[The Wyburn-Mason syndrome (author's transl)].

A patient aged 39 years with Wyburn-Mason syndrome is presented. He had an apple-sized intracranial arteriovenous angioma racemosum of the left temporo-medical region, which was inoperable, as it extended to the basal ganglia. It was diagnosed by carotid angiography and by sequential brain szintigraphy. More than 50 retinal arteriovenous anastomoses were observed in the left eye, involving all quadrants and the macular region. Some of these anastomoses were only found by fluorescence angiography. Clinical signs were hemiparesis and decreased sensibility of the right side, headaches, speech disorders and right homonymous hemianopia. Vision was decreased to 0.4. By partial embolisation via the right common carotid, it was possible to reduce the size of the intracranial hemangioma.