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An Abnormal Vaginal Opening in a Two-Year-Old



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Am Fam Physician. 2008 Feb 1;77(3):355-356.

A two-year-old girl's mother was concerned that her daughter's vaginal opening appeared to be closed. The mother had noticed this a few months earlier when her daughter had severe diaper rash. The patient did not have urinary problems or vaginal discharge, and her birth and development histories were unremarkable. Genital examination revealed a thin vertical raphe over the site of the vaginal opening (see accompanying figure). The labia majora were intact and separated, although only the upper third of the labia minora was identifiable.

[Figure unavailable]

[Figure unavailable]

Question

Based on the patient's history and physical examination, which one of the following is the most likely diagnosis?

A. Bartholin cyst.

B. Imperforate hymen.

C. Labial adhesions.

D. Transverse vaginal septum.

E. Vaginal atresia.

Discussion

The answer is C: labial adhesions. Labial adhesions are acquired abnormalities involving the labia minora, but not the labia majora. Adhesion is the most common interlabial abnormality in child urology patients.1 The adhesion usually begins at the posterior fourchette and extends to varying degrees superiorly to the clitoris. The abnormality is generally discovered between 13 and 23 months of age by the child's parents or by the physician during a routine well-child examination. Most cases occur before six years of age.2

Children with labial adhesions usually have a history of a local inflammatory process, such as diaper rash. Most patients are asymptomatic; however, the adhesions occasionally cause local inflammation, recurrent vulvovaginitis, or recurrent urinary tract infections. Adhesions do not occur in newborns, presumably because of the protective effect of circulating maternal estrogens.

Treatment of adhesions is nonsurgical and includes application of topical estrogen cream. Estrogen cream applied daily to the affected area for one to two weeks has an effectiveness rate between 49 and 90 percent.3,4 Occasionally, six to eight weeks of therapy is needed.5

Cleaning the affected area and keeping the labia separated with short-term (one to two months) application of a petrolatum-based barrier ointment (e.g., Vaseline) can help prevent recurrence. Simple hygienic measures may be sufficient for asymptomatic children because most adhesions resolve during early puberty.5 Topical steroids may also be effective, but they have not been prospectively studied.6 Surgical treatment is reserved for patients with unresponsive cases.

Bartholin's gland is a small vestibular gland located bilaterally between the labia minora and hymen. Occasionally, the duct of the gland becomes obstructed, causing unilateral vulvar swelling. Bartholin's gland abnormalities are uncommon in children.

An imperforate hymen is the most common congenital obstructive anomaly of the female reproductive tract,1 although it may not be diagnosed until adolescence. The labia are intact in affected patients. An imperforate hymen should be suspected in an adolescent presenting with primary amenorrhea; cyclic abdominal pain; and a bluish, bulging hymen. Newborns with this condition may have a bulge at the posterior introitus, representing retained vaginal fluid. Referral to a pediatric urologist is recommended for surgical repair of the hymen.

A complete transverse vaginal septum may occur at various levels inside the vagina, although most are located in the upper vagina.1 Patients have a vaginal opening, and the labia are intact and separated. Non-fusion or canalization of the urogenital sinus and müllerian ducts cause the abnormality. Children are usually asymptomatic, but they may present with amenorrhea and a distended upper vagina during adolescence. Transperineal ultrasonography and magnetic resonance imaging (MRI) can help establish the diagnosis and determine the location and thickness of the transverse septum. Treatment is surgical resection.

Vaginal atresia is suspected when a vaginal opening cannot be identified and, instead, a shallow dimple is seen inferior to the urethral opening. Failed formation of the lower portion of the vagina leads to the condition. The labia are intact and the upper vagina, cervix, and uterus are normal. Palpation of a distended vagina on rectal examination may help to distinguish vaginal atresia from agenesis (failed formation of the upper vagina or testicular feminization). Ultrasonography with or without MRI is necessary to define the abnormal anatomy. Patients should be referred to a pediatric urologist for surgical reconstruction.

Selected Differential Diagnosis of an Abnormal Vaginal Opening in a Child

Condition Characteristics

Imperforate hymen

Labia are intact; vaginal opening is intact, but obstructed by the hymen; congenital

Labial adhesions

Labia majora are intact; however, labia minora are fused together; vaginal opening is obstructed to varying degrees; not present at birth, but typically develops between 13 and 23 months of age

Transverse vaginal Septum

Labia are intact; vaginal opening is present, but obstructed by a transverse septum, typically in the upper vagina

Vaginal atresia

Labia are normal; distal vagina is absent; shallow dimple inferior to the urethral opening

Selected Differential Diagnosis of an Abnormal Vaginal Opening in a Child

View Table

Selected Differential Diagnosis of an Abnormal Vaginal Opening in a Child

Condition Characteristics

Imperforate hymen

Labia are intact; vaginal opening is intact, but obstructed by the hymen; congenital

Labial adhesions

Labia majora are intact; however, labia minora are fused together; vaginal opening is obstructed to varying degrees; not present at birth, but typically develops between 13 and 23 months of age

Transverse vaginal Septum

Labia are intact; vaginal opening is present, but obstructed by a transverse septum, typically in the upper vagina

Vaginal atresia

Labia are normal; distal vagina is absent; shallow dimple inferior to the urethral opening

Address correspondence to Nikhil Hemady, MD, FAAFP, at nhemady@nomc.org. Reprints are not available from the authors.

Author disclosure: Nothing to disclose.

REFERENCES

1. Rink R, Kaefer M. Surgical management of intersexuality, cloacal malformation, and other abnormalities of the genitalia in girls. In: Wein AJ, Kavoussi LR, Novick AC, et al., eds. Campbell-Walsh Urology. 9th ed. St. Louis, Mo.: Saunders; 2007.

2. Vulvovaginitis. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. St. Louis, Mo.: Saunders; 2004.

3. Muram D. Treatment of prepubertal girls with labial adhesions. J Pediatr Adolesc Gynecol. 1999;12(2):67–70.

4. Aribarg A. Topical oestrogen therapy for labial adhesions in children. Br J Obstet Gynaecol. 1975;82(5):424–425.

5. Omar HA. Management of labial adhesions in prepubertal girls. J Pediatr Adolesc Gynecol. 2000;13(4):183–185.

6. Myers JB, Sorensen CM, Wisner BP, et al. Betamethasone cream for the treatment of pre-pubertal labial adhesions. J Pediatr Adolesc Gynecol. 2006;19(6):407–411.

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