Multifocal arteriovenous malformations and facial nevus without leptomeningeal angioma: a variant form of Sturge-Weber syndrome? A case report and review of the literatures

Abstract

Sturge–Weber syndrome (SWS) is a congenital, nonfamilial neurocutaneous syndrome typically presenting with a facial port-wine stain within the trigeminal nerve distribution and ipsilateral leptomeningeal angiomatosis [1, 2, 3, 4, 5]. An arteriovenous malformation (AVM) is not an essential component of SWS, particularly if the above features are lacking [6]. An AVM may have similarities in common with a leptomeningeal angiomatosis especially when a fetal developmental error occurs within venous or capillary beds [7]. For this reason, an AVM can be regarded as another component of SWS and, in broader sense, may be defined as atypical SWS.We report a rare case of a 10-year-old boy with two AVMs and a facial port-wine nevus of the ipsilateral side. On the basis of the patient’s clinical and radiological features as well as a literature review, we carefully made a diagnosis of SWS in this patient.