Karima Sif Nasr, Siham El Haddad, Nazik Allali, Latifa Chat, Lina Belkouchi
Journal: Medpeer Publisher
ISSN: 3066-2737
Volume: 3
Issue: 10
Date of Publication: 2026/10/08
PHACE syndrome is an uncommon neurocutaneous disorder in which a segmental infantile hemangioma may reveal occult congenital brain and cerebrovascular abnormalities. We report the case of a 16-year-old girl with a persistent right facial hemangioma, first noticed at two months of age, with no neurological, developmental, or cardiorespiratory symptoms. Examination showed a segmental plaque larger than 5 cm involving the right frontotemporal, periorbital, upper eyelid, and nasal regions. CT confirmed a vascular soft-tissue lesion without intra orbital extension. MRI demonstrated right cerebellar hemispheric and vermian hypoplasia with communication between the fourth ventricle and the retrocerebellar cerebrospinal fluid space, while time-of-flight MR angiography identified a persistent primitive right dorsal ophthalmic artery arising from the cavernous internal carotid artery. These findings fulfilled the criteria for a definite PHACE syndrome. This case highlights the value of integrated neurovascular imaging in detecting clinically silent abnormalities and illustrates an uncommon cerebrovascular manifestation of PHACE syndrome.
PHACE syndrome, infantile hemangioma, posterior fossa malformation, cerebellar hypoplasia, magnetic resonance angiography
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