Prune belly syndrome with urethral hypospadiasis, hypoplasia, imperforate anus, recto - vesical fistula and bilateral CTEV: A case report

Association between Prune belly syndrome (PBS) with urethral, hypospadiasis, imperforate anus and recto-vesical fistula is an unusual condition. It is usually fatal unless there is a communication between the fetal bladder and the amniotic sac. We report a case of PBS with urethral hypospadiasis and hypoplasia, imperforate anus congenital recto-vesical fistula and b/l CTEV deformity in a male neonate. Patient had severe respiratory distress syndrome at birth (HMD) received prolonged mechanical ventilation and surfactant. Patient was passing meconium and urine through ventrally located urethral orifice. Patient survived early neonatal period, but died due to severe sepsis and multiorgan failure on 20th day of life.