Persistent urogenital sinus presenting as hydrometrocolpos and urinary ascites

Abstract

Persistent urogenital sinus (UGS) is a rare congenital cloacal anomaly whereby the urinary and genital tracts fail to separate during embryonic development. It can present as hydrometrocolpos (HMC) with severe vaginal stenosis/atresia, ambiguous genitalia, and/or urinary ascites. A term neonate born to a primigravida showed on antenatal ultrasound scan features suggestive of gross fetal ascites with severe oligohydramnios and hydronephrosis. Postnatally, the neonate presented with respiratory distress, urinary ascites, and abdominal mass and unilateral buphthalmos. An ascitic tap was done and HMC was drained. The cystogenitoscopy revealed vaginal stenosis for which vaginoplasty was done. The child was discharged and on follow-up is gaining weight. Our report shows an unusual presentation of a persistent UGS and also emphasizes the importance of early antenatal diagnosis and
prompt post-natal management to reduce morbidity.