Piatt, Joseph H.

OBJECTIVE Cerebral revascularization surgery (CRS) has been used to prevent stroke in children with sickle cell disease (SCD) and cerebral vasculopathy (e.g., moyamoya syndrome). While results suggest that it may be an effective treatment, surgical indications have not been well defined. This study sought to determine indications for offering revascularization surgery in centers with established sickle cell programs in the US. METHODS Three sequential surveys utilizing the Delphi methodology were administered to neurosurgeons participating in the Stroke in Sickle Cell Revascularization Surgery study. Respondents were presented with clinical scenarios of patients with SCD and varying degrees of ischemic presentation and vasculopathy, and the group’s agreement to offer surgical revascularization was measured. Consensus was defined as ≥ 75% similar responses. RESULTS The response rate to all 3 surveys was 100%. Seventeen neurosurgeons from 16 different centers participated. The presence of moyamoya collaterals (MMCs) and arterial stenosis matching an ischemic distribution yielded the strongest recommendations to offer surgery. There was consensus to offer revascularization in the presence of MMCs and at least 50% arterial stenosis matching an ischemic distribution. In contrast, there was no consensus to offer revascularization with 50%–70% stenosis not matching an ischemic presentation in the absence of MMCs. The presence of the ivy sign in the distribution of the stenotic artery also contributed to the consensus to offer surgery in certain scenarios. CONCLUSIONS There were several clinical scenarios that attained consensus to offer surgery; the strongest was moderate to severe arterial stenosis that matched the distribution of ischemic presentation in the presence of MMCs. Radiological findings of decreased cerebral flow or perfusion also facilitated attaining consensus to offer surgery. The findings of this study reflect expert opinion about questions that deserve prospective clinical research. Determination of indications for CRS can guide clinical practice and aid the design of prospective studies.

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.