Intracranial Aneurysms in White Patients with Moyamoya Disease: A U.S. Single-Center Case Series and Review

Background: Intracranial aneurysms (IA) are associated with moyamoya disease (MMD). There are no clinically tested treatment guidelines. Reporting of cases is vital to better understand the underlying pathophysiology and potential ethnic predispositions and improve patient selection for intervention. Methods: Records of all patients diagnosed with MMD with concomitant IA who presented to our institution were retrospectively reviewed. Data related to demographic, clinical, MMD characteristics, aneurysm characteristics, surgical intervention, and follow-up were collected from the records of each patient. Aneurysm location was categorized into circle of Willis (CoW) aneurysms (originating from the CoW or its major branches) and peripheral aneurysms (arising from choroidal or lenticulostriate arteries). Results: Ten patients were found to have a total of 14 IA. All patients were white. Ten aneurysms (71%) were classified as CoW aneurysms and 4 (29%) were classified as peripheral. Seven of 10 CoW aneurysms (70%) were located in the anterior circulation, whereas 3 (30%) were located in the posterior circulation. Aneurysms of anterior and posterior circulations were most commonly treated with coil embolization, whereas peripheral artery aneurysms were most commonly treated with either cerebral revascularization alone or aneurysm excision. Conclusions: Revascularization surgery seems to be an effective method of indirectly treating IA in patients with MMD. Previous literature in addition to our series shows that endovascular embolization is safe and efficacious in treating IA of most locations in patients with MMD. The use of open microsurgery for direct aneurysm treatment in this population poses many challenges.