Arteriovenous shunting as a new feature of PHACES. Case report.

Patients with the congenital neurocutaneous disorder PHACES are at a markedly increased risk of ischemic infarction during childhood. Although intracranial arterial anomalies have been well described, venous abnormalities have not been documented. The authors report on a unique case of a 3-month-old girl with PHACES and a skull base osteodural arteriovenous fistula. A separate arteriovenous shunt at T-5 may also have been present. Imaging findings and treatment strategies are discussed.