Encephaloduroateriosynangiosis (EDAS) in the management of Moyamoya syndrome in children with sickle cell disease

Alamri, Alexander; Hever, Pennylouise; Cheserem, Jebet Beverly; Gradil, Cátia; Bassi, Sanj; Tolias, Christos M.

doi:10.1080/02688697.2017.1339227

Cited by 15 publications

(10 citation statements)

References 20 publications

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“…183 Down syndrome (with a 26-fold increased likelihood of moyamoya), neurofibromatosis type I (with ≈2%-5% prevalence of moyamoya), SCD, and other associated conditions are summarized in Table 2. 177,181,[184][185][186][187][188][189][190][191][192][193][194][195][196][197][198][199] If moyamoya is identified on MRI, then DSA should be strongly considered because this modality has increased diagnostic sensitivity for moyamoya compared with MRI (including the ability to better differentiate vasculitis) and offers important data germane to preoperative planning. Specifically, transdural collaterals visualized on DSA are critical biomarkers of disease that can assess angiogenic potential (particularly in combination with proteomic assays), that can predict 1-year radiographic outcomes from surgery, and, when incorporated into surgical planning, have been demonstrated to reduce…”

Section: Intracranial Arteriopathymentioning

confidence: 99%

Management of Stroke in Neonates and Children: A Scientific Statement From the American Heart Association/American Stroke Association

Ferriero¹,

Fullerton²,

Bernard³

et al. 2019

Stroke

527

515

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Purpose-Much has transpired since the last scientific statement on pediatric stroke was published 10 years ago. Although stroke has long been recognized as an adult health problem causing substantial morbidity and mortality, it is also an important cause of acquired brain injury in young patients, occurring most commonly in the neonate and throughout childhood. This scientific statement represents a synthesis of data and a consensus of the leading experts in childhood cardiovascular disease and stroke. Methods-Members of the writing group were appointed by the American Heart Association Stroke Council's Scientific Statement Oversight Committee and the American Heart Association's Manuscript Oversight Committee and were chosen to reflect the expertise of the subject matter. The writers used systematic literature reviews, references to published clinical and epidemiology studies, morbidity and mortality reports, clinical and public health guidelines, authoritative statements, personal files, and expert opinion to summarize existing evidence and to indicate gaps in current knowledge. This scientific statement is based on expert consensus considerations for clinical practice. Results-Annualized pediatric stroke incidence rates, including both neonatal and later childhood stroke and both ischemic and hemorrhagic stroke, range from 3 to 25 per 100 000 children in developed countries. Newborns have the highest risk ratio: 1 in 4000 live births. Stroke is a clinical syndrome. Delays in diagnosis are common in both perinatal and childhood stroke but for different reasons. To develop new strategies for prevention and treatment, disease processes and risk factors that lead to pediatric stroke are discussed here to aid the clinician in rapid diagnosis and treatment. The many important differences that affect the pathophysiology and treatment of childhood stroke are discussed in each section. Conclusions-Here we provide updates on perinatal and childhood stroke with a focus on the subtypes, including arterial ischemic, venous thrombotic, and hemorrhagic stroke, and updates in regard to areas of childhood stroke that have not received close attention such as sickle cell disease. Each section is highlighted with considerations for clinical practice, attendant controversies, and knowledge gaps. This statement provides the practicing provider with much-needed updated information in this field. (Stroke. 2019;50:e00-e00.

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Section: Intracranial Arteriopathymentioning

confidence: 99%

Management of Stroke in Neonates and Children: A Scientific Statement From the American Heart Association/American Stroke Association

Ferriero¹,

Fullerton²,

Bernard³

et al. 2019

Stroke

527

515

View full text Add to dashboard Cite

show abstract

“…Risk of MA disease has been also attributed to mutations in GUCY1A3 gene, encoding the major nitric oxide receptor in vascular smooth muscle cells (vSMCs) in achalasia cases [ 11 ]. Other sporadic syndromic cases of MA have been reported, as resumed in Table 1 [ 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 ]. However, these observations are not able to fully explain the pathogenesis of MA, which is believed to be much more complex.…”

Section: Introductionmentioning

confidence: 99%

Microduplication of 15q13.3 and Microdeletion of 18q21.32 in a Patient with Moyamoya Syndrome

Sciacca

Rizzo

Bedini

et al. 2018

IJMS

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Moyamoya angiopathy (MA) is a cerebrovascular disease determining a progressive stenosis of the terminal part of the internal carotid arteries (ICAs) and their proximal branches and the compensatory development of abnormal “moyamoya” vessels. MA occurs as an isolated cerebral angiopathy (so-called moyamoya disease) or in association with various conditions (moyamoya syndromes) including several heritable conditions such as Down syndrome, neurofibromatosis type 1 and other genomic defects. Although the mechanism that links MA to these genetic syndromes is still unclear, it is believed that the involved genes may contribute to the disease susceptibility. Herein, we describe the case of a 43 years old woman with bilateral MA and peculiar facial characteristics, having a 484-kb microduplication of the chromosomal region 15q13.3 and a previously unreported 786 kb microdeletion in 18q21.32. This patient may have a newly-recognized genetic syndrome associated with MA. Although the relationship between these genetic variants and MA is unclear, our report would contribute to widening the genetic scenario of MA, in which not only genic mutation, but also genome unbalances are possible candidate susceptibility factors.

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“…Even so, the unilateral MMD might be a suitable model to examine the pathogenetic process of MMD, because the bilateral MMD exhibits complex and ambiguous pathological findings that do not allow identification of cause versus effect [ 18 ]. In order to accurately analyze the progression of the unilateral MMD, MMS was excluded in our study because the MMS-associated diseases such as sickle cell disease, Graves’ disease (GD), and Down syndrome [ 1 , 5 ] might have positive or negative effects on the angiographic and symptomatic progression. For example, Chen et al [ 5 ] observed that the disease progression was more frequent in MMS patients with GD than that in patients without GD during the follow-up period, especially in unilateral disease.…”

Section: Discussionmentioning

confidence: 99%

The contralateral progression in a cohort of Chinese adult patients with unilateral moyamoya disease after revascularization: a single-center long-term retrospective study

Tian

Zhang

2022

Acta Neurochir

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Background Moyamoya disease (MMD) is a chronic progressive cerebrovascular disease mainly existing in the Asian population, which can be divided into unilateral and bilateral types. Contralateral progression has been reported in pediatric patients with unilateral MMD, while large series about contralateral progression in Chinese adult patients were rare. The goal of this study is to elucidate the clinical features and incidence of contralateral progression in Chinese MMD adult patients. Methods One hundred one Chinese adult patients with unilateral MMD who received surgery treatments between January 2015 and January 2017 in our hospital were enrolled in this study. This study contained 89 patients. Digital subtraction angiography was performed in all patients for initial diagnosis, and magnetic resonance angiography was repeated 6 months from the initial operation and then annually. Clinical characteristics, contralateral progression, and risk factors were studied. Previous related studies were also reviewed and meta-analyzed. Results Of these 89 patients, contralateral progression was identified in 8 patients (9.0%) within a median follow-up period of 63 months, which was lower than that in previous studies (25.9%). Single-factor analysis and multivariate analysis did not reveal significant risk factors related to the contralateral progression. Conclusion The progress rate in this cohort of Chinese adult patients with unilateral MMD after revascularization was 9.0%, which indicates that some of the unilateral MMD were an early form of bilateral MMD rather than a separate condition. Trial registration. This work was approved by the Medical Ethics Committee of Zhongnan Hospital of Wuhan University (approval number: Kelun-2017005).

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Encephaloduroateriosynangiosis (EDAS) in the management of Moyamoya syndrome in children with sickle cell disease

Abstract: EDAS is a well-tolerated revascularisation procedure for children with MMS. The prevention of further infarcts in our group with sickle cell disease has allowed these children to resume normal school activities.

Cited by 15 publications

References 20 publications

Management of Stroke in Neonates and Children: A Scientific Statement From the American Heart Association/American Stroke Association

Management of Stroke in Neonates and Children: A Scientific Statement From the American Heart Association/American Stroke Association

Microduplication of 15q13.3 and Microdeletion of 18q21.32 in a Patient with Moyamoya Syndrome

The contralateral progression in a cohort of Chinese adult patients with unilateral moyamoya disease after revascularization: a single-center long-term retrospective study

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