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Moyamoya disease and syndrome
1Division of Neurosurgery, Hospital for Sick Children, Toronto, Canada.
Clinical Neurology and Neurosurgery
|December 31, 1997
Summary
Surgical interventions for Moyamoya disease in children between 1979 and 1995 yielded positive results, with most patients achieving excellent or good outcomes. Procedures like encephalo-myo-synangiosis (EMS) and encephalo-duro-arterio-synangiosis (EDAS) demonstrated efficacy in managing this condition.
Area of Science:
- Neurology
- Neurosurgery
- Pediatrics
Background:
- Moyamoya disease, first described in 1963, is a rare cerebrovascular disorder.
- Surgical management for Moyamoya disease commenced in the mid-1970s.
- This study reviews surgical outcomes at a single institution from 1979 to 1995.
Purpose of the Study:
- To evaluate the surgical outcomes of pediatric patients with Moyamoya disease and Moyamoya syndrome.
- To assess the long-term efficacy of surgical revascularization procedures.
Main Methods:
- Retrospective review of 30 pediatric patients treated between 1979 and 1995.
- Surgical interventions included encephalo-myo-synangiosis (EMS) and encephalo-duro-arterio-synangiosis (EDAS).
- Outcomes were categorized as excellent, good, or poor, with detailed follow-up.
Main Results:
- Twenty-one out of 30 children (70%) experienced an excellent outcome.
- Five children had a good outcome with significant neurologic deficits.
- Two children with persistent symptoms responded well to EMS; one child had a poor outcome.
- One patient with an excellent outcome post-EDAS died 7 years later from Fanconi's anemia.
Conclusions:
- Surgical revascularization offers favorable outcomes for pediatric Moyamoya disease.
- EMS and EDAS are effective procedures for improving cerebral blood flow.
- Long-term follow-up is crucial for assessing the overall impact of surgical management.