Hong-Thao Thieu and Meredith S. Thomas
History of present illness
The patient is a 19-month-old girl who presents to a tertiary care pediatric and adolescent gynecology clinic with acute dysuria and voiding difficulty. She has been followed by her pediatrician for labial agglutination for approximately one year. At the time of diagnosis she was noted to have thin labial adhesions that were transparent and affected only the posterior portion of the fold. As prescribed, her mother had been applying topical estrogen (Premarin®) cream to the labial adhesions twice daily for the past year but never achieved complete separation. Over the last week prior to presentation she has been fussy, crying with urination, and her mother notes a foul smell in her diaper. Review of systems is negative for fevers, back pain, nausea, vomiting, and diarrhea.
Physical examination
General appearance:Well-developed, well-grown
19-month-old girl who appears fussy, crying, and resistant to examiner
Vital signs:
Temperature: 37.0°C Pulse: 120 beats/min
Blood pressure: 95/60 mmHg Respiratory rate: 22 breaths/min Oxygen saturation: 100% on room air Height: 32.67 inches
Weight: 26 lb BMI: 17 kg/m2
Cardiovascular:Normal S1, S2 without murmurs, rubs, or gallops
Respiratory:Clear to auscultation bilaterally
Abdomen:Soft and nontender without masses or hernias;
no distention was appreciated
Gynecologic:Tanner stage I breast and pubic hair
development. There is nearly 100% agglutination of the labia except for a small urethral outlet (Fig. 76.1)
Laboratory studies:
Urine: Appears cloudy
Urinalysis: 1+ Leukocyte esterase, 1 RBC, 1 WBC, few bacteria, specific gravity 1.021
Urine culture: 10–50 000 cfu mixed bacterialflora
How would you manage this patient?
This 19-month-old girl with known labial adhesions presents after a year of treatment failure following conservative man- agement with topical estrogen. Given her voiding complaints, suspicion for urinary tract infection and treatment failure in the setting of high degree of agglutination, this patient qualifies for surgical management.
The patient was taken to the operating room where the labia were grasped bilaterally and extended to allow visualiza- tion of the thin line of adhesion and a small incision into the thin adhesion was made with electrocautery. The labia were then completely separated with gentle manual traction and blunt dissection. The line of adhesion remained hemostatic (Fig. 76.2).
Fig. 76.1 Labial adhesion in 19-month-old girl with one year of treatment failure, presenting with urinary difficulty.
Acute Care and Emergency Gynecology, ed. David Chelmow, Christine R. Isaacs and Ashley Carroll. Published by Cambridge University Press.
© Cambridge University Press 2015.
Labial adhesions
Labial adhesions are a relatively common pediatric gynecologic complaint and prevalence has been cited as up to 20% of pediatric populations presenting for routine primary care [1].
The prevalence differs largely among different studies, and this is likely secondary to the wide clinical presentation with a large percentage of cases being asymptomatic. The true prevalence is difficult to accurately calculate given the wide range of clinical presentations.
The above clinical scenario illustrates several of the common presentations of labial adhesions. Clinical presenta- tion varies from asymptomatic, thus an incidental finding of adhesions on routine exam, to acute urinary retention necessi- tating emergent surgical intervention. Common complaints include vulvovaginitis, positional pain, bleeding, urinary incontinence, and urinary tract infections. It is important to recognize the range of symptoms associated with labial adhe- sions in order to appropriately triage treatment goals. Practice Bulletin No. 93 by the American College of Obstetricians and Gynecologists (ACOG) recommends that treatment is reserved for those patients presenting with symptoms [2]. As above, the first-line treatment recommendation is topical estrogen cream.
As with any thorough evaluation, it is important to estab- lish a differential diagnosis prior to proceeding with treatment and to rule out alternative etiologies of the presenting com- plaint or clinicalfinding. The differential diagnosis for labial adhesions includes congenital anomalies such as vaginal agen- esis and vaginal septum, hymenal abnormalities such as an imperforate hymen, and clitoromegaly. Typically the patho- physiology of labial adhesions is thought to be secondary to a physiologic nadir in systemic estrogen and an inciting factor of vulvar irritation. This nadir corresponds with the peak inci- dence of agglutination presentation, which is 2.5 years of age [1]. A rare but important historical element that must be considered with labial adhesions is potential for abuse. Typical diaper rash is thought to be enough of an insult to initiate adhesions, but one would not want to miss abuse in the outpatient setting.
Approach to treatment depends on severity of symptoms.
ACOG recommends topical estrogen for symptomatic patients followed by one month of emollient cream to prevent reagglu- tiation of raw labial edges after separation [2]. Topical estrogen such as Premarin (0.625 mg/g) should be applied twice daily to the midline of the adherent labia [3]. If the twice-daily appli- cation is not successful, the application can be increased to three or four times per day. One alternative to estrogen cream that has been recently studied is topical steroids [2]. A study by Myers and colleagues looked retrospectively at a cohort of patients with labial adhesions who were treated with beta- methasone and found the treatment to be as effective as estro- gen [4]. However, a retrospective comparison by Eroglu and colleagues, published a few years later, looked at the use of betamethasone, estrogen, and a combination of the two and found essentially no difference [5]. Estrogen cream remains the first-line treatment but betamethasone may be a safe second- line treatment for those who fail estrogen therapy.
Some common pitfalls of treatment are not concentrating the application to the midline. Signs of too-wide application are treatment failure and hyperpigmentation. The patient in our case was treated conservatively for one year but never achieved complete separation. When she presented, her symp- toms had worsened. Presentation with urinary retention and suspected urinary tract infection is an indication for surgical intervention. Additional characteristics that may be an indication for surgical intervention are thick adhesions, greater than 90% of the vestibule affected, and small uretheral opening [6].
Reagglutination is a preventable complication of any labial separation, whether manual or passive with topical cream. The parents in this case were instructed to keep the raw edges of the adhesion moist with a topical agent such as Aquaphor®cream or A&D® ointment. When this patient presented for her 6-week postoperative visit she was doing well but was found to have a small 5 mm area of re-agglutination near the poster- ior forchette. At this time the parents were instructed to reintroduce Premarin topical cream until complete separation was achieved. By her second follow-up visit one month later,
Fig. 76.2 Labial separation achieved with electocautery and manual separation under general anesthesia.
she had achieved complete separation and this was further maintained with A&D ointment.
Key teaching points
Labial adhesions are a common presenting complaint in the pediatric population and treatment options are decided based on severity of symptoms.
Topical estrogen cream application is sufficient for most cases of labial adhesions, and manual or surgical separation should be reserved for only the most severe symptoms such as urinary dysfunction or retention.
Once separation is achieved, continued use of lubricants or moisturizers will help to maintain separation until the labial skin is completely healed.
References
1. Murram D. Treatment of prepubertal girls with labial adhesions.
J Pediatr Adolesc Gynecol1999;12:
67–70.
2. American College of Obstetricians and Gynecologists. Diagnosis and
management of vulvar skin disorders.
Practice Bulletin No. 93.Obstet Gynecol 2008;111:1243–53.
3. Tebruegge M, Misra I, Nerminathan V.
Is the topical application of oestrogen cream an effective intervention in girls suffering from labial adhesions?
Arch Dis Child2007;92:
268–71.
4. Myers J, Sorensen C, Wisner B, et al.
Betamethasone cream for the treatment of pre-pubertal labial adhesions.
J Pediatr Adolesc Gynecol2006;19:
407–11.
5. Eroglu E, Yip M, Oktar T, Kayiran S, Mocan H. How should we treat prepubertal labial adhesions?
Retrospective comparison of topical treatments: estrogen only,
betamethasone only, and combination estrogen and betamethasone.J Pediatr Adolesc Gynecol2011;24:389–91.
6. Bacon J. Prepuberal labial adhesions:
evaluation of a referral population.Am J Obstet Gynecol2002;187:327–32.
Case 76: A 19-month-old girl with labial adhesions and acute urinary retention