Review Fused Labia: a Paediatric Approach Authors Lina Michala / Sarah M Creighton Key Content: • Labial Fusion Is Common Among Prepubertal Girls

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The Obstetrician & Gynaecologist 10.1576/toag.11.4.261.27529 http://onlinetog.org 2009;11:261–264 Review

Review Fused labia: a paediatric approach Authors Lina Michala / Sarah M Creighton Key content: • Labial fusion is common among prepubertal girls. • In the majority of cases it is asymptomatic and should not be treated. • In symptomatic children, initial treatment with local estrogen is advised. Surgical treatment should be avoided if at all possible and considered only as a last resort. • Recurrence rates are high. The condition resolves with the onset of puberty. Learning objectives: • To learn how to diagnose labial adhesions. • To be able to reassure parents that the condition is self-limiting and benign. • To know the treatment modalities available for symptomatic children. Ethical issues: • There is no evidence that child sexual abuse causes labial adhesions, however, if the presentation is atypical or there are other concerns, advice should be sought from the lead professional for child protection in the organisation or Trust.

Keywords adhesions / labial agglutination / lichen sclerosus / local estrogen treatment / vulvovaginitis

Please cite this article as: Michala L, Creighton SM. Fused labia: a paediatric approach. The Obstetrician & Gynaecologist 2009;11:261–264. Author details

Lina Michala MRCOG Sarah M Creighton MD FRCOG Consultant Gynaecologist Consultant Gynaecologist Department of Obstetrics and Gynaecology, University College Hospital, London Alexandras Hospital, Athens, Greece WC1E 6AU, UK Email: [email protected] (corresponding author)

Review 2009;11:261–264 The Obstetrician & Gynaecologist

Introduction inflammation.Vulvovaginitis, diarrhoea and Fused labia (Figure 1),also known as labial scratching of the vulva can lead to local trauma of agglutination or labial adhesions,is a common the skin with superficial denudement of the labia. condition of the prepubertal girl and,along with During the healing process, the edges of the labia vulvovaginitis,it constitutes one of the most common fuse due to their close proximity and a thin complaints presenting to paediatric gynaecologists. membrane forms, starting at the level of the posterior fourchette and extending anteriorly, Epidemiology obliterating part or all of the vestibule. The prevalence of fused labia ranges from In some children labial fusion is associated with 1 1.8–3.3%, depending on the age of the children nappy rash and may resolve once the child is toilet assessed, but accurate estimates are difficult as a trained. Labial adhesions have been associated proportion of children are asymptomatic and the with lichen sclerosus, in which adhesions are condition may remain undetected. This was characteristically dense and difficult to treat and 2 demonstrated in a study that aimed to set can persist into puberty and adulthood.3 Lichen standards of normal genital anatomy among sclerosus can lead to the formation of adhesions prepubertal children, where labial adhesions were that involve the clitoral prepuce and can cause found in 38.9% of girls. This included adhesions as clitoral entrapment and neurovascular bundle small as 2 mm in length that were visible after compression with severe pain. colposcopic magnification. Unusual infections such as severe herpes and graft Fused labia occur when estrogen levels are low and versus host disease have also been associated with are therefore extremely rare in the first 3 months of labial adhesions and can occur in older patients.4,5 life when maternal estrogens are still abundant in the infant’s circulation. In a study of the genitalia of In the past there has been debate as to whether 1 9070 newborns, none had labial fusion at birth. labial adhesions can be caused by sexual abuse. The condition is self-limiting and corrects itself A few case reports from more than 20 years ago naturally in early puberty when the endogenous suggested this,6 but it has not been demonstrated in production of estrogen starts. The peak incidence is any more recent studies. However, both adhesions between 2 and 4 years of age. This also corresponds and child sexual abuse are common and may co- with the peak incidence of vulvovaginitis, which exist, but it is not now thought that the two are may coexist with labial fusion. linked. Predisposing factors Clinical presentation The skin of the vulva prior to puberty is fragile and (See Box 1.) Labial adhesions are, in the vast thin and, as a result, is prone to infection and majority of cases, asymptomatic. Rarely, however, they can be associated with urinary symptoms, Figure 1 vulvovaginitis and pain. Labial adhesions Urinary symptoms occur as urine accumulates behind the adhesions, leading to an altered urinary stream or dribbling after micturition. Stasis can also predispose to asymptomatic bacteriuria7 or overt urinary tract infection. Urinary tract obstruction is rare,8 although cases of hydronephrosis that resolve following lysis of labial adhesions have been described in the literature.9 Occasionally, an altered urinary stream interferes with toilet training or leads to soiling during micturition.

Vulvovaginitis can be perpetuated by labial adhesions as vaginal discharge and debris accumulate behind the adhesions.

Pain is present in approximately one-quarter of children with labial adhesions who are referred to a doctor.8 This is manifested by complaints or avoidance of activities that involve straddle positions, pain during walking or pulling at nappies or underwear.

The Obstetrician & Gynaecologist 2009;11:261–264 Review

Box 1 Usually asymptomatic Management Clinical presentation of labial (See Figure 2). Diagnosis of the condition is made by Can be associated with: adhesions inspection of the vulva alone. Unlike in cases of • urinary symptoms, e.g. altered urinary stream or dribbling ambiguous genitalia, the clitoris and labia majora are after micturition normal and a thin avascular membrane is clearly • vulvovaginitis • rarely, pain manifested by complaints or avoidance of visible between the labia minora. The adhesions activities that involve straddle positions, pain during usually involve the posterior part of the vestibule, walking or pulling at nappies or underwear starting from the posterior fourchette and extending anteriorly towards the level of the urethra. Figure 2 Social pressure and anxiety about the normality of Treatment algorithm the genitalia and the presence of the vagina are usually the driving forces behind bringing the child to the doctor. In these cases reassurance that the condition is benign and self-limiting is all that is needed. In asymptomatic cases, treatment is not required. The labial adhesions will resolve naturally when endogenous estrogen production starts, usually before the age of 9 years. There are no associated sequelae to the girl’s future reproductive and sexual life and the condition needs to be clearly differentiated in the parents’minds from other conditions that can lead to ambiguity or abnormality of the genitalia.

Irrespective of whether labial fusion is symptomatic or not, advice on meticulous hygiene of the vulva is recommended, as for vulvovaginitis. Regular cleaning is important, especially after defecation and urination.Avoiding local irritants, such as bubble bath, and choosing cotton rather than Topical treatment synthetic underwear is also advisable.Where In cases where labial adhesions are symptomatic vulvovaginitis coexists, a swab should be taken and cause pain or urinary symptoms, treatment from the introitus and, if a pathogen is isolated, will become necessary.A conservative approach is appropriate antibiotic treatment should be usually suggested, consisting of local application instituted. of estrogen cream on the labia for no longer than 6 weeks.A pea-sized amount of estrogen cream Although labial adhesions are not thought to be (such as estriol 0.1%) is applied using a cotton bud caused by sexual abuse, both may occur at the same along the line of fusion. Parents need to be warned time.A clinician who has received appropriate child against longer or excessive use of estrogen cream, as protection training should be responsible for a proportion of the hormone is absorbed into the assessment of any genital symptoms in a child. circulation, leading to systemic effects such as If the presentation is atypical or there are other breast budding, uterine bleeding and local skin worrying features during the consultation, advice discoloration or erythema.All of these effects must be sought from the lead professional for child regress promptly after discontinuation of the protection with the organisation or Trust where the medication.10 Success rates range between child is being seen. It must be remembered that the 50–100%,11,12 depending mostly on the density of majority of children who are abused do not have the adhesions, the duration of estrogen application any physical complaints related to trauma or and whether or not there has been prior treatment infection. and recurrence.

If the adhesions are associated with a skin Based on observations on male children with condition, such as lichen sclerosus, this is usually phimosis, where local application of steroids can symptomatic and will need treatment. Lichen lead to resolution of the problem,13 betamethasone sclerosus leads to a typical appearance of the cream has been used in girls with labial adhesions. vulva, with parchment-like areas and foci of In a retrospective review of 19 girls,14 the majority inflammation that look like ‘blood blisters’.3 Initial of whom had previously undergone treatment of treatment will usually include local potent steroid labial adhesions with either estrogen cream or cream application. Histological confirmation may surgical lysis, a course of local betamethasone be necessary if resolution does not occur or the cream 0.05% for 4–6 weeks was shown to be appearances are atypical. successful in 68% of cases. Nevertheless, in almost

Review 2009;11:261–264 The Obstetrician & Gynaecologist

one-quarter of cases the condition recurred. Conclusion Although no adverse effects were reported in this Labial fusion is a relatively common condition that study, betamethasone is a potent steroid that can be can cause significant distress to parents because of associated with local skin weakening and irritation, worries regarding the normality of their daughter’s as well as potential concerns from systemic genitalia. Doctors must be firm that the condition is absorption. It is therefore not a recommended benign and resist any temptation to treat the management option. condition unless the child is clearly symptomatic. Recurrence is common and all treatment options Surgical treatment have potential physical or psychological adverse Where medical treatment has failed, and if there are effects. There are no large randomised trials on associated urinary symptoms, surgical correction management of labial adhesions that clearly clarify may be indicated.Whilst there are many reports of the ideal treatment and most treatment algorithms adhesion separation in the outpatient setting, this are based on the doctor’s experience or preference.17 procedure, albeit brief, can be painful and However, it is logical to start treatment with the less traumatising and is not common practice in the invasive method of local estrogen application and UK.According to a survey of parents whose only proceed to surgical separation under general children had undergone separation of labial anaesthesia when conservative treatment has failed. adhesions under local anaesthetic, the perception of their child's discomfort was rated as moderate or References 15 1 Leung AK, Robson WL, Tay-Uyboco J. The incidence of labial severe in half of the cases. The success of fusion in children. J Paediatr Child Health 1993;29:235–6. separation in the outpatient setting has been doi:10.1111/j.1440-1754.1993.tb00495.x 8 2 McCann J, Wells R, Simon M, Voris J. Genital findings in prepubertal girls reported at the rate of 80%, but this needs to be selected for nonabuse: a descriptive study. Pediatrics 1990;86:428–39. balanced against the distress caused to the child. 3 Breech LL, Laufer MR. Surgicel in the management of labial and clitoral hood adhesions in adolescents with lichen sclerosus. J Pediatr Adolesc Gynecol 2000;13:21–2. doi:10.1016/S1083-3188(99)00029-7 In those few cases where surgical treatment is 4 Muram D. Labial adhesions in sexually abused children. JAMA 1988;259:352–3. doi:10.1001/jama.259.3.352 required, this should be carried out under a brief 5 DeMarco BJ, Crandall RS, Hreshchyshyn MM. Labial agglutination general anaesthetic. The adhesions are divided by secondary to a herpes simplex II infection. Am J Obstet Gynecol 1987;157:296–7. using gentle labial traction or by running a sound 6 Opipari AW Jr. Management quandary. Labial agglutination in along the line of fusion. There is usually no or a teenager. J Pediatr Adolesc Gynecol 2003;16:61–2. doi:10.1016/S1083-3188(02)00215-2 minimal blood loss and suturing is not usually 7 Leung AK, Robson WL. Labial fusion and asymptomatic bacteriuria. Eur J necessary. Pediatr 1993;152:250–1. doi:10.1007/BF01956155 8 Muram D. Treatment of prepubertal girls with labial adhesions. J Pediatr Adolesc Gynecol 1999;12:67–70. doi:10.1016/S1083-3188(00)86629-2 Recurrence is a common problem in labial 9 Norbeck JC, Ritchey MR, Bloom DA. Labial fusion causing upper urinary tract obstruction. Urology 1993;42:209–11. adhesions after both application of estrogen cream doi:10.1016/0090-4295(93)90650-Y and surgical separation. Recurrence rates range 10 Bacon JL. Prepubertal labial adhesions: evaluation of a referral 4 population. Am J Obstet Gynecol 2002;187:327–31. between 10–16%, depending on the treatment. doi:10.1067/mob.2002.126201 These numbers are probably underestimations, as 11 Schober J, Dulabon L, Martin-Alguacil N, Kow LM, Pfaff D. Significance of topical estrogens to labial fusion and vaginal introital integrity. J Pediatr most published studies have short follow-up Adolesc Gynecol 2006;19:337–9. doi:10.1016/j.jpag.2006.06.004 periods. Recurring adhesions, particularly where 12 Leung AK, Robson WL, Kao CP, Liu EK, Fong JH. Treatment of labial fusion with topical estrogen therapy. Clin Pediatr (Phila) 2005;44:245–7. there has been prior mechanical separation, are doi:10.1177/000992280504400308 denser and therefore less likely to resolve with 13 Elmore JM, Baker LA, Snodgrass WT. Topical steroid therapy as an 16 alternative to circumcision for phimosis in boys younger than 3 years. conservative management. There is also some JUrol 2002;168:1746–7. doi:10.1016/S0022-5347(05)64404-7 suggestion that, following prior surgical separation, 14 Myers JB, Sorensen CM, Wisner BP, Furness PD, III, Passamaneck M, Koyle MA. Betamethasone cream for the treatment of pre-pubertal labial recurring adhesions may be less likely to resolve adhesions. J Pediatr Adolesc Gynecol 2006;19:407–11. with the onset of spontaneous puberty. In the doi:10.1016/j.jpag.2006.09.005 15 Smith C, Smith DP. Office pediatric urologic procedures from majority of these cases repeat surgical separation a parental perspective. Urology 2000;55:272–6. will be required. This reinforces the commonsense doi:10.1016/S0090-4295(99)00571-3 16 Kumetz LM, Quint EH, Fisseha S, Smith YR. Estrogen treatment success approach of only treating adhesions when they are in recurrent and persistent labial agglutination. J Pediatr Adolesc Gynecol symptomatic, as treatment can be futile and can lead 2006;19:381–4. doi:10.1016/j.jpag.2006.09.008 17 Omar HA, Muram D, Schroeder B. Management of labial adhesions in to the formation of stronger adhesions after some prepubertal girls. J Pediatri Adolesc Gynecol 2000;13:183–6. months. doi:10.1016/S1083-3188(00)00064-4