Stroke risk stratification in patients with PHACE syndrome

	Description	Surveillance
Low risk	Anomalous origin of arteries Variants in the circle of Willis Persistent fetal vessels	Baseline scan only. Repeat imaging as needed for new symptoms.
Intermediate risk	Narrowing proximal to the circle of Willis* Stenosis of the proximal ICA if there is normal collateral flow in ACA and PCA	Referral to neurologist. Repeat imaging when sedation no longer needed or sooner for new signs or symptoms. May be at increased risk later in life if additional risk factors arise (atherosclerosis or traumatic injury). Avoid contact sports and sports with extreme neck positions.
High risk	>25% narrowing of principal cerebral vessels In association with an incomplete circle of Willis Tandem stenosis that causes a risk of diminished cerebral perfusion Signs of chronic or silent Ischemia Existing infarction Border zone ischemic changes Presence of lenticulostriate collateral dilation Pial collaterals	Referral to neurology. Repeat imaging at 6 months and 1 year. Then to be determined based on symptoms and progression. Avoid contact sports and sports with extreme neck positions. Aspirin 4 to 5 mg/kg/day up to 81 mg per day. If progressive moyamoya, refer to tertiary care center with experienced neurologist and neurosurgeon.

PHACE: posterior fossa anomalies, hemangiomas, arterial anomalies, cardiac anomalies, and eye anomalies; ICA: internal carotid artery; ACA: anterior cerebral artery; PCA: posterior cerebral artery.

* Must have intact circle of Willis.

Adapted from: Garzon MC, Epstein LG, Heyer GL, et al. PHACE Syndrome: Consensus-Derived Diagnosis and Care Recommendations. J Pediatr 2016; 178:24.

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Stroke risk stratification in patients with PHACE syndrome