Cerebral revascularization surgery reduces cerebrovascular events in children with sickle cell disease and moyamoya syndrome: Results of the stroke in sickle cell revascularization surgery retrospective study

Abstract

Background Recent studies suggest that cerebral revascularization surgery may be a safe and effective therapy to reduce stroke risk in patients with sickle cell disease and moyamoya syndrome (SCD–MMS). Methods We performed a multicenter, retrospective study of children with SCD–MMS treated with conservative management alone (conservative group)—chronic blood transfusion and/or hydroxyurea—versus conservative management plus surgical revascularization (surgery group). We monitored cerebrovascular event (CVE) rates—a composite of strokes and transient ischemic attacks. Multivariable logistic regression was used to compare CVE occurrence and multivariable Poisson regression was used to compare incidence rates between groups. Covariates in multivariable models included age at treatment start, age at moyamoya diagnosis, antiplatelet use, CVE history, and the risk period length. Results We identified 141 patients with SCD–MMS, 78 (55.3%) in the surgery group and 63 (44.7%) in the conservative group. Compared with the conservative group, preoperatively the surgery group had a younger age at moyamoya diagnosis, worse baseline modified Rankin scale scores, and increased prevalence of CVEs. Despite more severe pretreatment disease, the surgery group had reduced odds of new CVEs after surgery (odds ratio = 0.27, 95% confidence interval [CI] = 0.08–0.94, p = .040). Furthermore, comparing surgery group patients during presurgical versus postsurgical periods, CVEs odds were significantly reduced after surgery (odds ratio = 0.22, 95% CI = 0.08–0.58, p = .002). Conclusions When added to conservative management, cerebral revascularization surgery appears to reduce the risk of CVEs in patients with SCD–MMS. A prospective study will be needed to validate these findings.

Comment on: Highlight: Is smoke the signal for surgery? Should the moyamoya syndrome “puff of smoke” trigger cerebral revascularization surgery in children with sickle cell disease?

“…Protective effect of aspirin against CVE may be another confounding variable" Contrary to the protective effect against CVE as stated, our study found that patients on antiplatelet therapy had significantly increased odds of CVE occurrence (OR = 7.45, p < .001; Table S1). 2 Despite the Surgery group having significantly more patients on antiplatelet therapy, which appeared to increase CVEs, they still had a lower odds of CVE occurrence.…”

“…

We read with interest the Highlight article by Dr. Hsu that reviewed our Stroke in Sickle Cell Revascularization Surgery (SiSCRS) study published in the same issue of Pediatric Blood & Cancer. 1,2 We agree that further research is needed to determine which children with sickle cell disease and moyamoya syndrome (SCD-MMS) would benefit from cerebral revascularization surgery (CRS).We want to highlight several inaccuracies and weaknesses in the arguments used in the critique.

…”