Pediatric moyamoya MRI score: an imaging-based scale to predict outcomes in surgically treated pediatric patients with moyamoya

OBJECTIVE Moyamoya is a progressive arteriopathy that predisposes patients to stroke due to stenosis of the intracranial internal carotid arteries and their proximal branches. Despite the morbidity caused by this condition, the ability to accurately predict prognosis for individual patients remains challenging. The goal of this study was to develop a systematic scoring method based on parenchymal findings on preoperative brain MRI to predict long-term outcomes for surgically treated pediatric patients with moyamoya. METHODS A retrospective surgical cohort of pediatric patients (≤ 18 years of age at the time of the initial surgery) with moyamoya from a single center were studied. Radiological variables with existing correlations between outcomes in moyamoya or other vascular diseases were chosen to score preoperative MRI based on easily defined parenchymal findings that could be rapidly assessed and used to make a numeric score. Calculated scores were correlated with clinical outcome measures using the Pearson correlation coefficient and area under the receiver operating characteristic curve (AUROC). RESULTS A total of 35 children with moyamoya disease or moyamoya syndrome were included in the study, with a median follow-up time of 2.6 years from the time of surgery. The pediatric moyamoya MRI score (PMMS) consists of ischemic changes (0–2; 0 = none, 1 = focal, 2 = diffuse), encephalomalacia (0–2; 0 = none, 1 = focal, 2 = diffuse), and hemorrhage (0–1; 0 = not present, 1 = present). PMMSs were highly correlated with pediatric modified Rankin Scale scores at the last follow-up (r = 0.7, 95% CI 0.44–0.84; p < 0.001) as a six-point scale, and when dichotomized (AUROC = 0.85). CONCLUSIONS The PMMS was found to be a simple tool based on preoperative MRI data that could be quickly and easily calculated and correlated with disability. This scoring method may aid future development of predictive models of outcomes for children with moyamoya disease and moyamoya syndrome.

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Long-term follow-up of surgically treated juvenile patients with moyamoya disease

Journal of Neurosurgery Pediatrics ◽

10.3171/2012.8.peds11539 ◽

2012 ◽

Vol 10 (5) ◽

pp. 451-456 ◽

Cited By ~ 22

Author(s):

Maki Mukawa ◽

Tadashi Nariai ◽

Yoshiharu Matsushima ◽

Yoji Tanaka ◽

Motoki Inaji ◽

...

Keyword(s):

Moyamoya Disease ◽

Daily Living ◽

Pediatric Patients ◽

Late Onset ◽

Current Status ◽

Long Term Outcome ◽

Initial Surgery ◽

Cerebrovascular Events

Object Surgical revascularization is considered an effective treatment for juvenile patients with moyamoya disease (MMD). Yet the long-term outcome in surgically treated patients still needs to be clarified. More than 30 years have passed since the authors' department started intensively treating pediatric patients with MMD using indirect anastomosis techniques. In this study the authors surveyed the current status of these patients. Methods Activities of daily living (ADLs) were surveyed and present clinical status was assessed based on the modified Rankin Scale (mRS). Cerebrovascular events subsequent to surgical treatment were also recorded. Results Since 1979, 208 patients younger than 19 years of age with MMD were surgically treated and followed up for > 3 years. Data were available on 172 patients (83%), who had been followed up for a mean of 14.3 years (range 3–32 years). Activity of daily living outcomes were as follows: 138 patients (80.2%) had mRS scores of 0–2, 29 (16.9%) a score of 3, 1 (0.6%) a score of 4, 1 (0.6%) a score of 5, and 3 (1.7%) a score of 6. Cerebrovascular events occurred 8 or more years after surgery in 6 patients (3.4%), that is, 6 hemorrhages and 3 infarctions. The cumulative risk of late-onset stroke at 10, 20, and 30 years after surgical intervention was 0.8%, 6.3%, and 10.0%, respectively. Conclusions This long-term survey demonstrated that most surgically treated pediatric patients with MMD maintain good ADL outcomes. However, a significant number of new cerebrovascular events occurred more than 10 years after the initial surgery. Additional follow-up will help to identify which events may occur during the adult years of patients treated as children.

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Abstract TP397: Clinical And Functional Outcome Of Adult Patients Diagnosed With Moyamoya Disease In Childhood

Stroke ◽

10.1161/str.48.suppl_1.tp397 ◽

2017 ◽

Vol 48 (suppl_1) ◽

Author(s):

David Fam ◽

Nomazulu Dlamini ◽

Cheryl Jaigobin

Keyword(s):

Ischemic Stroke ◽

North America ◽

Natural History ◽

Functional Outcome ◽

Moyamoya Disease ◽

Vascular Event ◽

Moyamoya Syndrome ◽

The Mean

Background: Moyamoya disease is a progressive occlusive arteriopathy of the terminal ICA and its branches, leading to the formation of unstable collateral vessels. The disease is found worldwide, and is associated with a number of predisposing conditions, termed moyamoya syndrome. Currently there is a paucity of data on the natural history of moyamoya disease in North America, especially the long-term outcome of pediatric moyamoya. Objective: Our objectives were to determine the rate of recurrent TIA, ischemic stroke and intracerebral hemorrhage in patients presenting with pediatric Moyamoya disease and to assess long-term functional outcome. Methods: This study was a retrospective chart review of 52 patients presenting with pediatric moyamoya disease. We included patients aged 18 or older with a diagnosis of moyamoya disease or syndrome. All patients had confirmatory baseline vascular imaging (MRA or CTA +/- cerebral angiography). Baseline demographic variables and annual vascular event recurrence risk were obtained from the records. Modified Rankin Score (MRS) at presentation and last follow-up were determined from clinical records. Results: Of the original cohort, 34 patients were included for analysis. The mean age of the patients was 23.9 years. The mean age at presentation was 9 years (SD=4) with an average follow-up of 11.3 years (SD=5.1) for a total of 383 patient years. There was slight female predominance (1.4:1). Fifty percent of patients (17) presented with ischemic stroke. A total of 26 patients (76%) underwent surgery, with 7 (21%) requiring repeat surgery. The annual vascular recurrent event rate was 5.0% (19/383), which was not significantly different between surgical and non-surgical groups or between different moyamoya syndrome subtypes. Most of the recurrent events were TIA (annual recurrence 3.7%). There was no significant difference between initial MRS, MRS at last follow-up and mean change in MRS between surgical and non-surgical groups. Conclusion: Our study is the largest North American natural history study of pediatric moyamoya. Our observations indicate that pediatric moyamoya in North America have low recurrent vascular event rates and long-term functional outcomes are good, even in conservatively managed patients.

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Abstract 84: Late (5-20 Years) Outcome After STA-MCA Anastomosis and Ultimate Indirect Bypass in Patients With Moyamoya Disease

Stroke ◽

10.1161/str.51.suppl_1.84 ◽

2020 ◽

Vol 51 (Suppl_1) ◽

Author(s):

Satoshi Kuroda ◽

Naoki Nakayama ◽

Shusuke Yamamoto ◽

Daina Kashiwazaki ◽

Haruto Uchino ◽

...

Keyword(s):

Disease Progression ◽

Moyamoya Disease ◽

Hemorrhagic Stroke ◽

Short Term ◽

Post Surgery ◽

Initial Surgery ◽

Cerebrovascular Events ◽

Late Morbidity

Background and Purpose: Surgical revascularization is now known to improve the outcome in patients with moyamoya disease. However, majority of previous studies reported their short-term (<5 years) outcome. Therefore, this study was aimed to evaluate long-term (5 to 20 years) outcome after STA-MCA anastomosis and ultimate indirect bypass, encephalo-duro-myo- arterio-pericranial synangiosis (EDMAPS). Methods: Cumulative incidence of late morbidity/mortality and disease progression were evaluated among 93 patients who underwent STA-MCA anastomosis and EDMAPS. All of them were prospectively followed up for longer than 5 years post-surgery (mean, 10.5±4.4 years). There were 35 pediatric and 58 adult patients. Clinical diagnosis included TIA or ischemic stroke in 80 patients, hemorrhagic stroke in 10, and asymptomatic in 3. STA-MCA anastomosis and EDMAPS were performed onto their 141 hemispheres. MRI and MRA were performed every 6 or 12 years during follow-up periods. Results: During follow-up periods, 92/93 patients were free from any stroke or death, but one recurred hemorrhagic stroke (0.10% per patient-year). Disease progression occurred in the territory of the contralateral carotid or posterior cerebral artery (PCA) in 19 hemispheres of 15 patients (1.5% per patient-year). The interval between initial surgery and disease progression varied from 0.5 to 15 years. Repeat bypass surgery for anterior and posterior circulations resolved ischemic attacks in all 10 patients. Conclusion: STA-MCA anastomosis and EDMAPS would be the best choice to prevent further cerebrovascular events for longer than 10 years by widely providing surgical collaterals to both the MCA and ACA territories. However, regular follow-up would be essential for longer than 10 years post-surgery to identify the disease progression in the territory of contralateral carotid artery and PCA and prevent late cerebrovascular events.

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Effectiveness of surgical revascularization for stroke prevention in pediatric patients with sickle cell disease and moyamoya syndrome

Journal of Neurosurgery Pediatrics ◽

10.3171/2017.1.peds16576 ◽

2017 ◽

Vol 20 (3) ◽

pp. 232-238 ◽

Cited By ~ 13

Author(s):

Wuyang Yang ◽

Risheng Xu ◽

Jose L. Porras ◽

Clifford M. Takemoto ◽

Syed Khalid ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Stroke Prevention ◽

Pediatric Patients ◽

Moyamoya Syndrome ◽

Cell Disease ◽

Surgical Revascularization ◽

Transfusion Therapy ◽

Indirect Revascularization

OBJECTIVESickle cell disease (SCD) in combination with moyamoya syndrome (MMS) represents a rare complication of SCD, with potentially devastating neurological outcomes. The effectiveness of surgical revascularization in this patient population is currently unclear. The authors’ aim was to determine the effectiveness of surgical intervention in their series of SCD-MMS patients by comparing stroke recurrence in those undergoing revascularization and those undergoing conservative transfusion therapy.METHODSThe authors performed a retrospective chart review of patients with MMS who were seen at the Johns Hopkins Medical Institution between 1990 and 2013. Pediatric patients (age < 18 years) with confirmed diagnoses of SCD and MMS were included. Intracranial stroke occurrence during the follow-up period was compared between surgically and conservatively managed patients.RESULTSA total of 15 pediatric SCD-MMS patients (28 affected hemispheres) were included in this study, and all were African American. Seven patients (12 hemispheres) were treated with indirect surgical revascularization. The average age at MMS diagnosis was 9.0 ± 4.0 years, and 9 patients (60.0%) were female. Fourteen patients (93.3%) had strokes before diagnosis of MMS, with an average age at first stroke of 6.6 ± 3.9 years. During an average follow-up period of 11.6 years, 4 patients in the conservative treatment group experienced strokes in 5 hemispheres, whereas no patient undergoing the revascularization procedure had any strokes at follow-up (p = 0.029). Three patients experienced immediate postoperative transient ischemic attacks, but all recovered without subsequent strokes.CONCLUSIONSIndirect revascularization is suggested as a safe and effective alternative to the best medical therapy alone in patients with SCD-MMS. High-risk patients managed on a regimen of chronic transfusion should be considered for indirect revascularization to maximize the effect of stroke prevention.

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Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease

Journal of Neurosurgery Pediatrics ◽

10.3171/2014.12.peds14522 ◽

2015 ◽

Vol 16 (1) ◽

pp. 64-73 ◽

Cited By ~ 23

Author(s):

Christoph J. Griessenauer ◽

Jeffrey D. Lebensburger ◽

Michelle H. Chua ◽

Winfield S. Fisher ◽

Lee Hilliard ◽

...

Keyword(s):

Sickle Cell ◽

Stroke Prevention ◽

Pediatric Patients ◽

Case Series ◽

Secondary Stroke Prevention ◽

Moyamoya Syndrome ◽

Cell Disease ◽

Transfusion Therapy ◽

The Mean

OBJECT Pediatric patients with sickle cell disease (SCD) and moyamoya syndrome (MMS) are at significant risk for cerebrovascular accidents despite chronic transfusion therapy. Encephaloduroarteriosynangiosis (EDAS) and encephalomyoarteriosynangiosis (EMAS) are additional therapeutic options for these patients. To date, the incidence of complications after and efficacy of EDAS and EMAS in stroke prevention in this population have been described in several institutional case series reports, but no randomized prospective trials have been reported. METHODS The authors retrospectively reviewed the cases of all pediatric patients at the University of Alabama at Birmingham with a history of homozygous hemoglobin S (HbS) and sickle cell/β-thalassemia (SB0 thalassemia) and on chronic transfusion therapy, including 14 patients with MMS who underwent EDAS or EMAS. RESULTS Sixty-two patients with SCD and on chronic transfusion therapy were identified. After exclusion of patients on chronic transfusion therapy for indications other than stroke prevention, 48 patients (77.4%) remained. Of those patients, 14 (29.1%) underwent EDAS or EMAS. Nine (18.8%) and 25 (52.1%) patients were on chronic transfusion therapy for primary or secondary stroke prevention, respectively, but did not undergo EDAS or EMAS. The 14 patients with SCD and radiological evidence of MMS and on chronic transfusion therapy for primary or secondary stroke prevention underwent 21 EDAS or EMAS procedures for progressive vascular disease (92.9% of patients), stroke (71.4%), and/or seizure (7.1%). The mean (± SD) time from initiation of chronic transfusion therapy to EDAS or EMAS was 76.8 ± 58.8 months. Complications included 1 perioperative stroke, 1 symptomatic subdural hygroma, 1 postoperative seizure, and 1 case of intraoperative cerebral edema that required subsequent cranioplasty. Before EDAS or EMAS, the stroke rate was calculated to be 1 stroke per 7.8 patient-years. One additional stroke occurred during the follow-up period (mean follow-up time 33.7 ± 19.6 months), resulting in a post-EDAS/EMAS stroke rate of 1 stroke per 39.3 patient-years, a 5-fold reduction compared with that in the pre-EDAS/EMAS period. The patients’ mean pre-EDAS/EMAS HbS level of 29.5% ± 6.4% was comparable to the mean post-EDAS/EMAS HbS level of 25.5% ± 6.1% (p = 0.104). CONCLUSIONS The results of this retrospective case series in a large cohort of pediatric patients with SCD and MMS suggest that EDAS/EMAS provides a stroke-prevention benefit with an acceptably low morbidity rate. Given the combined experience with EDAS and EMAS for this indication at this and other institutions, a prospective clinical trial to assess their efficacy compared with that of chronic transfusion therapy alone is warranted.

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The role of left atrial strain in patients with functional tricuspid regurgitation before and after annuloplasty: a long-term follow-up study

Cardiovascular Ultrasound ◽

10.1186/s12947-021-00264-z ◽

2021 ◽

Vol 19 (1) ◽

Author(s):

Qing-long Meng ◽

Hong Meng ◽

Jia Tao ◽

Shu Yang ◽

Hao Wang

Keyword(s):

Tricuspid Regurgitation ◽

Mitral Stenosis ◽

Left Atrial ◽

Pearson Correlation ◽

Characteristic Curve ◽

Control Group ◽

Rheumatic Mitral Stenosis ◽

Functional Tricuspid Regurgitation

Abstract Background Functional tricuspid regurgitation (TR) is common among patients with left heart disease and may recur during the follow-up period after selective tricuspid valve annuloplasty (TVA). This study aims to analyse the relationship between left atrial (LA) strain and the degree of preoperative functional TR and to explore the role of LA strain in predicting TR recurrence. Methods This study included 63 patients with rheumatic mitral stenosis who underwent mitral valve replacement and concomitant TVA. Additionally, 20 healthy controls were enrolled. Preoperative conventional LA echocardiographic parameters and LA strain were measured. The association between LA strain and preoperative functional TR severity was analysed by Pearson correlation. Predictors of recurrent TR were determined by multivariate logistic regression analyses. Results Compared with the control group, the mitral stenosis group developed a significant impairment in terms of LA strain. The degree of preoperative functional TR exhibited moderate correlations with LA reservoir strain (r = − 0.57) and LA conduit strain (r = 0.48). During a median follow-up period of 66.4 ± 36.4 months, TR recurred in 18 patients. Preoperative LA reservoir strain and the mean transmitral gradient were predictors of postoperative TR recurrence. When the two indexes were combined to establish a prediction, the sensitivity and specificity of prediction increased. The area under the receiver operating characteristic curve of the combined indicator was higher than those of the single indicators (0.90 vs. 0.70 and 0.72). Conclusions LA strain correlates with preoperative functional TR severity in patients with rheumatic mitral stenosis. The LA reservoir strain and preoperative mean transmitral gradient are independent predictive factors for recurrent TR after TVA.

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Abstract TMP13: The Prevalence of Microbleeds in Moyamoya Disease and Moyamoya Syndrome in the United States

Stroke ◽

10.1161/str.47.suppl_1.tmp13 ◽

2016 ◽

Vol 47 (suppl_1) ◽

Author(s):

Mo Chen ◽

Sepand Salehian ◽

Maki Mukawa-Sawada ◽

Marco Pinho ◽

Babu Welch ◽

...

Keyword(s):

United States ◽

Moyamoya Disease ◽

Cerebral Hemorrhage ◽

Hemorrhagic Stroke ◽

Asian Population ◽

The United States ◽

Moyamoya Syndrome ◽

Increased Risk ◽

Ischemic Events

Introduction: Silent cerebral microbleeds (CMB) are common in Moyamoya Disease (MMD) and Moyamoya syndrome (MMS) in Asia. The incidence was reported to be 30-40%. The presence of CMB was found to be a predictor for subsequent cerebral hemorrhage in MMD. The significance of CMB in MMD/MMS in non-Asian population has not been reported. We try to investigate the prevalence of CMB in MMD/MMS in United States and its predictive value for subsequent cerebral hemorrhage. Methods: Moyamoya Database was established in our institution after reviewing patients with ICD9 code of Moyamoya Disease or Moyamoya Syndrome or cerebrovascular occlusive disease from 2007 to 2015. Patients in the database were reviewed retrospectively and included in the study if there were MR images (including GRE, SWI or T2* sequences) at diagnosis or during follow up and available for review. Patients with poor image quality were excluded. Patients were noted to have microbleeds if it was found on initial or follow up MRI. Multivariate logistic regression analysis was used to identify clinical and imaging predictors of CMB. Results: Sixty-three females and fourteen males were included with average age of 39 ± 13 at the time of diagnosis. The majorities were MMD (79.2%) and presented with ischemic events (79.2%). Hemorrhagic stroke was found in 9 (11.7%) patients before diagnosis. Ethnicity included Caucasian (61%), Asian (10.4%), Black (11.7%), and Hispanic (16.9%). Of total 77 patients, 7 (9.1%) had CMB but none of them presented with hemorrhagic stroke. During follow up of average 36 ± 29 months in these 7 patients, no cerebral hemorrhage was reported while ischemic events occurred in 2 (28.6%) patients. Of 70 patients without CMB, 51 had follow up of average 47 ± 46 months. One (2%) had cerebral hemorrhage while 12 (23.5%) had ischemic events. No independent predictors for CMB were identified among age, gender, ethnicity, etiology, presenting stroke, and comorbidities. Conclusions: Silent CMB was less prevalent in MMD/MMS in United States than in Asia. No specific risk factors were identified to be associated with the presence of silent CMB. It was not associated with increased risk of subsequent cerebral hemorrhage in non-Asian population. Further studies are needed to confirm these findings.

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Encephaloduroarteriosynangiosis for Pediatric Moyamoya Disease: A Single-Center Experience With 67 Cases in China

Journal of Child Neurology ◽

10.1177/0883073818798515 ◽

2018 ◽

Vol 33 (14) ◽

pp. 901-908 ◽

Cited By ~ 1

Author(s):

Chengjun Wang ◽

Meng Zhao ◽

Jia Wang ◽

Shuo Wang ◽

Dong Zhang ◽

...

Keyword(s):

Moyamoya Disease ◽

Pediatric Patients ◽

Positive Impact ◽

Case Series ◽

Stroke Recurrence ◽

Long Term Outcomes ◽

Single Center Experience ◽

The Mean

The purpose of this study is to investigate the surgical results and long-term outcomes of encephaloduroarteriosynangiosis for moyamoya disease in pediatric patients. We performed a retrospective analysis of 67 pediatric patients with moyamoya disease who underwent encephaloduroarteriosynangiosis in Beijing Tiantan Hospital. The case series included 36 boys and 31 girls. All the patients underwent surgical revascularization, and a total of 93 encephaloduroarteriosynangiosis procedures were performed (41 unilateral, 26 bilateral). The mean follow-up period after surgery was 30 months. During follow-up, ischemic stroke events were detected in 5 patients and the stroke rate for pediatric patients who underwent encephaloduroarteriosynangiosis procedure was 7.1% per patient-years. At the last follow-up, favorable outcomes (modified Rankin Scale score ≤ 2) were observed in 65 cases (97.0%). Our study suggests that long-term surgical outcome of encephaloduroarteriosynangiosis in pediatric moyamoya disease patients is satisfactory, and this technique has a positive impact on the prevention of stroke recurrence.

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No evidence of disease activity is associated with reduced rate of axonal retinal atrophy in MS

Neurology ◽

10.1212/wnl.0000000000004736 ◽

2017 ◽

Vol 89 (24) ◽

pp. 2469-2475 ◽

Cited By ~ 20

Author(s):

Marco Pisa ◽

Simone Guerrieri ◽

Giovanni Di Maggio ◽

Stefania Medaglini ◽

Lucia Moiola ◽

...

Keyword(s):

Disease Activity ◽

Operating Characteristic ◽

Characteristic Curve ◽

Brain Mri ◽

Area Under The Curve ◽

Fiber Layer ◽

Additional Tool ◽

Disability Status ◽

Reduced Rate

Objective:To explore, in a longitudinal study, the usefulness of optical coherence tomography (OCT) in monitoring people with multiple sclerosis (MS) by testing the association between retinal nerve fiber layer (RNFL) thinning and clinical and brain MRI criteria of no evidence of disease activity (NEDA).Methods:OCT, visual evoked potentials (VEPs), and disability, using the Expanded Disability Status Scale (EDSS), were tested at baseline and after 2 years in 72 patients, 63 with routine yearly brain MRI.Results:Longitudinal mean binocular RNFL thinning, in absence of optic neuritis during follow-up, was correlated with EDSS worsening, also controlling for baseline EDSS, RNFL, disease duration, and MS subtype (Spearman ρ −0.462, p < 0.001; partial correlation coefficient −0.437, p < 0.001). At follow-up, patients classified as NEDA (20; 31.7%) had RNFL loss of −0.93 μm ± 1.35 SD, while patients with active disease had −2.83 μm ± 2 SD thinning (t test; p < 0.001). At logistic regression, mean RNFL reduction correctly classified 76.2% of patients as NEDA at 2 years (R2 0.355; p = 0.003). A cutoff of −1.25 μm RNFL loss classified NEDA status with specificity 81.4% and sensitivity 80% (receiver operating characteristic curve: area under the curve 0.8; p < 0.001). No significant longitudinal correlations were found between changes in RNFL and in VEP latencies or scores.Conclusions:NEDA is associated with a relatively preserved RNFL over 2 years. A greater neuroretinal loss was detected even in patients with clinical evidence of disease activity independently from changes in brain MRI lesions, prompting further validation of OCT as an additional tool in MS monitoring.

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Surgical Outcomes for Moyamoya Angiopathy at Barrow Neurological Institute With Comparison of Adult Indirect Encephaloduroarteriosynangiosis Bypass, Adult Direct Superficial Temporal Artery–to–Middle Cerebral Artery Bypass, and Pediatric Bypass

Neurosurgery ◽

10.1227/neu.0000000000000017 ◽

2013 ◽

Vol 73 (3) ◽

pp. 430-439 ◽

Cited By ~ 46

Author(s):

Adib A. Abla ◽

Gurpreet Gandhoke ◽

Justin C. Clark ◽

Mark E. Oppenlander ◽

Gregory J. Velat ◽

...

Keyword(s):

Pediatric Patients ◽

Artery Bypass ◽

Superficial Temporal Artery ◽

Adult Patients ◽

Consecutive Series ◽

Modified Rankin Scale ◽

Moyamoya Angiopathy ◽

Initial Surgery ◽

Direct Bypass

Abstract BACKGROUND: Untreated, moyamoya angiopathy is a progressive vaso-occlusive process that can lead to ischemic or hemorrhagic stroke. OBJECTIVE: To review 1 institution's surgical experience with both direct and indirect bypass (encephaloduroarteriosynangiosis) in adult and pediatric groups. METHODS: A retrospective review was conducted of a consecutive series of patients treated for moyamoya angiopathy between 1995 and 2009. RESULTS: Thirty-nine adult patients underwent indirect bypass as their initial therapy; 29 adult patients underwent direct bypass. Twenty-four pediatric patients included 20 indirect bypasses and 4 direct bypasses. Overall, 140 hemispheres were treated; 48 patients received revascularization of both hemispheres. There were 14 additional revascularization procedures (10% per hemisphere) performed over a site of continued hypoperfusion postoperatively. Fourteen postoperative ischemic strokes occurred during the entire follow-up (10% per hemisphere), and the Kaplan-Meier analysis was not significantly different between groups (P = .59). Four grafts (9.09%) had failed at radiographic follow-up of the 44 direct bypasses performed. Before the initial surgery, the modified Rankin Scale score was 1.58 ± 0.93, 1.48 ± 0.74, and 1.8 ± 1.1 in the pediatric, adult direct, and adult indirect groups (P = .39). At last follow-up, it was 1.29 ± 1.31, 1.09 ± 0.90, and 1.94 ± 1.51 (P = .04) in the pediatric, adult direct, and adult indirect groups. CONCLUSION: This series demonstrates that both direct and indirect bypasses can be equally effective in preventing stroke. However, in adult patients, direct bypass patients had significantly greater improvement in symptoms, as seen in modified Rankin Scale scores. Pediatric patients, despite undergoing predominantly indirect bypasses, fared roughly the same as the adults in the direct bypass group.

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