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Background: Indirect revascularization is simple and safe, but it is not commonly used in adult moyamoya disease owing to its unreliable effect. This prospective study aimed to evaluate the effectiveness of indirect revascularization in treating adult moyamoya disease after refinement of the surgical technique and perioperative care., Methods: Adult patients who underwent indirect revascularization as the primary treatment for moyamoya disease between November 2013 and January 2017 were studied. The indirect revascularization procedures included encephalo-duro-arterio-synangiosis and encephalo-myo-synangiosis in the temporal region and encephalo-pericranio-synangiosis in other hypoperfusion areas. The preoperative and postoperative clinical conditions, cerebral angiography findings, and time-to-peak prolongation areas on magnetic resonance perfusion studies were assessed to evaluate the revascularization effect. Refinements of the surgical technique included the selection of surgical area based on perfusion imaging, craniotomy size, and number of affected hemispheres, along with the inclusion of a dural flap in every procedure., Results: Nineteen patients (17 females and 2 males), and a total of 31 cerebral hemispheres, were included. The mean patient age was 32.7 ± 11.4 years. After a mean follow-up of 38.6 ± 11.5 months, all patients showed clinical improvement or stabilization. Available postoperative angiography demonstrated Matsushima grade A or B in 19 of 21 hemispheres. The mean time-to-peak prolongation index of all 31 hemispheres improved from 24.09 ± 9.83% preoperatively to 12.82 ± 6.75% at 3-6 months after surgery (P < 0.001). The complication rate was 12.9%, and all complications were transient., Conclusions: With refinements of surgical techniques and perioperative care, indirect revascularization is more reliable and can be a viable alternative treatment for adult moyamoya disease. In our cohort, outcomes were satisfactory, with mean 38.6-month follow-up., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Moyamoya disease (MMD) is a disorder characterized by stenosis of bilateral internal carotid arteries with compensatory angiogenesis of the perforating blood vessels. Familial transmission in MMD is common. Recently, mutations in human RNF213 and ACTA2 genes were identified to be responsible for MMD. The present study was to determine whether Taiwanese MMD patients carried mutations in these two genes. Of the 36 MMD patients, eleven was found to have RNF213 mutations. Direct genetic sequencing identified four different RNF213 mutations in the 11 patients from 8 families: five with a p.R4810K, one with p.A1622V, one with p.V3933M, and the other one with p.R4131C. The latter three represent novel missense mutations. No mutation in ACTA2 gene was identified. Clinically, cerebral infarction was common in patients with an RNF213 mutation (9/11). In addition, four mutant patients had developmental delay (4/11) and two had mental dysfunction (2/11). The magnetic resonance angiography of asymptomatic mutant carriers demonstrated high incidence of multiple stenosis of intracranial vessels (3/6, 50%). Since 30.6% (11/36) of Taiwanese moyamoya patients carry an RNF213 mutation and intracranial arterial stenosis was found in half of the asymptomatic mutant carriers, it is suggested that the RNF213 mutation should form part of the diagnostic workup for MMD in clinical practice., (Copyright © 2015 Elsevier B.V. All rights reserved.)

Objective: It has been noted that the posterior circulation serves as an important source of collateral blood supply in moyamoya disease. Since most of the literature has focused on non-operative cases and many symptomatic patients receive surgical revascularization, we evaluated the posterior circulation changes after revascularization and found that progressive posterior cerebral artery (PCA) steno-occlusive changes after revascularization caused cerebral hemodynamic compromise and clinical deterioration in a significant portion of patients., Methods: Twenty-three moyamoya disease patients with ischemic presentation who received revascularization with complete angiography and xenon CT during a minimum of 3 years' clinical follow-up were enrolled. Revascularization was performed in 38 hemispheres. Pre- and postoperative angiography were reviewed to determine the internal carotid artery (ICA) stage, PCA stage, leptomeningeal collateral (LMC) grade, and Matsushima synangiosis grade. The postoperative regional cerebral blood flow (CBF) and cerebral vascular reserve (CVR) were recorded and correlated with angiographic findings and clinical outcome., Results: Progression of ICA staging was noted in 23 sides (55.2%), and progression of PCA staging was noted in 18 sides (47.4%). Among the 18 cases of PCA stage progression, an associated decrease in LMC grade was noted in 12 sides (66.7%). These changes were associated with decreased regional CBF and CVR, which also explained the recurrent ischemic symptoms in 27.8% of these patients. In contrast, LMC grade increased in 15 (65.2%) sides of patients with ICA progression., Conclusions: Progressive steno-occlusive change in the PCA after revascularization is associated with a reduction in LMC blood flow and cerebral ischemia in moyamoya patients. This phenomenon might cause recurrent ischemic symptoms in 27.8% of patients., (Copyright (c) 2009 S. Karger AG, Basel.)

The authors present a rare case of an infarction complication 15 days following acute intraventricular bleeding due to moyamoya disease. Before the infarction occurred, perfusion CT imaging disclosed early but reversible ischemic injury on the day of hemorrhage. Dehydration and hypotension are both possibly contributing factors of progressive injury from reversible ischemia due to infarction. Although the patient underwent successful bypass surgery, 1 month after the ictus the neurobehavior evaluation still showed marked executive dysfunction. The authors address that, in hemorrhagic-type moyamoya disease, early perfusion CT scanning is not only a powerful tool to identify the high-risk group of patients who could experience subacute infarction, but also alarms neurosurgeons to eliminate any predisposing factors when it shows reversible ischemic injuries.