Surgical Revascularization in Children with Sickle Cell Disease and
Moyamoya Syndrome
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.