Image from Pediatric EM Morsels (pedemmorsels.com).
Clinical scenario: different patients, same problem
A 5-year-old fully toilet-trained girl whose parents notice her underwear is always slightly wet.
An 18-month-old girl with high fever for 3 days and no obvious source.
A 3-year-old girl who has been scratching her genital area constantly and seems uncomfortable.
A 2½-year-old toilet-trained girl whose urine, since she started daycare, seems to take a long time to come out and dribbles a little at a time.
Each of these patients clearly needs a urine sample to evaluate for UTI. Just as important, each needs a proper genital exam to look for labial adhesions. Have you ever gathered supplies to straight-catheterize a girl for UA/urine culture and noticed that something did not look right?
Why it matters
Labial adhesions are relatively common. Beyond treating any UTI, the adhesion itself may need treatment. Otherwise the patient may keep getting UTIs or ongoing discomfort.
Background
Also called labial agglutination or synechia vulvae.
Most common in infants and young children, with a peak incidence of up to 3% of girls in the second year of life.
One proposed mechanism is inflammation of the labia minora combined with the low-estrogen state of prepubertal girls. Contributing factors include poor perineal hygiene; trauma (for example, straddle injury, female genital mutilation, or sexual abuse); vaginal infection (for example, Candida, group A Streptococcus, Neisseria gonorrhoeae, Mycoplasma pneumoniae mucositis, or HSV); and lichen sclerosus.
Clinical presentation
Diagnosed on routine genital examination.
May be partial (upper or lower labia only) or complete.
With complete adhesions, a small pinhole opening allows urine to exit from behind the fused labia.
May be asymptomatic or cause:
a pulling sensation
difficulty urinating or urinary dribbling
vaginal pain or discharge
recurrent urinary tract or vaginal infections
Management
Treatment depends on whether the adhesion causes symptoms, and how severe they are.
Asymptomatic
No treatment is necessary if the adhesion is asymptomatic, involves only a small portion of the labia, and does not affect the urine stream. Adhesions typically resolve when estrogen production increases at puberty.
Symptomatic: treat if any of the following
Extensive labial fusion
Diversion of the urinary stream
Inability to collect a clean-void urine culture in a child with recurrent febrile illness without an obvious source
Recurrent urinary or vaginal infections
Vaginal pain or discharge
First-line: topical therapy (not manual separation)
Choosing a topical agent requires shared decision-making with caregivers about the side effects of systemic absorption.
Topical estrogen or estradiol (most common)
Conjugated estrogen cream 0.625 mg/g or estradiol cream 0.01%, applied twice daily with a fingertip (rarely a cotton swab) directly to the thin white midline line of fusion.
Apply gently so the adhesion is not torn traumatically.
Continue, with re-evaluation, until the adhesion resolves. The most common reason for treatment failure is cream placed in the wrong spot or too little cream.
Stop once the adhesion has completely resolved, to limit systemic absorption.
Possible effects: local irritation, vulvar hyperpigmentation, breast bud formation, and minimal vaginal bleeding. Breast budding and bleeding resolve after the cream is stopped, and are less likely when the cream is applied sparingly to the adhesion alone.
Adhesions resolve in nearly all girls treated with correct technique for 2–6 weeks.
Topical betamethasone 0.05%
Applied twice daily for 4–6 weeks. Several studies report 68–89% efficacy.
Appears similarly effective to estrogen. It can be used as primary, secondary, or adjunctive therapy, and is a reasonable choice after estrogen fails.
Avoid use longer than 3 months because of the risk of skin atrophy and systemic corticosteroid effects.
If there is no improvement after 4–8 weeks of properly applied initial therapy, switch to the alternative cream for 2–4 more weeks. If there is still no improvement, refer to a specialist with pediatric gynecologic expertise.
Aftercare
After successful separation, recommend good vulvar hygiene, daily baths, and a bland ointment such as white petroleum jelly for 6–12 months to prevent re-adhesion.
Physical separation (rarely indicated)
Reserved for severe obstruction of urinary flow with retention, recurrent infections, or failed topical therapy. Topical therapy tends to fail with thick adhesions (3–4 mm wide) that have no thin, translucent raphe.
Non-surgical separation: should be performed only by experienced clinicians, with local analgesia (for example, topical lidocaine) and sedation as needed. A lubricated cotton swab is placed behind the labia minora and drawn gently forward along the midline raphe.
Surgical separation: reserved for rare complete obstruction of urine flow, under deep sedation or general anesthesia. Afterward, use topical estrogen for 1–2 weeks, then a bland emollient for 6–12 months.
What I do in the ED
Prescribe topical estrogen cream. Tell the parent to apply a small amount only directly on the adhesion twice daily for 2–6 weeks, stopping once it opens. Make sure they know exactly where to put the cream.
Counsel parents that breast budding or spotting can occur with prolonged use, and that it is temporary and resolves on its own.
Once the adhesion resolves, keep the area clean and free of irritation, and use petroleum jelly or another bland ointment for a few months to prevent re-adhesion.
A very thin adhesion may separate with gentle traction alone. This should not hurt or bleed.
Rarely, a complete adhesion with only a pinhole opening needs a pediatric urologist to separate it at the bedside. I have needed this once in my career.
Clinical pearls
In any girl being worked up for UTI, look at the labia before you catheterize.
Recurrent UTIs or dribbling in a toilet-trained girl should prompt an exam for adhesions.
Most treatment failures come from cream applied in the wrong place.
References and further reading
Adapted from UpToDate (labial adhesions: clinical manifestations and treatment), including the partial-adhesion image and management algorithm.
Pediatric EM Morsels. Labial adhesions in children.
