Vulvovaginal complaints in the prepubertal child INTRODUCTION Vulvovaginal complaints in prepubertal children may be the result of infection, congenital

l abnormalities, trauma, or dermatologic conditions. Vaginitis may have a nonspecific etiology or may be due to known pathogens. It is the commonest gynecological problem in prepubertal girls [1]. Symptoms include vaginal discharge, erythema, soreness, pruritus, dysuria, and bleeding. Occasionally, urinary tract or bowel symptoms may be interpreted by a child as vulvar or vaginal complaints.This topic will discuss common vulvovaginal disorders in this population. The gynecological history and physical examination and techniques for obtaining cultures in these children are reviewed separately. (See "Gynecologic examination of the newborn and child".) NONSPECIFIC VULVOVAGINITIS Nonspecific vulvovaginitis is responsible for 25 to 75 percent of vulvovaginitis in prepubertal girls [2]. There are a number of potential factors in children that increase their risk of vulvovaginitis: lack of labial development, unestrogenized thin mucosa, more alkaline pH (pH 7) than postmenarchal girls/women, poor hygiene, bubble baths, shampoos, deodorant soaps, obesity, foreign bodies, and choice of clothing (leotards, tights, and blue jeans). Chronic masturbatory activity can lead to vulvar irritation with occasional thickening of the clitoral hood. Sexual abuse is another etiology of nonspecific vulvovaginitis. Some girls with nonspecific vulvovaginitis seem to experience recurrences at the time of upper respiratory infections. Once a specific etiology (listed below) for the symptoms has been excluded, the following recommendation for parents may be of help: Avoid sleeper pajamas. Nightgowns allow air to circulate. Cotton underpants. Double-rinse underwear after washing to avoid residual irritants. Do not use fabric softeners for underwear and swimsuits. Avoid tights, leotards, and leggings. Skirts and loose-fitting pants allow air to circulate. Daily warm bathing is helpful as follows: Allow the child to soak in clean water (no soap) for 10 to 15 minutes. Adding vinegar or baking soda to the water has not been specifically studied but from our experience is not more efficacious than clean water alone. Use soap to wash regions other than the genital area just before taking the child out of the tub. Limit use of any soap on genital areas. Rinse the genital area well and gently pat dry. A hair dryer on the cool setting may be helpful to assist with drying the genital region. Do not use bubble baths or perfumed soaps. If the vulvar area is tender or swollen, cool compresses may relieve the discomfort. Wet wipes can be used instead of toilet paper for wiping. Emollients may help protect skin. Review hygiene with the child. Emphasize wiping front-to-back after bowel movements. If she has trouble remembering, try having her sit backwards on the toilet (facing the toilet). Children younger than five should be supervised or assisted in toilet hygiene. Avoid letting children sit in wet swimsuits for long periods of time after swimming. These techniques usually result in resolution of nonspecific mucoid discharge and/or odor within two to three weeks. If symptoms persist or recur, the possibility of a foreign body or specific infection should be considered. (See 'Foreign body' below and 'Specific infections' below.) Although not indicated for most nonspecific vaginal discharge, which resolves with the measures above, antibiotic therapy may hasten the resolution of a purulent vaginal discharge (despite a negative culture for specific infections, such as group A streptococcus) that does not respond to hygiene measures and for which other diagnoses have been excluded. Alternative regimens include: a 10-day course of amoxicillin or amoxicillin-clavulanate; topical metronidazole; or topical clindamycin. Occasionally, a short course of estrogen-containing cream (eg, Premarin) can thicken the vaginal mucosa and make it more resistant to recurrent nonspecific infections. Again, making sure to exclude the conditions described below is important. SPECIFIC INFECTIONS Pinworm Pinworms can cause vulvar symptoms, such as itching. Children with recurrent episodes of vulvar and/or perianal itching, especially at night, should be examined for pinworms and treated empirically, if indicated. (See "Enterobiasis and trichuriasis".) Respiratory and enteric flora Children may pass respiratory flora from the nose and oral pharynx to the vulvar area. Respiratory pathogens include: Streptococcus pyogenes (group A streptococcus), S. aureus, H. influenzae, S. pneumoniae, N. meningitidis, and B. catarrhalis. Likewise, enteric pathogens, such as Shigella and Yersinia, may cause vaginitis. Streptococcus pyogenes is the most commonly identified pathogen in prepubertal girls. Depending upon the definition of vulvovaginitis (vulvitis versus vaginitis only), the prior use of antibiotics, the type of culture obtained (vaginal versus introital), and the clinic setting, typically about 20 percent of girls with vulvovaginitis have S. pyogenes [3-7]. Vaginal cultures for respiratory and enteric flora should be obtained if vulvovaginitis persists or is purulent. Antimicrobial therapy for vulvovaginitis caused by respiratory or enteric bacteria depends upon the organism. Group A streptococcus is treated with penicillin or a similar antibiotic. S. aureus and H. influenzae may resolve with hygiene measures but should be treated if vulvovaginitis is persistent or purulent. Candida Colonization with Candida occurs in 3 to 4 percent of prepubertal girls. Candidal infection is most common in children who have had recent antibiotic therapy, are immunosuppressed, or who wear diapers. It is uncommon in normal prepubertal girls, in whom it is frequently overdiagnosed and wrongly assumed to be the etiology for patients' symptoms. (See "Overview of diaper dermatitis in infants and children", section on 'Candidal dermatitis' and "Overview of diaper dermatitis in infants and children", section on 'Antifungal agents' and "Candida vulvovaginitis".)

Gardnerella vaginalis Gardnerella vaginalis is generally not associated with a vaginal discharge in prepubertal girls. A possible relationship with sexual abuse has been disputed. In one study, Gardnerella was identified in 14.6 percent of sexually abused girls compared to only 4.2 percent of control girls [8]. In another report, however, the incidence of Gardnerella was equivalent in sexually abused girls and the female children of friends of the author (controls) [9]. Sexually transmitted diseases Sexually transmitted diseases in children typically result from sexual abuse. Pathogens include Neisseria gonorrhoeae, Chlamydia trachomatis, human papillomavirus, Treponema pallidum, and herpes simplex virus. (See "Evaluation of sexual abuse in children and adolescents", section on 'STI testing'.) Neisseria gonorrhoeae Neisseria gonorrhoeae usually presents with a green or mucoid vaginal discharge; the infection is rarely asymptomatic [10] but occasionally may be found without symptoms in girls being evaluated for sexual abuse. The diagnosis is made by culturing girls with an evident discharge. (See "Gynecologic examination of the newborn and child", section on 'How to obtain cultures from children' and "Diagnosis of gonococcal infections" and "Evaluation of sexual abuse in children and adolescents", section on 'Prepubertal victims'.) Chlamydia trachomatis Chlamydia trachomatis is primarily transmitted to newborns via exposure to an infected mother's genital flora during vaginal birth. C. trachomatis is also associated with vaginitis and sexual abuse, although patients may be asymptomatic. (See "Chlamydia trachomatis infections in the newborn", section on 'Epidemiology and transmission' and "Evaluation of sexual abuse in children and adolescents", section on 'Prepubertal victims'.) Trichomonas vaginalis Trichomonas vaginalis can occur in newborns, but is rare in the prepubertal child. Thus, except for infants, sexual contact is likely if trichomonal organisms are identified (by an experienced technician or clinician) in vaginal secretions by wet mount examination or culture. (See "Evaluation of sexual abuse in children and adolescents", section on 'STIs'.) Condylomata acuminata Condylomata acuminata are skin-colored or pink lesions that may be warty or smooth flattened papules. They are caused by the human papillomavirus (HPV). In children younger than two to three years of age, these lesions are likely the result of maternal-child transmission during vaginal birth, but may be acquired by sexual or nonsexual transmission. It is not necessary for the mother to be symptomatic or to have a history of HPV for this transmission to occur. HPV testing of mothers does not exclude sexual abuse and therefore is not generally performed; if sexual abuse is of concern, evaluation is needed. (See "Evaluation of sexual abuse in children and adolescents", section on 'STIs'.) In older children, sexual transmission and evaluation for potential sexual abuse should be considered and if there is a concern, children should be interviewed and evaluated by appropriately experienced professionals. Auto- and hetero-inoculation and indirect transmission via fomites are other possibilities. (See "Evaluation of sexual abuse in children and adolescents", section on 'STIs'.)The diagnosis is usually made clinically without a biopsy. A biopsy can, however, confirm the presence of HPV and leads to a conclusive diagnosis. HPV DNA typing may help the health care provider formulate a follow-up surveillance plan (table 1). There are over 100 distinct HPV subtypes; approximately 40 types are specific for the anogenital epithelium and have varying potentials to cause malignant change, such as cervical or anal cancer (table 1). (See "Virology of human papillomavirus infections and the link to cancer" and "Cervical cancer screening tests: Techniques and test characteristics of cervical cytology and human papillomavirus testing".)Spontaneous resolution occurs within five years in more than 50 percent of patients [11]. Expectant management is a potential initial approach to asymptomatic pediatric condylomata; however, many families choose to have them treated. Treatment options have not been well studied and have included laser therapy (which requires anesthesia), trichloroacetic acetic (which is not well tolerated in this age group), and topical imiquimod cream (which needs careful monitoring to assure vulvar reactions are not severe). (See "Condylomata acuminata (anogenital warts) in children".) FOREIGN BODY Foreign bodies in children can cause acute and chronic recurrent vulvovaginitis. Chronic vaginal discharge, intermittent bleeding or spotting, and/or a foul smelling odor are the usual clinical manifestations. Toilet paper is the most common foreign body found in the vaginas of children; small toys, hair bands, and paper clips are also common. The foreign body can often be removed by a calgi swab (for toilet paper) or with irrigation with warmed fluid, after the introitus has been treated with a topical anesthetic agent, such as a small amount of Xylocaine jelly or EMLA (although it is not FDA approved for utilization on mucus membranes). Examination under sedation and/or anesthesia may be necessary for extraction of larger foreign bodies and those that cannot be removed with irrigation. POLYPS OR TUMORS A vaginal polyp or tumor may also present with chronic discharge. Sarcoma botryoides can involve the hymen, lower urethra, or anterior vaginal wall. The peak incidence is at age two to five years. The tumor is more likely to arise higher up along the anterior vaginal wall towards the cervix in older preadolescent girls. (See "Rhabdomyosarcoma and undifferentiated sarcoma in childhood and adolescence: Epidemiology, pathology, and molecular pathogenesis" and "Vaginal cancer", section on 'Sarcoma'.)Benign polyps can also occur involving the vagina and hymenal area but are rare.Treatment is surgical; the specific procedure depends upon the pathology. A simple polyp can be removed with surgical excision. A vaginal sarcoma requires biopsy for diagnosis and then chemotherapy and conservative surgery with the aim to treat the tumor while maintaining future sexual and reproductive function. (See "Vaginal cancer", section on 'Treatment'.) SYSTEMIC ILLNESS Measles, chickenpox, scarlet fever, Epstein-Barr virus (EBV, infectious mononucleosis) StevensJohnson syndrome, Crohn's disease, and Kawasaki disease have all been associated with vulvovaginal signs and symptoms, such as vesicles, discharge, fistulae, ulcers, and inflammation. Severe cases of Stevens-Johnson syndrome or graft-versus-host disease after bone marrow transplant may result in vaginal stenosis or obstruction [12].Persistent complaints of vaginal bleeding or discharge that are otherwise unexplained may be a manifestation of a serious underlying medical problem (eg, rhabdomyosarcoma or other tumor) [13]. Pelvic examination under anesthesia, vaginoscopy, and/or cystoscopy may be necessary if genital examination and noninvasive imaging fail to provide an explanation for such complaints. VULVAR ULCERS Nonsexually transmitted vulvar ulcers, sometimes called Lipschutz ulcers, "virginal ulcers", or "aphthous ulcers", are typically seen in girls age 10 to 15 years (picture 1) [14]. Clinical features may include one or more acutely painful ulcers (often >1 cm) with a purulent base and raised edges and often systemic symptoms (eg, fatigue, malaise, fever, headache).The etiology often cannot be determined, although viral infections such as influenza A, EBV, and CMV have been

associated with these painful lesions [14-19]. A careful sexual history should be taken and testing for STDs, such as herpes simplex, performed even though in most cases the ulcers occur before the onset of genital or oral sexual activity. A CBC, differential, and monospot test, as well as acute and convalescent serum, can be obtained to look for EBV.Although most girls can be treated at home, at times patients require admission for foley catheter drainage due to the inability to urinate, antibiotics (if the ulcers are superinfected), topical anesthetics, and pain management. Oral corticosteroids have also been prescribed for these ulcers, but controlled studies are lacking. These ulcers often heal completely in one to three weeks, but may recur. Persistent ulcers can occur in girls with Crohn's disease. Recurrent vulvar ulcers, especially if associated with evidence of systemic involvement and vasculitis (oral lesions, uveitis, arthritis) should suggest the possibility of Behcet's disease. URINARY TRACT PATHOLOGY Ectopic ureter An ectopic ureter can cause chronic vulvar irritation and wetness in girls. The ectopic ureter arises from the upper pole of a duplex collecting system, or from a dysplastic kidney. An ultrasound evaluation may suggest this diagnosis, but an intravenous pyelogram is a more sensitive test. Treatment is surgical. (See "Ectopic ureter".) Urethral prolapse In prepubertal girls, the distal end of the urethra can prolapse either partially or in a complete circumferential fashion. The tissue may be friable and become infected, but rarely becomes necrotic. Children with urethral prolapse present with bleeding, dysuria, and/or difficulty with urination.We suggest that prepubertal girls with symptomatic urethral prolapse be treated with topical estrogen therapy. The distal urethra is estrogen-sensitive and may respond to treatment with estrogen, as in postmenopausal women with this problem. Sitz baths twice daily may also be helpful. Topical estrogen cream is applied twice daily after the sitz bath for two weeks, then the urethra is reassessed and treatment is continued if the prolapse has not resolved and is still present. The prolapse will usually resolve within a few weeks of topical estrogen treatment but can sometimes take longer. If the distal urethra is necrotic, it may require surgery. If there is persistence of the prolapse, assessment for a urethral polyp may be indicated. (See "The pediatric physical examination: The perineum", section on 'Females'.) CUTANEOUS VULVAR DISEASE Prepubertal hypoestrogenic tissues are atrophic and more susceptible to infection and irritation. The majority of vulvar symptoms in children are due to local irritants, foreign bodies, or infection [19].Lichen sclerosus Lichen sclerosus is a dermatologic abnormality of unclear etiology. Clinical manifestation in girls include itching, discomfort in the vulvar area, bowel or bladder symptoms, discharge, or bleeding. The vulvar tissue displays a white onion skinlike lesion, typically in an hourglass configuration with the whitened skin circumscribing the vulvar and perianal areas (picture 2). There may also be punctate hemorrhages, which can occur anywhere on the affected skin. (See "Vulvar lichen sclerosus".)The diagnosis in children is made by visual inspection; a biopsy is rarely required, in contrast to the adult population. Lichen sclerosus can be associated with underlying malignancies in adults, but this does not appear to be true for children.Treatment consists of topical, high-potency corticosteroids (eg, clobetasol 0.05 percent ointment or halobetasol 0.05 percent ointment), applied twice daily for two weeks. The vulva is then reassessed to determine response and further treatment. High-potency steroids are usually needed for 6 to 12 weeks, but may need to continue longer. It may take weeks to months of treatment for the condition to resolve. Once the symptoms and visible signs have resolved, the topical steroid is tapered; if the steroids are discontinued abruptly, there can be a "rebound" effect. An additional course of steroids may be necessary if the disease recurs.Untreated, lichen sclerosus can result in long-term sexual dysfunction due to loss of the normal labia and the normal architecture of the vulva. Scarring of the clitoral hood may occur with entrapment of the underlying glands, resulting in pain upon sexual excitation and engorgement of the clitoris. This may necessitate a surgical procedure to try to correct the abnormality, but repetitive scarring of the clitoral hood may occur. If there is clitoral hood scarring, then a "clitoral entrapment syndrome" can occur. We have described a technique of clitoroplasty with reconfiguration of the clitoral hood and placement of Surgicel in order to try to avoid rescarring of the area [20].An excellent information handout for parents is available through the North American Society for Pediatric and Adolescent Gynecology website: http://www.naspag.org/index.php/pagepediatricvulvar. Hemangiomas Hemangiomas occur on the vulva, as on other body surfaces. Most hemangiomas will involute between two and five years of age and require no further intervention. Once involuted, the remaining skin and fatty tissue can be evaluated to determine whether the lesion is of significant size to necessitate a surgical resection.Hemangiomas of the vulvar and clitoral region rarely bleed. A complete evaluation, including vascular magnetic resonance imaging, can be helpful for determining the extent of internal involvement of a larger hemangioma. Co-management with a vascular surgeon is recommended for hemangiomas that require surgical intervention.The clinical features, evaluation, and management of hemangiomas are discussed in detail separately. (See "Epidemiology; pathogenesis; clinical features; and complications of infantile hemangiomas" and "Evaluation and diagnosis of infantile hemangiomas" and "Management of infantile hemangiomas".) Labial adhesions The etiology and frequency of labial adhesions (also called labial agglutination and synechia vulvae) are unknown. They can be partial, involving only the upper or lower labia, or complete. A small pinhole orifice may be present that functions as a means for urine to exit from behind the fused labia.Labial adhesions may be asymptomatic or cause a pulling sensation, difficulty with urination, recurrent urinary tract infections, or recurrent vaginal infections. No treatment is necessary if the adhesions are asymptomatic, involve only a small portion of the labia, and are not affecting the urine stream. The adhesions may resolve when estrogen production increases at puberty.Labial adhesions should be treated if they affect urination by diverting a normal stream of urine. Treatment consists of topical estrogen cream (eg, Premarin cream) applied twice daily at the point of midline fusion where there is a thin white line [21]. With the application of the estrogen cream, great care should be taken so as not to traumatically tear the adhesion. Therapy is continued until the labial adhesions resolve. Breast bud formation is a possible side effect, which will resolve after the cessation of the topical estrogen cream. This complication is less likely if the cream is applied sparingly and directly to the adhesion.The response of labial adhesions to topical estrogen therapy was illustrated in a retrospective review of 109 girls (three months to 10 years of age) [22]. Topical estrogen therapy was successful in 79 percent of patients after a mean duration of four months. Minimal breast development occurred in six girls (5 percent), and vaginal bleeding in one (<1 percent). In our experience, labial adhesions resolve in nearly all girls treated with correct technique for two to six weeks. Successful separation

should be followed by attention to hygiene, daily baths, and the application of a bland ointment, such as A&D Ointment or white petroleum jelly, for 6 to 12 monthsFailure of medical therapy tends to occur with thick adhesions (3 to 4 mm in width) with no thin translucent raphe [23]. The most common reason for medical failure is placement of the cream in the wrong location or placement of too small an amount of cream.Surgical intervention for labial adhesions is reserved for rare patients with complete obstruction of urine flow in whom estrogen cream cannot be applied for psychosocial reasons or has been unsuccessful after an adequate trial (as described above). With these indications, the authors have performed only one surgical separation in the past 10 years. When necessary, surgical separation is performed with sedation and/or anesthesia and followed by topical estrogen cream for one to two weeks and then application of a bland emollient (eg, white petroleum jelly) for 6 to 12 months. Trauma Vulvar trauma can cause significant bleeding because the area is highly vascular and children do not have mature labia with fat pads, which protect the vulvar area of adults. Girls who sustain straddle or inline skating injuries may require surgical intervention [24]. (See "Straddle injuries".)A careful history should be obtained and physical examination performed. The history must correlate with the physical findings to confirm nonsexual trauma. If a child has a straddle injury, she will most likely have an injury to the anterior area of the vulva, including the mons, clitoral hood, and anterior aspect of the labia (picture 3A-E). An injury to the posterior fourchette and hymenal area suggest possible sexual abuse. As an example, a laceration to the lower half of the hymenal area (using a clock, the location would be three o'clock to nine o'clock) is consistent with a penetrating injury and must be further explored (picture 4A-B) [25]. Normal hymenal variants must be understood to be able to identify abnormal hymenal findings due to trauma [26-28]. (See "Gynecologic examination of the newborn and child", section on 'Evaluation of the hymen'.)Assessment of ability to urinate is essential as a large, obstructive hematoma can result from blunt trauma to the vulva. Ice to the area, bladder drainage, and pain medications are appropriate support measures for a girl with a large hematoma. Most vulvar hematomas will resolve spontaneously. Surgical drainage is not usually needed because the hematoma is usually the result of a compromise to small vessels that are difficult to identify if the hematoma is opened. In addition, surgical disruption of the skin facilitates introduction of bacteria and an abscess can result. (See "Evaluation and management of lower genital tract trauma in women".) Other Less common vulvar lesions in this age group include neurofibromas, leiomyomas or leiomyosarcomas, granular cell tumors, and nodular fasciitis [29]. INFORMATION FOR PATIENTS UpToDate offers two types of patient education materials, The Basics and Beyond the Basics. The Basics patient education pieces are written in plain language, at the 5 th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon. Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on patient info and the keyword(s) of interest.) Basics topic (see "Patient information: Lichen sclerosus (The Basics)") SUMMARY AND RECOMMENDATIONS Vulvovaginal complaints in prepubertal children may be the result of infection, congenital abnormalities, trauma, or dermatologic conditions. Vaginitis may have a nonspecific etiology; may be due to known pathogens, such as pinworm, candida, or respiratory or oral flora; or may be related to a foreign body. Sexually transmitted diseases in prepubertal children typically result from sexual abuse. A vaginal polyp or tumor may also present with chronic discharge and some systemic illnesses also affect the vagina. Prepubertal hypoestrogenic tissues are atrophic and thus more susceptible to local irritants, foreign bodies, and infection. The most common respiratory pathogen causing vaginitis is Streptococcus pyogenes (group A streptococcus); this infection should be promptly treated with penicillin or similar antibiotic. Mucoid discharge associated with organisms such as S. aureus or H. influenzae may resolve with hygiene, and thus antibiotics are usually prescribed for persistent symptoms or purulent discharge. Culture-negative but persistent vaginitis may resolve with treatments, such as a 10 day course of amoxicillin or amoxicillin-clavulanate, topical metronidazole, or topical clindamycin. (See 'Specific infections' above.) We suggest removing small foreign bodies with a swab or with irrigation with warmed fluid, after the introitus has been treated with a small amount of topical anesthetic agent, in some children with larger foreign bodies or those that are not removed by irrigation, conscious sedation or examination under anesthesia may be necessary (Grade 2C). (See 'Foreign body' above.) Tumors and polyps are surgically removed. The scope of the procedure depends upon the specific pathology. (See 'Polyps or tumors' above.) We suggest that children with urethral prolapse receive topical estrogen cream applied twice daily for two weeks (Grade 2C). Sitz baths may also be helpful during estrogen treatment. The patient should then be reassessed to see if the urethral prolapse has resolved. In some patients with necrotic tissue, surgical intervention may be required. (See 'Urethral prolapse' above.) Lichen sclerosus should be treated to prevent progression. We suggest administration of topical corticosteroids (Grade 2B). (See 'Lichen sclerosus' above.) We suggest treatment of labial adhesions with topical estrogen cream and gentle traction with care taken so as not to traumatically tear the adhesion (Grade 2B). (See 'Labial adhesions' above.)