Sirenomelia Associated with Truncus Arteriosus

Abstract

Sirenomelia is a sporadic congenital anomaly caused by a disruptive vascular defect. This syndrome is characterized by fusion of the lower extremities, almost invariably associated with bilateral renal agenesis and generally absence of sacrum, rectum and bladder. In this case, the bilateral renal agenesis was present with severe oligohydramnios, in association with other anomalies as single umbilical artery and ambiguous external genitalia, which are often found in other cases publicated, and truncus arteriosus type IV. This latter association has been never described before in the revision of the literature.