Cervical and Intracranial Arterial Anomalies in 70 Patients with PHACE Syndrome

• DOI: 10.3174/ajnr.a2206
• Publication Date: 2010-08-13T01:06:54Z
• Authors: C.P. Hess; H.J. Fullerton; D.W. Metry; B.A. Drolet; D.H. Siegel; K.I. Auguste; N. Gupta; A.N. Haggstrom; C.F. Dowd; I.J. Frieden; A.J. Barkovich
• Keywords: Dysgenesis; Abnormality

<h3>BACKGROUND AND PURPOSE:</h3> Cerebral and cervical arterial abnormalities are the most common non-cutaneous anomaly in PHACE syndrome, but location type of lesions that occur have not been systematically assessed a large cohort. Our aim was to characterize phenotypic spectrum arteriopathy, assess frequency with which different arteries involved, evaluate spatial relationships between brain structural lesions, hemangiomas syndrome. <h3>MATERIALS METHODS:</h3> Intracranial MRA and/or CTA images from 70 children accompanying MR 59 patients arteriopathy syndrome were reviewed identify abnormalities. Five categories identified used for classification: dysgenesis, narrowing, nonvisualization, primitive embryonic carotid-vertebrobasilar connections, anomalous course or origin. Univariate logistic regression analyses performed test associations location, hemangiomas, <h3>RESULTS:</h3> By study design, all had abnormalities, 57% &gt;1 form arteriopathy. Dysgenesis abnormality (56%), followed by origin (47%), narrowing (39%), nonvisualization (20%). Primitive connections present 20% children. Hemangiomas ipsilateral 1 case. The frontotemporal mandibular facial segments involved 97% cases, no other specific hemangioma sites detected. All cases posterior fossa anomalies either ICA persistent carotid-basilar connections. <h3>CONCLUSIONS:</h3> commonly involves its branches, cutaneous hemangioma, dysgenesis abnormal noted Brain also typically ipsilateral.