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Background <p>Moyamoya syndrome (MMS) associated with sickle cell disease (SCD) is a severe vasculopathy that significantly increases stroke risk. While cerebral revascularization is increasingly considered in this population, concerns about perioperative safety and long-term outcomes have limited its use in clinical practice.</p> Methods <p>We conducted a multicenter, retrospective cohort study of 553 patients with MMS who underwent surgical revascularization across 13 centers. Patients were grouped by SCD status (SCD-MMS vs. moyamoya disease (MMD)). Primary outcomes included perioperative stroke, perioperative complications, and functional status at discharge. Secondary outcomes included length of stay, and follow-up stroke.</p> Results <p>Of 553 patients, 32 (5.8%) had SCD. There were no significant differences in overall perioperative stroke (OR 1.05, 95% CI 0.19 to 5.54), symptomatic perioperative stroke (OR 0.94, 95% CI 0.09 to 8.94), perioperative complications (OR 1.66, 95% CI 0.47 to 5.86), or follow-up stroke (OR 0.88, 95% CI 0.17 to 4.55). Functional outcomes at discharge were similarly favorable in both groups (mRS 0–1: OR 0.84, 95% CI 0.29 to 2.40). SCD was associated with a longer hospital stay (beta 2.78 days, 95% CI 0.60 to 4.96).</p> Conclusion <p>Surgical revascularization for MMS in patients with SCD does not confer additional procedural risk and yields outcomes comparable to those of patients without SCD. These findings support the role of bypass surgery as a viable treatment option in this high-risk population.</p>

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Outcomes of Bypass Surgery in Patients with Moyamoya Syndrome Secondary To Sickle Cell Disease: a Multicenter Study

  • 期刊论文

  • 发表日期：

    2026年1月21日

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  • Basel Musmar,
  • Joanna M. Roy,
  • Hammam Abdalrazeq,
  • Celine Yacoub,
  • Nimer Adeeb,
  • Antony A. Fuleihan,
  • Sravanthi Koduri,
  • Michael Baldassari,
  • Matthews Lan,
  • Pious Patel,
  • Arbaz A. Momin,
  • Elias Atallah,
  • Rabab Alshahrani,
  • Spyridon Karadimas,
  • Saman Sizdahkhani,
  • Ajay Garg,
  • Kareem El Naamani,
  • Ching-Jen Chen,
  • Roland Jabre,
  • Hassan Saad,
  • Jonathan A. Grossberg,
  • Adam A. Dmytriw,
  • Aman B. Patel,
  • Mirhojjat Khorasanizadeh,
  • Christopher S. Ogilvy,
  • Ajith J. Thomas,
  • Andre Monteiro,
  • Adnan Siddiqui,
  • Gustavo M. Cortez,
  • Ricardo A. Hanel,
  • Rahim Abo Kasem,
  • Guilherme Porto,
  • Alejandro M. Spiotta,
  • Anthony J. Piscopo,
  • David M. Hasan,
  • Mohammad Ghorbani,
  • Joshua Weinberg,
  • Shahid M. Nimjee,
  • Kimon Bekelis,
  • Mohamed M. Salem,
  • Jan-Karl Burkhardt,
  • Akli Zetchi,
  • Charles Matouk,
  • Brian M. Howard,
  • Rosalind Lai,
  • Rose Du,
  • Rawad Abbas,
  • Abdelaziz Amllay,
  • Alfredo Munoz,
  • Nabeel A. Herial,
  • Stavropoula I. Tjoumakaris,
  • Michael Reid Gooch,
  • Christina Notarianni,
  • Bharat Guthikonda,
  • Jacques Morcos,
  • Robert H. Rosenwasser,
  • Pascal Jabbour

摘要

Background

Moyamoya syndrome (MMS) associated with sickle cell disease (SCD) is a severe vasculopathy that significantly increases stroke risk. While cerebral revascularization is increasingly considered in this population, concerns about perioperative safety and long-term outcomes have limited its use in clinical practice.

Methods

We conducted a multicenter, retrospective cohort study of 553 patients with MMS who underwent surgical revascularization across 13 centers. Patients were grouped by SCD status (SCD-MMS vs. moyamoya disease (MMD)). Primary outcomes included perioperative stroke, perioperative complications, and functional status at discharge. Secondary outcomes included length of stay, and follow-up stroke.

Results

Of 553 patients, 32 (5.8%) had SCD. There were no significant differences in overall perioperative stroke (OR 1.05, 95% CI 0.19 to 5.54), symptomatic perioperative stroke (OR 0.94, 95% CI 0.09 to 8.94), perioperative complications (OR 1.66, 95% CI 0.47 to 5.86), or follow-up stroke (OR 0.88, 95% CI 0.17 to 4.55). Functional outcomes at discharge were similarly favorable in both groups (mRS 0–1: OR 0.84, 95% CI 0.29 to 2.40). SCD was associated with a longer hospital stay (beta 2.78 days, 95% CI 0.60 to 4.96).

Conclusion

Surgical revascularization for MMS in patients with SCD does not confer additional procedural risk and yields outcomes comparable to those of patients without SCD. These findings support the role of bypass surgery as a viable treatment option in this high-risk population.