Funaki Takeshi, Takahashi Jun C, Takagi Yasushi, Kikuchi Takayuki, Yoshida Kazumichi, Mitsuhara Takafumi, Kataoka Hiroharu, Okada Tomohisa, Fushimi Yasutaka, Miyamoto Susumu
Departments of 1 Neurosurgery and.
J Neurosurg. 2015 Feb;122(2):400-7. doi: 10.3171/2014.10.JNS14231. Epub 2014 Nov 28.
Unstable moyamoya disease, reasonably defined as cases exhibiting either rapid disease progression or repeated ischemic stroke, represents a challenge in the treatment of moyamoya disease. Despite its overall efficacy, direct bypass for such unstable disease remains controversial in terms of safety. This study aims to reveal factors associated with unstable disease and to assess its impact on postoperative silent or symptomatic ischemic lesions.
This retrospective cohort study included both pediatric and adult patients with moyamoya disease who had undergone 140 consecutive direct bypass procedures at Kyoto University Hospital. "Unstable moyamoya disease" was defined as either the rapid progression of a steno-occlusive lesion or repeat ischemic stroke, either occurring within 6 months of surgery. The extent of progression was determined through a comparison of the findings between 2 different MR angiography sessions performed before surgery. The clinical variables of the stable and unstable disease groups were compared, and the association between unstable disease and postoperative diffusion-weighted imaging (DWI)-detected lesion was assessed through univariate and multivariate analyses with generalized estimating equations.
Of 134 direct bypass procedures performed after patients had undergone at least 2 sessions of MR angiography, 24 (17.9%) were classified as cases of unstable disease. Age younger than 3 years (p=0.029), underlying disease causing moyamoya syndrome (p=0.049), and radiographic evidence of infarction (p=0.030) were identified as factors associated with unstable disease. Postoperative DWI-defined lesions were detected after 13 of 140 procedures (9.3%), although only 4 lesions (2.9%) could be classified as a permanent complication. The incidence of postoperative DWI-detected lesions in the unstable group was notable at 33.3% (8 of 24). Univariate analysis revealed that unstable disease (p<0.001), underlying disease (p=0.028), and recent stroke (p=0.012) were factors associated with DWI-detected lesions. Unstable disease remained statistically significant after adjustment for covariates in both the primary and sensitivity analyses (primary analysis: OR 6.62 [95% CI 1.79-24.5]; sensitivity analysis: OR 5.36 [95% CI 1.47-19.6]).
Unstable moyamoya disease, more prevalent in younger patients and those with underlying disease, is a possible risk factor for perioperative ischemic complications. Recognition of unstable moyamoya disease may contribute to an improved surgical result through focused perioperative management based on appropriate surgical risk stratification.
不稳定型烟雾病合理定义为疾病进展迅速或反复发生缺血性卒中的病例，是烟雾病治疗中的一项挑战。尽管直接搭桥术总体疗效良好，但对于此类不稳定型疾病，其安全性仍存在争议。本研究旨在揭示与不稳定型疾病相关的因素，并评估其对术后无症状或有症状缺血性病变的影响。
这项回顾性队列研究纳入了在京都大学医院连续接受140例直接搭桥手术的烟雾病儿科和成年患者。“不稳定型烟雾病”定义为狭窄闭塞性病变迅速进展或反复发生缺血性卒中，且均发生在手术6个月内。通过比较手术前两次不同的磁共振血管造影检查结果来确定病变进展程度。比较稳定型和不稳定型疾病组的临床变量，并通过广义估计方程进行单因素和多因素分析，评估不稳定型疾病与术后弥散加权成像（DWI）检测到的病变之间的关联。
在患者至少接受两次磁共振血管造影检查后进行的134例直接搭桥手术中，24例（17.9%）被归类为不稳定型疾病病例。年龄小于3岁（p=0.029）、导致烟雾病综合征的基础疾病（p=0.049）以及梗死的影像学证据（p=0.030）被确定为与不稳定型疾病相关的因素。140例手术中有13例（9.3%）术后检测到DWI定义的病变，尽管只有4例病变（2.9%）可归类为永久性并发症。不稳定型组术后DWI检测到病变的发生率显著为33.3%（24例中的8例）。单因素分析显示，不稳定型疾病（p<0.001）、基础疾病（p=0.028）和近期卒中（p=0.012）是与DWI检测到的病变相关的因素。在主要分析和敏感性分析中对协变量进行调整后，不稳定型疾病在统计学上仍具有显著意义（主要分析：比值比6.62[95%可信区间1.79-24.5]；敏感性分析：比值比5.36[95%可信区间1.47-19.6]）。
不稳定型烟雾病在年轻患者和有基础疾病的患者中更为普遍，是围手术期缺血性并发症的一个可能危险因素。识别不稳定型烟雾病可能有助于通过基于适当手术风险分层的针对性围手术期管理改善手术结果。
