Disorders of the Labia

LABIAL AND VULVAR AGGLUTINATION (ADHESIONS) Agglutination (adhesion) of the labia minora or vulva is usually seen in girls 3 months to 6 years of age, although it is occasionally seen in girls who are approaching puberty. This condition is also called vulvar synechiae, gynatresia, vulvar or labial fusion, and labial coalescence. This disorder accounts for about 50 percent of prepubertal gynecologic complaints seen in an outpatient setting.

Prepubertal labia minora, covered by a very thin hypoestrogenized epithelium, are easily inflamed and denuded following infection, local trauma, or irritation. Often, the source of irritation is an environmental source previously tolerated during the diapered neonatal period and during earlier infancy (e.g., bubble bath, baby shampoos, and soaps). In addition, the anterior progression of labial agglutination from its origins at the posterior fourchette may lead to a significant "pocketing" of voided urine behind the agglutinated tissues, serving as a nearly constant source of additional irritation.

The girl with labial agglutination is most often asymptomatic, and the diagnosis comes to the physician's attention through parental concern or during routine examination. Some children have symptoms of urethritis; others have difficulties in toilet training due to the tendency for the perineum to remain moist from pocketed urine. Urinary tract infections are common.

The physical examination of a girl with labial agglutination is sufficient to distinguish this condition from its differential diagnostic considerations: ambiguous genitalia and imperforate hymen. The site of midline fusion of the labioscrotal folds seen in ambiguous genitalia, known as the median raphe, is a thick, raised, linear structure. In contrast, the site of midline labial agglutination is very characteristically thin, demonstrating agglutination of medial surfaces of the labia, not fusion of the labioscrotal folds. In addition, the agglutinated labia minora are distinct from and fused in the midline under the clitoral hood, while in ambiguous genitalia the labia minora are incorporated into the clitoral hood. Differentiation of labial agglutination from imperforate hymen is easily accomplished by noting that the hymen is located at the vaginal orifice, which is on the same plane as the urethra, as compared to the more exterior position of and posterior-to-anterior extension of labial agglutination. Occasionally, the presence of labial agglutination may give rise to parental concerns about sexual abuse if the adhesions are forcibly disrupted during straddle activity, resulting in a larger than previously recognized "opening," perhaps with a small amount of oozing blood.

Most girls exhibiting labial agglutination require no treatment, since the adhesions resolve spontaneously during puberty. Girls whose labial agglutination results in recurrent urinary tract infections, toileting difficulties, or difficulties in visualizing the urethra should probably be treated. Almost always, the agglutination responds to removal of the source of the caustic irritation coupled with careful application of topical estrogen cream (0.1% conjugated estrogen vaginal cream) twice daily for 2 to 4 weeks.

LICHEN SCLEROSIS ATROPHICA Although uncommon in prepubertal girls, lichen sclerosis is being increasingly recognized by emergency physicians and pediatricians. This increased recognition is in some measure a reflection of increased concern about and awareness of sexual abuse and the subsequently increased frequency of perineal examination of prepubertal girls. The affected girl complains of itch, irritation, dysuria, perineal and/or perianal pain, and bleeding. There may be a coexistent vaginal discharge. As perianal pain persists, the girl may develop problems with painful defecation, stool retention, constipation, and encopresis.

The characteristic appearance of the vulva is white, atrophic, and finely wrinkled. Ulcerations, blisters, excoriations, and inflammation are seen over the vulva, perineum, and perianal area, often giving rise to the terms hourglass or figure-eight to describe the pattern of skin involvement. Secondary infection is possible. Progression of the disease leads to distortion, thickening, and scarring of vulvar and perineal architecture. The condition is differentiated from vitiligo by absence of inflammation or atrophy in the latter. The diagnosis of lichen sclerosis atrophica is made on clinical grounds and may be confirmed histologically from biopsy specimen in atypical cases.

The friability of the affected tissues leads to an increased risk of bleeding, even as a consequence of very minor trauma (e.g., the friction associated with bicycling). This has given rise to concerns in some parents about possible sexual abuse. Typically, the child denies such contact. There is no evidence linking lichen sclerosis atrophica to sexual abuse.

Management involves removal of all perineal irritants accompanied by the use of systemic antipruritics and the local application of an emollient ointment, such as A & D ointment. A 2- to 3-month course of treatment with a low-potency topical steroid cream, such as 2.5% hydrocortisone cream, applied two to three times daily, is often useful. Topical antifungal creams and systemic antibiotics are indicated for treatment of yeast and dermatophyte infections, and bacterial superinfections, respectively.

Constipation Prescription

Constipation Prescription

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