Deniz Güven, Emel Örün, Muhammed Erkan Emrahoğlu, Şükriye Yılmaz
Trends in Pediatrics - 2025;6(3):208-214
Moyamoya disease (MMD) is a rare, chronic, progressive intracranial arteriopathy primarily affecting children. It is characterized by stenosis or occlusion of the internal carotid and cerebral arteries, often presenting with ischemia or hemorrhagic strokes. The co-occurrence of MMD and cerebral arteriovenous malformation (AVM) is exceedingly rare. We report the case of a seven-year-old male who presented with persistent intraoral bleeding. Physical examination revealed a cavernous hemangioma in the palatal mucosa and a hyperpigmented epidermal nevus. Magnetic resonance imaging identified an intracerebral AVM, subsequently diagnosed as MMD. The patient underwent anterior interhemispheric AVM resection and received prophylactic antiplatelet therapy. He remains under clinical follow-up with no complications or neurological sequelae. Recognizing dermatological manifestations associated with MMD is essential, particularly when concurrent vascular anomalies are present. A multidisciplinary approach, incorporating both endovascular and surgical strategies, is critical in managing such complex cases.
