Congenital urethrovaginal fistula associated with imperforate hymen causing fetal urinary ascites and abdominal cystic lesions: A case report and literature review

•We report a female baby of congenital urethrovaginal fistula associated with imperforate hymen.•The patient presented with massive urinary ascites and bilateral hydronephrosis in fatal period.•Obstruction of the uterine tube might cause the giant hydrometrocolpos and hydrosalpinx before birth.•Cystoscopy revealed a fistulous opening on the urethra, located approximately 5 mm distal from the internal urethral orifice.•Including our patient, only three cases of congenital urethrovaginal fistula associated with imperforate hymen have been reported.

A primipara presented to our hospital due to fetal ascites, bilateral hydronephrosis and hydrometrocolpos. The female baby had two giant abdominal cysts, a single atrium and foot polydactyly. Absence of the vaginal orifice was noticed, and the hymen was broken through; the left cyst collapsed after fluid drainage. The right cyst was punctured and drained, which improved the hydronephrosis. Contrast imaging showed medium pouring into the abdominal cavity via the uterine tube; the right cyst was a 6 × 5 cm closed cavity. Vaginal uroplania persisted even after hospital discharge. At the age of 4 years, cystoscopy showed a fistulous opening on the urethra 5 mm distal from the internal urethral orifice. A catheter inserted via the opening was found in the vagina. In our case, urine poured into the abdominal cavity through the fistula and uterine tube; following obstruction of the uterine tube, giant hydrometrocolpos and hydrosalpinx appeared. Three cases of congenital urethrovaginal fistula (CUVF) with imperforate hymen have been reported. A remnant of communication between the uterovaginal primordium and urogenital sinus may be responsible for CUVF.