PHACE syndrome

Case contributed by Ammar Haouimi

Presentation

Left orbital swelling with cardiac anomalies.

Patient Data

Age: 6 months
Gender: Female
mri

Left cerebellar and inferior vermian hypoplasia with a CSF cleft communicating with a dilated 4th ventricle. Supratentorial ventriculomegaly. The MRA reveals a hypoplasia of the ipsilateral internal carotid artery as well as the M1 middle cerebral artery segment. An ipsilateral facial (mainly periorbital) enhancing soft tissue mass is noted suggestive of a hemangioma. 

Case Discussion

MRI appearances suggestive of a PHACE syndrome.

Additional contributors: R Bouguelaa MD, Z Boudiaf MD.

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