Arterial Spin-Labeling Perfusion for PHACE Syndrome

Area of Science:

• Neurology
• Pediatric Radiology
• Vascular Malformations

Background:

• Posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities (PHACE) syndrome is associated with arterial stroke.
• Stroke risk is currently estimated by MRA-identified arterial anomaly severity, lacking evidence-based support.

Purpose of the Study:

• To evaluate if arterial spin-labeling (ASL) MR imaging perfusion can identify cerebral blood flow (CBF) alterations in PHACE syndrome patients.
• To correlate ASL findings with arterial anomalies and parenchymal brain findings.

Main Methods:

• Retrospective review of ASL MR imaging from 41 PHACE syndrome patients across three institutions (2000-2019).
• Qualitative assessment of CBF using ASL to detect decreased or normal perfusion.
• Characterization of arterial anomalies on MRA and evaluation of brain findings on conventional MRI.

Main Results:

• 10 out of 41 (24%) PHACE syndrome patients showed decreased CBF signal in a major arterial territory.
• Decreased CBF was observed in anterior circulation (10/10), with some involving posterior circulation or bilateral anterior circulation.
• 97.5% of patients had arteriopathies, which corresponded to the ASL-detected CBF alterations in all cases with reduced flow.

Conclusions:

• Arterial spin-labeling is a promising technique for characterizing hemodynamic changes in PHACE syndrome.
• ASL may provide objective evidence of perfusion deficits related to arterial anomalies in PHACE syndrome.