How I do it: operative nuances of the Encephalo-Duro-Mio-Synangiosis (EDMS) technique for pediatric moyamoya disease and syndrome.

BACKGROUND: Moyamoya disease, a progressive occlusive arteriopathy mainly affecting the supraclinoid internal carotid artery, leads to abnormal "Moyamoya vessels" and ischemic events in children due to decreased cerebral blood flow. Surgery, especially indirect revascularization, is suggested for pediatric Moyamoya cases. METHOD: We present the Encephalo-Duro-Mio-Synangiosis (EDMS) technique, illustrated with figures and videos, based on 14 years' experience performing 71 surgeries by the senior author (SGJ) and the Moyamoya Interdisciplinary Workteam at "Prof. Dr. J. P. Garrahan" Pediatric Hospital. CONCLUSION: EDMS is a simple and effective treatment for Moyamoya disease, enhancing procedure precision and safety, reducing associated risks, complications, and improving clinical outcomes.

Outcomes of Bypass Surgery in Adult Moyamoya Disease by Onset Type.

Importance: Moyamoya disease (MMD) is a rare chronic cerebrovascular disease, and the outcomes of bypass management in adult patients remain controversial. Objective: To categorize adult MMD based on asymptomatic, ischemic, and hemorrhagic onset and compare the outcomes (death, hemorrhagic stroke [HS], and ischemic stroke [IS]) of bypass surgery (direct or indirect) with those of conservative management. Design, Setting, and Participants: This retrospective, nationwide, population-based longitudinal cohort study used Korean National Health Insurance Research data to identify adults (aged ≥15 years) with MMD who were diagnosed between January 1, 2008, and December 31, 2020, and followed up until December 31, 2021 (median follow-up, 5.74 [IQR, 2.95-9.42] years). A total of 19 700 participants (3194 with hemorrhagic, 517 with ischemic, and 15 989 with asymptomatic MMD) were included. Data were analyzed from January 2 to April 1, 2023. Exposures: Bypass surgery and conservative management. Main Outcomes and Measures: Death constituted the primary outcome; secondary outcomes consisted of HS or IS. Kaplan-Meier survival curve and Cox proportional hazards regression analysis were applied. The propensity score-matching and stratified analyses were performed to control covariate effects. Results: A total of 19 700 patients (mean [SD] age, 45.43 [14.98] years; 12 766 [64.8%] female) were included. Compared with conservative management, bypass was associated with a reduced risk of death (adjusted hazard ratio [AHR], 0.50 [95% CI, 0.41-0.61]; P < .001) and HS (AHR, 0.36 [0.30-0.40]; P < .001) in hemorrhagic MMD; reduced risk of IS (AHR, 0.55 [95% CI, 0.37-0.81]; P = .002) in ischemic MMD; and reduced risk of death (AHR, 0.74 [95% CI, 0.66-0.84]; P < .001) in asymptomatic MMD. However, bypass was associated with an increased risk of HS (AHR, 1.76 [95% CI, 1.56-2.00]; P < .001) in asymptomatic MMD. Both direct and indirect bypass demonstrated similar effects in hemorrhagic and asymptomatic MMD, except only direct bypass was associated with a reduced risk of IS (AHR, 0.52 [95% CI, 0.33- 0.83]; P = .01) in ischemic MMD. After stratification, bypass was associated with a reduced risk of death in patients younger than 55 years with ischemic (AHR, 0.34 [95% CI, 0.13- 0.88]; P = .03) and asymptomatic (AHR, 0.69 [95% CI, 0.60-0.79]; P < .001) MMD, but an increased risk of HS in patients 55 years or older with ischemic MMD (AHR, 2.13 [95% CI, 1.1-4.16]; P = .03). Conclusions and Relevance: The findings of this cohort study of bypass outcomes for patients with MMD emphasize the importance of tailoring management strategies in adult patients based on onset types.

3D exoscopic versus microscopic superficial temporal artery to middle cerebral artery bypass surgery for moyamoya disease - a comparative series.

PURPOSE: Superficial temporal artery to middle cerebral artery (STA-MCA) direct bypass surgery is the most common surgical procedure to treat moyamoya disease (MMD). Here, we aim to compare the performance of the 3D exoscope in bypass surgery with the gold standard operative microscope. METHODS: All direct STA-MCA bypass procedures performed at a single university hospital for MMD between 2015 and 2023 were considered for inclusion. Data were retrospectively collected from patient files and surgical video material. From 2020 onwards, bypass procedures were exclusively performed using a digital three-dimensional exoscope as visualization device. Results were compared with a microsurgical bypass control group (2015-2019). The primary endpoint was defined as total duration of surgery, duration of completing the vascular anastomosis (ischemia time), bypass patency, number of stiches to perform the anastomosis, added stiches after leakage testing of the anastomosis and the Glasgow outcome scale (GOS) at last follow-up as secondary outcome parameter. RESULTS: A total of 16 consecutive moyamoya patients underwent 21 STA-MCA bypass procedures. Thereof, six patients were operated using a microscope and ten patients using an exoscope (ORBEYE® n = 1; AEOS® n = 9). Total duration of surgery was comparable between devices (microscope: 313 min. ± 116 vs. exoscope: 279 min. ± 42; p = 0.647). Ischemia time also proved similar between groups (microscope: 43 min. ± 19 vs. exoscope: 41 min. ± 7; p = 0.701). No differences were noted in bypass patency rates. The number of stiches per anastomosis was similar between visualization devices (microscope: 17 ± 4 vs. exoscope: 17 ± 2; p = 0.887). In contrast, more additional stiches were needed in microscopic anastomoses after leakage testing the bypass (p = 0.035). CONCLUSION: Taking into account the small sample size, end-to-side bypass surgery for moyamoya disease using a foot switch-operated 3D exoscope was not associated with more complications and led to comparable clinical and radiological results as microscopic bypass surgery.

Spontaneous occlusion of ruptured microaneurysm formed on postoperative transosseous anastomosis after indirect revascularization in a patient with moyamoya disease.

A 40-year-old female with a history of ischemic moyamoya disease treated with indirect revascularization at ages 12 and 25 years presented with a sudden severe headache. Imaging studies revealed focal parenchymal hemorrhage and acute subdural hematoma, confirming a microaneurysm formed on the postoperative transosseous vascular network as the source of bleeding. Conservative management was performed, and no hemorrhage recurred during the 6-month follow-up period. Interestingly, follow-up imaging revealed spontaneous occlusion of the microaneurysm. However, due to the rarity of this presentation, the efficacy of conservative treatment remains unclear. Further research on similar cases is warranted.

Vascular risk profile and changes of arterial hypertension after surgical revascularization in adult Moyamoya patients.

Moyamoya disease (MMD) is a rare stenoocclusive cerebral vasculopathy often treated by neurosurgical revascularization using extracranial-intracranial bypasses to prevent ischemic or hemorrhagic events. Little is known about the vascular risk profile of adult MMD patients compared to the general population. We therefore analyzed 133 adult MMD patients and compared them with data from more than 22,000 patients from the German Health Update database. Patients with MMD showed an age- and sex-adjusted increased prevalence of arterial hypertension, especially in women between 30 and 44 years and in patients of both sexes between 45 and 64 years. Diabetes mellitus was diagnosed significantly more frequently in MMD patients with increasing age, whereas the vascular risk profile in terms of obesity, nicotine and alcohol consumption was similar to that of the general population. Antihypertensive medication was changed one year after surgical revascularization in 67.5% of patients with a tendency towards dose reduction in 43.2% of all patients. After revascularization, physicians need to be aware of a high likelihood of changes in arterial hypertension and should adjust all other modifiable systemic vascular risk factors to achieve the best treatment possible.

[Imaging risk factors for postoperative cerebral infarction in adults with ischemic Moyamoya disease].

Objective: To investigate the imaging factors associated with postoperative cerebral infarction in adult patients aged 18 and above with ischemic Moyamoya disease. Methods: The clinical data of adult patients who underwent surgeries for ischemic Moyamoya disease in the Department of Neurosurgery at Peking University International Hospital from October 2015 to October 2020 were retrospectively analyzed. Of the 239 patients, 120 were male and 119 were female, with ages ranging from 18 to 63 (41.7±10.3) years. A total of 239 patients(290 cases) underwent direct and indirect combined revascularization (CR).Gender, age, surgical side, preoperative transient ischemic attack (TIA), presence of old cerebral infarction, and imaging features were compared between the patients with (48 cases) and without (242 cases) cerebral infarction within 1 week after surgery. Multivariate logistic binary regression model was used to analyze the imaging risk factors of postoperative cerebral infarction. Results: Cerebral infarction occurred in 48 cases(16.5%) among the 290 CR group within 1 week after surgery. The proportion of patients with TIA, old cerebral infarction, ICA stenosis, A1 segment stenosis, M1 segment stenosis, abnormal posterior cerebral artery (PCA), and unstable compensation before CR in the cerebral infarction group was higher than that in the non-cerebral infarction group (P<0.05).Preoperative TIA (OR=4.514, 95%CI: 1.920-10.611), old cerebral infarction (OR=2.856,95%CI:1.176-6.936), A1 stenosis (OR=7.027,95%CI:1.877-26.308), M1 stenosis (OR=6.968,95%CI:2.162-22.459), abnormal PCA (OR=4.114,95%CI:1.330-12.728)and unstable compensation (OR=4.488,95%CI:1.194-16.865) were risk factors for cerebral infarction after CR surgery (all P<0.05). Conclusion: Among the imaging factors, TIA, old cerebral infarction, A1 stenosis, M1 stenosis, abnormal PCA and unstable compensation were risk factors for cerebral infarction in adult patients with ischemic Moyamoya disease treated by combined revascularization.

Predictive value of the hemispheric magnetic resonance angiography score on the development of indirect pial synangiosis after combined revascularization surgery for adult moyamoya disease.

PURPOSE: It is difficult to precisely predict indirect bypass development in the context of combined bypass procedures in moyamoya disease (MMD). We aimed to investigate the predictive value of magnetic resonance angiography (MRA) signal intensity in the peripheral portion of the major cerebral arteries for indirect bypass development in adult patients with MMD. METHODS: We studied 93 hemispheres from 62 adult patients who underwent combined direct and indirect revascularization between 2005 and 2019 and genetic analysis for RNF213 p.R4810K. The signal intensity of the peripheral portion of the major intracranial arteries during preoperative MRA was graded as a hemispheric MRA score (0-3 in the middle cerebral artery and 0-2 in the anterior cerebral and posterior cerebral arteries, with a high score representing low visibility) according to each vessel's visibility. Postoperative bypass development was qualitatively evaluated using MRA, and we evaluated the correlation between preoperative factors, including the hemispheric MRA score and bypass development, using univariate and multivariate analyses. RESULTS: A good indirect bypass was observed in 70% of the hemispheres. Hemispheric MRA scores were significantly higher in hemispheres with good indirect bypass development than in those with poor indirect bypass development (median: 3 vs. 1; p < 0.0001). Multiple logistic regression analysis revealed hemispheric MRA score as an independent predictor of good indirect bypass development (odds ratio, 2.1; 95% confidence interval, 1.3-3.6; p < 0.01). The low hemispheric MRA score (< 2) and wild-type RNF213 predicted poor indirect bypass development with a specificity of 0.92. CONCLUSION: Hemispheric MRA score was a predictive factor for indirect bypass development in adult patients who underwent a combined bypass procedure for MMD. Predicting poor indirect bypass development may lead to future tailored bypass surgeries for MMD.

Natural course of hemodynamically stable hemispheres contralateral to operated hemispheres in adult patients with ischemic moyamoya diseases.

The necessity of bilateral bypass in adult moyamoya disease (MMD) remains unclear despite its recommendation for pediatric and hemorrhagic cases. We aimed to investigate the natural course of hemodynamically stable unoperated hemispheres after bypass surgery for symptomatic and hemodynamically unstable hemispheres in adult patients with ischemic MMD. Among 288 patients, the mean age at the first operation of the unstable hemispheres was 40.8 ± 12.2 years. The mean follow-up period was 62.9 ± 46.5 months. 45 patients (15.6%) experienced stroke events in the unoperated hemisphere, consisting of hemorrhagic stroke in 8 (2.8%) and ischemic stroke in 37 (12.8%), including progressive transient ischemic attack in 25 (8.7%) and infarction in 12 (4.2%). Among them, 39 patients (13.5%) underwent bypass surgery. The annual risk of total stroke is 3.0%/patient-year, with 2.5% for ischemic stroke and 0.5% for hemorrhagic stroke. The 5- and 10-year cumulative risks of ischemic stroke were 13.4% and 18.3%, respectively, and those of hemorrhagic stroke were each 3.2%. The natural course of hemodynamically stable hemispheres contralateral to the operated ones appeared fairly good. Additional bypass surgery on the unoperated hemispheres should be considered for symptomatic and hemodynamically unstable hemispheres in adult patients with ischemic MMD during the follow-up.

Development of the external carotid artery system including the middle temporal artery after direct-indirect revascularization surgery for moyamoya disease.

BACKGROUND: The aim of this study was to clarify when and from which blood vessels indirect revascularization develops after combined revascularization surgery for moyamoya disease and how the donor vessels that undergo direct revascularization change in the medium to long term. In particular, we focused on the middle temporal artery (MTA), which has not received much attention in indirect revascularization surgery for moyamoya disease until now. METHODS: We targeted 20 sides that were suitable for evaluating the diameter of the external carotid artery system involved in combined revascularization surgery among moyamoya disease patients who underwent a composite revascularization procedure utilizing a 'U'-shaped skin incision encircling the parietal branch of the superficial temporal artery (STA) at our institution from 2018 to 2023. We identified the STA parietal branch, MMA, DTA, and MTA in the TOF source MR images acquired preoperatively and three and six months after surgery; measured the long and short diameters of each blood vessel; approximated the blood vessel shape as an ellipse, and calculated its cross-sectional area. RESULTS: The cross-sectional areas of the MMA, DTA, and MTA involved in indirect revascularization significantly increased compared to presurgery three months after surgery, and this trend continued six months after surgery, but no significant change was observed between three and six months after surgery. There were no cases in which the MTA was clearly confirmed before surgery in the TOF reconstructed images, but the MTA was clearly confirmed in 55% (11/20 cases) of hemispheres three months after surgery and in 85% (17/20 cases) of hemispheres six months after surgery. The crosssectional area of the STA parietal branch, which was the donor for direct revascularization, had increased by more than 150% compared to before surgery in 55% (11/20 cases) of hemispheres three months after surgery. CONCLUSIONS: Indirect revascularization can be expected three months after combined revascularization surgery for moyamoya disease. The MTA, which has not received much attention in terms of indirect revascularization for moyamoya disease patients thus far, was found to be a useful blood flow source for indirect revascularization in combined revascularization surgery for patients with moyamoya disease. Whether or not the cross-sectional area of the superficial temporal artery used as a donor for direct revascularization increased in the medium to long term varied on a case-by-case basis.

Ultrasonography monitoring with Superb Microvascular Imaging during cerebrovascular surgery.

INTRODUCTION: Ultrasonography (US) is used as a real-time dynamic imaging modality during neurosurgery. A novel Doppler US technique, Superb Microvascular Imaging (SMI), can be used to visualize low-velocity flow of small vessels at high resolution with high frame rates. We visualized vessel flow using this US SMI technique and contrast agent during cerebrovascular surgery. METHODS: Forty-three patients with an unruptured cerebral aneurysm (control), ischemic and hemorrhagic moyamoya disease, carotid artery stenosis, hemangioblastoma, severe stenosis of the middle cerebral artery, venous angioma, and intracerebral hemorrhage (ICH) underwent neurosurgery with US SMI monitoring using a contrast agent. The diameter, length, and number of penetrating vessels were analyzed in patients with an unruptured cerebral aneurysm (control), moyamoya disease, and ICH. RESULTS: Diameter and length of cerebral penetrating vessels were significantly increased in patients with moyamoya disease and ICH compared to control patients. The number of penetrating vessels was increased in moyamoya disease patients compared to control and ICH patients. In hemorrhagic moyamoya disease, flow in the penetrating vessels originated from a deep periventricular point and extended to the cerebral surface. Pulsatile cerebral aneurysms during clipping surgery and carotid artery stenosis during carotid endarterectomy were easily identified by SMI. Drastically increased vessel flow in patients with a hemangioblastoma or a venous angioma was observed. CONCLUSION: Using the US SMI technique and contrast agent, we obtained useful flow information of the vascular disease structure and intracerebral deep small vessels during cerebrovascular surgery. Further quantitative analysis will be informative and helpful for cerebrovascular surgery.

Effect of revascularization surgery on cerebral hemodynamics in adult moyamoya disease.

OBJECTIVE: Few studies have reported local hemodynamic changes after revascularization surgery. This study aimed to identify regional hemodynamic changes after combined revascularization surgery for moyamoya disease using single-photon emission computed tomography with N-isopropyl-p-123I-iodoamphetamine. METHODS: A total of 46 adults with moyamoya disease who underwent combined revascularization surgery from August 2009 to July 2021 at our facility were enrolled. The combined bypass procedure comprised a single direct bypass to the motor area and encephalo-duro-arterio-myo-synangiosis. The preoperative and postoperative cerebral blood flow (CBF) and cerebral vascular reserve (CVR) in the genu; precentral, central, parietal, angular, temporal, and posterior regions; splenium; hippocampus; and cerebellum were measured. To modify the examination variability, the cerebral-to-cerebellar activity ratio (CCR) was calculated by dividing the counts in the region by those in the cerebellum (CBF-CCR and CVR-CCR). RESULTS: Postoperatively, asymptomatic cerebral infarction occurred in three (6.5%) patients. The CBF-CCR and CVR-CCR improved in the precentral, parietal, and temporal regions and in the overall middle cerebral artery (MCA) territory. Sub-analysis of anterior cerebral artery (ACA) and posterior cerebral artery (PCA) territory hemodynamics revealed that patients with normal preoperative hemodynamics showed no changes in the CBF-CCR and CVR-CCR postoperatively, whereas patients with preoperative perfusion impairment exhibited improved CVR-CCR in the ACA territory (0.13-0.3, p=0.019) and CBF-CCR in the PCA territory (0.93-0.96, p=0.0039). CONCLUSION: Combined revascularization surgery with single bypass to the motor area improved hemodynamics in the primary targeted MCA territory and in the ACA and PCA territories among patients with preoperative hemodynamic impairment.

Hemodynamic Effects of Tortuosity and Stenosis in Superficial Temporal Artery-Middle Cerebral Artery Bypass for Moyamoya Disease.

BACKGROUND: Superficial temporal artery (STA)-middle cerebral artery (MCA) bypass surgery has been widely adopted in treating moyamoya disease (MMD). Geometric variations including high tortuosity and stenosis exist in many cases, but the hemodynamic effects have not been comprehensively evaluated. We aim to evaluate the hemodynamic effects of bypass geometry variations based on patient-specific data. METHODS: In total, 17 patients with MMD who underwent STA-MCA bypass surgery with highly tortuous bypass geometry were included. For each patient, the original 3-dimensional structure of STA-MCA bypass was reconstructed from clinical imaging data. The bypass structure was virtually improved by removing the tortuosity and stenosis. Computational fluid dynamics simulation was performed on both bypass structures under identical patient-specific condition. The simulated hemodynamic parameters of the bypass and its distal branches were compared between the original and virtually improved bypass geometries in all cases using paired t-test or Wilcoxon signed-rank test. The changes of hemodynamic parameters were compared between the cases with and without mild-to-moderate stenosis (44.0-70.3% in diameter) in the bypass using t-test or Mann-Whitney U test. RESULTS: The virtual improvement of bypass geometry significantly increased the flow rate of the bypass and its distal branches (P < 0.05) and decreased the transcranial flow resistance (P < 0.05). The hemodynamic changes in cases with stenosis removal were significantly greater than those without stenosis (P < 0.05). CONCLUSIONS: High tortuosity and stenosis can significantly change the hemodynamics of STA-MCA bypass, and the optimization of bypass geometry deserves further consideration.

Evaluation of the contribution of individual arteries to the cerebral blood supply in patients with Moyamoya angiopathy: comparison of vessel-encoded arterial spin labeling and digital subtraction angiography.

PURPOSE: Vessel-encoded arterial spin labeling (VE-ASL) is able to provide noninvasive information about the contribution of individual arteries to the cerebral perfusion. The aim of this study was to compare VE-ASL to the diagnostic standard digital subtraction angiography (DSA) with respect to its ability to visualize vascular territories. METHODS: In total, 20 VE-ASL and DSA data sets of 17 patients with Moyamoya angiopathy with and without revascularization surgery were retrospectively analyzed. Two neuroradiologists independently assessed the agreement between VE-ASL and DSA using a 4-point Likert scale (no- very high agreement). Additionally, grading of the vascular supply of subterritories (A1-A2, M1-M6) on the VE-ASL images and angiograms was performed. The intermodal agreement was calculated for all subterritories in total and for the subdivision into without and after revascularization (direct or indirect bypass). RESULTS: There was a very high agreement between the VE-ASL and the DSA data sets (median = 1, modus = 1) with a substantial inter-rater agreement (kw = 0.762 (95% CI 0.561-0.963)). The inter-modality agreement between VE-ASL and DSA in vascular subterritories was almost perfect for all subterritories (k = 0.899 (0.865-0.945)), in the subgroup of direct revascularized subterritories (k = 0.827 (0.738-0.915)), in the subgroup of indirect revascularized subterritories (k = 0.843 (0.683-1.003)), and in the subgroup of never revascularized subterritories (k = 0.958 (0.899-1.017)). CONCLUSION: Vessel-encoded ASL seems to be a promising non-invasive method to depict the contributions of individual arteries to the cerebral perfusion before and after revascularization surgery.

Nonalcoholic fatty liver disease is an independent risk factor for ischemic stroke after revascularization in patients with Moyamoya disease: a prospective cohort study.

BACKGROUND: The study aimed to investigate the association between nonalcoholic fatty liver disease (NAFLD) and ischemic stroke events after revascularization in patients with Moyamoya disease (MMD). METHODS: This study prospectively enrolled 275 MMD patients from September 2020 to December 2021. Patients with alcoholism and other liver diseases were excluded. NAFLD was confirmed by CT imaging or abdominal ultrasonography. Stroke events and modified Rankin Scale (mRS) scores at the latest follow-up were compared between the two groups. RESULTS: A total of 275 patients were enrolled in the study, among which 65 were diagnosed with NAFLD. Univariate logistic regression analysis showed that NAFLD (P = 0.029) was related to stroke events. Multivariate logistic regression analysis showed that NAFLD is a predictor of postoperative stroke in MMD patients (OR = 27.145, 95% CI = 2.031-362.81, P = 0.013). Kaplan-Meier analysis showed that compared with MMD patients with NAFLD, patients in the control group had a longer stroke-free time (P = 0.004). Univariate Cox analysis showed that NAFLD (P = 0.016) was associated with ischemic stroke during follow-up in patients with MMD. Multivariate Cox analysis showed that NAFLD was an independent risk factor for stroke in patients with MMD (HR = 10.815, 95% CI = 1.259-92.881, P = 0.030). Furthermore, fewer patients in the NAFLD group had good neurologic status (mRS score ≤ 2) than the control group (P = 0.005). CONCLUSION: NAFLD was an independent risk factor for stroke in patients with MMD after revascularization and worse neurological function outcomes.

Risk Factors for Specific Postoperative Ischemic Complications in Patients with Moyamoya Disease: A Single-Center Retrospective Study.

AIM: To evaluate and compare postoperative ischemic complications to determine the risk factors for ischemic complications following revascularization surgery for Moyamoya disease (MMD). MATERIAL AND METHODS: This single-center retrospective study included 266 procedures between 2016 and 2021. Three types of revascularization approaches including direct bypass, indirect bypass, and combined bypass were performed. To identify risk factors for postoperative ischemic complications and contralateral cerebral infarction, preoperative clinical characteristics and radiographic features were examined using multivariate and ordinal logistic regression analyses. RESULTS: Postoperative ischemic complications occurred in 103 (6.6%) procedures. Ischemic presentation (p=0.001, odds ratios [OR] 5.59, 95% confidence interval [CI] 2.05-15.23), hypertension (p=0.030, OR 2.75, 95%CI 1.11- 6.83), advanced Suzuki stage (p=0.006, OR 3.19, 95%CI 1.40-7.26), and collateral circulation (p=0.001 OR 0.17, 95%CI 0.06-0.47) were risk factors for postoperative ischemic complications. Ordinal regression analysis revealed that unilateral involvement (p=0.043, OR 2.70, 95%CI 0.09-5.31), hemorrhagic presentation (p=0.013, OR 3.45, 95%CI 0.72-6.18), surgical approach (p=0.032, OR -1.38, 95%CI -2.65, -0.12), and collateral circulation [p=0.043, OR -1 .27, 95%CI -2.51, -0.04)] were associated with the type of ischemic complications. History of hypertension (p=0.031) and contralateral computed tomography (CT) perfusion stage (p=0.045) were associated with contralateral infarction. CONCLUSION: Inability of cerebral vessels to withstand changes in blood pressure induced by revascularization-related hemodynamic instability might be associated with postoperative complications in patients with Moyamoya disease.

[Moyamoya disease in adults: treatment methods in modern era]. / Sovremennyi podkhod k lecheniyu angiopatii moyamoya u vzroslykh.

Moyamoya angiopathy is a chronic progressive cerebrovascular disease characterized by stenosis and occlusion of the distal segments of the internal carotid arteries and/or proximal segments of the middle and anterior cerebral arteries, with a gradual compensatory restructuring of the cerebral circulation to the system of the external carotid arteries. Today, the main treatment method for Moyamoya angiopathy is surgical revascularization of the brain. A search and analysis of publications on the treatment of adult patients with Moyamoya angiopathy was carried out in the PubMed and Medscape databases over the past 10 years. We present a case of an adult female patient with a hemorrhagic form of Moyamoya angiopathy stage IV according to J. Suzuki, who underwent staged combined revascularization of both cerebral hemispheres. Surgical revascularization included the creation of a low-flow extra-intracranial shunt combined with a combination of indirect synangiosis. The combination of direct and indirect methods of surgical revascularization enables to achieve the development of an extensive network of collaterals and fully compensate for cerebral circulatory disorders both in the early and late postoperative periods, which is confirmed by instrumental diagnostic data. Combined revascularization is the most effective modern method of treating patients with Moyamoya angiopathy due to the complementary influence of direct and indirect components of revascularization.

Safety of antiplatelet therapy during the perioperative period of revascularization surgery for moyamoya disease patients with ischemic onset.

For patients with moyamoya disease, antiplatelet agents are often used during the perioperative periods of revascularization surgeries to prevent ischemic events. However, antiplatelet therapy is associated with the risk of hemorrhagic complications. Further, the influence of antiplatelet therapy on perioperative ischemic or hemorrhagic complications has not been investigated. This study aimed to determine the impact of antiplatelet agents on adult moyamoya disease patients with ischemic onset during the perioperative period. From January 2016 to December 2020, 183 consecutive combined (direct and indirect) revascularization surgeries for moyamoya disease patients were performed. Among these surgeries, 96 consecutive combined revascularization surgeries for adult moyamoya disease patients with ischemic onset were analyzed and perioperative ischemic and hemorrhagic complications were reviewed. Antiplatelet agents were continued during the perioperative period including on the day of surgery and the day after the surgery. Among 96 surgeries, no hemorrhagic complications occurred postoperatively. Infarction occurred in five cases (5.2%). Among the five cases, neurological deficits persisted in two cases and improved in three. The median value of bleeding volume was 112.5 mL (interquartile range, 80.0 - 200.0). Twenty-five cases (26.0%) needed blood transfusion. The modified Rankin Scale score deteriorated in two cases due to cerebral infarction. The incidence of hemorrhagic and ischemic complications after combined revascularization surgery in patients with ischemic moyamoya disease under antiplatelet therapy was low, indicating the safety of continued antiplatelet therapy.

[Various Bypass Surgery:Preoperative Simulation for STA-MCA Bypass].

In STA-MCA bypass surgery, it is important to select the optimal recipient using preoperative simulation to avoid complications. We report a preoperative simulation for STA-MCA bypass using the Brain LAB iPLAN platform®BRAIN LAB)and the 3DCG simulation software GRID®Kompath). Here, we introduce the basics and applications of preoperative simulation for occlusive atherosclerotic lesions and present a target bypass for periventricular anastomosis and peripheral vessels of aneurysms in Moyamoya disease. By creating and visualizing 3D fusion images, the optimal donor and recipient can be selected. Determining the skin incision and extent of craniotomy according to the case is also applicable to the minimally invasive STA-MCA bypass. Preoperative simulations enable accurate pinpoint bypass surgery and prevent complications.

Coexistence of moyamoya syndrome with arteriovenous malformation. Systematic review and illustrative case report.

The coexistence of Moyamoya Syndrome with Arteriovenous Malformation is exceedingly rare. Here, we present the case of a 37-year-old female patient diagnosed with AVM in the right parietal lobe, accompanied by severe stenosis of the right middle cerebral artery and right anterior cerebral artery, along with moyamoya collateral induction. Our objective was to investigate the frequency and mutual influence of these conditions, and to determine a preferable treatment strategy by conducting a comprehensive review of previous case reports. We conducted a thorough search of PubMed, Scopus, and Web of Science databases, adhering to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Our review encompassed 36 publications, reporting a total of 64 cases of AVM coexisting with Moyamoya Syndrome. Notably, bilateral stenosis was observed in half of the cases. Among patients with unilateral stenosis, AVM was localized in the opposite hemisphere only in 2 cases. Treatment approaches varied, with 18 cases undergoing active treatment for both stenosis and AVM, 11 cases treating stenosis prior to AVM, 5 cases addressing AVM first, and 3 cases treating both AVM and stenosis simultaneously. Embolization, either standalone or supplemented by stereotactic radiosurgery, was employed in six cases. Stereotactic radiosurgery alone was utilized in 12 cases, while 15 patients underwent surgical removal of the AVM. Our findings provide valuable insights for neurosurgeons managing patients with concurrent AVM and Moyamoya Syndrome. The variety of treatment approaches observed in the literature underscores the complexity of these cases, emphasizing the need for individualized strategies. This information may guide future systematic reviews and meta-analyses, contributing to a better understanding of the optimal management of these rare coexisting vascular pathologies.