Effectiveness of Ipsilateral Stroke Prevention Between Conservative Management and Indirect Revascularization for Moyamoya Disease in a North American Cohort

Porras, Jose L.; Yang, Wuyang; Xu, Risheng; Garzón-Muvdi, Tomás; Caplan, Justin M.; Colby, Geoffrey P.; Coon, Alexander L.; Ahn, Edward S.; Tamargo, Rafael J.; Huang, Judy

doi:10.1016/j.wneu.2017.11.113

Cited by 25 publications

(17 citation statements)

References 23 publications

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“…In our cohort, children seemed to have fewer recurrent strokes and a more favorable functional outcome than adults, as has been described by others [22,23]. One explanation could be that children are referred for surgical treatment faster than adults.…”

Section: Discussionsupporting

confidence: 80%

“…Because surgical treatment is not without risk, the indication for, and timing of revascularization surgery should be decided in a multidisciplinary team, taking into account the individual patients characteristics, hemodynamic studies, and technical considerations regarding surgery [9,10]. In our cohort, we had a complication risk of 9.5%, which is within the range of other studies [22,26].…”

Section: Discussionmentioning

confidence: 82%

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Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome

Kronenburg

Kleinloog

Zwan

et al. 2021

JCM

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Information on presentation and outcome of moyamoya vasculopathy (MMV) in European countries is limited. We investigated patient characteristics, treatment and outcome of patients with MMV. We retrieved patient characteristics and treatment information and determined functional outcome (modified Rankin Score (mRS); type of school/work) by structured telephone interviews. We performed uni- and multivariable logistic regression analysis to determine predictors of poor outcome. We included 64 patients with bilateral MMV. In children (31 patients), median age was 5 years (interquartile range (IQR) 2–11) and in adults (33 patients), it was 33 years (IQR 28–41). Predominant mode of presentation was ischemia (children 84%; adults 88%). Modified Rankin Scale (mRS) at presentation was ≤2 in 74%. Revascularization was performed in 42 patients (23 children). Median follow-up time was 46 months (IQR 26–90). During this period, 16 patients had recurrent stroke(s) and four patients died. In 73% of the patients (83% surgical group; 55% medically treated group), mRS was ≤2; 46% were able to return to regular school or work, of whom only 41% were on the same level. Univariable analysis revealed that surgical treatment was associated with lower odds of poor outcome ((mRS ≥ 3), OR 0.24; p = 0.017). This association was no longer statistically significant (OR 3.47; p = 0.067) in the multivariable model, including age and diagnosis (moyamoya disease or moyamoya syndrome). In this cohort of patients with MMV who presented in a single European center, a large proportion had good functional outcome. Nevertheless, less than half were able to attend regular school or were able to work at their previous level, indicating a large impact of the disease on their life.

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Section: Discussionsupporting

confidence: 80%

Section: Discussionmentioning

confidence: 82%

Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome

Kronenburg

Kleinloog

Zwan

et al. 2021

JCM

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“…Studies comparing the superiority of direct versus indirect bypass show that indirect methods offer a more favorable clinical course when attempted at an earlier stage of MMD. Patients who receive indirect revascularization surgery early in the disease process have a lower overall incidence of stroke compared to those who receive conservative management with observation and medical therapy [ 45 ]. In asymptomatic patients without ischemic or hemorrhagic events, early surgical intervention has been consistently shown to have improved clinical outcomes with the prevention of future symptoms and ischemic or hemorrhagic events [ 46 ].…”

Section: Reviewmentioning

confidence: 99%

Moyamoya: An Update and Review

Berry¹,

Cortez²,

Toor³

et al. 2020

Cureus

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This article is a clinical review of Moyamoya disease (MMD) and Moyamoya syndrome (MMS). We review the incidence, epidemiology, pathology, historical context, clinical and radiographic findings, diagnostic imaging modalities, radiographic grading systems, the effectiveness of medical, interventional, and surgical treatment, and some of the nuances of surgical treatment options. This article will help pediatricians, neurologists, neurosurgeons, and other clinical practitioners who are involved in caring for patients with this rare clinical entity. MMD is an intrinsic primary disease process that causes bilateral progressive stenosis of the anterior intracranial circulation with the involvement of the proximal portions of the intracranial internal carotid artery (ICA) extending to involve the proximal portions of the anterior cerebral artery (ACA) and middle cerebral artery (MCA); posterior circulation involvement is very rare. This causes a compensatory response where large numbers of smaller vessels such as the lenticulostriate arteries begin to enlarge and proliferate, which gives the angiographic appearance of a "Puff of Smoke", which is translated into Japanese as "Moyamoya”. MMS is a secondary process that occurs in response to another underlying pathological process that causes stenosis of intracranial blood vessels, such as radiation. For example, an external source of radiation causes stenosis of the ICA with a compensatory response of smaller blood vessels, which then enlarge and proliferate in response and has the same "Puff of Smoke" appearance on the diagnostic cerebral angiogram (DCA). Histological findings include an irregular internal elastic lamina with luminal narrowing, hyperplasia of the tunica media, and intimal thickening with vacuolar degeneration in smooth muscle cells in the tunica media. Compensation for diminishing blood supply occurs through angiogenesis, which causes the proliferation and enlargement of smaller collateral blood vessels to increase blood supply to under-perfused areas of the brain. MMD is rare in the United States, with just 0.086 newly diagnosed cases per 100,000 individuals per year, which is approximately one per million new cases annually. Risk factors for MMD include Eastern Asian ancestry and predisposing conditions such as neurofibromatosis and Down's syndrome. Clinically, patients often present with stroke signs and symptoms from cerebral ischemia. The proliferation of collateral blood vessels within the basal ganglia can produce movement disorders. Catheter-based DCA is the current gold standard for obtaining a diagnosis. CT perfusion allows preoperative identification of ischemic vascular territories, which may be amenable to surgical intervention. MRI enables rapid detection of acute ischemic stroke using diffusion-weighted Imaging (DWI) and apparent diffusion coefficient (ADC) sequences to assess for any diffusion restriction. Non-contrast CT of the head is used to rule out acute hemorrhage in the presentation...

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“…Surgical BR in MMD can prevent future cerebrovascular insults and avoid cognitive decline [116,117,227]. It is indicated in asymptomatic patients at the slightest sign of disease progression or patient neurological or mental impairment [103,234,235], particularly in the paediatric age group [62,103].…”

Section: Results Surgical Techniques Of Cerebral Revascularization In Moyamoya Diseasementioning

confidence: 99%

“…Endovascular treatment has been attempted in some cases with unsatisfactory results [110][111][112]. The surgical treatment with BR offloads the haemodynamic stress [113] and reduces the risk of subsequent ischemic and haemorrhagic cerebrovascular events [104,[114][115][116][117][118][119][120][121][122], providing symptom improvement to 87% of patients [104].…”

Section: Treatment Modalities In Moyamoya Diseasementioning

confidence: 99%

Surgical Treatment of Moyamoya Disease

Vanaclocha¹,

Saiz-Sapena²,

Vanaclocha³

2021

Moyamoya Disease - A Disease to Count on in Your Daily Practice

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Moyamoya disease is a rare cerebrovascular disease most prevalent in East Asian Countries. Thanks to the new diagnostic capabilities, the number of cases discovered has been rising steadily in the latest years, including many asymptomatic patients. But asymptomatic from the clinical point of view does not necessarily mean that there are no subjacent problems and that there will be no disease progression. Indeed, many patients harbour cognitive decline long before they start with clinical or even radiological manifestations. The only effective treatment is surgical revascularization, with all its possibilities: direct, indirect, and combined. While direct techniques are more useful in adult moyamoya patients, children seem to benefit most from indirect techniques. Additionally, indirect or combined procedures can be used as salvage procedures in case of unsatisfactory outcomes. Thus, many surgeons posit that surgical treatment should be considered in moyamoya patients, even if asymptomatic, particularly in the paediatric age group.

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Effectiveness of Ipsilateral Stroke Prevention Between Conservative Management and Indirect Revascularization for Moyamoya Disease in a North American Cohort

Cited by 25 publications

References 23 publications

Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome

Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome

Moyamoya: An Update and Review

Surgical Treatment of Moyamoya Disease

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