Anesthesia for Complex Cardiovascular Surgery in a Patient With PHACES Syndrome and Review of the Literature

Abstract

NFANTILE HEMANGIOMAS ARE a common benign vascular tumor occurring in 5% to 10% of infants 1 and resolve without complication or medical intervention. A subgroup of patients with hemangiomas have associated structural anomalies causing significant physiologic changes such as posterior fossa brain malformations, hemangiomas of the face, arterial cerebrovascular, cardiovascular, and eye anomalies that occur along with ventral defects (sternal defects or supraumbilical raphe). 1,2 These anomalies constitute the acronym PHACES, formally defined in 1996 by Freiden, 2 yet suspicion of the syndrome arose in 1978. It was recognized that certain cervicofacial hemangiomas were associated with intracranial abnormalities. 3 Patients are stratified into 2 categories: PHACES syndrome or possible PHACES syndrome depending on the number and type of anomalies. 4 Approximately 20% of infants with large cervicofacial hemangiomas 5 will have one of the associated anomalies of PHACES. 1 Two-thirds of patients with large segmental hemangiomas have at least 1 extracutaneous manifestation and 42% have at least 2. 4 Cerebrovascular anomalies are most common among PHACES-syndrome patients (87%) followed by cardiovascular (37%), eye (16%), ventral developmental defects (sternal defects; 7%-21%), as well as endocrine abnormalities (6%). 6,7 Hemangiomas are not always detectable in PHACES syndrome. 4 They can be absent or reduced in size due to