Ischemic Stroke in PHACE Syndrome

Aplasia, hypoplasia, and occlusion of a major cerebral artery appear to be significant risk factors for arterial ischemic stroke in children with PHACE syndrome.

PHACE is a syndrome defined by its acronym: Posterior fossa brain malformations, Hemangiomas, Arterial anomalies, Coarctation of the aorta/Cardiac defects, and Eye abnormalities (see also JW Dermatol Dec 2 2011). Abnormal craniocervical arteries, common in PHACE patients, may lead to ischemia, thromboembolic events, or arterial ischemic stroke (AIS).

To better delineate risk factors for AIS, these authors searched the literature and two PHACE registries and queried PHACE researchers. They identified 22 patients with PHACE and AIS. Of these, 15 had cardiovascular anomalies (68%) and 5 had posterior fossa brain abnormalities (23%). Imaging of head and neck arteries was available for 20 patients. Of these, 19 had either narrowing or nonvisualiz…

Reviewing Author

Mary Wu Chang, MD

Disclosures

Consultant / Advisory board Pierre Fabre; Valeant

Speaker’s bureauPierre Fabre

Mary Wu Chang, MD

Disclosures

Consultant / Advisory board Pierre Fabre; Valeant

Speaker’s bureauPierre Fabre

Ischemic Stroke in PHACE Syndrome

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Reviewing Author