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Surgical Revascularization in Children with Sickle Cell Disease and Moyamoya Syndrome
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  • Matthew Kruchten,
  • Sabrina Han,
  • Kelsey Hayward,
  • Joseph Piatt,
  • Corinna Schultz,
  • Kelly Gassie,
  • Eliza Carroll,
  • Ricardo Hanel,
  • Philipp Aldana
Matthew Kruchten
University of Florida College of Medicine
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Sabrina Han
University of Florida College of Medicine
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Kelsey Hayward
University of Florida College of Medicine
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Joseph Piatt
AI DuPont Hospital for Children
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Corinna Schultz
AI DuPont Hospital for Children
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Kelly Gassie
Mayo Clinic Hospital Jacksonville
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Eliza Carroll
University of South Florida College of Public Health
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Ricardo Hanel
Lyerly Neurosurgery
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Philipp Aldana
University of Florida College of Medicine
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Abstract

Background: Pediatric patients with sickle cell disease (SCD) and moyamoya syndrome (MMS) are at high risk for recurrent cerebrovascular accidents (CVA) despite best conservative management with chronic blood transfusions (CBT). The role of surgical revascularization in this patient population is not clearly defined. We aim to compare the risk of stroke occurrence in patients undergoing conservative treatment to those additionally undergoing surgical revascularization with conservative treatment. Methods: The authors performed a retrospective cohort study of pediatric SCD-MMS patients seen in Jacksonville and Delaware between 2006 and 2018. Patient characteristics and outcomes, including stroke occurrence, were compared between those who underwent conservative treatment and those who underwent surgical revascularization. Results: A total 17 patients diagnosed with SCD-MMS were on CBT, of which 12 (70.6%) were female. The average age at CBT initiation was 7.27 years. Prior to its initiation, 10 patients (83%) in the surgical and 6 (75%) in the conservative group experienced strokes. Twelve patients underwent surgical revascularization on 18 hemispheres (17 indirect, 1 direct) for severe vasculopathy or CVA - this included 2 patients who experienced a stroke while receiving CBT. The degree of vasculopathy was worse in the surgical group. The mean follow-up for conservative and surgical groups were 11.8 and 4.4 years, respectively. Three patients experienced strokes in the conservative group, while no strokes or major complications were observed in the surgical group. Conclusion: Surgical revascularization appears safe and may effectively reduce stroke rates in pediatric patients with SCD-MMS. Larger studies are needed to confirm these findings.