Perioperative Care of a Pediatric Patient With PHACES Syndrome

Abstract

PHACE syndrome is a neurocutaneous syndrome that was originally described by Dr. Ilonia Frieden in 1996. The term PHACE is an acronym for the main components of the syndrome including: P osterior fossa malformations of the central nervous system (CNS); H emangioma typically involving the skin of the head or neck; A rterial lesions usually involving the cerebral vasculature; C ardiac abnormalities, most commonly aortic coarctation; and Eye abnormalities. When accompanied by ventral developmental defects, it is termed PHACES syndrome, with the “S” representing sternal cleft or supraumbilical raphe. Patients may present for a variety of surgical procedures including the airway, CNS or the heart. Airway compromise secondary to hemangiomas and the potential for alterations in cerebral blood flow due to abnormalities of the cerebral arterial vasculature present major challenges for the anesthetic management of these patients. The perioperative implications of PHACES syndrome are discussed and previous reports of anesthetic care are reviewed. J Med Cases • 2013;4(12):811-815 doi: http://dx.doi.org/10.4021/jmc1374w