Facing PHACES Syndrome; Anesthesiologist’s Point of View

PHACES syndrome is a neurocutaneous syndrome that consists of posterior fossa brain malformation, hemangiomas of the face, arterial cerebrovascular malformation, cardiovascular anomalies (Coarctation of the aorta), eye anomaly, and sternal defect or supraumbilical raphe. Most of these manifestations would interfere with anesthesia and hence need major considerations.

A 2.5-month-old female weighing 2100 gr was a candidate for laser therapy due to retinopathy of prematurity. She was diagnosed with PHACES syndrome. Her anesthesia was induced and maintained with sevoflurane. LMA #1 was used for airway management. No complications occurred during or after anesthesia, and she was discharged the next day.

PHACES syndrome interacts with numerous anesthesia-related characteristics, such as airway management, cardiovascular disease, and CNS malformations. Based on the accompanying characteristics, perioperative care for these patients should be tailored.