a b s t r a c tA 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 Â 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.Ó 2015 The Authors. Published by Elsevier Inc. All rights reserved.Congenital urethrovaginal fistula (CUVF) is an extremely rare genitourinary anomaly, and etiology of CUVF remains unknown [1]. Only three CUVF cases associated with imperforate hymen (IH) have been reported [2,3]. Here, we present a newborn of CUVF with IH causing massive urinary ascites and large abdominal cystic lesions in fetal period. Furthermore, the pathology of the patient and etiology of CUVF are discussed.
CaseA 28-years-old primipara was referred to our hospital with fetal ascites and bilateral hydronephrosis at 28 weeks of gestation. Amniotic fluid examination showed a normal female karyotype.Fetal magnetic resonance imaging (MRI) demonstrated bilateral hydronephrosis and massive ascites that significantly pushed up the thoracic cavity. In the lateral view, hydrometrocolpos, dilated urinary bladder, and a luminal structure between them were visible ( Fig. 1). At 30 weeks of gestation, the ascites gradually decreased; however, cystic lesions appeared in the fetal abdominal cavity at 32 weeks of gestation.A newborn baby, weighing 3,107 g, was born at 34 weeks of gestation by emergency Caesarean section due to fetal distress. Tracheal intubation, artificial ventilation, and nitric oxide inhalation were performed immediately in response to the patient's severe abdominal distension and respiratory failure. Abdominal ultrasonography revealed two giant cystic lesions. She had a single atrium and left-foot polydactyly. One day after birth, bulging of the vaginal vestibule and absence of the vaginal orifice were both observed. The external urethral orifice and anus were at normal position. Perineal ultrasonography showed a part of the cyst across a thin membrane. She was subsequently diagnosed with IH; the hymen was then punctured with forceps. The left cystic lesion collapsed after drainag...