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It is unclear how rare RNF213 variants, other than the p.R4810K founder variant, affect the clinical phenotype or the function of RNF213 in moyamoya disease (MMD). This study included 151 Japanese patients with MMD. After performing targeted resequencing for all coding exons in RNF213, we investigated the clinical phenotype and statistically analyzed the genotype-phenotype correlation. We mapped RNF213 variants on a three-dimensional (3D) model of human RNF213 and analyzed the structural changes due to variants. The RNF213 p.R4810K homozygous variant, p.R4810K heterozygous variant, and wild type were detected in 10 (6.6%), 111 (73.5%), and 30 (19.9%) MMD patients, respectively. In addition, 15 rare variants were detected in 16 (10.6%) patients. In addition to the influence of the p.R4810K homozygous variant, the frequency of cerebral infarction at disease onset was higher in pediatric patients with other rare variants (3/6, 50.0%, P = 0.006) than in those with only the p.R4810K heterozygous variant or with no variants (2/51, 3.9%). Furthermore, on 3D modelling of RNF213, the majority of rare variants found in pediatric patients were located in the E3 module and associated with salt bridge loss, contrary to the results for adult patients. The clinical phenotype of rare RNF213 variants, mapped mutation position, and their predicted structural change differed between pediatric and adult patients with MMD. Rare RNF213 variants, in addition to the founder p.R4810K homozygous variant, can influence MMD clinical phenotypes or structural change which may contribute to the destabilization of RNF213., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: Patients sometimes experience transient neurological events (TNEs) during the early postoperative period after revascularization surgery for childhood moyamoya disease. The clinical background and pathophysiology of TNEs remain unclear. This study aimed to evaluate the incidence rate of TNEs and discuss the factors associated with pediatric moyamoya disease., Methods: The authors retrospectively reviewed 110 cerebral hemispheres of 61 pediatric patients younger than 15 years who were treated from 2011 to 2020. All children underwent either simple double-direct or combined revascularization surgery. Of these 61 patients, 52 underwent bilateral surgery and 9 underwent unilateral surgery. The authors calculated the incidence of TNEs in accordance with a previously reported TNE definition. Cerebral blood flow (CBF) was evaluated in all eligible cases with xenon CT immediately after revascularization surgery., Results: The incidence rate of TNEs in patients with childhood moyamoya disease was 26.4%. TNEs occurred after an average (range) of 6.26 (2-12) days postoperatively without triggers, and all identified TNEs spontaneously resolved within 2 weeks. The most common symptoms were dysarthria, facial palsy, and numbness around the mouth, followed by sensory disturbance of the upper extremities. The presence of focal hyperperfusion on xenon CT performed immediately after revascularization surgery was strongly correlated with the incidence of TNEs (p = 0.0001). Focal hyperperfusion was observed in 43 of 110 operative sides (39.1%). Notably, only 25.6% of patients with focal hyperperfusion showed numerical global hyperperfusion. In addition, a decrease in CBF compared with the thalamic region in the contralateral side was observed in TNE-affected cases (p = 0.0443)., Conclusions: TNEs occurred more frequently in childhood moyamoya disease patients than expected. The clinical background, including symptoms, timing, and duration, was almost identical to TNEs in adults. Focal hyperperfusion, rather than numerical global hyperperfusion, was strongly correlated with the incidence of TNEs. Furthermore, the authors advocate the notion that a clinical course where symptoms occur without triggers may be a unique characteristic of TNEs, especially in childhood moyamoya disease.

Objective: Postoperative intracerebral hemorrhage (ICH) after direct bypass surgery for Moyamoya disease could contribute to neurologic deterioration. The aim of this study was to evaluate the effectiveness of 5-day bed rest in reducing the occurrence of postoperative ICH., Methods: This study included 122 consecutive hemispheres in 87 Japanese adult MMD patients, composed of 80 control hemispheres from historical data and 42 hemispheres after 5-day bed rest. They all underwent direct bypass surgery. The incidence of postoperative ICH and neurologic deterioration assessed via the modified Rankin Scale were investigated and statistically analyzed., Results: Postoperative ICH was observed in 9 out of the 80 (11.3%) control patients, but not in the 42 patients with 5-day bed rest. The incidence of postoperative ICH and neurologic deterioration via the modified Rankin Scale were significantly different between the 2 groups (P = 0.0268 and 0.0078, respectively). Univariate logistic analysis revealed that 5-day bed rest significantly reduced the incidence of postoperative ICH (P = 0.0048)., Conclusions: Five-day bed rest after direct bypass surgery dramatically can reduce the incidence of postoperative ICH and neurologic deterioration after direct bypass surgery., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: Patients with pediatric moyamoya disease (PMMD) showing recurrent symptoms or decreased cerebral blood flow after initial revascularization therapy may require additional revascularization to improve their clinical condition. The authors evaluated the clinical and hemodynamic benefits of an occipital artery (OA)-middle cerebral artery (MCA) bypass for patients with PMMD who have undergone an initial revascularization procedure., Methods: The authors retrospectively identified 9 patients with PMMD who had undergone OA-MCA bypass between March 2013 and December 2017, and who had received a previous superficial temporal artery-MCA bypass. The following clinical data were collected: initial revascularization procedure, symptoms (presence or recurrence), pre- and postoperative cerebral blood flow and cerebrovascular reactivity (CVR) changes, posterior cerebral artery (PCA) stenosis, PCA-related and nonrelated symptoms, and latest follow-up., Results: Preoperatively, all patients (n = 9) suffered non-PCA-related recurrent symptoms, and 4 had PCA-related symptoms. At 1-year follow-up, all patients with PCA-related symptoms showed complete recovery. Additionally, 8 (89%) patients with non-PCA symptoms experienced improvement. Only 1 (11%) patient showed no improvement after the surgical procedure. The mean pre- and postoperative CVR values of the MCA territory were 14.8% and 31.3%, respectively, whereas the respective mean CVR values of the PCA territory were 22.8% and 40.0%., Conclusions: The OA-MCA bypass is an effective rescue therapy to improve the clinical condition and hemodynamic changes caused by PMMD in patients who experience recurrent symptoms after initial revascularization.

Introduction: Moyamoya disease (MMD) is a rare cerebrovascular disease associated with cerebral infarction or hemorrhage. Hyperperfusion is the most significant complication of direct bypass surgery. Previous research has shown that an increase in cerebral blood flow (CBF) is strongly related to symptomatic hyperperfusion and highlighted the importance of postoperative assessment of CBF., Objective: The principal aims of this study were to quantitatively analyze the relationship between intraoperative graft flow and increase in CBF and to evaluate the effectiveness of intraoperative graft flow measurement during bypass surgery for patients with MMD., Methods: This study included 91 surgeries in 67 consecutive adult patients with MMD who underwent direct revascularization surgery at our institution between November 2013 and September 2018. Intraoperative graft flow of the branches and main trunk was measured in all patients, after anastomosis had been established. Postoperative CBF measurements were performed under sedation, immediately after surgery. Radiological hyperperfusion was defined as focal high uptake, as determined by CBF imaging immediately after surgery. Patients were divided into two groups (radiological hyperperfusion and nonradiological hyperperfusion groups), and the relationship between intraoperative graft flow and radiological hyperperfusion was analyzed., Results: Significant differences were observed between the radiological hyperperfusion and nonradiological hyperperfusion groups in terms of intraoperative graft flow of both the branch (median 72 vs. 42 mL/min, respectively; p < 0.01) and main trunk (median 113 vs. 68 mL/min, respectively; p < 0.01). A receiver-operating characteristic analysis was performed to test the utility of intraoperative flow as a quantitative measure. We set the cutoff values for the intraoperative branch and main trunk flow at 57 mL/min (sensitivity: 0.707, specificity: 0.702; area under the curve [AUC]: 0.773; 95% confidence interval [CI]: 0.675-0.871) and 84 mL/min (sensitivity: 0.667, specificity: 0.771; AUC: 0.78; 95% CI: 0.685-0.875), respectively., Conclusions: Measuring intraoperative graft flow during bypass surgery may be an effective means of predicting hyperperfusion and could serve to facilitate early therapeutic intervention such as strict blood pressure control., (© 2020 The Author(s) Published by S. Karger AG, Basel.)

Background: Moyamoya syndrome (MMS), distinguished from definite moyamoya disease (MMD), is characterized by moyamoya vasculopathy thought to develop secondary to underlying conditions (e.g., hyperthyroidism). Recent studies have shown that a proportion of East Asian (EAS) patients with MMS possess the p.R4810K variant of RNF213 (rs112735431), the foremost susceptibility variant among EAS patients with MMD. We evaluated the association between hyperthyroidism-associated MMS (hMMS) and sequence variants in RNF213., Methods: We performed next-generation sequencing of RNF213 in 15 patients with hMMS. Candidate coding variants for the association analysis were defined by allelic frequencies of <1%, based on the p.R4810K frequency in the Japanese population. The association with hMMS was tested using a collapsing method, and 260 unrelated EAS women from the 1000 Genomes Project served as population-based controls., Results: All patients were female, reflecting female predominance in both moyamoya and hyperthyroid conditions. Five candidate missense variants in RNF213 were identified in 8 of 15 patients (53.3%): p.C118R, p.R4062Q, and p.R4810K as heterozygous; and p.A3468V and p.S3986N as compound heterozygous with p.R4810K. Among 260 EAS female controls, 36 (13.8%) had putatively functional variants. All identified variants were missense variants and were significantly overrepresented among patients compared with EAS controls (permuted P = 0.00010; odds ratio = 7.03; 95% confidence interval, 2.09-24.3)., Conclusions: Rare and low-frequency missense variants in RNF213 confer susceptibility to both MMD and hMMS. This finding indicates that susceptibility variants in RNF213 may require additional clinical factors with an effect equivalent to hyperthyroidism in order to develop moyamoya vasculopathy., (Copyright © 2019 Elsevier Inc. All rights reserved.)

The effects of superficial temporal artery to middle cerebral artery (STA-MCA) double bypass on recurrent hemorrhage in the operated hemisphere in hemorrhagic moyamoya disease (HMD) have not been clearly demonstrated. This study evaluated the effectiveness of STA-MCA double bypass in the prevention of further hemorrhagic or ischemic events in the operated hemispheric sides in comparison to the conservatively treated non-operated sides. We retrospectively analyzed 52 hemispheres of 36 patients with adult-onset HMD treated with STA-MCA double bypass. Twenty and 16 patients underwent unilateral (unilateral group) and bilateral (bilateral group) surgery, respectively. In addition, the perioperative and long-term outcomes of the 52 operated sides and 20 non-operated sides in the unilateral group were compared. All bypass surgeries were successful, but 21% of the operated sides showed hyperperfusion as estimated by our methods. Perioperative mortality and morbidity rate were 0% and 5.6%, respectively. Concerning long-term follow-up, the annual rebleeding rate (ARR) in the unilateral and bilateral group was 2.7% and 2.6%/person-year, respectively (p = 0.256). The ARR in the operated and non-operated sides was 1.1% and 1.8%/side-year, respectively (p = 0.163). Two of 20 non-operated sides suffered from ischemic infarction during the follow-up period, while none of the 52 operated sides experienced ischemic events (p < 0.05). Although the long-term rebleeding rate in the operated hemisphere tended to be lower after STA-MCA double bypass compared with that in the non-operated hemisphere, the difference was not statistically significant. In conclusion, while STA-MCA double bypass could not clearly prevent rebleeding, it can prevent further ischemic attacks in patients with HMD.

Background: Homozygosity of this p.R4810K founder variant of RNF213moyamoya disease (MMD) susceptibility gene is known to influence the severity of the clinical disease phenotype at disease onset. However, the association between this genotype and long-term clinical manifestations has remained unclear., Objectives: The principal goal of this study was to investigate whether and how the p.R4810K variant of RNF213influences the long-term phenotype in Japanese patients with MMD., Method: This retrospective cohort study included 94 Japanese patients with MMD who underwent direct or combined bypass for revascularization with the p.R4810K genotype determined in our hospital. The following phenotypic parameters were analyzed at disease onset and over a long-term period: age and initial presentation at onset, recurrent stroke after initial revascularization, and final modified Rankin Scale., Results: The p.R4810K genotype was significantly associated with the phenotype at onset, especially in younger patients. Over a median follow-up period of 100 months, recurrent stroke occurred in 6 out of 94 patients: none out of 5 patients with the homozygous variant, 5 out of 64 with the heterozygous variant, and 1 out of 25 in the wild-type group. There were no significant differences among the genotypes. In particular, recurrent cerebral hemorrhage occurred in 5 patients, all possessing the heterozygous variant. The log-rank test showed no difference between the genotypes in the stroke-free survival rate. Furthermore, the p.R4810K genotype was not associated with a poor functional condition., Conclusions: The p.R4810K founder variant of RNF213 affects the phenotype at disease onset. However, the optimal revascularization may be effective, regardless of the genotype, even for the homozygous variant, which has been thought to be the most pathogenic. This genotype may not strongly influence the long-term clinical manifestations or poor prognosis in MMD., (© 2019 S. Karger AG, Basel.)

Objective: Superficial temporal artery-middle cerebral artery anastomosis is an established treatment for moyamoya disease. However, hemorrhagic cerebral hyperperfusion syndrome (CHS) leads to poor outcomes. This study aimed to identify predictors of hemorrhagic CHS based on regional cerebral blood flow (rCBF) in patients with moyamoya disease., Methods: The study included 251 hemispheres in 155 patients with moyamoya disease who underwent preoperative and postoperative rCBF measurements and superficial temporal artery-middle cerebral artery double anastomosis. We used rCBF increase rate for predicting hemorrhagic CHS. rCBF increase rate was calculated by 2 methods. In method 1, the rCBF value on the operated side was compared with the rCBF value on the nonoperated side. In method 2, the postoperative rCBF value on the operated side was compared with the preoperative rCBF value on the operated side. Patients were classified into 4 groups according to rCBF increase rate to predict risk of hemorrhagic CHS., Results: Hemorrhagic CHS occurred in 7 (2.8%) hemispheres (no children). Severe hemorrhagic CHS occurred in only 1 (0.4%) hemisphere. Hemorrhagic CHS was observed in patients with ≥30% rCBF increase according to method 1 and ≥50% rCBF increase according to method 2 and was most frequently noted in ≥100% rCBF increase., Conclusions: Predictors for hemorrhagic CHS were ≥30% rCBF increase when using method 1 and ≥50% increase when using method 2., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: Postoperative hyperperfusion is an important complication after direct bypass for moyamoya disease, which sometimes occurs late after initial postoperative cerebral blood flow (CBF) measurement. This study aimed to clarify the incidence of hyperperfusion with management using postoperative continuous sedation and repeated postoperative CBF measurement and to identify factors associated with delayed hyperperfusion., Methods: This retrospective study evaluated 72 consecutive hemispheres in 56 adult Japanese patients with moyamoya disease who underwent direct bypass. Postoperative continuous sedation was routinely administered based on CBF evaluation. First, the incidence of symptomatic hyperperfusion was investigated. Second, radiologic hyperperfusion (RHP), which was strictly defined as >30% increase in CBF compared with the contralateral side, and factors associated with delayed RHP were statistically analyzed., Results: Postoperative symptomatic hyperperfusion occurred in 3 hemispheres (4.2%), including subarachnoid hemorrhage in 1 hemisphere (1.4%). RHP immediately after surgery was identified in 16 hemispheres (22.2%). In 8 hemispheres (11.1%), RHP appeared or worsened several days after initial CBF study. In univariate logistic regression analysis, decreased preoperative cerebral vasoreactivity was significantly associated with delayed RHP., Conclusions: The incidence of symptomatic hyperperfusion was 4.2% with management. Delayed hyperperfusion was significantly associated with decreased cerebral vasoreactivity. Therefore, repeated CBF measurements evaluating preoperative cerebral vasoreactivity may decrease complications., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Pediatric moyamoya cases may be very arduous, even more so in a developing country, where access to specialized centers may be prevented by different factors., Case Description: Herein we report a challenging case, which was managed in the new Neurosurgical Center of Trujillo, regarding the direct anastomosis between the left superficial temporal artery and a cortical branch of the left middle cerebral artery in a 8-year-old Peruvian boy with moyamoya disease. Postoperatively, the patient's motor deficits and aphasia improved. To the best of our knowledge, this is the first performance of a direct revascularization for a pediatric moyamoya case in Peru., Conclusions: The creation of highly specialized neurosurgical centers in the main strategic places of developing countries may allow optimal treatment of neurosurgical patients with complex diseases., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: Surgical revascularization for pediatric moyamoya disease improves cerebral blood flow (CBF) and consequently may prevent further ischemic events. However, the timing of the treatment is controversial especially for patients with no ischemic symptom and normal CBF. The purpose of this case report is to inform and infer the surgical treatment timing for pediatric moyamoya disease patients., Case Report: A 10-year-old female patient with unilateral moyamoya disease complaining of only headache as a symptom and whose Suzuki stage was II or in transition to III by angiography and CBF was almost normal was admitted to Tokyo Women's Medical University Hospital. We performed superficial temporal artery-middle cerebral artery (STA-MCA) double anastomoses for the patient because we estimated her headache was derived from low perfusion in the brain. STA-MCA bypass surgery was not only effective for relief of her severe headache but also valid for her cerebral perfusion. Her angiography showed much supply from external carotid artery to intracranial arteries via bypass grafts in 7 months., Conclusion: Our case showed early surgical treatment was beneficial for relief of severe headache even for early staged pediatric moyamoya disease patients by improving perfusion pressure and cerebral circulation.

Background: Moyamoya disease (MMD) concurrent with Graves' disease (GD) is rare. There is no guideline about optimizing thyroid hormones and the appropriate timing of surgical treatment for MMD with GD., Methods: We encountered eight patients with MMD and GD presenting with cerebral ischemia who were treated by direct bypass. Thyroid hormones [free thyroxin (fT4) and free triiodothyronine (fT3)], thyroid-stimulating hormone (TSH), and TSH receptor antibody (TRAb) were measured sequentially. After thyrotoxic conditions were medically optimized, revascularization surgery was performed by superficial temporal artery-middle cerebral artery (STA-MCA) double bypass in all cases. Clinical outcomes were estimated by modified Rankin scale (mRS) at discharge and 3 months after surgery., Results: In six patients with thyrotoxicosis, the fT4, fT3, and TRAb (range) at the onset of cerebral ischemia were 4.81-10.30 pg/ml, 13.08-31.90 pg/ml, and 3.5-83.8 IU/l, respectively. At surgery, mean (range) fT3 and fT4 were optimized to 3.02 (1.01-4.87) pg/ml and 1.09 (0.41-1.68) ng/dl, respectively. In the thyrotoxic cases, it took 70-310 days (mean, 142 days) to optimize thyroid hormones before surgery. There was no neurological aggravation after surgery, and outcome was excellent at 3 months with mRS scores ≤2 in all cases., Conclusions: For MMD concurrent with GD, optimizing thyroid hormones followed by STA-MCA double bypass was successful to prevent cerebral ischemic events.

Subjects: Little is known about the pathology and pathogenesis of the Moyamoya disease (MMD). To better understand the molecular processes involved in MMD gene expression analysis of Moyamoya artery tissue to a control artery, this study was conducted., Methods: Tissue samples of two MMD and two non-MMD were profiled using oligonucleotide microarrays. Gene ontology classification of the differentially expressed genes was analyzed, and regulatory functional networks and pathways were identified with a network-based computational pathway analysis tool. Analysis of MMD and control tissue revealed 104 differentially expressed genes., Results: The two major significantly associated gene ontology terms was cellular development and cellular movement. Further network-based analysis showed significant interaction between RNF213 downstream gene networks and the top 3 score gene networks of MMD. Three major nodes of this network were evident in the merged network and were showing interactions with downstream network of RNF213., Conclusions: Our results demonstrate that cellular development and cellular movement in MMD are the key role of mechanisms.

Moyamoya disease (MMD) and atherosclerotic cerebrovascular disease (ACVD) differ in angiographic appearance and probably hemodynamics. Pediatric MMD (PMMD) usually presents with cerebral ischemia, while intracranial hemorrhage is more common in adult MMD (AMMD), suggesting differences in cerebral hemodynamics. We analyzed the cortical flow velocity and direction of recipient arteries using micro-Doppler ultrasonography to evaluate the cortical circulation before and after anastomosis in MMD and ACVD. Twenty-eight patients with adult MMD (AMMD), 7 with pediatric MMD (PMMD), 16 with ACVD, and 12 control patients were studied. A micro-Doppler probe was applied on the cortical recipient artery (A4 or M4) before and after anastomosis. Systolic maximum flow velocity (V max) and blood flow direction were investigated at proximal and distal parts of anastomosed sites in recipient arteries. Pre- and postoperative regional cerebral blood flow was measured by cold xenon-computed tomography (Xe-CT). Before anastomosis, retrograde cortical flow was significantly more common in PMMD patients, and V max in cortical artery was significantly lower in AMMD patients. Bypass surgery changed the direction of blood flow from the anastomosis site to proximal and distal sites of the recipient artery in most patients, but pre-anastomosis flow direction was preserved more frequently in PMMD patients. The rate of V max increase after anastomosis was significantly higher in AMMD than in PMMD (11.6 ± 9.8 vs. 3.9 ± 1.8; P = 0.01). Micro-Doppler ultrasonography identified differences in cortical circulation among AMMD, PMMD, and ACVD. In AMMD, significantly low velocity in the cortical artery was observed before anastomosis, and bypass surgery reversed the flow and significantly increased flow velocity. The data of PMMD showed unique hemodynamics of the cortical artery before anastomosis, characterized by a higher frequency of retrograde flow and preserved velocity. The V max increase rate was significantly higher in patients with postoperative cerebral hyperperfusion on Xe-CT, and further study is warranted to validate the clinical use of intraoperative micro-Doppler monitoring to predict postoperative hyperperfusion.

Object: Some patients with moyamoya disease complain of severe headache, which may be closely related to cerebral ischemia. The efficacy of superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis in these patients was evaluated by clinicoradiological studies., Methods: Of 117 consecutive patients with ischemic moyamoya disease, 25 complained mainly of severe headache (headache group) and 92 had no significant headache (nonheadache group). Intensity of headache was evaluated pre- and postoperatively. Furthermore, regional cerebral blood flow (rCBF) and cerebrovascular reactivity (CVR) were assessed pre- and postoperatively., Results: The headache group was significantly younger than the nonheadache group. In a group corrected for the age distribution, preoperative rCBF and CVR were similar in headache and nonheadache groups. After STA-MCA anastomosis, 16 patients with headache experienced complete relief from headache, 7 patients showed remarkable improvements and discontinued medications for headache, and the remaining 2 patients had some alleviation of headache but sometimes required medication. In the headache group, the postoperative rCBF was significantly greater than the preoperative value. The postoperative rCBF in this group was also significantly greater than the postoperative rCBF in the nonheadache group., Conclusions: These data suggest that severe headache is one of the main symptoms in young patients with moyamoya disease probably related to cerebral circulatory disturbances. An STA-MCA anastomosis is effective in relieving headache in patients with ischemic moyamoya disease manifesting severe headache, probably by improving perfusion pressure and cerebral circulation.

We investigated whether postoperative hyperperfusion in moyamoya disease can be predicted using intraoperative laser Doppler flowmetry and/or thermography. A prospective study was conducted on 27 patients (39 hemispheres) with moyamoya disease who underwent superficial temporal artery-middle cerebral artery (STA-MCA) bypass. During surgery, regional cerebral blood flow (rCBF) was measured with a laser Doppler flowmeter and the temperature of the cortical surface was measured with an infrared thermograph. Postoperative hyperperfusion was assessed immediately after surgery based on CBF study under sedation (propofol) as >100% increase in corrected rCBF compared to preoperative values. Postoperative hyperperfusion on CBF was observed in two patients (7.4%). A significant correlation was observed between intraoperative rCBF changes and postoperative rCBF increase (Pearson's method: r=0.555, p=0.0003; simple regression: Y=1.22X+3.289, r (2)=0.308, p=0.0004). Furthermore, the rCBF changes measured by laser Doppler flowmetry were significantly greater in patients with postoperative hyperperfusion (p=0.0193) and CHS (p=0.0193). The present study suggests that intraoperative rCBF measurement using laser Doppler flowmetry may predict a risk of post-EC-IC bypass cerebral hyperperfusion in moyamoya disease.

Background: Although some patients with moyamoya disease need revascularization in the anterior cerebral artery (ACA) territory, there are few reports on direct bypass in the ACA territory because of the difficult surgical technique., Objective: To report our technical strategy for superficial temporal artery (STA)-ACA bypass., Methods: We performed simultaneous STA-ACA and STA-middle cerebral artery direct bypasses in 7 patients with moyamoya disease using the following strategies: creating 2 separate craniotomies for the 2 bypasses, dissecting a long STA graft and securing a recipient ACA around the bregma for the STA-ACA bypass, and using loose stitches at the anastomoses. One branch of the STA was dissected for a length of approximately 10 cm. The graft coursed on the brain surface under the bone bridge and was directly anastomosed to the cortical branch of the ACA. At the anastomoses, the stitches were widely spaced and loose to facilitate expansion of the orifice., Results: This method prevented kinking of the graft. Postoperative angiograms revealed good patency of the STA-ACA bypass in all patients. After the bypasses, 5 patients no longer had transient ischemic attacks or stroke, 1 patient was almost completely free of transient ischemic attacks, and 1 patient had only residual contralateral symptoms. In all 7 patients, patency of the bypass was satisfactory during follow-up periods ranging from 9 to 23 months (mean 16.4 months)., Conclusion: This method of STA-ACA bypass provides successful and reliable direct revascularization of the ACA territory in patients with moyamoya disease. Further investigation of the possible merit of this surgery in improving cognitive function is warranted.

Table 2 of the original version of this article contained an added data. Correct Table 2 is presented here. [ABSTRACT FROM AUTHOR]