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Failures in Revascularization for Pediatric Moyamoya Disease and Syndrome: A Scoping Review
Nathan A Shlobin1, H Gregory Frankel1, Sandi Lam1
1Department of Neurological Surgery, Northwestern University Feinberg School of Medicine, Division of Pediatric Neurosurgery, Lurie Children's Hospital, Chicago, Illinois, USA.
Background:
Moyamoya disease (MMD) and moyamoya syndrome (MMS) are a rare cause of stroke commonly managed surgically. We conducted a scoping review to identify the current scope of the literature regarding factors associated with failure of revascularization surgery for MMD and MMS in pediatric patients and to catalyze future research.
Methods:
A scoping review was conducted to explore failures of revascularization surgery for MMD and MMS in pediatric patients using the PubMed, Embase, and Scopus databases. Titles and abstracts returned from searches were screened for full-text review. Studies meeting inclusion criteria were reviewed in full, and relevant data were extracted.
Results:
Of 2450 resultant articles, 15 were included. Angiographic outcomes were reported for 900 hemispheres, of which 442 (49.1%) were denoted as Matsushima grade A, 299 (33.2%) as Matsushima grade B, and 159 (17.7%) as Matsushima grade C. Patients with MMS had poorer angiographic outcomes than did patients with MMD. Patients with poor neovascularization had a greater degree of moyamoya vessels on follow-up angiogram. Suzuki stage was not associated with angiographic outcome in individual patients. Angiographic outcomes differed by surgical approach and were not associated with clinical outcomes. Literature identifying factors was sparse.
Conclusions:
The existing literature indicates that factors such as cause, degree of moyamoya vessels, and surgical approach may affect the likelihood of Matsushima grade C revascularization in pediatric patients with MMD and MMS. Future studies are necessary to definitively elucidate factors associated with failure of revascularization surgery for pediatric MMD.
Insights
Revascularization surgery for moyamoya disease (MMD) and moyamoya syndrome (MMS) in children shows variable outcomes. Factors like cause and surgical approach may influence success, but more research is needed.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Stroke
Background:
- Moyamoya disease (MMD) and moyamoya syndrome (MMS) are rare cerebrovascular disorders causing stroke, often treated with surgery.
- Pediatric MMD and MMS present unique challenges in surgical management and outcomes.
Purpose of the Study:
- To conduct a scoping review of literature on factors influencing revascularization surgery failure in pediatric MMD and MMS.
- To identify gaps in current research and guide future investigations.
Main Methods:
- A comprehensive scoping review of PubMed, Embase, and Scopus databases was performed.
- Studies were screened, and relevant data on surgical outcomes and influencing factors were extracted.
Main Results:
- Fifteen studies were included, analyzing 900 hemispheres.
- Poorer angiographic outcomes (Matsushima grade C) were observed in MMS compared to MMD.
- Poor neovascularization correlated with increased moyamoya vessels; surgical approach impacted outcomes, but clinical outcomes were not associated.
Conclusions:
- Factors such as the underlying cause, degree of moyamoya vessels, and surgical technique may influence revascularization success in pediatric MMD/MMS.
- Further research is essential to fully understand and mitigate factors leading to surgical failure in pediatric MMD.

