraconazole effective against dermatophytes, Candida and Pityriasis versicolor.! Fluconazole - used in dermatophyte and Candida infections !

! In nail infections Itraconazole can be given:! ! 4. Alkylamines:!

200mg daily for 3 months ! OR ! 200mg bd for 7days, subsequent course repeated after 21 days; fingernails 2 courses, toenails 3 courses ! Terbinafine (Lamisil) may be used systemically in dermatophyte infections.! In nail infections, 250mg daily for 6 weeks for fingernails, 250mg daily for 3 months for toenails.!

! PITYRIASIS VERSICOLOR! ! Clinical features:!

-

Caused by yeast, which is normally a commensal but becomes pathogenic in warm humid conditions.! Particular individuals seem susceptible and it tends to be recurrent in these persons.! Caused by the unicellular yeast Pityrosporum orbiculare and Pityrosporum ovale. They become pathogenic by budding and producing filaments known as pseudohyphae in this state, the organism is sometimes known as Malassezia furfur.! Well-defined, irregular patches (sometimes slightly raised).! Mildly scaly.! Hypo- or hyperpigmented.! May have mild itching.! Mainly occurs on the upper part of the body.! Typically affects adolescents and young adults.!

! Treatment:!

Topical:! 1. Half strength Whitfields ointment irritation is a common side effect.!! ! ! 2. Sodium thiosulphate ! 3. Imidazoles e.g. clotrimazole (Canesten), miconazole (Daktarin), ketaconazole shampoo or cream (Nizoral)! 4. Selenium sulphide 2.5% shampoo (Selsun)! 5. Terbinafine cream (Lamisil)! 6. Naftifine (Exoderil)! 7. Ciclopirox olamine!

! ! Systemic:!

1. Ketaconazole 200mg daily for 5 days or 400mg stat.! 2. Itraconazole 100mg daily for 5 days.!

! CANDIDA!
-

Candidosis (Candidiasis) is a common infection of the mouth, genitalia, flexures and nails usually caused by Candida albicans.! Eighty percent of normal individuals harbour the yeast as a commensal in the oropharynx, gastrointestinal tract or vagina.!

! Factors that favour candidosis:!

Occlusion! Damage to the stratum corneum! Immunosuppression! Neutropenia! Endocrinopathies eg. diabetes mellitus, Cushings disease! Uraemia and malignant disease! Extremes of age, menstruation and pregnancy! ! ! Iatrogenic factors e.g. antibiotics, oral contraceptives, steroids.!

! Chronic paronychia:!
-

! Oral candidosis (thrush):! ! Candida intertrigo: ! ! Treatment:!

Inflammation of the nailfolds! Occurs in persons whose hands are much exposed to water! Common in diabetes! Loss of cuticle! Bolstering of the nailfold! Dark pigmentation of the nails! White patches on the mucous membranes, which on removing shows an erythematous base.! Susceptible individuals include: neonates, immunosuppressed, healthy individuals who are edentulous and neglect oral and dental hygiene.!

(Intertrigo is a dermatological term that indicates an eruption, which occurs between two apposing skin surfaces)! - Maceration! - Erythema! - White exudate! - Satellite lesions! Topical:! Nystatin, Amphotericin, imidazoles.! ! ! ! ! ! ! Systemic:! Imidazoles e.g. Ketaconazole! Triazoles e.g. Fluconazole (Diflucan), Itraconazole (Sporanox).! Intravenous Amphotericin B used in resistant cases of systemic candidiasis but toxic.!

! DEEP FUNGAL INFECTIONS! ! SPOROTRICHOSIS! ! Treatment:!

Caused by Sporothrix Schenkii, a saprophyte of vegetation.! Produces discrete fungating lesions, which run along the lymphatics.! Site of predilection limbs.! Oral potassium iodide 10 drops, 3 times daily after meals.! Alternatively, Amphotericin B given intravenously.!

! MYCETOMA! Caused by a number of organisms, which may be fungi (eumycetoma) or bacteria (actinomycetoma).! ! Aetiological agents of mycetoma:!
Eumycetoma! ! Madurella mycetoma! ! ! ! !

! Found on limbs and exposed areas.! ! Treatment:!

! ! ! !

Actinomycetoma! Actinomyces species! Nocardia species! Streptomyces species!

Usually acquired from trauma in agricultural work.! Has chronic discharging sinuses with granules.! Excise small lesions! Actinomycetoma may respond partially to penicillin, sulphonamides or tetracyclines.! Eumycetoma may respond to itraconazole.!

! CHROMOBLASTOMYCOSIS! ! Treatment:!

Caused by several fungi including Cladosporium and Phialophora species.! Limbs affected.! Discrete fungating lesions.! Surgical excision is the treatment of choice for early lesions.! Amphotericin B sometimes used intralesionally.! 5 fluorocytosine may be used alone or in combination with Ketaconazole or Itraconazole.!

! ! ! SCABIES! ! Causative mite Sarcoptes scabiei.! ! Clinical features:!

PARASITIC INFESTATIONS!

Transmitted by prolonged physical and usually intimate contact.! The newly fertilized female burrows into the epidermis and lays her eggs.! Itching is the most obvious and manifestation of scabies and in some cases may be the only one.! Lesions, which may be seen, are:! - papules! - pustules! - burrows (pathognomonic)! - secondary eczema!

! Sites of predilection:!
-

! Diagnosis:!
1.! 2.! 3.! 4.!

Wrists! Sides of fingers and finger webs! Elbows! Axillary folds! Around the nipples! Peri-umbilical area! Penis and scrotum in men! ! Natal cleft! Infragluteal fold! Ankles and feet in children!

! Treatment:!

Identify burrow.! Scrape off debris and mite.! Place on slide with few drops of potassium hydroxide soln.! View under microscope.!

1. Benzyl benzoate (Ascabiol) do not use in infants, avoid in children, commonly irritates the skin.! 2. Gamma benzene hexachloride (Rid) avoid in infants! 3. Permethrin (Nix) treatment of choice for infants.!

! All contacts must be treated.!

! ! ! ! ! ! ! Bathe and wash all bed linen.! Apply lotion or cream and keep this on for 24 hours without bathing. (Application should be renewed after the first 12 hours.)! A further treatment for 24 hrs may be required for benzyl benzoate, gamma benzene hexachloride.!

Permethrin should be used for 12-14 hours in infants and children up to 2 years. Permethrin does not usually require a second treatment.! In infants and children up to 2 years, application should be extended to scalp, neck, face, ears.! Itching may persist for weeks after treatment completed.!

! Crusted scabies:!

Presents clinically with numerous crusts over the body especially on prominent areas such as knees and elbows as well as the usual areas of predilection for scabies.! Crusts are hyperinfested with mites.! Patients are usually immunosuppressed.!

! Treatment:! Same as above but several applications may be required.! ! ! VIRAL INFECTIONS OF THE SKIN! ! Varicella (chickenpox)"

Clinical Features! Caused by varicella zoster, a double-stranded DNA virus belonging to the herpes group! Incubation period 9 23 days! Mild prodrome of fever, malaise! Then crops of macules, which become vesicles surrounded by erythema! The contents of the vesicles become turbid and crusted! Individual lesions are at different stages of development! The distribution is centripetal which means that the trunk is most extensively affected, then the face and scalp, followed by the upper arms and thighs! The disorder is quite pruritic! Lesions are found in the mouth! " Management! Uncomplicated varicella only requires symptomatic treatment.! In complicated cases or immunosuppressed individuals, acyclovir may be given orally or intravenously.! Complications include: varicella pneumonia and encephalitis. !

! ! Herpes Zoster (shingles)!

"

Caused by the same virus that transmits varicella (chickenpox)! The virus lies dormant in the dorsal root or cranial nerve ganglion after a patient recovers from chickenpox! There may be a latent period of several decades before the virus is reactivated and commences to spread along the cutaneous nerve!

Clinical Features! The first symptom is the abrupt onset of pain or discomfort! Examination reveals an eruption which corresponds to a dermatome! Each individual lesion begins as a red macule which rapidly becomes a papule and then a vesicle surrounded by erythema! The vesicles may become confluent and evolve to become pustular haemorrhagic and finally scabbed! " Complications! Involvement of certain dermatomes e.g. the ophthalmic branch of the trigeminal nerve! Disseminated herpes zoster! Postherpetic neuralgia! " Management! Acyclovir! Analgesia and bed rest!

Topical antibiotics may help to prevent bacterial infection! REMEMBER live chickenpox virus is present in the lesions until the scabs have formed so someone who has never had chickenpox can catch it from a patient with shingles! Molluscum Contagiosum! Caused by a pox virus! Common in children and young adults ! Close contact in children and sexual transmission in adults are well recognized as means of spread! " Clinical Features! Typical lesion is an asymptomatic flesh-coloured or dome-shaped papule with a central depression! This umbilication is the most important diagnostic sign! An individual lesion lasts about 2 months! The outbreak resolves spontaneously within a year! " Management! Some dermatologists leave them alone! Cryotherapy with liquid nitrogen can be done! The lesions can be pierced with a cocktail stick dipped in iodine! Imiquimod (Aldara) an immune modulator!

! Viral Warts!
"

Due to an infection of skin or mucous membranes by the human papilloma virus! Warts are contagious and spread more easily if there is local trauma to the skin! Commonly contracted from institutions where there are communal bathing and changing facilities!

Clinical Types! Common warts (verrucae vulgaris)! Plane warts! Palmar and plantar warts! Mosaic warts! Anogenital warts (condylomata acuminata)! " Management! Salicylic acid preparations with or without lactic acid! Podophyllin! Glutaraldehyde or formaldehyde! Imiquimod! Liquid nitrogen! Cautery!

! ! ! ! ! Carbuncle!

BACTERIAL INFECTIONS OF THE SKIN!

! Management! ! Impetigo!

A carbuncle is an acute abscess of contiguous hair follicles caused by Staphylococcus aureus! Staphylococcus aureus is usually also present in the nose axillae and perineum! It is most common in adolescents and young adults! The patient is usually healthy! Incise and drain pus! A swab should be taken for culture and sensitivity! Cloxacillin, amoxycillin + clavulinic acid, erythromycin are usually effective!

 "

! Syphilis!

An acute, contagious and superficial infection of the skin caused by either Staphylococcus aureus or ! haemolytic streptococcus or both! Starts as large or small blisters with yellow pus! The blisters rupture and purulent exudate dries to form honey-coloured crusts! Occasionally nephritis may occur three weeks after infection with the nephritogenic strain of streptococcus! An infection with the spirochaete Treponema pallidum! Syphilis is usually sexually transmitted but may be acquired from maternal disease in utero or from infected blood products or instruments! The disease is divided into stages known as primary, secondary, latent or tertiary! The former two stages are infectious! The primary lesion or chancre occurs at the site of inoculation, between 10 days and 3 months after infection! The chancre is a painless ulcer with an indurated edge! Spirochaetes may be demonstrated by dark-ground microscopy! Local lymph nodes are enlarged but quite painless! The chancre heals spontaneously within 1 3 months! This occurs 6 8 weeks after the chancre! The patient usually presents with a fever and rash! The eruption is widespread! It does not itch! There is a generalized lymphadenopathy! Initially the rash is macular and pink (roseola)! It becomes papular and more widespread! Annular configurations may occur! In the intertriginous areas the papules may become eroded condylomata lata! Snail-track ulcers appear in the mouth! Moth-eaten alopecia is common! The gumma is the hallmark of tertiary syphilis! Dermal gummata are firm brownish-red papules or nodules which are usually arranged in an annular manner! Mucosal gummata may occur and the tongue may be diffusely infiltrated!

! Primary Syphilis!

! Secondary Syphilis!

! Tertiary Syphilis!

Management! Intramuscular long-acting penicillin treatment of choice! Tetracycline! Erythromycin!

! Leprosy(Hansens Disease)!

! Management!

A chronic infection principally of the skin and nervous system caused by Mycobacterium leprae! The disease is transmitted by inhalation or ingestion of infected nasal droplets! The degree of involvement depends largely on the immunological status of the patient and the bacteriological load! Leprosy is classified into tuberculoid, borderline and lepromatous based on the immunological status of the patient! Designated TT BT BB BL LL! Neurological involvement occurs ! Dapsone! Rifampicin! Clofazimine!

! !

! ACNE VULGARIS! ! Definition: A chronic inflammatory disorder affecting the pilosebaceous unit which is characterized by the formation of comedones, erythematous papules and pustules.! !- 90% of teenagers have acne!
- Thought to be physiological! - Commoner in females than in males.! - Lesions are papules, pustules, comedones, cysts.! (Comedones = whiteheads + blackheads)!

! Common sites:!
-

! Pathogenesis:!

Forehead! Cheeks ! Chin! Nose! Upper chest! Back! Shoulders!

4 major aetiological factors:! 1. Increased production of sebum! 2. Excessive keratinisation at follicle mouth causing blockage of the pilosebaceous canal! 3. Presence of skin micro-organisms! 4. The production of inflammation!

! 1. Increased sebum production:!

-

! 2. Blockage of pilosebaceous canal:!

-

Sebaceous glands directly under androgen hormonal control! May be target organ over-activity (increased conversion of testosterone by enzyme 5a reductase at androgen receptor of sebaceous glands.! - It has been shown in some women that sex-hormone binding globulin is reduced leading to elevated levels of serum unbound testosterone! - Less commonly, it can be a feature of a virilizing disease i.e. high overall androgen production.! The comedone represents blockage of the pilosebaceous duct by a keratin plug! Caused by failure of the epidermis lining the duct to keratinize properly so keratin not shed correctly! If blockage superficial, comedones, papules and pustules result. If deeper, nodules, cysts and scarring may occur!

! 3. Presence of skin micro-organisms:!

Pilosebaceous duct and skin surface colonized by important micro-organisms:! a) Propionibacterium acnes - bacteria! b) Staphylococcus epidermidis - bacteria! c) Pityrosporum ovale yeast, of which P. acnes is dominant.!

! Skin bacteria contribute to the pathogenesis by:! ! ! 4. The production of inflammation:!

disrupting the follicle wall! causing inflammation! attracting white blood cells.!

Free fatty acids produced in the follicle by action of enzymes associated with P. acnes act as a primary irritant! P. acnes elaborates small molecular weight peptide which attracts polymorphonuclear leukocytes to microcomedones and initiates inflammation! P. acnes is also a potent activator of complement via the classical pathway!

! Aggravating factors:!

Activation of complement releases neutrophil hydrolytic enzymes which rupture the follicular wall! Other exo-enzymes produced by P. acnes may also contribute to rupture.!

1. Endocrine factors! - There may be premenstrual exacerbation! - Some oral contraceptives may cause acne to flare especially those which contain the androgenic and antioestrogenic progestogens norgestrel, norethindrone and norethindrone acetate!

! 2. Climatic conditions! - Often becomes worse in hot humid climate! ! 3. Medicaments!

Heavy oils, greases! Oily or greasy cosmetics!

! 4. Occlusive circumstances! - Tight fitting clothes.! ! 5. Mechanical trauma! - Pressure, friction, rubbing and squeezing from clothing or behavioural habits! ! 6. Drugs!
Hormones: Topical and systemic corticosteroids! ACTH!! ! ! ! Androgens! ! ! ! Danazol! ! ! ! ! ! ! ! ! ! ! ! - Halogens: ! Iodides in cough mixtures, in radiological materials and in drugs used in the treatment of thyroid disease. Iodide in seaweeds if eaten in large amounts.! Bromides (rarely given nowadays).! Chlorides! Halothane!

!-

Antiepileptic drugs:! Phenobarbitone and derivatives! Phenytoin and hydantoin derivatives! ! ! ! ! ! - Anti-tuberculosis drugs! Isoniazid! Rifampicin!

! 7. Industrial:!
-

! MANAGEMENT:!

Halogonated hydrocarbons! Tar! Lubricating oil!

Determine effect of disorder on patients morale.! Avoid aggravating factors.! Mild acne topical treatment! Moderate and severe acne topical and oral treatment! !

! TOPICAL TREATMENT!

Topical preparations largely a) antibacterial b) keratolytic (exfoliating) i.e. reducing the faulty keratinization at the pilosebaceous duct.!

! 1. Chemical exfoliating agents!

! ! !

! 2. Benzoyl peroxide!

1-2% ppt sulphur! 1-2% salicylic acid! 1-2% resorcinol!

- antibacterial! - exfoliating! Available in 2.5, 4, 5, 10% concentrations as cream or gel.! ! ! ! Also available in 5% and 10% soaps and washes.! Sometimes combined with other antibacterials.! ! ! ! ! Irritates the skin, therefore start with weakest strength nightly. Increase to twice daily to the point of mild dryness and erythema but not discomfort.!

! 3. Topical antibiotics!

Clindamycin! - Available in 1% concentration in alcoholic solution, gel or lotion!

! Erythromycin! - Available as solution, ointment or gel sometimes in combination with other anti-acne agents! ! Tetracycline!
! 4. Retinoids (vitamin A analogues)!

Less effective.! May produce temporary yellow discolouration that may be washed off after 1 hr with no decrease in drug effectiveness! Will fluoresce under long wave ultraviolet light eg. discos. !

Tretinoin (Retin A, Stieva-A) cream or gel! - Keratolytic! - Increases basal cell mitosis (epidermal cell turnover) and decreases the cohesiveness of horny cells, thus inhibiting the formation of comedones while helping existing comedones to become loosened and expelled.! - Causes erythema, peeling and increased sensitivity to sunlight.! - Available in 0.025%, 0.050% and 0.1% concentrations.!

! Isotretinoin (Isotrex) gel! - Similar to Tretinoin but less irritating.! ! Adapalene (Differin) gel! - Similar to Tretinoin but less irritating.! ! Tazarotene (Tazorac) gel! - Not yet available in Jamaica! ! ! !

ORAL TREATMENT:! ANTIBIOTICS:! 1. Oral tetracycline! - Has to be given for a minimum of 6 months.! - Least expensive, few side effects.! - Minor gastrointestinal tract irritation! - Candida vaginitis! ! ! ! ! ! ! ! ! - Should not be given to children under the age of 12 nor administered during pregnancy.! - Must be taken on an empty stomach.! - Drug interaction with metallic irons Al, Mg, Ca present in antacids and milk.!

Initiate therapy at 250mg qds or 500mg bd (half hour before meals or 2 hours after) until clear improvement; then decrease to maintenance dosage 250 500mg per day.!

2. Minocycline! - More expensive.! - Overall most effective antibiotic to treat acne.! - Can be taken with food drink and with milk.!

! 3. Doxycycline!
-

! 4. Oral erythromycin! - Used in a similar way to tetracycline but more expensive and may cause abdominal cramps! ! HORMONAL THERAPY!
RETINOIDS! Isotretinoin (Roaccutane)! - Given for a course of 4 months! - Side effects include dryness of skin and lips, increased plasma lipids and liver enzymes! - Teratogenic: females must be on adequate contraception! - Indicated in severe nodulo-cystic acne.!

Less expensive than minocycline but more expensive than tetracycline.! Absorption not decreased by milk.!

Diane - 35! - Special oral contraceptive pill with an anti-androgen, cyproterone acetate 2 mg and oestrogen, ethinyloestradiol 35 micrograms.!

! ALTERNATIVE MEDICATION ! ! ! ! HYPOPIGMENTATION! ! Congenital!

1.!

Spironolactone! - Has anti-androgenic properties! - Prime effect is by reducing sebum excretion! In female patients over the age of 30 years oral spironolactone for 6 months is of considerable benefit.! DISORDERS OF PIGMENTATION!

! 2.!

Piebaldism (partial albinism)! - Inherited as an autosomal dominant gene! - Characterised by patch of white hair in front (forelock) associated with patches of depigmented skin usually on the forehead! - In Waardenburgs syndrome, piebaldism is associated with deafness! - No cure or treatment except camouflage with cosmetic cover creams! Oculocutaneous albinism! - Heterogenous group of at least four distinct autosomal recessive disorders! - The two most common varieties are the tyrosinase-negative and tyrosinase-positive types! - Clinical features:! Total lack of body pigment! Nystagmus! Photophobia! Poor visual acuity! Prone to developing skin cancer at an early age! - No treatment except protection from the sun!

Acquired! 1.! Post inflammatory! - May cause hypo- or depigmentation! - Occur following eczema, psoriasis, burns, infection, discoid L.E. etc.!

! 2.! !

Exposure to chemicals! - Ethers of hydroquinone: ! Component of many creams! Bleaches skin unevenly leading to confetti-like depigmentation! Pigmentation returns after discontinuing use! Phenol may cause hypopigmentation!

3.!

! May be associated with other auto-immune conditions e.g. thyroid disease, pernicious anaemia, Addisons disease,
myasthenia gravis, alopecia areata! - 10 20% show spontaneous repigmentation! - Lesions are frequently periorificial in that it occurs around mouth, nose, eyes, nipples, umbilicus and anus! - May be intertriginous e.g. in groin and axillae! - May occur on extensor surfaces such as elbows, knees, front of the shins, backs of the hands and feet; on the flexor surfaces of the wrists and in the oral mucosa as well! - No universally successful treatment! - Management consists of:! Photoprotection: Sunscreens which block UVB and UVA to prevent burning of affected skin! Topical glucocorticosteroids: Potent or very potent topical steroids may be effective in producing repigmentation of areas of vitiligo! PUVA: Requires anywhere between 100 and 300 treatments! Camouflage: using cosmetic cover creams! Bleaching of normal skin: in those with extensive vitiligo!

Vitiligo! - Skin usually depigmented! - Electron microscopy studies confirm the absence of melanocytes in affected areas! - Thought to be auto-immune in origin!

! HYPERPIGMENTATION!

Congenital! 1.! Mongolian blue spot! - Pigmented melanocytes present in the deeper layers of the reticular dermis! - Represent a migratory arrest of melanocytes from the neural crest! - Lesion eventually disappears! - Disappearance of lesion related to gradual reduction of melanogenesis!

! 2.! ! 3.!
! ! 4.!

Blue naevi! - Failure of melanocytes migrating from the neural crest to arrive at the dermo-epidermal junction! - Melanocytes found in the lower dermis! - Lesions slightly elevated! ! - Persist into adult life ! ! Naevus of Ito shoulders! Naevus of Ota around eyes! ! ! ! ! ! ! Caf au lait spots of neurofibromatosis! - Greater than 5 spots abnormal! - Associated with axillary freckling!

! !

GENERALIZED PRURITUS! Pruritus means itching for which there is no obvious cause in the skin. Scratch marks and prurigo papules, which result from scratching, are allowed.!

! Causes:!

1. Skin disorders! 2. Systemic disorders!

! Skin disorders:!

1. Parasitic infestations! - In scabies generalized itching may precede skin lesions.! - Pediculosis corporis.!

! 2. Urticaria and dermographism! - Itching may be present but no lesions.! ! 3. Aquagenic pruritus!
-

! 4. Drug reaction to topically administered drugs! !5. Dermatitis herpetiformis! - Itching may precede skin lesions! ! 6. Bullous pemphigoid! - Itching may precede skin lesions! ! 7. Xeroderma (dry skin)! ! Systemic disorders:! ! 2. Endocrine!

Distinct from aquagenic urticaria.! If the condition is not recognized, sufferers are liable to be labelled neurotic.!

1. Drug reaction from oral or parenteral drugs! Two mechanisms:! i) Allergic e.g. penicillin! ii) Non-specific liberation of histamine e.g. aspirin, codeine, morphine.! i) Pregnancy! - Prurigo of pregnancy! In the last trimester of pregnancy may get generalised itching due to hormonal changes in pregnancy. Recurs with each pregnancy and severity increases.!

! ii) Hypothyroidism and hyperthyroidism!

iii) Diabetes mellitus! ! iv) Diabetes insipidus! v) Hypoparathyroidism! !

! 3. Hepatic disease!

Pruritus in liver disease is a symptom of biliary obstruction (cholestasis).! In primary biliary cirrhosis, itching may precede jaundice.! Pruritus is thought to be due to deposition of bile salts in the skin. !

! 4. Chronic renal disease! Exact mechanism unknown.! ! 5. Haematological disorders!

i) Iron deficiency anaemia! ii) Leukaemias and lymphomas especially Hodgkins lymphoma! iii) Polycythaemia rubra vera!

! 6. Malignant tumours! Carcinomatosis occasionally causes pruritus.! ! 7. Tropical and intestinal parasites!
Infestation with hookworm, round worm.! Onchocerciasis, filiariasis.!

! ! Management:!

Thorough history.! Examine skin thoroughly as well as full systemic examination.! If no cause found do:! - CBC, ESR, urea & electrolytes, liver function tests, glucose, thyroid function tests.! - Stools examination for parasites, ova and cysts.! Further investigation e.g. chest x-ray, abdomino-pelvic ultrasound may be indicated in particular cases depending on history and examination findings.!

! Treatment:!

Symptomatic treatment includes:! - Antihistamines! - Sedatives! - Baths! - Cool clothing! - Cholestyramine in cases where cholestasis is a cause!

! If underlying cause found, treat the cause.! ! ! Commonest side effects of drugs.!
1.

DRUG ERUPTIONS!

! 2.!
! 3.!

Exanthematous (Toxic Erythema)! - Commonest of all cutaneous reactions! - May simulate scarlet fever or measles! - If previously sensitised starts within 2 to 4 days, if first time 9 to 10 days! - May start after drug discontinued! - Usually short-lived; lasts 1 to2 weeks if drug discontinued! - Commonest causes: Ampicillin, Gentamicin, Sulphonamides, Gold salts.! Urticaria! - Caused by (a) direct histamine liberators e.g. morphine, codeine! (b) Allergic reaction! - Aspirin may cause urticaria by an allergic or a pharmacological mechanism! - Commonest causes: Penicillins, Cephalosporins, Salicylates, Animal sera.! Photosensitivity! - Eczematous rash in light exposed areas! - Commonest causes: Amiodarone, Tetracycline, Phenothiazines, Sulphonamides (and related drugs), Thiazides, Nalidixic acid.! Fixed drug eruptions! - Starts with prodrome of itching and burning! - Area becomes erythematous and oedematous within 24 hrs! - May form blisters if severe!

! 4.!

! 5.! ! 6.! ! 7.! ! 8.!

! !

Heals with slate blue colour! Commonest causes: Phenolphthalein (found in laxatives), Quinine (found in tonic water), Tetracycline, Barbiturates, Sulphonamides, Dapsone.!

Drug-induced lichenoid eruptions! - More pigmented and pruritic than idiopathic L. P.! - Commonest causes: Methyldopa, Dapsone, Antimalarials, Thiazides, Beta blockers.! Drug-induced acne! - Commonest cause is systemic steroids e.g. Prednisone, Dexamethasone! - See Acne Lecture notes! Drug-induced alopecia! - Anagen alopecia may be caused by cytotoxic drugs! - Telogen hair loss has been caused by thiouracils and heparin! ! Drug-induced lupus erythematosus-like syndrome! - Abnormal lab findings are usual but anti-DNA antibodies are absent! - Commonest causes: Hydrallazine, Methyldopa, Griseofulvin, Penicillin, Procainamide, Isoniazid.! Bullous eruptions! -! Bullae often at pressure areas may be seen in-patients comatose after overdosage with barbiturates, methadone, and nitrazepam! - Erythema multiforme (see notes)! - Pemphigus and pemphigoid may be drug induced! Toxic epidermal necrosis! - Clinical features include dusky erythema, blistering and peeling of necrotic skin! - Common causes: Sulphonamides, Phenolphthalein, Phenytoin.! Eczematous! - Topical applications! - Systemic drugs!

! 9.! ! 10.! ! 11.!

12.! !

! 13.! ! ! 14.! ! ! ! ! ! ! ! ! ! ! ! !

Erythroderma / Exfoliative dermatitis! - Common causes: Ampicillin, Gold, Sulphonamides, Gentamycin.! Hyperpigmentation! - Common causes: Tetracycline, Minocycline, Mepacrine, Chloroquine.! Odd pigmentation! - Mepacrine: yellow discolouration! - Lead: grey discolouration! - Silver: grey discolouration! - Phenothiazines: bluish black pigmentation!

! ! ! ! ! !

SKIN MANIFESTATIONS OF HIV INFECTION!

Skin lesions are often the first manifestation of HIV infection, and may occur:! 1. As a manifestation of the primary HIV infection! 2. As a consequence of immunosuppression (late stage HIV infection and AIDS).!

! Lymphadenopathy may occur without other signs of the disease.! ! Acute HIV infection (seroconversion illness) is associated with clinical changes in about a half of cases. Fever, malaise,
headache, lymphadenopathy and gastrointestinal upset occur. Sometimes there is an effect on the central nervous system. A transient maculopapular eruption occurs with erythema and erosion of the palate in some patients. At this time HIV antibody testing may still give negative results, although an antigenaemia soon develops. For this reason it may be important to retest suspected cases 6 8 weeks later.! inflammatory skin diseases are more florid, infections are frequent and severe and opportunistic infections occur. In addition, Kaposis sarcoma occurs in 34% of homosexual men and in 5% of other cases.!

! Skin manifestations of AIDS and late stage HIV disease are many and variable. The main features are that the common ! Seborrhoeic eczema is common and may be the only evidence of HIV infection initially. It is more extensive and inflamed than usual.! ! The role of Pityrosporum organisms is indicated by the response to imidazole antifungal drugs.! ! Psoriasis is more widespread, severe, and resistant to treatment in patients with late HIV infection.! ! Thinning and straightening of the hair occurs at low CD4 counts.! ! Acquired ichthyosis and xerosis occur commonly in late stage HIV disease and AIDS.! ! Any type of opportunistic infection is more likely in patients with AIDS and will generally be more severe.! ! Eosinophilic folliculitis (papular prurigo of HIV disease) an itchy folliculitis, occurs in many cases. The cause is unknown, but it is possible that Demodex spp. play a part.! ! Herpes zoster virus infection is common, with florid lesions and systemic spread. Often two dermatomes are affected.! ! Herpes simplex virus produces widespread and sometimes persistent ulcerating lesions.! ! Perianal warts can proliferate and cervical intraepithelial neoplasia occurs.! ! Fungal infections may be widespread and with increased inflammation and hyperkeratosis. Candidiasis, often with
associated bacterial infection infection is very common, particularly at the corners of the mouth, on the palate, and in the pharynx!

! Cryptococcus neoformans and Histoplasma capsulatum are occasional opportunistic pathogens and can produce inflammatory, papular, and necrotic lesions.! ! Mycobacteria may cause both cutaneous and systemic lesions.! ! Oral hairy leukoplakia caused by Epstein-Barr virus, may occur in 30-50% of patients with AIDS.! ! !

Kaposis sarcoma presents with polychromic plaques and nodules, varying from red and purple to brown. They are common on the palate and nose, often found on the trunk, but can be disseminated or produce lesions in the classical site the ankle.!

! HIV infection may thus present with a wide variety of skin conditions, commonly with several present at the same time.
Any unusually florid skin condition that is resistant to treatment should raise the suspicion that HIV infection may be present.!

! ! 1. Pruritus! ! 2. Acanthosis nigricans!

SKIN DISORDERS ASSOCIATED WITH MALIGNANCY!

In malignant acanthosis nigricans, the skin becomes thrown into folds and hyperpigmented.! The surface feels and looks velvety.! Skin changes occur on flexural and exposed areas.! Other features include warty lesions and skin tags.! ! ! ! ! ! In over 50% of patients, the mucous membrane of the tongue is thrown into folds and warty lesions occur on the oral mucosa.! The majority of patients with malignant acanthosis nigricans have an adenocarcinoma usually of the gastrointestinal tract, less commonly in the genito-urinary system.! May also be associated with lymphomas.!

! 3. Dermatomyositis!

Juvenile dermatomyositis is not associated with malignancies.! Adult dermatomyositis is associated with malignancies in # of cases.! Skin and skeletal muscles affected.! Skin lesions:! - Scaling and mauve erythema (heliotrope discolouration - pathognomonic) on the eyelids.! - Distinctive mauve erythema on dorsum of hands.! - Erythematous papules over the knuckles (Gottrons sign).! - Nailfold telangiectasia.! - Ragged, hypertrophic cuticles.! - Photosensitive dermatitis.!

! Muscle involvement:!

- Proximal myopathy i.e. limb girdle (shoulder and pelvic girdle).! - May spread to involve intercostal muscles and muscles used in swallowing.! Associated malignancies include carcinoma of breast, bronchus or ovary.!

! 4. Pagets disease of the nipple!

-

Looks like unilateral eczema of the nipple.! Contains malignant cells.! 100% have underlying intraductal carcinoma.! Pigmentation! Acquired ichthyosis! Erythroderma! Bullous pemphigoid! Paraneoplastic pemphigus! !

! 5. Other conditions associated with malignancy!

! ! ! ! ! ! ! All the previously described conditions except Pagets disease of the nipple are non-metastatic. Breast and renal carcinoma, malignant melanoma and lymphoma may metastasize to the skin.! ! ! ! !

BLISTERING DISORDERS! ! A blistering disorder is an autoimmune condition in which the primary lesion is a blister! ! Pemphigus Vulgaris! "

A serious autoimmune disorder of intraepidermal cell cohesion, resulting in flaccid blisters and painful erosions of the skin and mucous membranes! Aetiology! Rare autoimmune disorder! Most common in Jews! Strongly associated with HLA- DR4! There is an association with various autoimmune diseases such as rheumatoid arthritis! The IgG antibodies are directed against the intercellular cement substance! " Clinical Features! In 50 percent of patients the disorder starts in the mouth with flaccid blisters which result in painful erosions! However, all stratified squamous epithelial mucosal surfaces may be involved, including the pharynx, larynx, oesophagus, conjunctivae, urethra,vulva, penis, cervix and rectum.! All skin may be involved! Blisters are flaccid and may arise on normal or erythematous skin! They break easily to leave erosions which are painful! These erosions do not heal spontaneously! " Diagnosis! The diagnosis may be confirmed by a skin biopsy! There is oedema and disappearance of the intercellular bridges in the lower epidermis! This gives rise to acantholysis (separation of the cells in the prickle cell layer)! Subsequently, suprabasal intraepidermal blisters occur! Linear deposits of IgG may be demonstrated between the epidermal cells in 100% of patients during active disease! The majority of patients manifest circulating antibodies against the intercellular cement!

! Pemphigus Foliaceus"
"

A clinical variant of pemphigus with blistering just below the stratum corneum that occurs in idiopathic, epidemic or druginduced forms! Aetiology! Most cases are idiopathic.! There is a distinctive endemic variety which occurs in rural South America and in particular Brazil.! In a small proportion of patients, the disorder is drug-induced.! " Clinical Features! Recurrent superficial erosions occur, seen with erythema, scaling and crusting.! Intact flaccid blisters are rarely observed.! The scalp, face, chest and back are involved in a distribution similar to seborrhoeic dermatitis.! However, eventually, the whole cutaneous surface may be affected.! Itching, often severe, associated with burning, is present.! The Nikolsky sign is a helpful positive physical sign.! Oral lesions are unusual.! " Diagnosis! Skin biopsy for routine histology and immunofluorescence is indicated.! The histology shows a subcorneal acantholytic blister.! IgG is deposited intercellularly in the granular layer! Circulating IgG autoantibodies, which react with intercellular cement, are also present in most patients!

Management! Prior to the introduction of systemic glucocorticosteroids, the disorder was fatal in the majority of cases! These drugs seem to act by decreasing autoantibody levels! Initially very high doses of systemic glucocorticosteroids are employed! The advent of other immunosuppressant agents, such as methotrexate, cyclophosphamide, azathioprine and cyclosporin A permits the dose of glucocorticoid to be reduced.! Other agents such as gold, dapsone and plasmapheresis have been used.!

! ! Bullous Pemphigoid"
"

A serious condition of spontaneous blistering, characterized by itchy tense blisters and caused by an autoimmune process that affects the dermoepidermal junction! Aetiology! Relatively common in the elderly! Affects both sexes equally and all races! Considered to be an autoimmune process and is often associated with other autoimmune disorders! Circulating IgG autoantibodies to the basement membrane zone of the dermoepidermal junction have been demonstrated in the sera.! However, malignant disease should be suspected in patients with oral ulceration and in seronegative patients! " Clinical Features! The condition is usually intensely itchy! The patient presents with blisters which are usually symmetrical in distribution! Blisters usually involve the limbs first and then the trunk! Blisters are tense and are usually but not always surrounded by erythema! The blisters eventually become haemorrhagic and break leaving denuded eroded skin! Occasionally in the early stages, the lesions are of amore urticarial nature! The mucous membranes are involved in a minority of patients! " Diagnosis! Skin biopsy is taken for histopathology and immunofluorescence! The pathology shows that the entire epidermis makes up the roof of the blister! Almost all patients show IgG and C3 at the epidermal basement membrane zone in a linear manner! " Management! The condition is more benign than pemphigus! Lower doses of corticosteroids e.g. Prednisone 40 mg. per day are required! Azathioprine has a steroid-sparing effect! Other therapeutic regimens: sulphones, cyclosporin A and high dose intravenous pulse therapy with methylprednisolone!