Encephalo-duro-arterio-myo-synangiosis Research Articles

Abstract 042: Cognitive outcomes in patients with carotid insufficiency after undergoing direct extracranial‐intracranial bypass

Introduction Extracranial‐intracranial (EC‐IC) bypass is a niche procedure that has been selectively used for flow augmentation or flow restoration in patients with intracranial carotid insufficiency.1 The underlying etiology varies but is most commonly related to Moya Moya disease (MMD) or intracranial carotid atherosclerotic disease (ICAD).2 Patients are often managed non‐operatively until they begin to develop symptoms such as transient ischemic attacks (TIAs) or strokes that are recalcitrant to maximal medical management, at which point bypass is pursued.1 This lag period, however, could put patients at risk for long term deficit, not only in the form of focal neurological deficits but also in terms of overall neurocognitive capability.3 Several reports have previously demonstrated some level of association between EC‐IC bypass and neurocognitive outcomes, though in isolated populations of either MMD or ICAD.4,5 We therefore aim to further assess the relationship between EC‐IC bypass and neurocognitive outcomes in patients with intracranial carotid insufficiency. Methods Patients ages 18 years and up undergoing EC‐IC bypass were identified from a single institution database between January 2020 and December 2021. Patient records were reviewed for demographic data including age and sex, as well as preoperative clinical information including comorbidities, operative indication, anticoagulation/antiplatelet therapy usage, prior ischemic stroke, modified Rankin score (mRS), and Montreal Cognitive Assessment (MOCA) score. Radiographic data was also collected including Suzuki grade, collateralization, and perfusion. Post‐operative clinical information including complications, discharge mRS, and discharge MOCA scores were identified. Follow‐up duration, MOCA scores, and mRS were also identified and recorded. Results Of the forty‐four patients that underwent EC‐IC bypass during the specified period, ten had cognitive assessments before and after surgery available for review. The indications for bypass in these cases were ICA steno‐occlusive disease resulting in strokes despite maximal medical management in 9 cases, and MMD in one case. Suzuki grade was IV in the MMD case. Pial collaterals were present in 7 cases, and ECA collaterals were present in 5. Prior ischemic strokes were present in 8 cases, with a preoperative mRS range from 1‐4 (median 1), and average MOCA of 20. Perfusion imaging demonstrated a Tmax mismatch in eight cases, with an average volume of 73.6 cc at risk. All patients underwent direct superficial temporal artery to middle cerebral artery bypass, with additional encephaloduroarteriomyosynangiosis (EDAMS). Follow up MOCA score was improved in 8 cases, unchanged in one case, and worsened in one case (average MOCA increased from 20 to 23). Follow up mRS ranged from 0‐2. Additionally, CT perfusion demonstrated notable improvements in Tmax >4 seconds (decrease from 176 cc to 110 cc), Tmax >6 seconds (decrease from 59 cc to 29 cc), and mismatch (decrease from 59 cc to 17 cc) at 6 month follow up. Conclusion Patients with carotid insufficiency from MMD or ICAD undergoing EC‐IC bypass demonstrate well below normal preoperative MOCA scores. This is suggestive of ongoing neurocognitive decline, which is an often overlooked issue in patients with these pathologies. MOCA and mRS both appear to improve in patients after bypass at longer term follow‐up, suggesting that bypass may be an effective method of addressing the issue of neurocognitive decline in patients with carotid insufficiency.

A single - center retrospective observational study on patients undergoing Encephalo-Duro-Arterio -Myo-Synangiosis in patients with moyamoya disease.

BACKGROUND:Many cases of moyamoya disease are refractory to conventional medical therapy, hence surgical revascularization techniques have emerged as one of the primary choices of treatment. In this study, we present the functional and angiographic outcomes of patients undergoing encephalo-duro-arterio-myo-synangiosis (EDAMS).METHODS:This is a retrospective observational cohort study, done over 8 years (2012–2020) in a neurological center in Eastern India. Data were retrieved from the hospital's electronic system, recorded in a standard data abstract sheet, and analyzed.RESULTS:This study included 75 patients, with a male (n = 42; 56.0%) preponderance. Majority belonged to the pediatric age group (≤18 years) (n = 70; 93.3%); remaining adult population included 5 (6.6%) patients. The most common presenting complaint was that of an ischemic cerebrovascular accident (CVA) (n = 57; 76.0%). Symptomatic hemispheres (n = 69; 92.0%) were treated and later followed if they had progressed to bilateral disease formation. Preoperative DSA showed 50 (71.4%) to have Suzuki grade 3 type of angiographic findings. Postoperative complications included worsening unilateral hemiparesis 4 (40%), slurring of speech (n = 2; 20.0%), hematoma (n = 2; 20.0%), and surgical site infection (n = 2; 20.0%). One patient succumbed to his illness on the second postoperative day. A postoperative angiogram showed regression of moyamoya vessels in the majority (n = 69; 93.3%) of patients. All (n = 74; 100%) had an intensification of transdural vessels; none had a regression. None of the study participants showed an intensification of moyamoya vessels. Many of our patients (83.8%) had a good grade of revascularization (modified Matsushima and Inaba A and B), while 16.2% had low-grade revascularization (grade C). On assessing outcomes using a modified Rankin Score, a large number of our patients had an excellent (n = 45, 60%) neurological outcomes.CONCLUSION:There was a bimodal age distribution with most of them presenting with ischemic CVA. This procedure (EDAMS) had good angiographic (Matsushima and Inaba) and functional (modified Rankin Score) outcomes.

Indirect revascularization in an Iraqi child with Moyamoya Disease

Background: Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by bilateral stenosis starting at the supraclinoid internal carotid artery (ICA), with the development of a collateral network of vessels. It is an established cause of stroke in the pediatric age group. Despite its increasing prevalence in various parts of the world, it remains largely underrecognized in the Middle East, particularly in Iraq. This is the first case of MMD in an Iraqi patient undergoing surgery.
 Case description: A 12-year-old boy presents with a 3-months history of progressive behavioural changes. MRI revealed diffuse infarcts of different ages. MRA and CT angiography revealed extensive asymmetrical steno-occlusive changes of the supraclinoid ICAs extending into the anterior and middle cerebral arteries, with the development of a collateral network in the basal ganglia. Indirect revascularization of the right side by encephaloduroarteriomyosynangiosis (EDAMS) was performed. The clinical status of the patient improved during the follow-up and the MRA showed a re-establishment of the blood flow to the MCA.
 Conclusion: MMD should be recognized as a cause of stroke or recurrent TIAs in the Iraqi population, particularly in pediatric patients. EDAMS is an effective revascularization procedure with good results in pediatric patients.

Postoperative neovascularization, cerebral hemodynamics, and clinical prognosis between combined and indirect bypass revascularization procedures in hemorrhagic moyamoya disease

ObjectiveWe evaluated what few studies emphasized the postoperative collateral formation and cerebral hemodynamics of hemorrhagic moyamoya disease (MMD). MethodsHemorrhagic MMD patients treated surgically were retrospectively collected and dichotomized into combined bypass (CB) and indirect bypass (IB) groups. CB used superficial temporal artery-to-middle cerebral artery anastomosis combined with encephaloduroarteriomyosynangiosis (STA-MCA+EDAMS), and IB used encephaloduroarteriomyosynangiosis (EDAMS) for revascularization. Postoperative complications and clinical prognosis, as well as pre- and post-operative Modified Rankin Scale (mRS), collateral circulation status, and cerebral hemodynamics were observed and compared between the CB and IB groups. ResultsA total of 37 patients with hemorrhagic MMD were identified. Of the 68 cerebral hemispheres, 47(69.1%) were combined revascularization, and the rest were indirect. During an average follow-up of 16.5 ± 8.7 months, the recurrent stroke events were significantly lower, as well as having a postoperative mRS scores≤ 2. A satisfactory postoperative collateral formation, and an improved dilation or extension of the anterior choroidal/posterior communication artery (AchA/PcoA) were significantly higher in the CB group than in the IB group (all P < .05). Compared with preoperative cerebral hemodynamics, relative cerebral blood flow (rCBF), relative cerebral blood volume (rCBV), mean transit time (MTT), and relative time to peak (rTTP) in the CB group; rCBF, rCBV, and MTT in the IB group were significantly improved (all P < .001). The CB group’s postoperative rCBF was significantly improved compared with the IB group (P < .001). ConclusionsSTA-MCA bypass combined with EDAMS can obtain better postoperative collateral formation, cerebral hemodynamics, and clinical prognosis than EDAMS alone.

Outcome Following Surgical Revascularization in Patients of Moyamoya Disease with Focus on Graft Patency and Angiographic Changes.

Surgical revascularization is the mainstay of treatment in symptomatic patients of moyamoya disease (MMD). The present study analyzed the postoperative angio-architecture in pediatric and adult patients of moyamoya disease. Patients with MMD, both ischemic and hemorrhagic, were subjected to surgery. A superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis was attempted in all. It was augmented by an encephalo-duro-myo-synangiosis), this was labelled as the combined surgical group. In patients where a direct bypass was not possible encephalo-duro-arterio-myo-synangiosis (EDAMS) was performed and these patients were put in the indirect surgery group. In the postoperative period, MRA was performed in all patients to look for (a) graft patency, (b) regression of moyamoya vessels, and (c) degree of surgical neovascularization (as quantified on adapted Matsushima and Inaba grading system). Eighty-two patients underwent 131 surgical revascularization procedures. A combined surgery (STA-MCA bypass and EDAMS) was performed in 100 hemispheres and indirect surgery (EDAMS) on 31 sides. In children less than 5 years of age, STA-MCA anastomosis was possible in more than 50% of patients. Clinical improvement was seen in 85.4% of patients. Postoperative MRA demonstrated a patent bypass graft in 97% of cases. Regression of moyamoya vessels was seen in half of the cases and good surgical revascularization (type A and B) was seen in more than 80% of hemispheres in the combined surgery and indirect surgery group. Revascularization procedures led to a regression of moyamoya collaterals, appearance of surgical neo angiogenesis, and a graft patency rate of 97%. Surgical group with combined revascularization had a trend towards better collateral development.

PedsQL Score Post Encephalo-duro-arterio-myo-synangiosis Procedure for Moyamoya Disease: A Single Center Experience

Moyamoya disease is a progressive, occlusive arteriopathy of the intracranial vessels causing an increased risk of stroke. It often results in functional impairment and decrease in quality of life, both in the presence and absence of stroke. Revascularization is the accepted treatment for patients with symptomatic moyamoya disease, preventing further stroke. At Leeds Children's Hospital we use the encephalo-duro-arterio-myo-synangiosis (EDAMS) technique to facilitate revascularization. We aim to assess the quality of life outcomes of patients who have undergone operative intervention at our unit for moyamoya disease. Pediatric patients with operated moyamoya disease from Leeds Children Hospital between February 2009 and January 2019 were included. Patients awaiting primary surgery were excluded. Patients were contacted via telephone and the Pediatric Quality of Life Inventory 4.0 (PedsQL) questionnaire administered via parent proxy. Quality of life outcomes were assessed using the PedsQL questionnaire score, which measures physical, emotional social and school functioning domains. This score was then converted to a health-related quality of life score. Scores in each domain and mean scores were compared with normative data. This study included 11 children (5 boys), median age 6.8 years (range: 22 months to 15.5 years), and 9 children underwent bilateral operations. Mean parent proxy PedsQL score was 66 (range: 12.5-98.4), with a mean score of 61.9 in physical function, 88.9 in emotional function, 70.9 in social function, and 58.7 in school function. This was lower than healthy controls overall and in each individual domain except emotional function, which was similar to normative data. Children with moyamoya disease have a lower quality of life than healthy controls within this series. This suggests that children with moyamoya should be offered additional psychosocial support within the community.

Spatial coefficient of variation applied to arterial spin labeling MRI may contribute to predict surgical revascularization outcomes in pediatric moyamoya vasculopathy.

In moyamoya vasculopathy, prolonged arterial transit time may increase the arterial spin labeling (ASL) signal heterogeneity, which can be quantitatively expressed by the spatial coefficient of variation of ASL-CBF (ASL-sCoV). The aim was to compare the accuracy of ASL-sCoV and ASL-CBF with dynamic susceptibility contrast (DSC)-CBF and time-to-peak (DSC-TTP) in the evaluation of perfusion changes and clinical outcome after encephalo-duro-arterio-myo-synangiosis (EDAMS) in pediatric moyamoya patients. A total of 37 children with moyamoya vasculopathy (mean age 6.31years (1.12-15.42)) underwent ASL and DSC perfusion imaging at 3T before and up to 24months after EDAMS. Mean DSC-CBF, mean DSC-TTP, mean ASL-CBF, and ASL-sCoV were calculated in middle cerebral artery territories. Generalized linear model analyses were used to evaluate temporal variations of postoperative perfusion changes and to compare these variations between patients developing valid pial collateralization and those without angiographic improvement. Relationship between perfusion parameters and clinical outcome after surgery was tested using multivariate regression analysis. Significant reduction was observed after EDAMS for ASL-sCoV (P = .002; eta-squared (η2) = 0.247) and DSC-TTP (P < .001; η2 = 0.415), whereas only a trend of increase was observed for DSC-CBF and ASL-CBF, with larger discrepancy before and 6months after surgery. At last follow-up, children developing pial collateralization showed lower absolute ASL-sCoV (P = .002 Cohen's d = 0.84) and DSC-TTP (P = .027; Cohen's d = 0.64) and higher DSC-CBF (P = .002; Cohen's d = - 0.55) compared with those without vascular improvement. Low preoperative and early post-surgical ASL-sCoV predicted better long-term neurological outcome (P < .001; ß = - 0.631). ASL-sCoV may contribute to predict surgical outcomes in pediatric moyamoya patients undergoing EDAMS.

Quantitative analysis of revascularization in ischemic moyamoya disease via whole-brain computed tomography perfusion: A retrospective single-center study.

Ischemic moyamoya disease (MMD) can be treated with the revascularization of superficial temporal artery to middle cerebral artery (STA-MCA) bypass combined with encephalo-duro-arterio-myo-synangiosis (EDAMS) effectively. The purpose of the present study was to quantify the revascularization of STA-MCA bypass combined with EDAMS via whole-brain computed tomography perfusion (WB-CTP).Seventy-nine consecutive patients with ischemic MMD who admitted to our hospital from August 2012 to October 2018 were carried out STA-MCA bypass combined with EDAMS. WB-CTP was performed at 24 hours prior to operation and 3 months following bypass with a follow-up WB-CTP, respectively. Both automatic analysis of WB-CTP (MIStar, Apollo Medical imaging Technology, Melbourne, Australia) for analyzing values of brain volume in delayed time (DT) >3 seconds and DT > 6 seconds, relative cerebral blood flow (γCBF) < 30% and its mismatch ratio or percentage and diffusion-weighted imaging of magnetic resonance imaging in the ischemic penumbra and the infarct core at the 2 time points were studied for verifying the effectiveness of the combined revascularization. Changes in DT values at MCA-terminal territory after revascularization had been investigated. The dynamic data were with reference to the individual cerebellar arteries.All patients with ischemic MMD underwent STA-MCA bypass combined with EDAMS successfully. The preoperative brain volume in DT > 3 seconds in MCA-terminal territory was significantly larger than that of postoperative one (P < .05) in the ischemic penumbra in ischemic MMD. The mismatch ratio in brain volume of 24 hours prior to revascularization in MCA-terminal territory was significantly lower than that of 3 months (P < .05) following combined revascularization. The percentage of mismatch in brain volume of 24 hours prior to revascularization vs that of 3 months and the value of γCBF < 30% were similar to the above mismatch ratio (P < .05). The ratio of postoperative brain volume in DT > 3 seconds vs DT > 6 seconds indicated no significant differences compared with that of preoperative one (P > .05).The WB-CTP can be regarded as a choice for quantifying the combined revascularization in the ischemic penumbra and the infarct core in ischemic MMD. As proposed methods, brain volume in DT > 3 seconds, the value of γCBF < 30% and mismatch ratio in brain volume in MCA-terminal territory should be paid more attention in assessing the validity of STA-MCA bypass combined with EDAMS in ischemic MMD.

Clinical efficacy of different vascular reconstruction on adult moyamoya disease

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.

Combined (direct and indirect) revascularization in adult patients with moyamoya disease

The study objective is to analyze the results of combined (direct and indirect) brain revascularization in adult patients with moyamoya disease. Materials and methods. From February 2015 to August 2016, 12 operations were performed on 12 hemispheres in 7 patients (2 men, 5 women) with moyamoya disease. Six patients had bilateral disease, 1 – unilateral. The age of patients ranged from 25 to 60 year old, the average – 41 year old. Multislice computed tomography (MSCT) perfusion imaging of the brain with a stress test was made in every patient, MSCT angiography of the extraand intracranial arteries was perfomed. Stages of the disease were determined according to the Suzuki–Takaku classification: stage III was revealed in 5 patients, stage IV – in 2. To evaluate the clinical course of the disease the Matsushima classification was used: type VI – in 2 cases, type III – in 2, type IV – in 1, type V – in 1. In 1 case, the pathological vascular network of 1 hemisphere was detected by accident. Extracranial-intracranial (EC–IC) bypass and encephaloduromyosynangiosis (EDMS) was used in 3 cases; a double-barreled EC–IC bypass and EDMS – in 3; a double-barreled EC–IC bypass and encephaloduroperiosteomyosynangiosis – in 1; EC–IC bypass and encephaloduroarteriomyosynangiosis (EDAMS) – in 3. In 1 case was perfomed EC–IC bypass and encephaloduroperiosteosynangiosis. Only indirect revascularization was performed in 1 case – EDAMS. We assessed the neurological status in the follow-up period (5–11 months); MSCT angiography and MSCT perfusion imaging of the brain were performed. Results. All patients in follow-up period demonstrated the increase of cerebrovascular reserve according to MSCT perfusion imaging of the brain with stress tests. Stroke, epilepsy, surgical complications were not detected in the postoperative period. In 1 case, the left-hand monoparesis regressed.Conclusion. The combined (direct and indirect) revascularization may be effective in adult patients with moyamoya disease. To obtain more objective conclusions it is necessary to analyze the long-term results of interventions.

Outcome of revascularization in moyamoya disease: Evaluation of a new angiographic scoring system.

Background:Moyamoya disease (MMD) is a chronic progressive cerebrovascular occlusive disease affecting commonly the anterior circle of Willis. Matushima grade inadequately reflects the angiographic changes postrevascularization procedure.Aims:To analyze the clinical and angiographic outcome of revascularization procedures (direct [ST-middle cerebral artery (MCA) anastomosis] and indirect [encephalo-duro-arterio-myo-synangiosis (EDAMS)]) in MMD and validate a new angiographic scoring system.Materials and Methods:Retrospective study included symptomatic patients of MMD who underwent revascularization; both indirect and combined methods between January 2002 and April 2012. Follow-up angiography was done after at least 3 months. We devised a novel scoring system the “angiographic outcome score” (AOS) including reformation of distal MCA and anterior cerebral artery, regression of basal moyamoya vessels, leptomeningeal collaterals and overall perfusion. AOS was applied to the angiograms independently by a neuroradiologist and a neurosurgeon that were blinded toward its preoperative or postoperative status.Results:Totally 33 patients underwent 36 EDAMS and 4 combined procedures (EDAMS + ST-MCA bypass). The mean follow-up was 20 months. None had recurrent transient ischemic attack or fresh infarct. Postoperative AOS was significantly higher than preoperative AOS. The Spearman rho showed positive correlation between Matushima grade and postoperative AOS. Significant regression of basal moyamoya vessels and increase in number of loci of transdural collaterals was seen.Conclusions:EDAMS is a simple yet effective method of revascularization in both pediatric as well as adult age groups. AOS is a simple, precise and easily reproducible scoring system, which reflects the favorable angiographic changes after revascularization.

Quantitative Angiographic Comparison With the OSIRIS Program Between the Direct and Indirect Revascularization Modalities in Adult Moyamoya Disease

Up-to-date, quantitative angiographic measurement of revascularization extent after bypass surgery has not been reported. To measure the extent of angiographic revascularization quantitatively 6 months postoperatively with the OSIRIS program (University Hospital of Geneva, version 3.1). A total of 75 bypass procedures were performed in 65 consecutive adult moyamoya disease patients, and 71 bypass surgeries in 61 adult moyamoya disease patients were studied 6 months postoperatively with angiography. We performed 5 different types of bypass surgeries: encephaloduroarteriosynangiosis (EDAS), superficial temporal artery-middle cerebral artery anastomosis (SMA), SMA with encephalomyosynangiosis (EMS), SMA with EDAS, and SMA with encephaloduroarteriomyosynangiosis (EDAMS). We also investigated single-photon emission computed tomography (SPECT) results after 6 months postoperatively and compared the angiographic revascularization extent with the SPECT results. The mean values for the extent of revascularization among 5 different bypass surgeries were as follows: 32.4% for EDAS only, 57.4% for SMA only, 58.4% for SMA with EMS, 66.1% for SMA with EDAS, and 70.8% for SMA with EDAMS. There was a statistically significant difference in the extent of revascularization among the 5 surgical modalities (P = .000) and between the EDAS-only group and various SMA groups (P = .000). Statistical analysis between angiographic revascularization extent and SPECT results demonstrated significant statistical correlation (P = .000). The extent of angiographic revascularization in adult moyamoya disease patients was highest in the SMA with EDAMS group and lowest in the EDAS-only group. In addition, angiographic revascularization extent was well correlated with the change in SPECT.

Angiographic Results of Indirect and Combined Bypass Surgery for Adult Moyamoya Disease

ObjectiveThe aim of this study was to compare the efficacy of indirect and combined bypass surgery for treatment of adult moyamoya disease (MMD). The definition of combined bypass surgery is a combination of superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis and indirect anastomosis. Development of collateral circulation after surgery was investigated.MethodsForty three patients (58 hemispheres) with MMD were followed by cerebral angiography for at least six months after surgery, between May 2002 and July 2011. Indirect and combined revascularization surgeries were performed in 33 and 25 cases, respectively. Good outcome was defined as more than group B, in accordance with the method suggested by Matsushima.ResultsDevelopment of collateral circulation was not affected by sex (p = 0.493), clinical features (p = 0.206), or Suzuki stage (p = 0.428). Based on postoperative cerebral angiography, the combined bypass surgery group showed a better angiographic outcome, than the encephaloduroarteriomyosynangiosis (EDAMS) group (p = 0.100, odds ratio [OR] 4.107, 95% confidence interval [CI] 0.700 - 24.096). The combined bypass group showed a better response than the encephaloduroarteriogaleosynangiosis (EDAGS) group (p = 0.088, OR 4.600, 95% CI 0.721 - 29.332). Similar responses were observed for EDAGS and EDAMS (p = 0.886, OR 1.120, 95% CI 0.239 - 5.251). The combined bypass group showed a better response than the indirect group (p = 0.064, OR 4.313, 95% CI 0.840 - 22.130).ConclusionResults of this study demonstrate that combined bypass results in better revascularization on angiographic evaluation in adult MMD. Therefore, among surgical procedures, combined bypass is a choice that can be recommended.

Effect of preoperative skull block on pediatric moyamoya disease

Stable hemodynamics, normocapnia, and adequate pain relief are considered important factors in the reduction of neurological complications in pediatric patients undergoing encephaloduroarteriomyosynangiosis (EDAMS) operations for the treatment of moyamoya disease. A preoperative skull block may reduce hemodynamic fluctuations and hypo- or hyperventilation due to emergence delirium or oversedation and provide adequate pain relief, thereby reducing postoperative morbidity. Pediatric patients (age 3-13 years) undergoing EDAMS surgery for moyamoya disease were randomly divided into a nerve block (NB) group (18 cases) or control group (21 cases). The treatment group patients received a preoperative NB (0.25% 5-8 ml bupivacaine mixed with 20-40 mg methylprednisolone) targeting the supraorbital, supratrochlear, auriculotemporal, and posterior auricular nerves. Patients in the control group did not receive NB. General anesthesia with sevoflurane was induced in both groups. In the NB group, stable hemodynamic parameters were obtained with a lower sevoflurane concentration than in the control group. For delirious awakening, the odds ratio in the control group was 4.9 compared with the NB group. Pain and analgesic requirement were higher in the control patients than in the NB-treated patients during the postanesthesia care unit stay. However, the arterial CO(2) tension in the postanesthesia care unit did not differ between the 2 groups. The odds ratio in the control group for the rate of morbidity (cerebral infarction and reversible ischemic neurological deficits) during the first 24 hours following the operation was 3.2 compared with the NB group. The use of skull block during EDAMS surgery provided easy hemodynamic control, calm awakening, and better pain relief and may be related to the reduced postoperative morbidity.

もやもや病に対する前大脳動脈，中大脳動脈領域への直接血行再建とその効果

Vascular reconstruction for moyamoya disease can be achieved by STA-MCA anastomosis, STA-ACA anastomosis as direct revascularization, EDAMS (encephalo-duro-arterio-myo-synangiosis) and EGAS (encephalo-galeo-arterio-synangiosis) as indirect vascular reconstruction. We revascularized 19 hemispheres of 13 patients—4 pediatric and 9 adult patients—using STA-ACA and STA-MCA anastomosis, EGAS for the medial frontal lobe, and EDAMS for the lateral frontal lobe. All of these procedures improved cerebral blood flow in the frontal lobe, resulting in improved mental and intellectual acuity in pediatric patients, which was apparent from obviously improved concentration and intellectual ability soon after surgery.

Surgical results in pediatric moyamoya disease: Angiographic revascularization and the clinical results

Objecive We retrospectively reviewed the pediatric patients with moyamoya disease (MMD) who underwent bypass surgery at our institution to compare the surgical results according to the surgical procedures. Patients and methods There were 24 total patients (age range: 2–15 years; mean age: 8.2 years). Twelve patients underwent encephalo-duro-arterio-synangiosis (EDAS) on 16 sides, 5 patients underwent encephalo-duro-arterio-myo-synangiosis (EDAMS) on 8 sides and 7 patients underwent combined superficial temporal artery–middle cerebral artery (STA–MCA) anastomosis with EDAMS (STA–MCA–EDAMS) on 12 sides. The postoperative results were evaluated between 4 months and 5 years following surgery in terms of the angiographic revascularization and the clinical outcome. Results EDAMS, regardless of the combined STA–MCA anastomosis, was significantly effective for achieving a good extent of the postoperative angiographic revascularization as compared with simple EDAS ( P < 0.05). STA–MCA–EDAMS tended to be better with respective to the relief of preoperative ischemic symptoms as compared with simple EDAS, although there was no significant statistical difference. Conclusion These results suggest that EDAMS with or without the combination of STA–MCA anastomosis was very useful for the formation of collateral circulation in comparison with simple EDAS for treating the pediatric patients with MMD, although these findings were not well correlated with the clinical outcomes.

Indirect revascularisation for paediatric moyamoya disease: The EDAMS technique

Moyamoya disease can have devastating effects on paediatric patients as a result of cerebral ischaemia. Several direct and indirect surgical methods have been devised in order to facilitate revascularisation. Debate has long ensued about which methods are most efficacious and yet safe. The authors describe their experience with a straightforward method for performing the EDAMS (encephalo-duro-arterio-myo-synangiosis) technique.

Moyamoya disease initially mimicking MELAS syndrome in a 14-month-old child.

Moyamoya disease is a neurological disease rarely seen in children outside Japan. It is difficult to differentiate moyamoya disease in its early stage from mitochondrial disorders in children when nontraumatic ischemic stroke is considered. We present a 14-month-old Taiwanese female child who had suffered from sudden onset of nontraumatic seizure attack, after which progressive left limb weakness was noted. Initial brain imaging and a series of laboratory studies were performed which suggested mitochondrial disorders, especially mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome. The patient underwent an oral glucose lactate stimulation test (OGLST), and mitochondrial disorders were not favored. MR angiography of the circle of Willis showed moyamoya disease. The patient then underwent an encephalo-duro-arterio-myo-synangiosis (EDAMS) operation. Evaluation of the neurodevelopmental and intelligence outcome required close long-term follow-up.

Long-Term Outcomes of Pediatric Moyamoya Disease Treated by Encephalo-Duro-Arterio-Synangiosis

To investigate the efficacy of encephalo-duro-arterio-synangiosis (EDAS) for the treatment of pediatric moyamoya disease, we analyzed 11 patients who were followed up for more than 100 months. Among 22 sides in 11 patients, we performed EDAS on 16 sides in 10 pa- tients, encephalo-duro-arterio-myo-synangiosis (EDAMS) on 5 sides in 4 patients and encephalo-myo-synangiosis (EMS) on 1 side. Of the 11 patients, 8 patients showed normal development and had no neurological deficit. The remaining 3 patients showed mild to moderate neurological deficits, but in these cases, pre- and perioperative insults were considered to be attributable to the morbidities. Of 13 sides treated by EDAS, well-developed neovascularization was observed in 12. However, well-developed neovascularization was observed in only 3 out of 6 sides treated by EMS or EDAMS. These data might indicate that EDAS is a therapeutic alternative for the surgical treatment of pediatric moyamoya disease.

もやもや病に対するRibbonen encephalo-duro-arterio-myo-synangiosis (Ribbon EDAMS) 施行例における術前・術後の脳循環動態の検討

To evaluate the efficacy of Ribbon encephalo-duro-arterio-myo-synangiosis (EDAMS) for patients with childhood moyamoya disease, we analyzed pre- and postoperative cerebral hemodynamics at resting and at acetazolamide-challenging in the areas of the anterior cerebral arteries (ACAs) and the middle cerebral arteries (MCAs).We reviewed 10 children with moyamoya disease treated with Ribbon EDAMS. The value of cerebral perfusion (CP) was obtained by dividing the radioisotope uptake in each ROI by the mean uptake in the cerebellum measured using 99mTc-hexamethyl-propyleneamine oxime single-photon emission CT. The value of vasodilatory capacity (VDC) was defined as the change in CP of each ROI after acetazolamide-challenging.Pre-existing clinical symptoms disappeared after Ribbon EDAMS in 8 patients. The other 2 patients needed another supplementary reconstructive surgery to resolve the neurological problems. CP at resting in the areas of the MCAs (n=38) changed significantly from 0.98±0.18 to 1.04±0.16 (P<0.05). VDC in the areas of MCAs (n=26) also improved from -15±21% to +6±19% (P<0.01). Although the changes of CP at resting in the areas of the ACAs (n=32, from 0.93±0.19 to 0.96±0.11) were not significant, VDC in the areas of the ACAs (n=20) improved from -22±29% to +5±19% (P<0.01).We conclude Ribbon EDAMS is effective to improve hemodynamic compromise in both areas of the ACAs and the MCAs in patients with childhood moyamoya disease.