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Pediatric Gynecology

The gynecologic evaluation of the neonate should begin with a general examination, noting any abnormal findings (webbed neck, abdominal mass, inguinal hernia) that suggest a genital anomaly. Next, the breasts and genitalia should be inspected, noting the appearance, size, and location of each structure.

First, each breast should be inspected. A small amount of breast tissue is often palpable in the newborn as a result of maternal estrogen exposure in utero. Furthermore, a small amount of discharge may be expressed from the nipples. Repetitive breast examinations are not necessary and should be avoided as they may lead to bruising. To visualize the external genitalia, place the infant in the supine position with the thighs flexed against the abdomen. Estrogen effects are also apparent on inspection of the genitalia. The labia majora appear thick and bulbous. A white vaginal discharge is usually present. A small amount of withdrawal bleeding may also be observed during the first few days.

The clitoris often appears large in relation to the other genital structures. If it appears larger than normal, the clitoral index (glans length x width) may be calculated. A normal index is 6 mm2 or less; values above this require evaluation. Testing for congenital adrenal hyperplasia should be initiated immediately when clitoromegaly is observed because a delay in diagnosis can lead to dehydration and death of the neonate. Other causes of clitoromegaly to consider include true hermaphroditism, teratogenic agents ingested.

The vaginal orifice may be difficult to visualize in the newborn due to the redundancy of the hymenal tissue. To confirm patency, slight pressure may be applied to the vestibule, which will produce drops of mucus. If this is unsuccessful, the tip of a well-lubricated, soft rubber catheter may help.

Failure to locate an opening is suggestive of vaginal agenesis or an imperforate hymen, which occurs in 0.1% of births. An ultrasound examination can distinguish between these two disorders by identifying a normal cervix and uterus above the obstruction in patients with an imperforate hymen. To correct this disorder, remove the central portion of the membrane (sutures are usually unnecessary). Other hymenal abnormalities (septate, cribriform, or microperforate) do not require treatment at birth but should be closely followed because their persistence may cause problems with sexual activity or tampon use.

Pediatric Patients

The pelvic examination of the prepubertal child requires a gentle, patient approach. To decrease anxiety, reassure the child that the examination will not be painful. Use dolls or demonstrations to explain what will take place during the examination, and allow her to touch any instruments to be used. It may be helpful to allow the child to view the examination with a mirror. This gives the child a sense of control over the procedure.

To examine children younger than 4 years of age, place the child in the supine position with the hips fully abducted and the feet together in the "frog-leg" position. Alternatively, the child can be placed on the mother's lap with the child's legs straddling the mother's thighs. Children older than 4 years can easily use stirrups. The knee-chest position is important if sexual abuse is suspected because it exposes the most inferior portion of the hymen where injuries.

To view the vaginal introitus, grasp the labia along the inferior portion between the thumb and index finger and gently pull outward and downward (labial traction). This technique is more effective in opening the vaginal introitus than labial separation alone (98% versus 86%). In some cases, it may be necessary to separate the edges of the hymen with a moistened cotton-tipped swab.

Due to a lack of estrogen, the genitalia appear different during childhood (ages 4-7 years) compared with the neonatal period. The labia majora flatten out, and the labia minora become thinner. The vasculature of the mucosal surfaces is easily visualized. The unestrogenized hymenal membrane is flat and thin and can be easily lacerated. The vagina is 4-5 cm in length in the prepubertal child and has a thin, red epithelium that is very sensitive.

Vaginal rugae are not present during this period of development, although longitudinal ridges on the mucosa are common. After the neonatal period, the uterus regresses and does not again reach its natal size until approximately 5-6 years of age. The young child's cervix is approximately twice as large as the fundus, but may be hard to visualize because it is flush with the vaginal vault.

Endocrine activity of the ovaries and adrenal and pituitary glands increases between 7 and 10 years of age. Vulvovaginal features display the effects of estrogen: the labia majora fill out, the labia minora thicken and become rounded, and the hymen thickens and becomes more redundant. The vagina increases in length.

The vagina enlarges to 10-12 cm in length between the ages of 10 and 13 years. Physiologic leukorrhea increases, and lactobacilli are present. The vaginal mucosa thickens and is no longer as sensitive to touch. The cervix becomes a distinct "knob," protruding into the vagina. The cervix to uterus ratio reverses, with the uterus twice the size of the cervix. During the peripubertal years, the ovaries descend into the pelvic cavity and follicular development begins.

Pediatric Gynecologic Problems

Vulvar Disorders

Numerous disorders may result in irritation or inflammation of the vulva. Infections (molluscum contagiosum, condyloma acuminata, herpes simplex) may be transmitted to the child sexually or by close nonsexual contact. The vulva may be exposed to irritants (contact dermatitis), and systemic diseases may have vulvar manifestations (atopic dermatitis, Crohn's disease).

The most common cause of vulvitis in children under 2 years of age is Candida albicans. Most cases present as mild erythema and edema accompanied by pruritus. Severe cases may result in intense, red, macerated, weeping, eczematoid dermatitis with satellite pustules. Because C albicans prefers an estrogenic environment, infection is uncommon after the diaper period unless other risk factors, such as diabetes mellitus or completion of a course of antibiotics, are present. Topical antifungal agents are usually effective.

Another common vulvar disorder in the pediatric patient is labial agglutination, or fusion of the edges of the labia minora. Extensive agglutination from the posterior fourchette to the urethra is present in approximately 5% of prepubertal girls. It is usually asymptomatic, although urinary dribbling or retention, urinary tract infections, or urethritis may result. If it is symptomatic, estrogen cream should be applied directly to the line of adhesions. Asymptomatic cases usually require no treatment. Recurrent adhesions tend to be thick and may not be responsive to estrogen cream. If medical treatment fails, adhesions may be lysed with a scalpel or Bovie cautery under general anesthesia. Mechanical separation of the adhesions should not be attempted in the office setting without anesthesia because the procedure can be very painful.

Molluscum contagiosum is characterized by 1- to 5-mm discrete, skin-colored, dome-shaped, smooth papules with a central cheesy plug. It is often accompanied by pruritus. This viral infection occurs most commonly in children of school age, especially if conditions of poverty, overcrowding, and poor hygiene are present. In tropical climates, which are conducive to the growth of the virus, it occurs in 10% of children. Because the disease can be spread by sexual or nonsexual contact, abuse should be ruled out. Lesions may spontaneously involute in a few months, so initial treatment should not be overly aggressive. If lesions do not resolve, removal of the central core by curettage is effective. A local anesthetic should be administered before curettage.

Condyloma acuminata may occur in children as a result of perinatal transmission or by sexual or nonsexual contact. Although the latency period between birth and the first appearance of warts is controversial, a period of at least 1 year is acceptable. Determination of the etiology beyond this period is difficult because maternal infections present at birth can spontaneously resolve. Furthermore, viral typing has not proven helpful in determining the mode of transmission because all members (male and female) of a family may be infected with the same type. Because sexual transmission is possible, a careful history should be obtained, and an evaluation for other sexually transmissible infections should be performed. If appropriate, authorities should be notified. However, it should be recognized that fomite transmission is a possibility.

The diagnosis of condyloma acuminata usually can be made by visual inspection. Obtaining tissue for biopsy necessitates a general anesthetic in children, so biopsy should be reserved for those cases in which the diagnosis is in question or operative treatment is planned. Treatment options for the clinic setting include application of podophyllin, trichloroacetic acid, or liquid nitrogen. However, these are all poorly tolerated by children because they are painful and require multiple applications. Furthermore, treatment failures approach 50% for all modalities. Recent studies using topical, intralesional, or systemic interferon show similar results. Carbon dioxide or potassium-titanyl-phosphate (KTP) laser should be reserved for very large or symptomatic lesions or those that are unresponsive to other modalities because their use requires a general anesthetic and the long-term cure rate is no better than that of more conservative methods. In planning the therapy, it is critical to discuss the risks, benefits, and limitations of the proposed treatment with the parent (and child, when appropriate).

Genital herpes is uncommon in children. After the neonatal period, most cases result from sexual abuse, although infection is possible through autoinnoculation or close nonsexual contact. A common presenting complaint among young patients is urinary retention; otherwise, the clinical presentation in children is similar to that of adults. Vesicular, blistering lesions on the genitalia may also result from primary infection with the varicella-zoster virus. Culture (with typing) is the gold standard for diagnosis of herpes simplex virus. Direct fluorescent antibody testing should also be performed to differentiate between varicella and herpes simplex vims type I or type 2. When herpes is present, oral acyclovir may provide partial relief of symptoms. When used for recurrences, it must be given within 2 days of the outbreak of lesions to be effective. Topical acyclovir should not be prescribed because it is less effective.

Approximately 10-15% of cases of lichen sclerosus occur in children. The etiology of this disorder is unknown, but may be related to an autoimmune disorder. Diagnosis is usually apparent on visual inspection: the skin is thin and has a pale, parchment-like appearance. Children often present with bleeding because the skin is easily traumatized. Alternatively, vaginal discharge may be the presenting complaint because secondary infection is common. Chronic cases may result in atrophy of the labia majora or clitoris, constriction of vaginal introitus or urethral meatus, anal stenosis, or anal fissures. Topical application of a moderate- to high-potency corticosteroid preparation for 1-3 months effectively treats the symptoms of lichen sclerosus in most children but is not curative. Hydroxyzine hydrochloride also may be administered to relieve itching. At or near menarche, lesions spontaneously resolve in 66% of children.

Atopic dermatitis, an inflammatory skin disease that begins in infancy, affects approximately 2-8% of children. It characteristically affects the face, neck, chest, and extremities. Chronic cases may result in crusty, weepy lesions of the vulva that are accompanied by intense pruritus and erythema. Scratching often results in excoriation of the lesions and secondary infection. Serum immunoglobulin E (IgE) levels are elevated in 80% of patients and may aid in diagnosis. Application of topical fluorinated corticosteroid preparations may be used during acute flare-ups. Secondary infection can be treated with erythromycin or cephalexin.

Contact dermatitis in children is associated with exposure to an irritant, such as perfumed soaps, bubble baths, or nylon panties. Erythematous, edematous, or weepy vulvar vesicles or pustules, which become thick and lichenified, may result. The localized nature of this condition distinguishes it from atopic dermatitis, although treatment is the same for both.

Psoriasis of the vulva, in contrast to the red, well-demarcated plaques of classic psoriasis, usually appears as poorly demarcated, scaly patches. Lesions are most commonly found on the mons pubis, although symmetrical fissures between the labia minora and labia majora.

to the preclitoral area may also be observed. The diagnosis is confirmed by locating other affected areas on the body, such as crusting behind the ears, on the scalp, or in the nasolabial folds. Vulvar lesions may be treated topically with moderate- to high-potency fluorinated corticosteroid preparations.

Vaginal Discharge and Bleeding

Vaginal discharge is the most common gynecologic problem seen in children. The vagina is particularly susceptible to infection.

A foul-smelling, bloody discharge is suggestive of Shigella infection or a foreign body. Tissue paper is the most common foreign body identified.

Other causes of vaginal bleeding in the pediatric patient include withdrawal bleeding, urethral prolapse, neoplasms, precocious puberty, and trauma. Urethral prolapse occurs most commonly in African-American girls under 10 years of age. Bleeding is the most common presenting complaint.

Straddle-type injuries, which often result in only minor lacerations or abrasions of the labia, account for 75% of genital trauma in children. If a vulvar hematoma results, apply pressure to the wound with ice packs. Large hemato-mas may necessitate drainage and vessel ligation.

Developmental Anomalies

Müllerian anomalies usually present during the adolescent years with a complaint of primary amenorrhea (müllerian aplasia, vaginal atresia) or cryptomenorrhea (transverse septum, incomplete longitudinal septum).

Müllerian aplasia (Mayer-Rokintansky-Kiister-Hauser syndrome) is congenital absence or hypoplasia of the fallopian tubes, uterine corpus, cervix, and proximal vagina. After gonadal dysgenesis, this is the second most common cause of primary amenorrhea.

Creation of a neovagina should be deferred until the patient has a partner and desires to have intercourse. The Frank procedure has a success rate of more than 90% and is the treatment of choice for these young women. Surgical creation of a neovagina by the McIndoe procedure.

Other procedures that have been described include the William vaginoplasty, colovaginoplasty, amnion vaginoplasty, tissue expansion technique, and rectus abdominis musculocutaneous flap. Because at least eight cases of vaginal carcinoma have been reported after creation of a neovagina.

Transverse septa occur less commonly than müllerian aplasia. This disorder results from a failure of fusion of the müllerian duct derivatives.

Failure of fusion of the lower müllerian ducts that form the vagina results in a longitudinal vaginal septum. Septa may be sagittal or coronal. This anomaly may result in difficulty with sexual intercourse or tampon insertion, or it may result in an arrest of the second stage of labor. If an imperforate septum does not extend the entire length of the vagina and is associated with a didelphic uterus, outflow obstruction will occur, resulting in the formation of a hematocolpos. symptomatic, the septum should be

Vaginal atresia is failure of the urogenital sinus to form the lower vagina with normal mullerian structures above.