MANAGEMENT OF AN INFECTED HEMATOMETROCOLPOS IN A PATIENT WITH CONGENITAL ADRENAL HYPERPLASIA AND VAGINAL STENOSIS

Abstract

A 12-year-old girl presented with abdominal pain associated with menses. She had a history of congenital adrenal hyperplasia, and had undergone clitoral recession and posterior flap vaginoplasty at age 18 months. Evaluation revealed abdominal tenderness and pyuria. She was treated for urinary tract infection. The pain worsened and was associated with fever, and the patient was seen by a urologist. Ultrasound demonstrated a pelvic mass consistent with a dilated, fluid filled vagina. The patient underwent endoscopy through the common urogenital sinus but the vaginal introitus could not be identified. Symptoms improved significantly on intravenous antibiotics, and she was referred for further evaluation. Physical examination demonstrated suprapubic tenderness, an enlarged clitoris and a single perineal opening. Rectal examination revealed a large bulging mass anteriorly. Urinalysis showed no infection. The perineal opening was cannulated and a voiding cystourethrogram showed mass effect with anterior displacement of the bladder. Despite several bladder fillings and voidings, no contrast material entered the vagina. MRI was performed to characterize the suspected hematometrocolpos. The sagittal fast spin echo series showed a large midline pear-shaped, fluid filled mass displacing and compressing the bladder and rectum (see figure). Superiorly the mass was contiguous with a small fluid filled uterus and inferiorly it appeared obstructed just above the urogenital diaphragm. The signal intensity of the vaginal and uterine contents was suggestive of proteinaceous fluid and did not follow the signal characteristics of simple blood products. Based on MRI and history, infected hematometrocolpos due to vaginal stenosis was suspected. At cystoscopy the bladder neck was 2 cm. from the perineal opening of the urogenital sinus. It was elevated due to compression from the posterior mass. The bladder contained abundant whitish debris but was otherwise unremarkable. The vaginal introitus was not visible along the urogenital sinus. Based on the MRI and using real-time transabdominal ultrasound guidance, the stifF end of a 0.038-inch polytetrafluorwthylene coated guide wire was passed through the wall of the urogenital sinus and into the vagina. The tract