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The Management of Symptomatic Moyamoya Disease in Pediatric Patients: A Systematic Review and Meta-Analysis
Ataollah Shahbandi1, Shahab Aldin Sattari2, Tej D Azad2
1Department of Neurosurgery, School of Medicine, Tehran University of Medical Sciences, Tehran , Iran.
Insights
Surgical revascularization improves outcomes for pediatric moyamoya disease (MMD). Both direct/combined bypass and indirect bypass techniques are effective, with surgery reducing stroke risk compared to conservative management.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Medicine
Background:
- Optimal management for symptomatic pediatric moyamoya disease (MMD) remains unclear.
- This study systematically reviews and analyzes surgical versus conservative treatment strategies.
- It also compares direct/combined bypass (DB/CB) with indirect bypass (IB) techniques.
Purpose of the Study:
- To compare the efficacy of surgical versus conservative management for pediatric MMD.
- To evaluate the effectiveness of direct/combined bypass (DB/CB) versus indirect bypass (IB) techniques.
- To identify optimal revascularization strategies for improving clinical outcomes in pediatric MMD patients.
Main Methods:
- Systematic review and meta-analysis of studies from MEDLINE and PubMed (up to March 17, 2024).
- Primary outcomes: follow-up ischemic stroke (surgical vs. conservative; DB/CB vs. IB).
- Secondary outcomes: hemorrhagic events, transient ischemic attacks, seizures, functional outcomes (mRS), and symptom improvement.
Main Results:
- Surgical management was associated with significantly lower odds of ischemic stroke (OR=0.33) and a trend towards fewer hemorrhagic events compared to conservative care.
- Direct/combined bypass (DB/CB) showed a trend towards better Matsushima grade A compared to indirect bypass (IB) (OR=3.44).
- No significant differences were found between DB/CB and IB for stroke, TIA, seizures, symptomatic improvement, or functional outcomes (mRS ≤2).
Conclusions:
- Surgical revascularization offers superior clinical outcomes compared to conservative management in pediatric symptomatic moyamoya disease.
- Both direct/combined bypass and indirect bypass techniques demonstrate comparable efficacy in preventing ischemic events.
- Surgical flow augmentation, regardless of technique, appears beneficial for pediatric patients with symptomatic MMD.
Background And Objectives:
The optimal management strategy for pediatric patients with symptomatic moyamoya disease (MMD) is not well established. This systematic review and meta-analysis compares surgical vs conservative management and direct/combined bypass (DB/CB) vs indirect bypass (IB) for pediatric patients with symptomatic MMD.
Methods:
MEDLINE and PubMed were searched from inception to March 17, 2024. For analysis of surgical vs conservative treatment, the primary and secondary outcomes were follow-up ischemic stroke and intracranial hemorrhagic events, respectively. For analysis of DB/CB vs IB, the primary outcome was follow-up ischemic stroke, and secondary outcomes included follow-up transient ischemic attack, new or worsened seizures, symptomatic improvement, modified Rankin Scale score ≤2, and Matsushima grade A at the last follow-up.
Results:
Twenty-two included studies yielded 1091 patients, with a median follow-up duration of 35.7 months. Regarding surgical vs conservative management, 428 patients were analyzed. Surgical treatment was associated with lower odds of ischemic stroke (odds ratios [OR] = 0.33 [95% CI, 0.11-0.97], P = .04), and intracranial hemorrhagic events tended to be lower with surgery (OR = 0.25 [0.06-1.03], P = .05). Regarding DB/CB techniques vs IB, 875 patients were analyzed. The groups had similar rates of ischemic stroke (OR = 0.79 [0.31-1.97], P = .61), transient ischemic attack (OR = 1.27[0.46-3.55], P = .64), new or worsened seizures (OR = 1.05[0.3-3.65], P = .93), symptomatic improvement (OR = 2.45[0.71-8.45], P = .16), and follow-up modified Rankin Scale ≤2 (OR = 1.21 [0.16-8.85], P = .85). CB was associated with higher Matsushima grade A relative to IB (OR = 3.44 [1.32-9.97], P = .01).
Conclusion:
Surgical revascularization yielded more favorable clinical outcomes than conservative management in this meta-analysis. Clinical outcomes were similar between DB/CB vs IB techniques. Surgical flow augmentation, either by DB/CB or IB, seems to benefit pediatric patients with symptomatic MMD.
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