MRI evaluation of venous abnormalities in children with Sturge-Weber syndrome

The fundamental abnormality in the SturgeWeber syndrome (SWS) is considered to be the lack of superficial cortical draining veins, which results in numerous collateral pathways of venous flow. Venous abnormalities have been described in detail using angiography but magnetic resonance imaging (MRI) has replaced angiography in the diagnosis of SWS. In this paper we aim to demonstrate the range and evolution of venous abnormalities in the SWS as seen on standard MRI sequences. Retrospective review of 16 MRI scans in a group of ten children with SWS was performed by two pediatric radiologists with emphasis on venous abnormalities. Eight patients had unilateral angiomas and five of these had ipsilateral choroid plexus hypertrophy. Two patients had bilateral angiomas both of which had bilateral choroid plexus hypertrophy. In two patients, the vein of galen was enlarged. Four children had abnormal venous structures including intramedullary veins and enlarged subependymal veins. There was evolution of the venous abnormalities in four of the five cases with follow-up imaging. Three patients had associated cerebral malformations. The predominant venous abnormalities in patients with SWS demonstrated by conventional MRI sequences included anomalous parenchymal veins. Deep venous enlargement as previously described on angiography, was an uncommon finding using MRI. Evolution of venous abnormalities was demonstrated in four of five patients who underwent follow-up examination. (J Pediatr Neurol 2004; 2(1): 29-32).