Clinical Features and Outcomes of Intracranial Aneurysm Associated with Moyamoya Disease

Abstract

Background and Purpose Moyamoya disease (MMD) is a rare form of intracranial stenoocclusive disease that can be associated with intracranial aneurysms. We evaluated the clinical features and outcomes of MMD-associated aneurysms while focusing on their locations. Methods Between January 1998 and December 2018 there were 1,302 adult and pediatric patients diagnosed as MMD at a single institution. These patients included 38 with 44 MMD-associated aneurysms. The MMD-associated aneurysms were classified into two groups based on their locations: major-artery aneurysms and non-major-artery aneurysms. The clinical and radiological data for patients with MMD-associated aneurysms were reviewed retrospectively. Results The 44 MMD-associated aneurysms comprised 28 in major arteries and 16 in nonmajor arteries. All of the major-artery aneurysms were initially unruptured lesions, and follow-up angiography showed that 23 (82.1%) had an improved or stable status and 5 (17.9%) had a worse status. The non-major-artery aneurysms comprised 10 ruptured and 6 unruptured lesions, and follow-up angiography showed that 11 (68.8%) had improved or were stable and 5 (31.2%) had worsened. At the latest follow-up, there were four cases of unfavorable outcome: two initial hemorrhagic insults, one treatment-related morbidity, and one repeatedhemorrhage case. Conclusions MMD-associated aneurysms occurred in 3.3% of the MMD cohort in this study, of which 63.6% were major-artery aneurysms and 36.4% were non-major-artery aneurysms. The major-artery group included 17.9% that became angiographically worse, while 31.2% were growing or hemorrhaging in the non-major-artery group.

Endovascular treatment of main trunk aneurysms in the residual anterior circulation in moyamoya disease

“…22 Due to hemodynamic stress and attenuated arterial wall integrity, the main trunks of intracranial arteries can grow aneurysms. 5 In Kim et al’s 13 study of MMD-associated aneurysms, 63.6% of aneurysms were located on the main trunks of intracranial arteries.…”

“…In MMD, when main trunk aneurysms were initially unruptured lesions, follow-up angiography showed that 82.1% of aneurysms had a stable status and 17.9% had a worse status. 13 Therefore, the close follow-up was necessary. 14 However, in the real world, most aneurysms in MMD cannot be found until they rupture.…”

“…By searching PubMed, the report contained more cases than other previous reports. 3,[9][10][11][12][13][14]…”

Successful shrinkage of anterior communicating artery aneurysm after ACA–ACA bypass with interposed occipital artery graft in pediatric moyamoya disease: illustrative case

“…The natural clinical course of MMD-associated ACoA aneurysm remains unknown. While some researchers have stressed the devastating nature of MMD-associated aneurym, 16,17 others have suggested that such aneurysms can spontaneously disappear with the progression of MMD. 18,19 Spontaneous disappearance of ACoA aneurysm can occur in unilateral MMD if the contralateral ICA gradually becomes occluded.…”

Surgical Treatment of Moyamoya Disease

“…They are usually located in deep areas [11], small in size and with very fragile walls, making their treatment, endovascular or surgical, extremely challenging [11,12,14]. They can also be found at the circle of Willis [9,11,15,16], where they usually have a fusiform shape [11,17] and can lead to a subarachnoid haemorrhage [11]. The prognosis in the case of this type of haemorrhage is poor [11].…”