Concurrent Moyamoya-like Intracranial Steno-occlusive Disease and Dural Arterio-Venous Fistulae

Introduction: The simultaneous presentation of Moyamoya disease or moyamoya-like vasculopathy and dural arteriovenous fistulas (DAVFs), two rare cerebrovascular entities, in patients has been documented in very few case reports worldwide. We aimed to better characterize this association by retrospectively reviewing and analyzing four cases evaluated in our institution.

Methods: We reviewed the clinical and radiological findings of four patients with concurrent Moyamoya or moyamoya-like vasculopathy and DAVFs evaluated in our institution.

Results: All four patients were of Asian descent. One patient presented with ischemic stroke secondary to intracranial stenosis, two presented with symptoms related to the DAVF, and the diagnosis was incidental in the fourth patient. Three patients underwent embolization of the DAVF, which was followed by surgical ligation in 2. One patient underwent extracranial-intracranial bypass for Moyamoya-like intracranial steno-occlusive disease. One patient is being managed conservatively with close follow-up.

Conclusion : Our case series suggests a causal association between Moyamoya-like intracranial vasculopathy and DAVF based on proposed pathophysiologic mechanisms and cohort characteristics.