Aortic arch tortuosity with PHACE syndrome : a rare case scenario

Abstract

PHACE syndrome is a rare neurocutaneous disorder characterised by an association of infantile haemangiomas with structural anomalies of brain, cerebral vasculature, eye, aorta and chest wall.1 Coarctation of aorta (COA) is most the common cardiac anomaly reported in PHACE syndrome. COA or interrupted aortic arch in PHACE is unique and complex both in location and character compared to the typical coarctation anatomy. Arterial tortuosity of the cerebral vasculature has been well described in literature in PHACE syndrome. We present a rare case of tortuous aortic arch continuing as descending aorta in an infant with PHACE syndrome.