The Management of Symptomatic Moyamoya Disease in Pediatric Patients: A Systematic Review and Meta-Analysis

Ataollah Shahbandi; Shahab Aldin Sattari; Tej D Azad; Yuanxuan Xia; Kurt Lehner; Wuyang Yang; James Feghali; Rebecca A Reynolds; S Hassan A Akbari; Mari L Groves; Risheng Xu; Justin M Caplan; Chetan Bettegowda; Alan R Cohen; Judy Huang; Rafael J Tamargo; L Fernando Gonzalez

doi:10.1227/neu.0000000000003277

The Management of Symptomatic Moyamoya Disease in Pediatric Patients: A Systematic Review and Meta-Analysis

Neurosurgery. 2024 Nov 14. doi: 10.1227/neu.0000000000003277. Online ahead of print.

Authors

Ataollah Shahbandi¹, Shahab Aldin Sattari², Tej D Azad², Yuanxuan Xia², Kurt Lehner², Wuyang Yang², James Feghali², Rebecca A Reynolds³, S Hassan A Akbari⁴, Mari L Groves², Risheng Xu², Justin M Caplan², Chetan Bettegowda², Alan R Cohen², Judy Huang², Rafael J Tamargo², L Fernando Gonzalez²

Affiliations

¹ Department of Neurosurgery, School of Medicine, Tehran University of Medical Sciences, Tehran , Iran.
² Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore , Maryland , USA.
³ Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City , Iowa , USA.
⁴ Department of Neurosurgery, Johns Hopkins All Children's Hospital, St. Petersburg , Florida , USA.

PMID: 39560368
DOI: 10.1227/neu.0000000000003277

Abstract

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.