Intracranial Infantile Hemangiomas Associated With PHACE Syndrome

Abstract

BACKGROUND AND PURPOSE: Head and neck infantile hemangiomas are common lesions that are rarely observed in an intracranial location. We report 4 patients with orbital infantile hemangiomas and ipsilateral enhancing intracranial lesions, presumed to be infantile hemangiomas.

METHODS: Imaging studies and medical records of 4 infants with orbital hemangiomas and enhancing intracranial lesions were reviewed. The intracranial lesions were evaluated in terms of their location, signal intensity characteristics, enhancement pattern, and degree of involution following treatment. Additional findings associated with PHACE syndrome were also noted and a literature review of intracranial infantile hemangiomas and PHACE syndrome was also performed.

RESULTS: The intracranial masses were primarily in or adjacent to the internal auditory canal and demonstrated imaging characteristics and treatment response similar to the ipsilateral orbital lesions. Ipsilateral internal carotid artery hypoplasia, ipsilateral cerebellar hemisphere hypoplasia, and/or other head and neck hemangiomas were present in all patients.

CONCLUSION: These cases collectively support the diagnosis of intracranial infantile hemangiomas and suggest a unique radiographic association between PHACE syndrome and intracranial infantile hemangiomas.