Hemifacial Infantile Hemangioma with Intracranial Extension: A Rare Entity

Abstract

Intracranial hemangiomas are uncommon, especially in the absence of diffuse hemangiomatosis or the syndrome consisting of posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities, sternal clefting, and/or supra-umbilical raphe (PHACES). We saw an 8-month-old ex-premature girl with a large left-sided ocular and facial hemangioma that had been growing since early infancy. Examination revealed a 7 x 13 cm violaceous tumor involving the left periocular region and face. Ophthalmologic examination revealed deprivation amblyopia, anisometropia with myopia and astigmatism. Magnetic resonance imaging demonstrated a vascular tumor mass involving the scalp, face, and calvarium with extension into the orbit, infratemporal fossa, nasopharynx, lateral medullary cistern, internal auditory canal, and fourth ventricle. Marked enhancement was seen with contrast, and no posterior fossa malformations were noted. She was treated with prednisolone, which was tapered over 12 months. Follow-up magnetic resonance imaging examination at 25 months showed a marked decrease in the size of all lesions, with residual hemangioma in the periorbital soft tissues and small foci in the orbit and intracranial sites. The brain and ventricular system were normal. Intracranial hemangioma may occur in the setting of a large facial hemangioma (especially segmental) in the absence of the PHACES syndrome or diffuse hemangiomatosis. Radiological imaging should be considered to assess for intracranial hemangioma as well as posterior fossa or arterial anomalies.