Anesthetic Management of a Parturient with PHACE Syndrome for Cesarean Delivery

PHACE syndrome is a disorder that features posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. PHACE syndrome includes abnormalities in several organ systems that may influence anesthetic management. We discuss the anesthetic management of a 26-year-old woman with PHACE syndrome presenting for cesarean delivery. Management included careful airway examination, slowly dosed epidural anesthesia, close hemodynamic monitoring aided by a radial arterial line, and continuous intraoperative neurologic assessment.