Vulvovaginal disorders in prepubertal girls-including vulvovaginitis, lichen sclerosus, labial adhesions, urethral prolapse, and genital ulcers-are common and can be managed on an outpatient basis by a skilled clinician.
By Rachel K. Casey, MD, and Veronica Gomez-Lobo, MD
Dr. Casey is an obstetrician/gynecologist completing fellowship in pediatric and adolescent gynecology at MedStar Washington Hospital Center and Children’s National Health System in Washington, DC.
Dr. Gomez-Lobo is the director of pediatric and adolescent obstetrics and gynecology at Medstar Washington Hospital Center, Children’s National Health System, and clinical professor of obstetrics and gynecology at Medstar Georgetown University Hospital, Washington, DC.
Neither author has a conflict of interest to report with respect to the content of this article.
Vulvovaginal complaints in prepubertal girls are common and account for up to 80% of childhood visits to gynecologists.1 Some common complaints include vulvovaginal itching, irritation, rash, bleeding, and pain. Vulvovaginal complaints encompass a wide variety of conditions. Those most often encountered include vulvovaginitis (infectious and noninfectious), lichen sclerosus, genital ulcers, labial adhesions, and urethral prolapse.2 The symptoms associated with these conditions may be similar but with subtle differences. Frequently, a diagnosis can be made with a thorough history and physical exam (Table 1). All these diagnoses are benign and can often be managed in the outpatient setting with close follow-up.
The gynecologic exam is an essential part of the evaluation of a vulvovaginal complaint and should be approached in a delicate manner. Girls and their caregivers may feel anxious about what the exam may entail and any pain it may cause. It is important to allay fears, emphasizing that an internal exam is rarely warranted in prepubertal girls and a gentle external exam should not cause pain.
Many caregivers convey worry about the possibility of sexual abuse in girls with a vulvovaginal complaint. Physicians should emphasize that examination of the genital area should be performed only by a healthcare provider for medical reasons. Caregivers should be asked directly if they suspect abuse. If a concern about abuse arises, the patient should be referred to a child protection center for further investigation. This topic takes careful consideration because some vulvovaginal complaints can be mistaken for sexual abuse. For example, perinatally transmitted anogenital warts are common in girls younger than 2 years and do not necessarily indicate abuse.3
Enlisting the help of the patient and caregiver can reduce anxiety. The girl can be examined in a supine “frog-leg” position, which is often described to young patients as a “butterfly,” or in a prone knee-to-chest position. The patient may sit on the caregiver’s lap while in either position. A chaperone should be considered if the caregiver is not present or if the caregiver or patient requests one.4
A thorough genital exam can be performed with careful external inspection. An internal speculum exam is not indicated for an outpatient exam in prepubertal girls and utilized in postpubertal girls only for certain indications. If a prepubertal child requires an internal exam, it should be performed under anesthesia, preferably with vaginoscopy in the operating room. During external exam, separation of the posterior labia minora allows for adequate visualization of the clitoris, urethra, hymen, and vaginal introitus.5 This can be done by placing the hands on the buttocks and gently exerting downward and lateral traction (Figure 1). An alternative method involves gently grasping the labia majora and pulling outward (Figure 2). A rectal exam may be performed to evaluate internal pelvic organs, but is not routinely indicated.
The provider should note the presence and pattern of pubic hair, size of the clitoris, shape of the hymen, and any abnormalities of the labia. In infant girls, the hymen will be estrogenized and appear moist, pink, and fluffy. In older prepubertal girls, the hymen will appear thin and red due to the lack of estrogen. Various configurations of the hymen are normal, including crescentic, annular, and redundant. In girls with hymeneal lacerations or transsections, sexual trauma should be considered. These patients should be referred to child protective services for forensic evaluation.5
Anatomic and physiologic factors in prepubertal girls may predispose them to irritation. Girls often present with itching, irritation, pain, or rash. Initially, a newborn remains estrogenized from maternal hormones, which can protect the vaginal tissue from irritation and infection for months to years due to fluctuations in gonadotropins.2 Lack of estrogen and pubic hair, and the proximity of the vagina to the rectum, later increase susceptibility to irritation. As girls enter puberty, estrogenization of the genitalia in conjunction with adrenarche serve as protection against irritation and pathogens.2,6
Girls with vulvovaginitis may complain of generalized itching, irritation, discharge, and dysuria. Detailed history should elicit information regarding irritants such as bath soaps, feminine hygiene products, baking soda, and bleach baths. Associated illnesses such as recent upper respiratory infection or diabetes may also be uncovered. Bloody or purulent discharge may signify precocious puberty or indicate the presence of a foreign body. These patients should undergo further examination for pubertal development, and a vaginal lavage to evaluate for foreign body.6,7
Examination may reveal erythema, excoriations, and discharge, which is commonly thin and mucoid.6 Discharge that is copious and watery may indicate a specific pathogen, such as streptococcus pyogenes, and a culture should be obtained.7 Due to the nonspecific nature of this condition, an in-depth differential diagnosis should be considered.
Vulvovaginitis can be nonspecific or specific, secondary to a specific pathogen, and is also termed infectious or noninfectious. Nonspecific vulvovaginitis is far more common, and thus, initial treatment involves changes to hygiene practices, specifically elimination of any irritants (Table 2).2,6,7 To relieve symptoms, patients should be instructed to sit in warm-water baths, without soap, twice a day. Antihistamines may also be helpful to relieve itching at night. Measures to protect the skin may include using an emollient, wearing loose-fitting clothing and cotton underwear, and avoiding soaps in the bathtub by using a shower prior to bathing. Reviewing bathroom practices, such as wiping front to back and sitting with legs wide apart while urinating, may also be helpful. Adequate treatment and prevention of constipation may assist in preventing perianal bacterial overgrowth and vulvovaginal irritation.2,6,7
Specific vulvovaginitis typically presents as an acute episode and treatment should be initiated only after a positive vaginal culture. Even if nonspecific vulvovaginitis is suspected, persistent or atypical discharge should be cultured. One of the more common infections in prepubertal girls is S pyogenes. Vulvovaginal streptococcal infection is often associated with a nasopharyngeal infection and should be treated with penicillin V or amoxicillin.1,7
Recurrent episodes of vulvovaginitis may require further investigation and consideration of uncommon causes, including foreign bodies, congenital malformations, tumors, trauma, and skin diseases.6 Some providers may consider empiric treatment for pinworm with mebendazole or an alternative antibiotic.7 If an empiric course of treatment is given, it is important to avoid overuse of antibiotics, both oral and topical, as this often worsens symptoms. Candidal infections are more commonly seen in infants in diapers because the basic pH in prepubertal girls is not conducive to Candida overgrowth.2,7 Vulvovaginitis due to Candida is thus more commonly seen after antibiotic use or in conjunction with another risk factor, such as diabetes or immunosuppression.1,7
Lichen sclerosus is a chronic inflammatory process with an unclear etiology. Research suggests that genetic factors, autoimmune factors, and local irritation may contribute.8 The condition is most commonly seen in prepubertal girls and postmenopausal women, with an increasing prevalence in childhood, estimated at 1:900–1000.9,10 It is estimated that 7% to 15% of all vulvar lichen sclerosus is found in prepubertal girls.10 Girls complain of symptoms similar to those of vulvovaginitis, such as itching, irritation, pain, and dysuria.
Exam findings typically reveal lightening and thinning of the skin, sometimes associated with small tears, bruises, or bleeding due to subepithelial hemorrhages. The dermatologic abnormality is usually seen in a “figure of eight” pattern surrounding the vulva and anus and may involve the labia minora, majora, clitoris, introitus, or perianal tissue (Figure 3).1 Progressive disease may cause scarring and loss of normal architecture, resulting in distorted-appearing anatomy.9
Diagnosis is made clinically and a biopsy in a prepubertal girl is typically not indicated. High-potency steroid ointment is the most effective treatment in young girls. Treatment goals include improvement in both symptoms and evidence of scarring.10 Clobetasol ointment applied 1-2 times per day for 2-4 weeks is first-line treatment. The ointment may then be tapered and a less-potent steroid can be used, if needed, for symptomatic relief. Although symptoms may improve, hypopigmentation of the skin may persist. It is important that topical steroids are used sparingly and tapered when adequate improvement is achieved to avoid side effects.7,10 It is, however, also important not to undertreat to avoid persistent symptoms and possible scarring of the vulva. Symptoms and severity of lichen sclerosis may remit and recur over many years.10 In some patients, symptoms improve after puberty, but evidence suggests that most are at risk of recurrence, underscoring the importance of long-term follow-up. In addition, good vulvar hygiene is important for symptom management.10
Agglutination of the labia minora most commonly occurs in girls aged 3 months to 6 years.7 This prepubertal age group is most susceptible due to lack of systemic estrogen.11 Common complaints may include abnormal-appearing anatomy, postvoid dribbling and sometimes concern about an absence of the vaginal introitus.7
Physical exam reveals thin, filmy adhesions visualized between labia minora (Figure 4). It is possible for the adhesions to cover the vaginal introitus and sometimes the entire urethra. Diagnosis is made based on visual exam alone.
Without treatment, most adhesions will resolve spontaneously at puberty. Asymptomatic adhesions do not require treatment. Adhesions resulting in urinary tract infection, vulvar irritation, or postvoid dribbling may warrant treatment. Initial therapy involves topical estrogen cream applied directly to the adhesion with some pressure, twice daily for 3 weeks, then another 2 to 4 weeks. Adhesions will resolve after 2 to 3 weeks of treatment in approximately 50% of girls.7 The majority will resolve after a 6-week course of therapy. Topical betamethasone is an alternative for girls with a contraindication or intolerance to topical estrogen. Those with persistent or obstructive symptoms may require separation of labial adhesions, which can be done with a cotton swab using topical lidocaine on a cooperative older girl in an outpatient setting or in the operating room under sedation for young children. Rarely, labial adhesions persist beyond puberty and appear dense, requiring surgical separation.
Vaginal bleeding in the prepubertal girl may indicate trauma, vulvar dermatologic abnormality, precocious puberty, foreign body, or urethral prolapse.12 Urethral prolapse typically presents as painless vaginal bleeding, commonly in girls aged 5 to 8 years.1,7 The condition is most commonly seen in premenarche and postmenopause, times when estrogen is low.12
Physical exam will reveal a circular red or blue protrusion typically arising from or obscuring the vaginal introitus.12 Surrounding genital anatomy should appear normal, without findings of vaginal irritation or discharge. Often, the mass is found incidentally by the patient or on routine physical exam.
Options for treatment include observation or medical therapy. If the protrusion is asymptomatic and not bothersome, no treatment is necessary. During puberty, estrogenization of the tissue typically results in spontaneous resolution. If the mass is bothersome or causing bleeding, topical estrogen cream can be applied to the affected area twice daily for 2 to 4 weeks. If necrosis is noted on exam, resection of the area by a pediatric urologist may be indicated. It is important to discuss with patients that even after resolution, urethral prolapse may recur. Recurrences during the prepubertal period should be managed in the same fashion because most will resolve after estrogenization of the tissue.7,12
Vulvar ulcers are increasingly being reported in young adolescent patients who are not sexually active.13-15 They are less commonly seen in prepubertal girls. A detailed history should be obtained and should include exposure to infectious disease, recent travel, and pets to uncover rare entities. A comprehensive sexual history should be taken confidentially, with caregivers out of the room, to help guide a differential diagnosis.
Ulcers in young adolescents who are not sexually active are often identified as “aphthous” or “Lipschutz” ulcers. Ulcers commonly appear following a prodromal viral illness, such as a gastrointestinal, upper respiratory, or nonspecific febrile illness.15 Evaluation of underlying sexually transmitted infections, such as herpes simplex virus, chancroid, and syphilis, is important. Other etiologies include nonsexually transmitted infection, such as varicella, and systemic illnesses such as Crohn’s disease, Behcet’s disease, Epstein-Barr virus, and cytomegalovirus.15
Patients will often present complaining of severe vaginal pain and dysuria. The pain may be so intense that it interferes with daily activities. Some patients may require narcotics for pain control and others may even require inpatient pain management.1
Examination typically reveals black, necrotic, shallow, or deep ulcers in the labia minora and/or majora and often labial swelling.15 Lesions are exceedingly painful and may be associated with an overlying purulent discharge. A culture of the ulcer may be obtained to rule out infectious etiology. Serum evaluation of systemic illnesses should be undertaken if cultures are negative.
Treatment is supportive. Warm-water baths may be used for comfort and during urination. Topical lidocaine may help some patients to urinate without pain. In certain circumstances, the pain may become so severe that hospitalization is required.1,7
Vulvovaginal disorders in prepubertal girls are common and can be managed safely on an outpatient basis. An in-depth medical history guides evaluation and treatment. Refer to a child protection team if concern for abuse arises. The majority of these conditions do not require surgical intervention and can be managed on an outpatient basis by a skilled clinician (Table 3). Improved hygiene measures are very helpful in improving bothersome vulvovaginal symptoms.
References
1. Trager JDK. Vuvar Dermatology. In: Emans SJ, Laufer MR. Emans, Laufer, Goldstein’s Pediatric and Adolescent Gynecology. 6th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2012:60–99.
2. Van Eyk N, Allen L, Giesbrecht E, Jamieson AM, Kives S, Morris M, et al. Pediatric vulvovaginal disorders: a diagnostic approach and review of the literature. J Obstet Gynaecol. 2009;31:850–862.
3. Varma S, Lathrop E, Haddad LB. Pediatric condyloma acuminata. J Pediatr Adolesc Gynecol. 2013;26; e121–122.
4. Curry ES. Committee on Practice and Ambulatory Medicine. Policy Statement: Use of Chaperones During the Physical Examination of the Pediatric Patient. Pediatrics. 2011;27:991–993.
5. Emans SJ. Office Evaluation of the Child and Adolescent. In: Emans SJ, Laufer MR. Emans, Laufer, Goldstein’s Pediatric and Adolescent Gynecology. 6th ed. Philadelphia, PA:Lippincott Williams and Wilkins; 2012:1–20.
6. McGreal S, Wood P. Recurrent vaginal discharge in children. J Pediatr Adolesc Gynecol. 2013;26:205–208.
7. Emans SJ. Vulvovaginal Problems in the Prepubertal Child. In: Emans SJ, Laufer MR. Emans, Laufer, Goldstein’s Pediatric and Adolescent Gynecology. 6th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2012:42-59.
8. Trager JDK. Pediatric vulvar lichen sclerosus. North America Society for Pediatric and Adolescent Gynecology. http://www.naspag.org/index.php/pagepediatricvulvar. Accessed March 3, 2014.
9. Focseneanu MA, Gupta M, Squires KC, Bayliss SJ, Berk D, Merritt DF. The course of lichen sclerosus diagnosed prior to puberty. J Pediatr Adolesc Gynecol. 2013;26:153–155.
10. Bercaw-Pratt JL, Boardman LA, Simms-Cendan JS. Clinical recommendation: pediatric lichen sclerosus. NASPAG Clinical Recommendation. Pediatric and Adolescent Gynecology. 2014;27:111–116.
11. Muram D. Treatment of prepubertal girls with labial adhesions. J Pediatr Adolesc Gynecol. 1999;12:67–70.
12. Vunda A, Vandertuin L, Gervaix A. Urethral prolapse: an overlooked diagnosis of urogenital bleeding in pre-menarcheal girls. J Pediatr. 2011;158:682–683.
13. Deitch HR, Huppert J, Adams Hillard PJ. Unusual vulvar ulcerations in young adolescent females. J Pediatr Adolesc Gynecol. 2004;17:13–16.
14. Huppert JS, Berber MA, Beitch HR, Mortensen JE, Allen Staat M, Adams Hillard PJ. Vulvar ulcers in young females: a manifestation of aphthosis. J Pediatr Adolesc Gynecol. 2006;29:195–204.
15. Rosman IS, Berk DR, Bayliss SJ, White AJ, Merritt DF. Acute genital ulcers in nonsexually active young girls: case series, review of literature, and evaluation and management recommendations. Pediatr Dermatol. 2012;29:147–153.
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