Mild Tessier No. 7 cleft with PHACE syndrome: the case for pulmonary vascular steal

Abstract

We describe an unusual case of PHACE syndrome that provides a demonstration of the role of vascular anomalies in the causation of external ear and facial anomalies. The child in our case was characterized by a small segmental hemangioma of the face, tetralogy of Fallot, and anomalous origin of left common carotid artery from pulmonary artery with retrograde blood flow. This presumably resulted in hypoperfusion of the left side of the face resulting in a Tessier number 7 cleft and left ear anomaly explained by pulmonary vascular steal phenomenon. The absence of posterior fossa anomalies may reflect normal perfusion via the Circle of Willis.