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Source: Comprehensive Gynecology, 5th Edition and Novaks Gynecology, 14th edition

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Pediatric and Adolescent Gynecology

Gynecologic Disorders Uncommon in children

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Knowledgeable approach PHYSICAL EXAMINATION INSPECTION Diagnosis for some conditions may require only this step Postero-lateral pressure on inferior portion of labia and surrounding skin Knee chest position (Emans and Goldstein)- to visualize vagina and cervix in children >2y/o Normal findings during prepubertal Hymen: diverse variation (cresentic, annular, collar, septate) Vagina: narrower, epithelium redder and thinner, lacks distensibility, 4-6

Evaluation techniques are age dependent, requiring a physiologic and gynecologic considerations Child as an informant is unreliable Informant is usually the parent or caretaker Gynecologic evaluation pointers Proceed at a slow pace Give ample time Show gentleness and patience o Establish rapport with the child o May defer pelvic exam for the Constantly reassure child Positioning of the child May sit at the edge of the examining table May be examined on the mothers lap Lithotomy position- children age 4-5 y/o Frog-leg position on caretakers lap Prone-knee position-much deeper structures seen; vagina and cervix seen Other helpful techniques Place childs hands on top of physicians and make her a partner by involving her Place child on mothers lap Physician should sit, unhurried approach No drapes nor restraints for preadolescents Make examination short, but complete. Avoid interruptions Room ambiance. Instruments out of sight Use of general anesthesia for optional and selected cases Examination of Adolescent Female adolescents do not want other observers in the examining room They have preconceived ideas of painful examination Inform and counsel the patient INTERNAL EXAM - Rarely needed RECTAL EXAM Distressing so sometimes omitted Instrumentation Indication: recurrent vulvovaginitis, persistent bleeding, suspicion of foreign body, neoplasm, congenital anomalies Speculum Vaginoscope Cystoscope Hysteroscopy Obtain samples of vaginal secretions for micro exam or culture sensitivity: Plastic medicine dropper NSS-saturated cotton-tipped aspirator Catheter-in-catheter aspirator 2nd visit if child is very anxious

cm length, neutral pH Cervix: transverse ridge/pleat redder than vagina Normal findings during puberty Acidic pH, influence of bacilli or glycogenated estrogen-dependent vagina Breast budding is a reliable sign that the vaginal pH is shifting to an acidic environment.

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Indications: genital tract bleeding, pelvic pain, suspicion of foreign body or pelvic mass

lichen sclerosus figure of 8 or hourglass rash, from bubble bath ectopic ureter (ureter in vagina) pinworm infection o nocturnal perineal pruritus (laying of eggs) - 20% with vulvovaginitis

Normal findings non palpable uterus and ovaries cervix- 1.2cm uterus to cervix ratio (1:2) FINDINGS VULVOVAGINITIS 80-90% of OPD consultations present with introital irritation Discharge of varying severity Initially starts as irritation of the vulva followed by secondary involvement of the lower third of vagina 75% with nonspecific virology o GIT flora - nonspecific vaginitis 25% Neisseria gonorrhea, Trichomonas vaginalis, Chlamydia vaginalis, Chlamydia trachomatis, HSV, Shigella boydii, mycotic/bacterial vaginosis (STDs) Vulvitis and vaginitis = vulvovaginitis

foreign body - foul smelling and bloody

Childrens susceptibility to vulvovaginitis Physiologic factors Lack of labial fat pads and pubic hair o Lower 1/3 of vagina open and unprotected especially when squatting Lack of protective effects of estrogen so more prone to infection and irritation Thin vaginal epithelium, has neutral vaginal pH Vagina lacks Lactobacilli, glycogen and antibodies, thus is favorable medium for bacterial growth Poor perineal hygiene o Due to proximity of anus to vagina Unsupervised toilet training o Behavioral factors Poor / unsupervised toilet training contaminates vulvar skin with intestinal flora Autoinoculation of vulva a) b) c) scratch-itch cycle coupled with rare washing of hands upper respiratory tract infection (group ! beta-hemolytic strep) tight-fitting clothes - moist, warm, vulvar skin conducive to infection

Manifestations of vulvovaginitis Usually nonspecific Discharge: white to gray, yellow to green, Minimal to copious Mother notes stain in underwear Suspected foreign body if foul or bloody Associated symptoms: introital irritation, pain, pruritis, dysuria Signs: vulvar erythema, edema, excoriation Usual presentation: bloody, pruritus, dysuria

Pathophysiology of vulvovaginitis Primary irritation of the vulva with secondary involvement of the lower third of vagina Causes Topical allergy Infection (skin, respiratory, UTI) Persistent/recurrent o Foreign body primary vulvar skin disease, ectopic ureter, pinworm infection, sexual abuse (Trichomonas, Differential diagnoses Skin disease: seborrheic dermatitis, psoriasis, atopic dermatitis Gonorrhea, Chlamydia) o o o o Treatment improve local perineal hygiene/ education o o o o wiping from front to back after defecation loose-fitting cotton undergarments avoid allergens/ irritants keep area dry with calamine lotion or a nonirritating cornstarch powder acute weeping lesion wet compress with Burrows solution Sitz bath with water with 2 tbsp baking soda Causes darkening of skin Has systemic absorption

Estrogen cream 3-5weeks use only

Oral antibiotics 10-14 days

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Mebendazole for pinworms 0.5% hydrocortisone cream severe pruritus

Familial true posterior labial fusion autosomal dominant trait with incomplete penetrance Combination of labial adhesions and scarring of the posterior fourchette consider highly the possibility of sexual abuse

VAGINAL DISCHARGE Treatment Prepubertal age: caused by inflammation and irritation o Primary site: vulva 4% of gynecologic visits History not helpful because usually there is no witness, nor does the child remember putting a foreign body into the vagina Objects inserted: toilet paper wads, hairpins, toy parts, crayons, sand/gravel o Object may embed in vaginal mucosa

No special treatment if without problems with voiding Spontaneous separation within 6-8mos If symptomatic: tropical estrogen cream 2x a day for 2-4 weeks (max) o Separation of adhesion in 2 weeks Forceful separation should not be done

ABNORMAL VAGINAL BLEEDING IN INFANCY AND CHILDHOOD o Before menarche, occurring no younger than age 9 years, any bleeding requires evaluation o o slight vaginal bleeding occur within the 1st few days due to withdrawal from exposure to high levels of estrogen urinary, GI factors and sexual abuse should be considered

Physiologic vaginal discharge a.) Neonatal leucorrhea Physiologic vaginal discharge in the newborn Gelatinous, sticky, gray discharge Secondary to high maternal levels of estrogen Cause temporary stimulation to the genital tract epithelium Disappear spontaneously few days post delivery Foreign body o o Common cause of vaginal discharge (purulent/bloody) Most common are small pieces of toilet paper

b.) Premenarchal physiologic vaginal discharge Occur 6-12 mos before menarche due to high levels of estrogen superficial cells can be seen Non-irritating grayish white discharge Microscopic finding: vaginal epithelial cells LABIAL ADHESIONS (ADHESIVE VULVITIS) Common in age 2-6 y/o Agglutination denuded adjacent labia minora Maybe mistaken for congenital absence of vagina Self-limiting consequence of chronic vulvitis

Vulvar Lesions o o Vulvar irritation can lead to pruritus with excoriation, maceration of the vulvar skin, or fissures that can bleed. External causes include urethral prolapse, condylomas, lichen sclerosus or molluscum contagium Endocrine causes o Precocious puberty Vaginal bleeding in the absence of other secondary sexual characteristics Pubertal development younger than 8 y/o (Breast budding and pubic hair precedes menses) o o Granulosa cell tumor PCOS (polycystic ovary syndrome)

Manifestations Usually asymptomatic Difficulty in voiding and UTI In advance cases, fusion over both the urethra and vaginal orifice Pathognomonic: translucent vertical midline at site of agglutination Differentials Imperforate hymen Vaginal agenesis o Genital trauma o

Fall - 75% straddle injuries affect the anterior and lateral vulvar area Lacerations - penetration of the perineum and injury of the internal pelvic organs; automobile/ bicycle accidents

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o o o

Kicks from fighting Self-inflicted wounds Sexual molestation Forms of abuse such as fondling/digital penetration may not result in visible genital lesions

TUMORS OF CHILDHOOD Tumors of labia and vulva Hematocolpos o o o Blood in the vagina (due to obstruction) Imperforate hymen Longitudinal repair

Tumors Rare; most common tumor is Rhabdomyosarcoma Sarcoma bortyoides grapelike clustered mass plus bleeding Causes of bleeding Prepubertal Adolescent Anovulation Exogenous hormone use Pregnancy Coagulopathy

Vaginal cyst Hemangioma/lymphangioma Hydradinoma Teratoma Glanular cell myoblastoma Rare Sarcoma botyroides grape-like masses most common Clear cell carcinoma Functional cyst Physiologic Nothing to worry about, just observe Follicle cyst, corpus luteum cyst

Tumors of the vagina

Vulvovaginitis Foreign body Precocious puberty Tumor Management

Ovarian cyst and tumor

Hematoma - by a blunt object; bluish-red mass o Non enlarging hematoma or non-expanding type o Bed rest with feet elevation Ice pack/ sitz bath for the first 24hour (then apply warm compress/ water bath) Enlarging/ expanding hematoma If the hematoma continues to grow Evacuation and ligation of blood vessel

Germ cell tumors Common epithelial tumor Sex cord stromal tumor Premenarcheal children: ovarian tumors constitute approximately 1% of all neoplasms Preadolescent female: both benign and malignant ovarian tumors are usually unilateral Lipoid cell tumors Germ cell tumors most common ovarian malignancy in preadolescent females Secondary metastatic tumors Dermoid cyst Usually unilateral so only inspection and no biopsy of contralateral ovary is done Complications: torsion, rupture, hemorrhage, infection, malignant degeneration

Laceration - sharp object (e.g. fence post) o Depth may be more extensive than suspected May require inspection under GA to determine extent of vaginal laceration May be associated with injury to the bladder, SI, peritoneal cavity

Hematuria o o o o o Gross: CT scan Micro: cytoscopy Irrigation, debridement, vessel ligation Retroperitoneal hematoma years Explorative laparotomy Tetanus toxoid booster if last immunization is more than 5

Benign teratoma most common tumor in children 75-85% requiring surgery are benign; 15-25% are malignant Manifestations o o o Abdominal pain - most common symptom Palpation of an abdominal mass - most common sign Pain may be vague, frequently associated with complications (torsion, hemorrhage, rupture) causing excruciating pain

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Differentials o o Cyst of mesentery/ omentum - most common non-ovarian cause Benign uterine tumors - rare

related to an ovarian or adrenal disorder increased circulating levels of estrogen may lead to estrogen-induced uterine stimulation and bleeding without any normal ovarian follicular activity most common type granulosa cell tumor > 8cm (60%) most common cause estrogen- or HCG-secreting ovarian tumor other cause: McCune-Albright syndrome (polyostotic fibrous dysplasia) rare triad of cafau-lait spots, fibrous dysplasia, cysts of skull and long bones tx: aromatase inhibitors

Diagnosis o o o Ultrasound MRI CT scan for abdomen Dermoid cyst: UTZ shows calcification

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Treatment Observation for functional luteal and follicular cysts Conservative surgery/cystectomy (benign) (3) Isosexual

PUBERTY o Process of biological change and physical development resulting in the possibility of sexual reproduction Accelerated linear growth and development of secondary sexual characteristics Normal sequence Breast pubic/axillary hair accelerated skeletal growth menarche PRECOCIOUS PUBERTY appearance of any signs of secondary sexual maturation at an early age short stature related to the limited duration of the rapid growth spurt accelerated bone maturation and premature closure of the distal epiphyseal growth centers Types of Precocious Puberty (1) GNRH-dependent complete, true premature maturation of the hypothalamic-pituitary-ovarian (HPO) axis normal menses, ovulation, possibility of pregnancy increased circulating levels of estrogen 30% are secondary to CNS disease high incidence of abnormal EEGs shy and withdrawn from peers GNRH agonists - drug of choice

(4) Heterosexual secondary sex characteristics are discordant with the genetic and phenotypic sex premature virilization in a female child and includes development of masculine secondary sexual characteristics androgens usually come from the adrenal gland

Premature pubarche isolated early development of pubic hair Premature adrenarche isolated early devt of axillary hair Premature thelarche isolated unilateral or bilateral breast development as the only sign of secondary sexual maturation usually occurs 1-4 y/o absent nipple development benign, self-limiting condition does not require treatment breast enlargement spontaneously regresses

CONGENITAL ABNORMALITIES OF THE GENITAL TRACT caused by genetic errors or teratologic events during embryonic development minor abnormality little consequence major abnormality severe impairment of menstrual and reproductive function may be present at birth, before puberty, with the onset of menses, or during pregnancy with adverse pregnancy outcome Genital Tract System

(2) GNRH-independent incomplete, pseudo

Genetic sex is determined at fertilization indifferent gonad stage external genitalia look the same

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 Mesonephric: Wolffian duct - Paramesonephric: Mullerian duct - Y chromosome necessary for the development of testes (4th-6th week) No Y chromosome female Gender is not apparent until the 12th week of embryonic life (phenotype) Development of testes from the effects of testis-determining factor (TDF) Development of ovaries in the absence of TDF Female external genital structures also contain androgen receptors Exposure to high androgen levels early in gestation can lead to masculinization of the female genitalia Maternal estrogen has no direct influence on male genital development Polycystic Ovarian Syndrome obese female; likes like a male does not ovulate; irregular menses high levels of androgen Examination of the Newborn for Sexual Ambiguities Clitoral Abnormalities ** clitoris generally 1 to 1.5 cm long and 0.5cm wide in the non-erect state Clitoral Hyperthrophy o o Male with androgen insensitivity syndrome (AIS) CAH, exposed to exogenous endogenous androgens

Tx: replacement of cortisol, corrective surgery, psychological support (identity issues) IMPERFORATE HYMEN hymen - represents the junction of the sinovaginal bulbs with the urogenital sinus embryonic life: hymen is perforated childhood: mucocolpos or hydrocolpos collection of secretions behind the hymen that builds up and may form a mass puberty: 1 amenorrhea cyclic cramping pain without menstrual flow hematocolpos/hematometrium in advanced cases, the fallopian tubes may be distended with menstrual flow, and the flow may back up through the tubes and form endometrial implants in the peritoneal cavity PE: bulging membrane in introitus Tx: cruciate incision into the hymen extending to 10, 2, and 6 o'clock positions TRANSVERSE VAGINAL SEPTUM mllerian ducts join the sinovaginal bulb at a point (mullerian tubercle) normal vaginal lumen: canalization of the mllerian tubercle and sinovaginal bulb transverse vaginal septum: area of junction between these 2 structures is not completely canalized Complete Septum Partial Septum May bleed but can develop hematocolps /hematometrium NO bulging at introitus Foul smelling vaginal discharge

Enlarged clitoris with fused labia o androgen effect from CAH (congenital adrenal hyperplasia) o maternal ingestion of androgen o high androgen production Bifid clitoris o associated with extrophy of the bladder, short vagina, fused labia LABIAL FUSION May occur without clitoromegaly due to exogenous androgen; associated with defects of the anterior abdominal wall Most common CAH with inborn error of metabolism of 21-hydroxylase diminished cortisol, elevated 17-OH-progesterone conversion to ANDROGEN ANDROSTENEDIONE increasing androstenedione fetal masculinization may develop adrenal crisis due to salt loss

Same sx as imperforate hymen

lies at the junction of the upper 1/3 and lower 2/3 of vagina diagnosed at puberty

VAGINAL AGENESIS associated with Mayer-Rokitansky-Kster-Hauser syndrome congenital absence of vagina and uterus 46, XX karyotype ovaries normal, fallopian tubes present

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 patient cannot bear a child MULLERIAN FUSION ABNORMALITIES polygenic or multifactorial non-obstructive or obstructive complete duplication of the vagina, uterus and cervix may be asymptomatic until the woman begins to menstruate (puberty) earliest symptom: tampons do not obstruct menstrual flow for the Px inserts tampon but the other vagina is open

Arcuate - uterus has normal shape, with small septate indentation at the upper end of the fundus - no negative effects on pregnancy Diagnosis Hx of 1 amenorrhea with cyclic pain, infertility, recurrent fetal loss, ectopic pregnancy, preterm labor UTZ Hysterosalpingography Hysteroscopy MRI/CT scan

can lead to ectopic pregnancy and reproductive wastage (early 2nd trimester) Diagnosis o anomaly indicated by Hx, PE and confirmed by imagine Vaginal Agenesis Unicornuate uterus o destruction of 1 mllerian duct o often related to lack of development of the mesonephric system on one side associated with lack of the appropriate development of the mllerian system o there is almost always a missing kidney and ureter w/ the fallopian tube on the same side o can support a pregnancy Anomalies of lateral fusion of mullerian ducts Didelphus - complete duplication of the vagina, uterus and cervix Bicornuate - single-chamber vagina and cervix with a complete/partial septate uterus and two uterine bodies - most common congenital uterine anomaly - pregnancy wastage

Management Therapy not needed for unobstructed abnormalities (unicornuate and didelphus uteri) Excision for obstructed functional rudimentary horn OVARIAN ABNORMALITIES

Accessory ovary o o Excess ovarian tissue is noted near a normally placed ovary and connected to it Rare

Supernumerary ovary o o A third ovary is normally situated ovaries Rare

Ovotestes o o o o Ovaries that have an SRY antigen Majority in true hermaphrodites Degree to which mullerian and mesonephric development occurs depends to the developing duct system Considerable amount of testicular tissue may descend toward the labial-scrotal area

Diagnosis: Septate - uterus appears as a single organ but contains a complete/partial midline septum - pregnancy wastage Palpation of the gonad in the inguinal canal or within the labialscrotal area Ovulation and menstruation, as well as spermatogenesis may occur

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Treatment: testicular tissue increased risk for malignant degeneration and gonads must be removed after puberty
KEY TERMS AND DEFINITIONS Adhesive Vulvitis. A self-limited condition in which denuded epithelium of the adjacent labia minora agglutinates and fuses the two labia together. A transitional period of life during which an individual matures physiologically and occasionally psychologically from a child into an adult. Agglutination of the adjacent labia minora from a denuded epithelium that fuses the two labia together. A skin dystrophy involving the labia seen typically in prepubertal children and postmenopausal women. A rare syndrome of caf-au-lait spots, fibrous dysplasia, and lesions in the skull and long bones, accompanied by precocious puberty. The disorder is caused by a somatic mutation (thus noninherited) in neural crest cells with a mutation in the G protein system. The process of biologic and physical development after which sexual reproduction first becomes possible. Prolapse of the distal urethral mucosa seen typically in prepubertal children. Inflammatory process involving the vulva and vagina.

Adolescence. Labial adhesions. Lichen Sclerosus Atrophicus. McCune-Albright Syndrome (Polyostotic Fibrous Dysplasia). Puberty. Urethral prolapse. Vulvovaginitis.

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