Kim Sunghan, Jang Chang Ki, Park Eun Kyung, Shim Kyu Won, Kim Dong Seok, Chung Joonho, Kim Yong Bae, Lee Jae Whan, Park Keun Young
Department of Neurosurgery, Bucheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Department of Neurosurgery, Severance Stroke Center, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
J Clin Neurol. 2020 Oct;16(4):624-632. doi: 10.3988/jcn.2020.16.4.624.
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.
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.
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 repeated-hemorrhage case.
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.
烟雾病（MMD）是一种罕见的颅内狭窄闭塞性疾病，可伴有颅内动脉瘤。我们评估了烟雾病相关动脉瘤的临床特征和预后，重点关注其位置。
1998年1月至2018年12月期间，一家机构共诊断出1302例成人和儿童烟雾病患者。这些患者中有38例患有44个烟雾病相关动脉瘤。烟雾病相关动脉瘤根据其位置分为两组：主要动脉动脉瘤和非主要动脉动脉瘤。对烟雾病相关动脉瘤患者的临床和放射学数据进行回顾性分析。
44个烟雾病相关动脉瘤中，28个位于主要动脉，16个位于非主要动脉。所有主要动脉动脉瘤最初均为未破裂病变，随访血管造影显示23个（82.1%）病情改善或稳定，5个（17.9%）病情恶化。非主要动脉动脉瘤包括10个破裂和6个未破裂病变，随访血管造影显示11个（68.8%）病情改善或稳定，5个（31.2%）病情恶化。在最近一次随访时，有4例预后不良：2例最初有出血性损伤，1例与治疗相关的并发症，1例反复出血病例。
本研究中烟雾病相关动脉瘤在烟雾病队列中的发生率为3.3%，其中63.6%为主要动脉动脉瘤，36.4%为非主要动脉动脉瘤。主要动脉组中血管造影显示病情恶化的占17.9%，而非主要动脉组中有31.2%在生长或出血。
