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Whether surgical revascularization can prevent recurrent hemorrhage in hemorrhagic moyamoya disease (HMD) patients remains a matter of debate. This study mainly aims at the comparison of treatment effect between surgical revascularization and conservative treatment of adult HMD patients. We retrospectively enrolled 322 adult HMD patients, including 133 in revascularization group and 189 in conservative group. The revascularization group included patients who underwent combined (n = 97) or indirect revascularization alone (n = 36). Ninety-two and forty-one patients underwent unilateral and bilateral revascularization respectively. The modified Rankin scale (mRS) was used to assess the functional status. The comparison was made based on initial treatment paradigm among two categories: (1) revascularization vs. conservative, (2) unilateral vs. bilateral revascularization. The rebleeding rate was significantly lower in revascularization group than that in conservative group (14.3% vs. 27.0%, P = 0.007). As for the functional outcomes, the average mRS was significantly better in revascularization group (1.7 ± 1.5) than that in conservative group (2.8 ± 1.9) (P < 0.001). The death rate in revascularization group was 8.3% (11/133), comparing to 20.1% (38/189) in conservative group (P = 0.004). While comparing between unilateral and bilateral revascularization within the revascularization group, the result demonstrated lower annual rebleeding rate in bilateral group (0.5%/side-year) than that in unilateral group (3.3%/side-year) (P = 0.001). This study proved the better treatment efficacy of surgical revascularization than that of conservative treatment in HMD patients, regarding both in rebleeding rate and mortality rate. Furthermore, bilateral revascularization seems more effective in preventing rebleeding than unilateral revascularization. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to develop and validate a predictive nomogram model for long-term rebleeding events in patients with hemorrhagic moyamoya disease (HMMD)., Methods: In total, 554 patients with HMMD from the Fifth Medical Center of the Chinese PLA General Hospital (5-PLAGH cohort) were included and randomly divided into training (390 patients) and internal validation (164 patients) sets. An independent cohort from the First Medical Center and Eighth Medical Center of Chinese PLA General Hospital (the 1-PLAGH and 8-PLAGH cohort) was used for external validation (133 patients). Univariate Cox regression analysis and least absolute shrinkage and selection operator (LASSO) regression algorithm were used to identify significant factors associated with rebleeding, which were used to develop a nomogram for predicting 5- and 10-year rebleeding., Results: Intraventricular hemorrhage was the most common type of cerebral hemorrhage (39.0% of patients in the 5-PLAGH cohort and 42.9% of the 1-PLAGH and 8-PLAGH cohort). During the mean ± SD follow-up period of 10.4 ± 2.9 years, 91 (16.4%) patients had rebleeding events in the 5-PLAGH cohort. The rebleeding rates were 12.3% (68 patients) at 5 years and 14.8% (82 patients) at 10 years. Rebleeding events were observed in 72 patients (14.3%) in the encephaloduroarteriosynangiosis (EDAS) surgery group, whereas 19 patients (37.3%) experienced rebleeding events in the conservative treatment group. This difference was statistically significant (p < 0.001). We selected 4 predictors (age at onset, number of episodes of bleeding, posterior circulation involvement, and EDAS surgery) for nomogram development. The concordance index (C-index) values of the nomograms of the training cohort, internal validation cohort, and the external validation cohort were 0.767 (95% CI 0.704-0.830), 0.814 (95% CI 0.694-0.934), and 0.718 (95% CI 0.661-0.775), respectively. The nomogram at 5 years exhibited a sensitivity of 48.1% and specificity of 87.5%. The positive and negative predictive values were 38.2% and 91.3%, respectively. The nomogram at 10 years exhibited a sensitivity of 47.1% and specificity of 89.1%. The positive and negative predictive values were 48.5% and 88.5%, respectively., Conclusions: EDAS may prevent rebleeding events and improve long-term clinical outcomes in patients with HMMD. The nomogram accurately predicted rebleeding events and assisted clinicians in identifying high-risk patients and devising individual treatments. Simultaneously, comprehensive and ongoing monitoring should be implemented for specific patients with HMMD throughout their entire lifespan.

Background: The treatment strategy for hemorrhagic moyamoya disease (MMD) due to a ruptured aneurysm at the distal portion of the anterior choroidal artery remains controversial. The authors successfully treated the ruptured aneurysm with neuroendoscopic hematoma evacuation, followed by endovascular coil embolization., Observations: The authors encountered two patients with massive hemorrhagic MMD whose MMD had already been diagnosed and who had a periventricular anastomosis due to a ruptured aneurysm of the distal portion of the anterior choroidal artery involving the periventricular anastomosis. In both cases, neuroendoscopic hematoma evacuation was performed for hemorrhagic MMD in the acute phase, followed by endovascular coil embolization of the ruptured aneurysm in the chronic phase. In both endovascular treatments, the patient's condition was stabilized by hematoma evacuation, allowing a detailed preoperative evaluation of the anatomical findings of the vessel and functional findings of intraoperative neurophysiological monitoring using continuous monitoring of motor evoked potentials to preserve motor function., Lessons: Combination therapy can be useful for hemorrhagic MMD in patients with diagnosed MMD with a periventricular anastomosis. Additionally, a preoperative understanding of the vascular construction and intraoperative neurophysiological monitoring will aid in the successful coil embolization of aneurysms at the distal portion of the anterior choroidal artery with hemorrhagic MMD.

Association of moyamoya disease (MMD) with von Willebrand disease (vWD) is extremely rare. We first report a 27-year-old female case of MMD concurrent with vWD type 1, which presented as hemorrhagic stroke. The patient underwent a revascularization surgery with perioperative replacement therapy of von Willebrand factor and coagulation factor VIII. No hemorrhagic complications occurred. The patient overcame postoperative transient neurological events and fully recovered. We discuss appropriate perioperative supplementation of coagulation factors for a revascularization surgery for MMD with vWD. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Delayed anastomotic occlusion occurred in a considerable proportion of hemorrhagic moyamoya disease (MMD) patients undergoing direct revascularization. This study aimed to investigate the predictors and outcomes of delayed anastomotic occlusion in adult hemorrhagic MMD. The authors retrospectively reviewed 87 adult hemorrhagic MMD patients. Univariate and multivariate logistic regression analyses were performed. After an average of 9.1 ± 6.9 months of angiographic follow-up, the long-term graft patency rates were 79.8%. The occluded group had significantly worse angiogenesis than the non-occluded group (p < 0.001). However, the improvement of dilated anterior choroidal artery–posterior communicating artery was similar (p = 0.090). After an average of 4.0 ± 2.5 years of clinical follow-up, the neurological statues and postoperative annualized rupture risk were similar between the occluded and non-occluded groups (p = 0.750; p = 0.679; respectively). In the multivariate logistic regression analysis, collateral circulation Grade III (OR, 4.772; 95% CI, 1.184–19.230; p = 0.028) and preoperative computed tomography perfusion (CTP) Grade I–II (OR, 4.129; 95% CI, 1.294–13.175; p = 0.017) were independent predictors of delayed anastomotic occlusion. Delayed anastomotic occlusion in adult hemorrhagic MMD might be a benign phenomenon. Good collateral circulation (Grade III) and compensable preoperative intracranial perfusion (CTP Grade I–II) are independent predictors for this phenomenon. Moreover, the delayed anastomotic occlusion has no significant correlations with the long-term angiographic and neurological outcomes, except neoangiogenesis.

The present study aimed to investigate the clinical characteristics of hemorrhagic moyamoya disease (MMD) in Jilin province in northeast China. A total of 212 cases of hemorrhagic MMD were consecutively enrolled from the First Hospital of Jilin University in Changchun, China between January 2011 and January 2015. The patients' general clinical data, including age and gender characteristics, history of previous illnesses, hemorrhage type and onset symptoms, Hunt-Hess classification at admission, imaging characteristics, association with aneurysms, treatments and prognosis, were recorded and analyzed using SPSS 19.0. The results demonstrated that i) patients with hemorrhagic MMD in Jilin province were 47.7±11.5 years of age; ii) hemorrhagic MMD was primarily characterized by subarachnoid hemorrhage; iii) a total of 51.9% of the hemorrhagic MMD cases involved a unilateral artery; iv) a total of 24.1% of the hemorrhagic MMD cases were accompanied by anterior choroid artery and/or posterior communicating artery expansion; and v) following conservative or surgical treatment, patients with a prognostic Glasgow Outcome Scale score of 5 accounted for 65.6% of the study population. Therefore, the present study identified characteristics of MMD in Jilin province in northeast China. These results may improve understanding of the epidemiology of MMD in China, which at present remains not well established. Although the results are representative only of Jilin province in China, the study demonstrated high consistency with other studies, and thus may indirectly contribute to general understanding of hemorrhagic MMD etiology. [ABSTRACT FROM AUTHOR]

Our objective was a retrospective assessment of the management modalities that provided the most beneficial treatment in hemorrhagic moyamoya disease during the last 13 years at our institution. The clinical results of 44 patients with hemorrhagic moyamoya disease were investigated, comparing revascularization surgery (direct, indirect, and combined bypass) or conservative treatment. Angiographic features, rebleeding, and clinical outcome were investigated. Six of the 35 patients (17.1%) with revascularization surgery experienced rebleeding, as did 4 of 9 patients (44.4%) with conservative treatment. However, patients who underwent bypass surgery had a lower chance of rebleeding. No significant difference in chance of rebleeding was observed between bypass surgery and non surgery groups (p > 0.05). Cerebral angiography performed after bypass surgery showed that for achieving good postoperative revascularization, direct and combined bypass methods were much more effective (p < 0.05). While the risk of rebleeding in the revascularization group was generally lower than in the conservative treatment group, there was no statistically significant difference between treatment modalities and conservative treatment. Although statistical significance was not attained, direct and combined bypass may reduce the risk of hemorrhage more effectively than indirect bypass. [ABSTRACT FROM AUTHOR]

Background: Hemorrhagic moyamoya disease (MMD) and the fragile periventricular collaterals are known to have a causal relationship. Digital subtraction angiography and magnetic resonance angiography have shown the presence of fragile periventricular moyamoya vessels. However, dynamic fragile periventricular moyamoya vessels have never been observed under direct vision., Observations: The authors treated two patients with hemorrhagic MMD: a 42-year-old man with intraventricular hemorrhage and a 47-year-old woman with intracerebral hemorrhage. Endoscope-integrated indocyanine green video angiography (EICG angiography) could visualize the dynamic fragile periventricular collaterals. In particular, EICG angiography enabled visualization of invisible moyamoya vessels buried in the subependyma and characterization of the blood flow in the moyamoya vessels located inside the lateral ventricles and hematoma cavity., Lessons: EICG angiography can confirm the fragile periventricular collaterals associated with MMD by direct visualization. The high spatial resolution and real-time imaging can help to avoid accidental hemorrhage in and after evacuation of hemorrhage in patients with MMD.

Moyamoya disease (MMD) is a chronic progressive cerebrovascular disease, which can be divided into three types: ischemic, hemorrhagic, and asymptomatic. Hemorrhagic MMD has attracted considerable attention due to its distinctive imaging features and the controversy over the treatment. This report presents a comprehensive review of the literature on hemorrhagic MMD, focusing on the epidemiological characteristics, etiology and pathogenesis, imaging features, predictors of hemorrhage, and treatment options and their efficacy of hemorrhagic MMD. Hemorrhagic MMD mainly occurs in adult patients in Asian countries, and many factors may contribute to the etiology and development of this disease. Hemorrhagic MMD has two major imaging features: the dilatation and abnormal branching of anterior choroidal artery or posterior communicating artery, and multiple microbleeds, which may predict subsequent hemorrhage. The treatment for hemorrhagic MMD is not standardized, and large sample prospective randomized clinical trials may help to determine which method is better. In hemorrhagic MMD patients, more attention should be paid to cognitive function and quality of life, and these assessments should be included in the evaluation of effectiveness of treatment modalities. [ABSTRACT FROM AUTHOR]

Delayed anastomotic occlusion occurred in a considerable proportion of hemorrhagic moyamoya disease (MMD) patients undergoing direct revascularization. This study aimed to investigate the predictors and outcomes of delayed anastomotic occlusion in adult hemorrhagic MMD. The authors retrospectively reviewed 87 adult hemorrhagic MMD patients. Univariate and multivariate logistic regression analyses were performed. After an average of 9.1 ± 6.9 months of angiographic follow-up, the long-term graft patency rates were 79.8%. The occluded group had significantly worse angiogenesis than the non-occluded group (p < 0.001). However, the improvement of dilated anterior choroidal artery–posterior communicating artery was similar (p = 0.090). After an average of 4.0 ± 2.5 years of clinical follow-up, the neurological statues and postoperative annualized rupture risk were similar between the occluded and non-occluded groups (p = 0.750; p = 0.679; respectively). In the multivariate logistic regression analysis, collateral circulation Grade III (OR, 4.772; 95% CI, 1.184–19.230; p = 0.028) and preoperative computed tomography perfusion (CTP) Grade I–II (OR, 4.129; 95% CI, 1.294–13.175; p = 0.017) were independent predictors of delayed anastomotic occlusion. Delayed anastomotic occlusion in adult hemorrhagic MMD might be a benign phenomenon. Good collateral circulation (Grade III) and compensable preoperative intracranial perfusion (CTP Grade I–II) are independent predictors for this phenomenon. Moreover, the delayed anastomotic occlusion has no significant correlations with the long-term angiographic and neurological outcomes, except neoangiogenesis. [ABSTRACT FROM AUTHOR]

Objective: We evaluated what few studies emphasized the postoperative collateral formation and cerebral hemodynamics of hemorrhagic moyamoya disease (MMD)., Methods: Hemorrhagic 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., Results: A 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)., Conclusions: STA-MCA bypass combined with EDAMS can obtain better postoperative collateral formation, cerebral hemodynamics, and clinical prognosis than EDAMS alone., (Copyright © 2021. Published by Elsevier B.V.)

Objective: Here, the authors aimed to determine whether the presence of cerebral hemodynamic failure predicts subsequent bleeding attacks and how it correlates with the effect of direct bypass surgery in hemorrhagic moyamoya disease., Methods: Data from the Japanese Adult Moyamoya (JAM) Trial were used in this study: 158 hemispheres in 79 patients. A newly formed expert panel evaluated the SPECT results submitted at trial enrollment and classified the cortical hemodynamic state of the middle cerebral artery territory of each hemisphere into one of the following three groups: SPECT stage (SS) 0 as normal, SS1 as decreased cerebrovascular reserve (CVR), and SS2 as decreased CVR with decreased baseline blood flow. In the nonsurgical cohort of the JAM Trial, the subsequent hemorrhage rate during the 5-year follow-up was compared between the SS0 (hemodynamic failure negative) and SS1+2 (hemodynamic failure positive) groups. The effect of direct or combined direct/indirect bypass surgery on hemorrhage prevention was examined in each subgroup., Results: The hemodynamic grade was SS0 in 59 (37.3%) hemispheres, SS1 in 87 (55.1%), and SS2 in 12 (7.6%). In the nonsurgical cohort, subsequent hemorrhage rates in the SS0 and SS1+2 groups were 12 cases per 1000 person-years and 67 cases per 1000 person-years, respectively. Kaplan-Meier analysis revealed that hemorrhagic events were significantly more common in the SS1+2 group (p = 0.019, log-rank test). Cox regression analysis showed that hemodynamic failure was an independent risk factor for subsequent hemorrhage (HR 5.37, 95% CI 1.07-27.02). In the SS1+2 subgroup, bypass surgery significantly suppressed hemorrhagic events during 5 years (p = 0.001, HR 0.16, 95% CI 0.04-0.57), with no significant effect in the SS0 group (p = 0.655, HR 1.56, 95% CI 0.22-11.10). Examination of effect modification revealed that the effect of surgery tended to differ nonsignificantly between these two subgroups (p = 0.056)., Conclusions: Hemodynamic failure is an independent risk factor for subsequent hemorrhage in hemorrhagic moyamoya disease. Direct bypass surgery showed a significant preventive effect in the hemodynamically impaired hemispheres. Thus, hemodynamic failure, as well as previously proposed factors such as choroidal anastomosis, should be considered for the surgical indication in hemorrhagic moyamoya disease.Clinical trial registration no.: C000000166 (umin.ac.jp).

Objectives: Hemorrhagic moyamoya disease (MMD) is one common subtype in adult patients. However, the study about outcome of hemorrhagic MMD patients in the acute stage is still lacking. This study is aimed to explore the short-term prognostic factors for adult patients with hemorrhagic MMD in the acute stage., Patients and Methods: Adult hemorrhagic MMD patients in the acute stage awere retrospectively analyzed. Both clinical and imaging data were collected. Unfavorable functional outcome at discharge was considered when modified Rankin Scale score ≥3. Multivariate logistic regression was used to investigate the prognostic factors in patients with hemorrhagic MMD in the acute stage., Results: A total of 107 patients were included in this study. Among these patients, 17 died and 59 had unfavorable functional outcome at 9.6 ± 7.8 days. In multivariate logistic regression, admission blood glucose (odds ratio (OR) = 1.457, 95% confidence interval (CI) 1.156-1.836, P = 0.001), midline shift >5 mm (OR = 24.268, 95%CI 4.324-136.191, P < 0.001), and subarachnoid hemorrhage (OR = 13.067, 95%CI 2.020-84.512, P = 0.007) were independently associated with death at discharge. Moreover, admission Glasgow Coma Scale (GCS) score (OR = 0.420, 95%CI 0.296-0.598, P < 0.001), midline shift >5 mm (OR = 6.685, 95%CI 1.226-36.455, P = 0.028), and intraparenchymal hemorrhage (OR = 4.790, 95%CI 1.184-19.381, P = 0.028) were independently associated with unfavorable functional outcome at discharge., Conclusion: This study shows that admission blood glucose, midline shift >5 mm, and subarachnoid hemorrhage are independent predictors of short-term mortality in hemorrhagic MMD in the acute stage. In addition, admission GCS score, midline shift >5 mm, and intraparenchymal hemorrhage are independent predictors of short-term unfavorable functional outcome in hemorrhagic MMD in the acute stage., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Object: The outcome of patients with hemorrhagic moyamoya disease (MMD) after cerebral revascularization is uncertain. The purpose of this study was to delineate the efficacy of this surgical method in the treatment of hemorrhagic MMD., Methods: Between January 2007 and August 2011, a consecutive cohort of 113 patients with hemorrhagic MMD was enrolled into this prospective single-center cohort study. The surgical method was combined direct and indirect bypass. The cumulative probability of the primary end point (all stroke and deaths from surgery through 30 days after surgery and ipsilateral recurrent hemorrhage afterward) was analyzed. The angiographic outcome was measured by the following parameters: bypass patency, reduction of basal MMD vessels, improved degree of dilation, and branch extension of the anterior choroidal and posterior communicating arteries (AChA-PCoA)., Results: Of the 113 enrolled cases, CT scans revealed pure intraventricular hemorrhage (IVH) in 63 cases (55.7%), pure intracranial hemorrhage (ICH) in 14 cases (12.4%), and ICH with IVH in 36 cases (31.9%). In 74 of 113 hemorrhagic hemispheres (65.5%), the AChA-PCoA was extremely dilated with extensive branches beyond the choroidal fissure. A total of 114 surgeries were performed. No patient suffered ischemic or hemorrhagic stroke through 30 days after surgery. Ipsilateral rebleeding occurred in 5 patients, 4 of whom died of the rebleeding event. The cumulative probability of the primary end point was 0% at 1 year and 1.9% at 2 years. The annual rebleeding rate was 1.87%/person/year. The improvement in AChA-PCoA extension was observed in 75 of 107 operated hemispheres (70.1%), which was higher than that in 7 of 105 unoperated hemispheres (35.2%)., Conclusions: Revascularization may provide a benefit over conservative therapy for hemorrhagic MMD patients. The improvement of dilation and branch extension of AChA-PCoA might be correlated with the low rebleeding rate.