Affiliations: Department of Pediatric Radiology, Royal Children's
Hospital, Flemington Road, Parkville, Melbourne, Victoria, Australia | Department of Pediatric Radiology, Red Cross
Children's Hospital, School of Child and Adolescent Health, University of Cape
Town, South Africa
Note: [] Correspondence: Savvas Andronikou, F.R.C.R., Department of
Pediatric Radiology, Red Cross Children's Hospital, School of Child and
Adolescent Health, University of Cape Town, South Africa. Tel: +27 (0)21 658
5422; fax: +27 (0)21 658 5101; E-mail: docsav@mweb.co.za
Abstract: The fundamental abnormality in the Sturge-Weber 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).
