Facial Capillary Malformation,Ocular Anomalies and Cerebral Developmental Venous Anomalies

Abstract

To describe an uncommon vascular syndrome combining a facial Port-Wine-Stain (PWS) in the ophthalmic V1 trigeminal area, without evidencing the pial vascular malformation and subsequent cerebral lesions.

Six patients with facial PWS, comprising the ophthalmic V1 location, underwent clinical studies, plain radiographs and CT. Cerebral angiography was carried out in 4 patients, MR in 3, and MR, enhanced-MR and MR angiogram in 2 other cases.

All 6 patients had a facial PWS with V1 involvement, ocular anomalies (glaucoma or choroid angioma), ipsilateral to the facial port-wine-stain in 3 cases and bilateral in 3 cases, and extensive supratentorial DVAs. In all cases clinical examination was normal and no stigmates of leptomeningeal angioma, no brain anomalies were detected on radiological studies.

We focus on this particular vascular syndrome, a variant of classic SWS, with excellent brain tolerance, in contrast to what happens to patients with a leptomeningeal vascular malformation.