Successful Treatment of PHACE Syndrome with Oral Propranolol

Abstract

Abstract: An 8-week-old female infant presented with a large segmental plaque-like facial hemangioma involving her right eyelid, which caused a mechanical ptosis of the right eye with visual occlusion, and Dandy-Walker malformation, consistent with PHACE syndrome. Propranolol 2 mg/kg/day in 2 divided doses results in a dramatic change in the patient’s skin color in the first week, and she could open her eyelids normally after 3 weeks. At 6 months of treatment, the skin color had returned to almost normal. The propranolol dosage was then tapered to 1 mg/kg/day for 1 month and discontinued without a relapse. No undesirable side effects were reported during the patient’s follow-up visits. She had normal development and normal vision. We conclude that propranolol can be used as a front-line treatment for large facial hemangioma associated with PHACES syndrome.