A perfusion territory shift attributable solely to the secondary collaterals in moyamoya patients: a potential risk factor for preoperative hemorrhagic stroke revealed by t-ASL and 3D-TOF-MRA
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Abstract:
The authors conducted a study to noninvasively and nonradioactively reveal moyamoya disease (MMD) intracerebral perfusion and perfusion territory supplied by the unilateral internal carotid artery (ICA) and external carotid artery (ECA) and bilateral vertebral arteries (VAs) before surgery and to further identify risk factors for preoperative hemorrhage in adult MMD.Keywords:
Moyamoya Disease
Stroke
Univariate analysis
Moyamoya disease is a cerebrovascular disorder characterized by bilateral stenosis or occlusion of the terminal portions of the internal carotid arteries accompanied by typical net-like collateral vessels in the basal ganglia. Although the etiology of moyamoya disease remains unknown, hereditary and immunogenic as well as hemodynamic factors have been implicated in the underlying mechanism of moyamoya disease. We report two patients with confirmed moyamoya disease and a patient with probable moyamoya disease complicated with Graves' disease. We reviewed the literature and summarized 23 cases of moyamoya disease or probable moyamoya, coexisting with Graves' disease.
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Moyamoya Disease
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Moyamoya disease (MMD) is characterized by progressive occlusion of the internal carotid artery or its terminal branches, associated with formation of extensive collateral vessels (moyamoya vessels) at the base of the brain. Whether unilateral moyamoya disease, confirmed by typical angiographic evidence of moyamoya disease unilaterally and normal or equivocal findings contralaterally, is an early form of definite (bilateral) moyamoya disease remains controversial. Inherited or acquired disorders and conditions may present in conjunction with moyamoya disease. This condition is known as quasi-moyamoya disease (quasi-MMD). We attempted to determine the incidence and total patient number of moyamoya disease, unilateral MMD and quasi-MMD, who were treated during 2005 in Japan. Questionnaires were sent to 2,998 departments, which are listed in resident training programs of neurosurgery, neurology and pediatrics. Totally, 1,183 departments replied, and the response rate was 39.5%. The number of annual first-visit patients of MMD, unilateral MMD and quasi-MMD is 571, 118, and 53, respectively. Thus, the number of annual revisit patients of MMD, unilateral MMD and quasi-MMD is 2,064, 214, and 117 respectively. It is estimated that 6,670.9 MMD patient exists in Japan. The incidence rate of MMD, unilateral MMD and quasi-MMD is 1.13, 0.23 and 0.11/100,000, respectively, and the prevalence is 5.22, 0.66 and 0.34/100,000, respectively. This nationwide study revealed the present epidemic status of MMD, unilateral MMD and quasi-MMD.
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Moyamoya disease is a rare, progressive cerebral vasculopathy which most commonly presents in the first and fourth decades of life. The mainstay of treatment is surgical revascularization; without treatment, most patients experience ischemic or hemorrhagic strokes. This report reviews moyamoya disease, its associated conditions, surgical treatment techniques, and anesthetic management of patients with moyamoya disease.
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Moyamoya disease is a chronic progressive cerebral vascular disease. The initial onset of moyamoya disease is cerebral ischemia or intracranial hemorrhage. Aneurysm combined with hemorrhagic type moyamoya disease plays an important role on its prognosis. The major reason of intracranial hemorrhage in hemorrhagic type moyamoya disease is the rupture of anterior choroidal artery. Preventing the rebleeding of moyamoya disease is the key of treatment.【
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Moyamoya Disease
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Interventional radiology
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Objective: To investigate the clinical efficacy of superficial temporal artery -middle cerebral artery combined with encephalo-duro-arterio-myo-synangiosis (STA-MCA+EDAMS) and encephalo-duro-arterio-myo-synangiosis (EDAMS) in the treatment of adult moyamoya disease. Methods: The clinical data of 47 adult patients with moyamoya disease who received vascular reconstruction in the Department of Neurosurgery of Taizhou Hospital of Zhejiang Province from January 2014 to January 2018 were retrospectively analyzed. Among them, 21 patients received EDAMS alone (EDAMS group, 14 patients with hemorrhagic moyamoya disease, 7 patients with ischemic moyamoya disease), 26 patients received STA-MCA combined with EDAMS (STA-MCA+EDAMS group, 17 patients with hemorrhagic moyamoya disease, 9 patients with ischemic moyamoya disease). Cerebral hemodynamics at 1 day before surgery and 3 and 6 months after surgery were compared. The clinical efficacy and postoperative complications of the two methods were compared at 3 and 6 months postoperatively in hemorrhagic and ischemic types. Results: For hemorrhagic moyamoya disease, the remission rate (94.1%) at 6 months after surgery in the STA-MCA + EDAMS group was higher than that in the EDAMS group (57.1%), and the difference was statistically significant (P<0.05). The CBF and CBV in the STA-MCA+EDAMS group were higher than those in the EDAMS group at 3 and 6 months after operation, and the MTT and TPP were lower than those in the EDAMS group, but there was no significant difference between the two groups (all P>0.05). For hemorrhagic moyamoya disease and ischemic moyamoya disease, the total incidence of postoperative complications of the two surgical methods was different, but the difference was not statistically significant (both P>0.05). Conclusion: Superficial temporal artery -middle cerebral artery combined with encephalo-duro- arterio-myo-synangiosis (STA-MCA+EDAMS) and encephalo-duro-arterio-myo-synangiosis (EDAMS) can significantly improve neurological function and cerebral hemodynamics in adult moyamoya disease patients with high safety.目的： 探讨颞浅动脉-大脑中动脉搭桥联合脑-硬膜-动脉-颞肌贴敷术（STA-MCA+EDAMS）以及脑-硬膜-动脉-颞肌贴敷术（EDAMS）治疗成人烟雾病的安全性和有效性。 方法： 回顾性分析2014年1月至2018年1月在浙江省台州医院神经外科接受血管重建术治疗的47例成人烟雾病患者的临床资料，其中接受单纯EDAMS者21例（EDAMS组），其中出血型烟雾病14例，缺血型烟雾病7例；接受STA-MCA联合EDAMS者26例（STA-MCA+EDAMS组），其中出血型烟雾病17例，缺血型烟雾病9例。比较2组术前1 d及术后3、6个月的脑血流动力学指标，按出血型和缺血型分别比较2种术式术后3、6个月的临床疗效及术后并发症情况。 结果： 出血型烟雾病中STA-MCA+EDAMS组术后6个月的缓解率为94.1%（16/17），EDAMS组为57.1%（8/14），差异有统计学意义（P<0.05）。STA-MCA+EDAMS组术后3、6个月的脑血流量、脑血容量高于EDAMS组，平均通过时间、达峰时间低于EDAMS组，但差异均无统计学意义（均P>0.05）。不管是出血型烟雾病还是缺血型烟雾病，两种术式术后总并发症发生率差异均无统计学意义（均P>0.05）。 结论： 颞浅动脉-大脑中动脉搭桥联合脑-硬膜-动脉-颞肌贴敷术以及脑-硬膜-动脉-颞肌贴敷术治疗成人烟雾病均可显著改善患者神经功能和脑血流动力学指标，安全性较高。.
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✓ Whether a diagnosis of moyamoya disease is justified in patients with typical angiographic evidence of moyamoya disease unilaterally and normal angiographic findings contralaterally remains controversial. In this study the authors analyzed longitudinal angiographic change, familial occurrence, and basic fibroblast growth factor (bFGF) concentration in the cerebrospinal fluid (CSF) of patients with “unilateral” moyamoya disease. Over a 10-year period, 10 cases of unilateral moyamoya disease were followed using conventional angiography or magnetic resonance angiography. Basic FGF in CSF, obtained from the subarachnoid space of the cerebral cortex during revascularization surgery, was measured in five cases. Among the 10 cases of unilateral moyamoya disease, only one pediatric case showed obvious signs of progression to typical bilateral disease. The other nine cases (including six adults and three children) remained stable throughout follow-up radiological examinations (magnetic resonance angiography) with a mean observation period of 3.5 years. There was no familial occurrence in these cases of unilateral moyamoya disease. Levels of bFGF, which are high in typical moyamoya disease, were low in these patients. The progression from unilateral moyamoya disease to the typical bilateral form of the disease appears to be infrequent. The low levels of bFGF in the CSF of these patients and the lack of familial occurrence strongly suggest that most cases of unilateral moyamoya disease, especially those found in adults, are distinct from typical bilateral moyamoya disease.
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Magnetic resonance angiography
Subarachnoid space
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An unverified disease called "Moyamoya Disease" or "Spontaneous occlusion of the circle of Willis" has been recently reported as a disease entity by some Japanese researchers. Since the first report of this disease by Shimizu and the author in 1955, many cases have been reported not only in Japan but in many countries outside Japan. It has been already clarified either clinically or pathologically, that, in the Moyamoya Disease, the most important finding is the basal arterial occlusive change of unknown etiology and the Moyamoya Phenomenon is only nonspecific neuroradiological change as the extraordinary dilated collaterals via the striate arteries, perforators etc. However, the real cause of the arterial obstruction is still obscure in the so-called "true Moyamoya Disease". Further studies will be necessary in order to establish a new clinical entity related to the Moyamoya Disease. However, under existing situations, the Moyamoya Disease must be strictly differentiated from the Moyamoya Phenomenon which can be frequently observed among cases with basal occlusion of known and unknown origin.
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Circle of Willis
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