Introduction
Gynecological symptoms are relatively common before puberty, although serious pathology is rare. Nonspecific vulvovaginitis and labial adhesions contribute to a significant proportion of the workload of a pediatric gynecologist. Clinicians must be able to confidently identify and manage these common conditions as symptoms can be prolonged and last for many months before resolution. In addition, clinicians also need to be aware of the rarer causes of vulvar symptoms including dermatological disease, recurrent vulvar ulceration, and genital lesions. Vaginal bleeding is uncommon but can indicate more serious underlying pathology and should be carefully investigated.
Vulvovaginal Symptoms
Vulvovaginal symptoms are the commonest gynecological complaints in a prepubertal child and are the most frequent indication for referral to a pediatric gynecologist. Symptoms may include vulvar pruritus and burning and/or pain and may also be accompanied by complaints of vaginal discharge or in rare cases bleeding. A number of factors predispose the prepubertal girl to vulvovaginal conditions including lack of labial development and fat, absence of pubic hair, an unestrogenized mucosa, a more alkaline vaginal environment, and the close proximity of the vagina to the anus. These factors, often combined with difficulty with hygiene, make the vulvar skin in the prepubertal child particularly sensitive to irritants and infections.
Vulvovaginitis
Vulvovaginitis is the most common cause of symptoms before puberty. The peak age of presentation is between ages 3 and 7. Girls may complain of vulvar pain, pruritus, or dysuria, and parents may notice discharge on the child’s undergarments. The discharge may be green or yellow and foul smelling. Typically, examination will reveal a reddened “flush” around the vulva and anus. The skin may be excoriated and discharge may be evident. A culture swab of the perineal area can be taken, although this is often negative or contains nonspecific skin flora or mixed anaerobes from the gut. Most vulvovaginitis is noninfectious (74 percent to 80 percent), and often termed “nonspecific vulvovaginitis.” When identified, infectious agents include respiratory pathogens, likely from autoinoculation or enteric pathogens. The most common infective agents identified in prepubertal vaginitis include group A beta-hemolytic streptococci and Hemophilus influenzae. Others include Staphylococcus aureus, Moraxella catarrhalis, Streptococcus pneumonia, Niesseria meningitides, Shigella, and Yersinia enterolitica [1]. Specific pathogens should be treated with appropriate antibiotics. Recurrence is common for both nonspecific and infectious vulvovaginitis and the mainstay of treatment is improved vulvar hygiene.
Treatment of Vulvovaginitis
The mainstay of treatment is hygiene and the avoidance of irritants such as soaps. Improvement can be very slow and episodes of recurrent symptoms are common. Parents need to be clear that the measures should be continued long term. It is likely that resolution will occur as the child gets older. This is probably in part due to improved dexterity and skill in keeping the genital area clean. In addition, the rise in estrogen levels as puberty approaches will change the anatomy and skin quality. Parents should be counseled on measures outlined in Table 6.1.
| Measure | Action |
|---|---|
| Clothing | Wear loose-fitting, cotton underpants |
| Avoid tights, leggings, and leotards | |
| Bathing | Allow to sit in warm water for 10–15 minutes daily |
| Avoid soap to wash the genital area – no bubble baths! | |
| Wash hair and body just prior to removing child from tub | |
| Rinse the genital area well and pat dry | |
| General hygiene | Emphasize wiping front to back |
| May sit backward on toilet to avoid pooling of urine within the vagina | |
| Avoid constipation | |
| Other | Shower after swimming |
| Avoid sitting in wet swimsuits for prolonged periods | |
| Emollients | Can use soap-free emollients such as aqueous cream for washing |
| Barrier creams may help | |
| No antifungal creams unless fungal infection confirmed on culture |
Persistent or Recurrent Symptoms
In the case of persistent or recurrent symptoms despite adequate hygiene measures, particularly if a foul odor or vaginal bleeding is present, a specific infection or the possibility of a vaginal foreign body should be considered (see later). Persistent wetness or discharge from an ectopic ureter can be confused with “vaginal” discharge. In the case of upper urinary tract infection, purulent discharge may be present. Most commonly the ectopic ureter empties onto the perineum adjacent to the normal urethra; however, other sites include the cervix, vagina, uterus, and urethra. On examination, a small opening or drop of urine might be visible adjacent to the urethra. Further imaging with renal ultrasound and/or intravenous pyelogram can help with the diagnosis and a referral to a pediatric urologist is indicated.
Other Infective Causes
Infections with sexually transmitted organisms such as Gonorrhea, Chlamydia, or Trichomonas, if detected, indicate sexual abuse. Appropriate referral is necessary and is addressed in detail elsewhere. Vulvar pruritus and pain, particularly with symptoms predominantly at night, can be caused by pinworms (threadworms). If clinically suspected, empiric treatment with a single dose of mebendazole may be helpful. Worms may be visible at the anus or in feces, but the traditional “scotch (sellotape) test” suggested to parents in the past is not usually helpful. Candida vaginitis is a common cause of vulvar pruritus and irritation in estrogenized adolescents and adult women; however, it is uncommon in the toilet-trained prepubertal girl. Misdiagnosis and inappropriate treatment are common. If present on vaginal cultures, candidiasis should prompt consideration of diabetes mellitus in this age group.
Dermatological Causes
Dermatologic conditions, such as atopic dermatitis, psoriasis, and lichen sclerosis may also cause vulvar symptoms. Lichen sclerosus (LS) is a chronic dermatologic condition of unknown etiology. An association exists between LS and autoimmune diseases such as vitiligo, autoimmune thyroid disease, alopecia acreata, and rheumatoid arthritis. LS is often seen in adult women, but 10 percent to 15 percent of cases are seen in children [2]. A hormonal association is suggested due to peaks in the prepubertal and postmenopausal years. Additionally, many girls see an improvement in symptoms with the onset of puberty. Clinically, pruritus is the most common complaint, at times associated with pain, dysuria, or bleeding from erosions or fissures. With rectal involvement, constipation or pain with defecation may be present. The diagnosis is made on examination. LS typically appears as a sharply demarcated, hypopigmented lesion surrounding the vulva and anus. The skin appears thin and atrophic, often described as having a “parchment-paper” appearance. Purpura, fissuring, and erosions may be present. With time, significant scarring including fusion and resorption of the labia minora, introital narrowing, and clitoral phimosis may occur (Figure 6.1). In the prepubertal girl with a typical history and physical examination consistent with LS, biopsy is not necessary. Vulvar biopsy should be reserved for cases when the diagnosis is in question or those not responsive to standard treatment.
Figure 6.1 Lichen sclerosus
Treatment involves potent topical steroid such as 0.05 percent clobetasol propionate. Short courses of high-potency topical steroids appear to be safe, effective, and well tolerated [3]. However, prolonged use can be associated with atrophic skin changes, telangiectasias, secondary infection, and adrenal suppression.
A number of regimens have been suggested and have been shown in case series to lead to good response. No robust data in the pediatric population suggests an ideal treatment regimen. Following disease improvement with an initial twice daily or daily regimen of high-potency steroid, a taper to a lower-potency steroid such as 0.1 percent triamcinolone acetonide then 1 percent hydrocortisone should be considered [4].
Topical calcineurin inhibitors pimecrolimus and tacrolimus have also been used for treatment of LS. Data regarding their use in this setting is limited; however, multiple case reports have indicated success. A double-blind, randomized prospective study comparing topical clobetasol propionate 0.05 percent with topical tacrolimus 0.1 percent in 55 women and children with LS showed a significant decrease in symptoms (burning, pain, and pruritus) in both groups. However, the clobetasol group showed a significantly faster response in symptom control at one- and two-month follow-up. Moreover, significantly more patients treated with clobetasol achieved complete remission of clinical signs of disease on anogenital exam [5].
Both pimecrolimus and tacrolimus have an FDA black box warning concerning a possible relationship between long-term use and skin cancer and lymphoma. Given the black box warning and the known effectiveness of high-potency steroids for disease management, these should be considered second-line options in the setting of an inadequate response to high-potency steroid following confirmation of a diagnosis of LS with vulvar biopsy. Neither should be utilized in children under age 2.
Even with adequate clinical improvement, recurrence is common and patients should be monitored every 6–12 months to assess for this [6]. Postmenopausal women with lichen sclerosis are known to have an increased risk of squamous cell carcinoma of the vulva. It is uncertain what this risk may be in children and adolescents diagnosed with lichen sclerosis. Parents should be advised of this potential risk and the importance of long-term follow-up for surveillance of symptoms and clinical findings.
Genital Lesions
Prepubertal girls may present with a variety of genital lesions. These may be an incidental finding on routine examination or present as a genital swelling or mass, or at times with pain, irritation, discharge, or bleeding.
Labial Adhesions
Labial adhesions, also known as labial fusion or agglutination, are a common finding in prepubertal girls. The prevalence is likely to be underreported but is cited as between 2 percent and 39 percent [7]. Labial adhesions are uncommon before age 1 and are not present at birth. The etiology is unknown but thought to be due to a combination of vulvar irritation and hypoestrogenism in prepubertal girls. The appearance is characteristic and the diagnosis is made on clinical examination. Fusion of the labial skin is noted extending from the posterior vaginal introitus toward the urethra. There is often a clearly visible raphe consisting of a thin membranous line in the midline where the labia join. In severe fusion, only a pinhole opening may be visible (Figure 6.2). The majority of cases are asymptomatic and no treatment is required. It is thought that 80 percent of cases spontaneously resolve within one year and virtually all will resolve with the onset of puberty and endogenous estrogen production [8]. If symptoms are present, they are often urinary, including frequency, post-void dribbling, and urinary tract infection. There have been rare reports of urinary obstruction. Pooling of urine behind the adhesions may also lead to vulvar irritation and vaginitis.
Figure 6.2 Labial adhesions
Treatment of Labial Adhesions
Treatment is only required if the child is symptomatic. First-line therapy should be a conservative approach with topical estrogen. A pea-sized amount of estrogen cream is applied to the labia using a finger or Q-tip with gentle downward pressure along the line of fusion. The labia will start to “buttonhole” and then separate. Success rates are reported at 50 percent to 88 percent [9]. Once separated, parents should be counseled on vulval hygiene and use of bland emollients to prevent recurrence. However, even with meticulous care, recurrence following discontinuation of treatment is common. Published treatment regimens vary in their frequency and duration of use of estrogen; however, the majority will resolve in 2–6 weeks. Treatment should not exceed 6 weeks as even with topical vaginal administration alone, a small amount of estrogen is absorbed systemically. Although rare, side effects can include breast swelling and tenderness, vulvar pigmentation, and vaginal bleeding. Breast bud formation and vaginal bleeding will resolve after the cessation of treatment.
The use of topical steroid (betamethasone 0.05 percent) has been reported as an alternative or adjuvant conservative therapy for labial adhesions with varying rates of success (16 percent to 89 percent) [10,11]. Betamethasone 0.05 percent can be applied twice daily for 4–6 weeks. Skin atrophy and systemic steroid effects can occur with prolonged use and this should be avoided. Topical estrogen remains the first-line treatment; however, a trial of topical betamethasone can be considered in those who fail estrogen therapy before resorting to surgical separation.
Rarely, in the setting of severe urinary symptoms (urinary obstruction or recurrent urinary infections) and failed conservative management, surgical separation is required. This should be performed under a brief general anesthetic. Manual separation using local anesthesia in the outpatient setting has been reported with good success rates [12]; however, it has been associated with significant distress and discomfort for child and parent alike [13]. If surgical separation is performed, estrogen cream and emollients should be used for a period postoperatively to prevent recurrence. Despite this, recurrence remains common, even after surgery.
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