Large facial hemangioma; concern for PHACES syndrome; r/o intracranial malformation. Brain MRI: There is diffuse thickening and enhancement of the skin of the right side of the face and scalp, measuring up to 2 mm in thickness, which is compatible with a hemangioma. There is a T2 hyperintense enhancing mass centered in the right lacrimal fossa that measures up to approximately 25 mm with associated proptosis and remodeling of the lateral orbital wall, which also likely represents an hemangioma. There is mild right cerebellar hemisphere with associated prominence of the lateral posterior fossa CSF spaces. There is no midline shift or herniation. The degree of myelination is within normal limits. There is no evidence of intracranial hemorrhage or mass. There is a right tympanomastoid effusion.Brain MRA: There is a diffusely and markedly hypoplastic right internal carotid artery. There is an anomalous right ophthalmic artery that arises from the left cavernous carotid artery. There is a diffusely hypoplastic right vertebral artery There is an anomalous branch that supplies the right posterior fossa derived from the right external carotid artery. There is reconstitution of the circle of Willis vessels, with intact bilateral anterior, middle, and posterior cerebral arteries. The Neck MRI: There is a T2 hyperintense and enhancing lesion in the right posterior triangle of the neck that measures up to 25 mm, which likely represents an hemangioma. There are also multiple subcentimeter T2 hyperintense and enhancing lesions in the right buccal fat pad, right cheek, right temporal fossa subcutaneous tissues, and perhaps in the right parapharyngeal space, which also likely represent hemangiomas. Otherwise, there is no significant lymphadenopathy in the neck, and the thyroid and salivary glands are unremarkable. The cervical spine appears to be intact. Neck MRA: There is an aberrant right subclavian artery. There is moderate diffuse hypoplasia of the right common carotid artery and severe diffuse hypoplasia of the right internal carotid artery. There is also diffuse hypoplasia of the right vertebral artery diffusely. The left vertebral and internal carotid arteries are intact.
The findings are compatible with PHACES syndrome, including right orbital, facial, and upper neck hemangiomas, mild right cerebellar hypoplasia, and hypoplasia of the right carotid and vertebral arteries with anomalous collateral vessels supplying the right orbit and posterior fossa and reconstitution of the circle of Willis branches. Discussed with Dr. Rosenblatt at 1:30 PM on 4/23/15.