SynthesisChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2018
When and why is surgical revascularization indicated for the treatment of moyamoya syndrome in patients with RASopathies? A systematic review of the literature and a single institute experience.
Synthesis in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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14 citing papers in PubMed, 27 citations in OpenAlex.
- Exploration of the risk factor for infarction after revascularization in moyamoya disease.Annals of medicine · 2024Article
- Surgical revascularization as a procedure to prevent neurological complications in children with moyamoya syndrome associated with neurofibromatosis I: a single institution case series.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Article
- Neurofibromatosis Type 1: Optimizing Management with a Multidisciplinary Approach.Journal of multidisciplinary healthcare · 2024Review
- Neuropsychiatric Symptoms of Moyamoya Disease: Considerations for the Clinician.Neuropsychiatric disease and treatment · 2024Review
- Moyamoya Vasculopathy in Neurofibromatosis Type 1 Pediatric Patients: The Role of Rare Variants ofCancers · 2023Article
- Case report: Revascularization failure in NF1-related moyamoya syndrome after selumetinib: A possible pathophysiological correlation?Frontiers in pediatrics · 2023Article
- Hutchinson-Gilford progeria syndrome complicated with stroke: A report of 2 cases and literature review.Frontiers in pediatrics · 2022Article
- A rare triad of morning glory disc anomaly, moyamoya vasculopathy, and transsphenoidal cephalocele: pathophysiological considerations and surgical management.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2021Article
- Limits and pitfalls of indirect revascularization in moyamoya disease and syndrome.Neurosurgical review · 2021Review
- Late-onset chorea after cerebral revascularization as a clinical manifestation of moyamoya disease.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2021Article
- RNF213 variant in a patient with Legius syndrome associated with moyamoya syndrome.Molecular genetics & genomic medicine · 2021Article
- Vulnerability to shear stress caused by altered peri-endothelial matrix is a key feature of Moyamoya disease.Scientific reports · 2021Article
- Spatial coefficient of variation applied to arterial spin labeling MRI may contribute to predict surgical revascularization outcomes in pediatric moyamoya vasculopathy.Neuroradiology · 2020Article
- Review
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Authors and funding
7 authors at 2 institutions in 1 country.
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Abstract
backgroundMoyamoya disease (MMD) is a cerebrovascular disorder characterized by the progressive occlusion of the supraclinoid internal carotid artery (ICA), resulting in the formation of an abnormal cerebral vascular network. When MMD occurs in association with an underlying medical condition, including some distinctive genetic disorders, it is named moyamoya syndrome (MMS). The discrimination between MMD and MMS has been validated by recent genetic researches and international reviews. Similarly to patients suffering from MMD, patients with MMS generally become symptomatic because of ischemic complications, which lead to hemiparesis, transient ischemic events, seizures, and sensory symptoms. RASopathies are a group of neurodevelopmental disorders that can be associated with MMS.
resultsWe retrospectively reviewed 18 RASopathy patients with MMS treated at our institution from 2000 to 2015 (16 neurofibromatosis type 1, 1 Costello syndrome, and 1 Schimmelpenning syndrome). Here, we report clinical data, performed surgical procedures, and clinic-radiological outcome of these patients. Most of them received both indirect revascularization and medical therapy.
conclusionsAt the moment, there are no univocal recommendations on which of these two treatment strategies is the treatment of choice in patients with RASopathies and MMS. We suggest that patients with a good overall prognosis (primarily depending on the distinctive underlying genetic disorder) and initial cerebrovascular disease could benefit from a prophylactic surgical revascularization, in order to prevent the cognitive impairment due to the progression of the vasculopathy.
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