Superior sternal cleft associated with PHACES syndrome: postnatal sonographic findings.

Abstract

Sternal cleft is a rare congenital malformation caused by complete or partial failure of the sternum to fuse during the third month of embryologic development. Cleft sternums can be classified as superior, inferior, or complete. Sternal clefting is also associated with the PHACES syndrome, a neurocutaneous syndrome consisting of posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, cardiac defects, eye abnormalities, and sternal clefting with or without abdominal raphae. We present the case of a 1-day-old girl who was born at our institution with a variant of the PHACES syndrome. Although the sternal cleft was evident clinically, sonography provided additional information compared with plain radiography in showing the extent of the anterior chest wall abnormalities. The literature on this rare condition is reviewed.