Common Vulvar Disorders in Children

CONTRIBUTED BYNORTH AMERICAN SOCIETY FORPEDIATRIC AND ADOLESCENT GYNECOLOGYADOLESCENTGYNECOLOGYCommon Vulvar Disordersin ChildrenEllen S. Rome, MD, MPHAny vulvovaginal symptoms in childrenusually raise the spectre ofsexual abuse or disease. However,most conditions are benign andeasily treated, and provide a“teachable moment” that demandsall due respect and effort.Vulvar disorders are fairly commonin children, and require a specialapproach to evaluation and managementthat combines patiencewith an open manner that inspires trust inboth patients and parents. This articledescribes common vulvar disorders (eg,vulvovaginitis, lichen sclerosis, “bubblebath”vaginitis). Sexual abuse⎯a subspecialtyin itself⎯is not addressed here.VULVOVAGINAL EXAMINATION INCHILDRENVulvovaginal inspection should be a routinepart of the pediatric well-child visit,but is often omitted. The prepubertal genitalexamination should be viewed as anopportunity to educate the girl and herparents about issues such as hygiene, preventivecare, correct anatomical terms forthe genitalia, protection of privacy, and“stranger danger.”Before providing gynecologic services forchildren, the clinician must create anappropriate environment. The waiting areaand office should be “child-proofed”⎯eg, electric outlets covered, no scalpels orEllen S. Rome, MD, MPH, is Head, Section of AdolescentMedicine, Cleveland Clinic, Cleveland, OH.scissors within reach. Toys, games, andbooks should be available so that the childcan relax safely.HistoryWhen the genital examinationoccurs in the context of a problem,the parents may have fearsabout sexual abuse, cancer,potential infertility, or otherhidden anxieties. The clinicianshould ask the child directly ifshe has any concerns about herbody or her health; if the childlooks to the parent or caregiverfor guidance, questions can bedirected to the parent. The childshould be allowed to describeany symptoms. Specific questionsshould include symptomssuch as pain, bleeding, itching,odor, and discharge; duration ofsymptoms; past problems; and use ofbubble bath or perfumed body washes.If sexual abuse is a concern, the childshould be asked open-ended, nonjudgmentalquestions. Good eye contactshould be maintained with the child toABOUT NASPAGThe North American Society for Pediatricand Adolescent Gynecology (NASPAG) isa nonprofit organization dedicated to educatinghealth care professionals in pediatricand adolescent gynecology.For information, contact NASPAG:www.naspag.orgNASPAG1209 Montgomery HighwayBirmingham, AL 35216-2809FOCUSPOINTAllow the childto describe hersymptoms, and askquestions aboutpain, bleeding,itching, odor,and discharge.The Female Patient | VOL 33 JANUARY 2008 51

Romethan in asymptomatic girls. 6 In a study ofgirls with vulvovaginitis aged 2 to 12 years,pathogenic bacteria were isolated in 36%of cases, and group A beta hemolytic streptococcuswas found in 59%. 7 Pathogenicbacteria (including Streptococcus pyogenes,Haemophilus influenzae, Staphylococcusaureus, Moraxella catarrhalis, Streptococcuspneumoniae, Neisseria meningitides,Shigella, and Yersinia entercolitica) correlatedwith the presence of leukocytes invaginal secretions with a sensitivity of83% and a specificity of 59%. 1,7 Fewerpatients are presenting with H influenzaevaginitis today due to universal vaccination.Vaginal colonization with Escherichiacoli has been found in 36% of girls presentingwith vaginitis and in 23% of asymptomaticgirls 8 ; 90% of girls younger thanage 3 had vaginal E coli, compared with15% of asymptomatic girls aged 3 to 10years. 9 One case has been reported involvinga prepubertal girl with vulvovaginitiscaused by antibiotic-resistant Shigellaflexneri. 10 Cultures should be obtained forbloody discharge using a nasopharyngealswab moistened with nonbacteriostaticsaline solution. 10,11 A positive finding mayrepresent colonization rather than trueinfection: the latter is more likely when oneorganism predominates. Noninfectiouscauses are more common in prepubertalgirls, in whom infections are typicallyassociated with more severe inflammationand vaginal discharge. 12 Treatment usuallyconsists of sitz baths and removal of offendingagents such as bubble bath, ammonia,or bleach in the tub.Foreign bodies are the most commoncause of vaginal bleeding in children. Vulvovaginitis,lichen sclerosis, precociouspuberty, urethral prolapse, hemangioma,and tumor are other possibilities. In thepresence of bleeding, the history shouldinclude trauma, hematuria, rectal bleeding,symptoms of vaginitis, pubertal development,and potential abuse. In a childyounger than age 7, bleeding with acceleratedlinear growth may indicate precociouspuberty. Patients with thrombocytopeniamay have vaginal bleeding, as well as petechiae,easy bruising, or epistaxis. 3OTHER VULVOVAGINAL DISORDERSIN CHILDRENLichen Sclerosis et AtrophicusAssociated with hypoestrogenism, lichensclerosis can occur in prepubertal childrenand postmenopausal women. Girls maypresent with itching, vaginal or perinealbleeding, soreness, dysuria, and (rarely)constipation. Classic findings include an“hourglass” or “figure-eight” demarcationof hypopigmented skin from above the clitoristo the anus and scattered telangiectasia,with or without perineal fissuring.Treatment consists of midpotency or strongertopical corticosteroids, using the loweststeroid dosage that provides relief. Midpotencyoptions include hydrocortisone valerate,0.2%, while fluocinonide, 0.05%, is ahigher-potency choice. For rarecases requiring a super-potent steroid,clobetasol propionate, 0.05%,or halobetasol propionate, 0.05%,may be used. Creams tend to stingmore than ointments, and emollientssuch as diaper ointmentsand petroleum jelly can be appliedas well. The steroid cream is usuallyapplied sparingly twice dailyfor 2 weeks, then once daily for 2 to4 weeks, and then every other dayfor 2 weeks. Chronic use of topicalsteroids can lead to atrophy, telangiectasias,striae, hypopigmentation,and superinfection (fungal orviral), so the clinician must educatethe parents and closely followthese patients.Atopic DermatitisSome children may be overly sensitive tospecific diaper brands, diaper creams, orwipes. They commonly present with abrightly erythematous perineum, butwithout the satellite lesions seen withCandida. Usually, a simple brand changewill suffice, but in extreme cases, the parentcan be advised to use water ratherthan commercially available wipes. Atopicdermatitis tends to spare the “diaperarea,” aiding in diagnosis. Superinfectionwith S aureus or Streptococcus sp mayFOCUSPOINTLichen sclerosiscan occur in prepubertalchildren and istreated with topicalcorticosteroids, butthe patient must befollowed closely.The Female Patient | VOL 33 JANUARY 2008 53

Romeoccur if the child scratches, but S aureusinfections rarely involve the vagina. 13 Useof emollients, hypoallergenic soaps, andpossibly 1% hydrocortisone cream mayhelp relieve symptoms.PsoriasisChildren are more likely than adults tohave psoriasis in the vulvar area. It maypresent as an itchy, well demarcated, nonscaly,brightly erythematous, symmetricalplaque involving the vulva, perineum, andgluteal folds. Nail pitting, posterior auricularerythema, or rash with scaling in otherareas may occur as well. Treatment involveslow-potency or midpotency topical corticosteroids,using the lowest effective dosage.Concurrent infection should beexcluded. Low-dose topical steroids maybe needed for maintenance therapy.Systemic DiseasesInfectious mononucleosis and other systemicillnesses may present with vulvarfindings, and vulvar ulcers can mimicherpes simplex virus. 13,14 Children withKawasaki syndrome or Stephens-Johnsonsyndrome may have desquamation ofthe perineum. Crohn’s disease tends toaffect the perianal area. Behcet diseasemay present first as vulvovaginitis; oralapthous ulcers may be followed by genitalulcers, with ocular symptoms in the teenageyears. 13,15,16 The parent or child mayfear a sexually transmitted infection inthese cases. Acrodermatitis enteropathicamay occur with zinc deficiency, presentingwith lateral cracking of the lips/mouthand an eroded, bilateral vulvar rash with awell demarcated edge. 17TumorsAlthough rare, genital tumors should beconsidered in any child presenting withchronic genital ulceration, nontraumaticswelling of the external genitalia, a vaginalmass, foul-smelling or bloody discharge,and no evidence of a foreign body,virilization, or precocious puberty. Botyroidsarcoma of the vagina has a peakincidence at age 2, and 90% of cases occurin children younger than age 5. This tumoris often found in the lower vagina in youngchildren, and in the upper vagina/cervixin children older than age 10. 13 Originatingin the lamina propria from undifferentiatedmesenchyme, this tumor spreadsrapidly below the vaginal epithelium toinfiltrate the vaginal wall. It may appearas a cluster of “grapes,” bulging outwardwith polypoid growths containing edematousstroma and dilated blood vessels.Treatment consists of chemotherapy withor without radiation therapy and conservativesurgery, with a survival rate of 80%to 90% in girls with localized pelvic rhabdomyosarcoma.3,18,19 Other rare findingsinclude vulvar malignant melanoma,Langerhans cell histocytosis,congenital perineal lipoma,condyloma acuminatum, andprolapsed ectopic ureterocoele.Labial AdhesionsLabial adhesions are relativelycommon in babies and children,sometimes with secondary dermatitisfrom pooling urine or vaginalsecretions due to adhesionsabove the urethra that extend pastthe vagina. Urinary tract infectionsmay rarely occur with adhesions.Labial adhesions usuallybother the parent more than thechild, but are easily treatable with topicalestrogen cream applied directly to the areadaily in very small amounts. Althoughadhesions often recur, asymptomaticadhesions do not require treatment.CONCLUSIONVulvar dermatologic problems in childrencan provide an excellent opportunity forreassurance, education, and discussion,enhancing communication for parent,child, and clinician. With simple officetools and proper positioning, foreign bodies,infections, and systemic disease arereadily identified and managed. In childrenwith vulvovaginitis, simple rules fordiagnosis and treatment apply: the presenceof pus warrants culturing; abscessesshould be incised and drained; anysystemic and/or topical treatment optionFOCUSPOINTChildren are morelikely than adultsto have psoriasisin the vulvar area.Tumors are rare, butlabial adhesionsare common.The Female Patient | VOL 33 JANUARY 2008 57

ADOLESCENTGYNECOLOGYCommon Vulvar Disorders in Childrenshould be considered, as appropriate; andsitz baths should be recommended to helprelieve symptoms.REFERENCES1. Emans SJ, Laufer MR, Goldstein DP, eds. Pediatricand Adolescent Gynecology, 5th ed. Philadelphia,PA: Lippincott Williams & Wilkins;2005:1-15, 83-119.2. Pokorny SF, Stormer J. Atraumatic removal ofsecretions from the prepubertal vagina. Am JObstet Gynecol. 1987;156(3):581-582.3. Emans SJ, Goldstein DP. The gynecologic examinationof the prepubertal child with vulvovaginitis:use of the knee-chest position. Pediatrics.1980;65(4):758-760.4. Heller RH, Joseph JM, David HJ. Vulvovaginitisin the premenarchal child. J Pediatr. 1969;74(3):370-377.5. Piippo S, Lenko H, Vuento R. Vulvar symptomsin paediatric and adolescent patients. Acta Paediatr.2000;89(4):431-435.6. Stricker T, Navratil F, Sennhauser FH. Vulvovaginitisin prepubertal girls. Arch Dis Child.2003;88(4):324-326.7. Emans SJ, Woods ER, Allred EN, Grace E.Hymenal findings in adolescent women: impactof tampon use and consensual sexual activity. JPediatr. 1994;125(1):153-160.8. Alexander ER. Misidentification of sexuallytransmitted organisms in children: medicolegalimplications. Pediatr Infec Dis J. 1988;7(1):1-2.9. Whittington WL, Rice RJ, Biddle JW, Knapp JS.Incorrect identification of Neisseria gonorrhoeaefrom infants and children. Pediatr Infect Dis J.1988;7(1):3-10.10. Johnson RE, Newhall WJ, Papp JR, Knapp JS,Black CM, Gift TL, et al. Screening tests to detectChlamydia trachomatis and Neisseria gonorrhoeaeinfections⎯2002. MMWR Recomm Rep.2002;51(RR-15):1-38.11. Sexually transmitted diseases treatment guidelines2002. Centers for Disease Control and Prevention.MMWR Recomm Rep. 2002;51(RR-6):1-78.12. Paradise JE, Campos JM, Friedman HM, FrishmuthG. Vulvovaginitis in premenarchal girls:clinical features and diagnostic evaluation.Pediatrics. 1982;70(2):193-198.13. Mroueh J, Muram D. Common problems in pediatricgynecology: new developments. Curr OpinObstet Gynecol. 1999;11(5):463-446.14. Sisson BA, Glick L. Genital ulceration as a presentingmanifestation of infectious mononucleosis.J Pediatr Adolesc Gynecol. 1998;11(4):185-187.15. Uziel Y, Brik R, Padeh S, et al. Juvenile Behcet’sdisease in Israel. The Pediatric RheumatologyStudy Group of Israel. Clin Exp Rheumatol.1998;16(4):502-505.16. Eldem B, Onur C, Ozen S. Clinical features ofpediatric Behçet’s disease. J Pediatr OphthalmolStrabismus. 1998;35(3):159-161.17. Fischer GO. Vulval disease in pre-pubertal girls.Australas J Dermatol. 2001;42(4):225-234.18. Copeland LJ, Gershenson DM, Saul PB, Sneige N,Stringer CA, Edwards CL. Sarcoma botyroides ofthe female genital tract. Obstet Gynecol. 1985;66(2);262-266.19. Arndt CA, Donaldson SS, Anderson RJ, et al.What constitutes optimal therapy for patientswith rhabomyosarcoma of the female genitaltract? Cancer. 2001;91(12): 2454-2468.Have you seen an interesting casein your practice recently?We invite you to write it up andsubmit it to our Case Reportdepartment. Please include anyrelevant images, and email the caseto carol.saunders@QHC.com.Please see our author guidelinesat www.femalepatient.comfor further information.58 The Female Patient | VOL 33 JANUARY 2008