A case of left facial port-wine stain with right hemiparesis is reported. Radiologically, two arteriovenous malformations (AVM) and diffuse venous abnormalities were observed in the left hemisphere. AVM were seen in the basal ganglia. One was fed by a perforating artery from the MCA and drained into the superior petrosal sinus, and the other was fed by a perforating artery from the basilar artery and drained through the vein of Rosenthal into an extremely dilated vein of Galen. Venous abnormalities were obstruction and a decrease in the superficial cortical veins, as well as the formation of collateral veins in both the cortex and white matter. This case resembles Sturge-Weber syndrome (SWS), but there were no signs of leptomeningeal angiomatosis or venous angioma. We have been unable to find any case reports on SWS with AVM or AVM with diffuse venous abnormalities. We discuss the differences between our case and SWS.
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