Sturge Weber syndrome


Sturge Weber syndrome in a 20 month old child.

The left cerebral hemisphere is small in keeping with previously demonstrated atrophy related to extensive cortical surface malformation of Sturge-Weber syndrome

Extensive surface enhancement is shown over the left cerebral hemisphere and this is particularly prominent over the occipital lobe . There is relative sparing of the surface over the temporal lobe. The ventricular size is normal.

There is anomalous deep venous drainage of both hemispheres. Small veins in the left frontal white matter pass to join a vein beneath the left caudate head which travels along the septum pellucidum in the midline, enlarges as it goes posterior where it is tortuous and joins a single internal cerebral vein which appears to arise from the right and continues to join the vein of Galen. There is a tortuous and large vein from the region of the left choroidal fissure which joins the vein of Galen and this is likely a variant of the basal vein of Rosenthal. It receives a collection of small veins originating more superiorly adjacent to the left trigone.

The major venous sinuses show normal appearances. Numerous cortical veins reach the sagittal sinus from the right but there is only a single cortical vein of any size on the left which is at about the parieto-occipital junction. Its course is quite tortuous. A large surface vein is shown on the right overlying the temporal lobe coursing along the sylvian fissure to reach the right transverse sinus. There is apparently normal cortical venous drainage evident over the left temporal lobe.


Preoperative evaluation showing extensive pial malformation over the left cerebral hemisphere with volume loss. There is a anomalous deep venous drainage. No right sided cortical abnormality is shown.