Moyamoya disease associated with a brain stem glioma

An 8-year-old boy was found to have primary moyamoya disease associated wit h a brain stem glioma. For over 3 years the child had experienced transient ischemic attacks induced by hyperventilation. One month before referral to our hospital he had presented with progressive left facial nerve palsy. Ma gnetic resonance imaging showed a cystic mass in the lower pens. Angiograph y revealed severe bilateral stenosis of the internal carotid arteries and p rominent moyamoya vessels in the basal ganglia. Partial resection of the tu mor yielded a histological diagnosis of pilocytic astrocytoma. Local radiat ion therapy reduced the size of the tumor. Anastomosis of the superficial t emporal arteries and middle cerebral arteries on both sides was then perfor med. After direct bypass surgery, the patient remained in a good condition for a 5-year follow-up period. Clinical investigation of the coincidence of primary moyamoya disease and brain stem glioma led the authors to conclude that these two diseases coexisted independently.