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.

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.

Combined Neuroendoscopic Techniques in the Management of Pediatric Brain and Skull Base Tumors: A Single-Institutional Case Series.

BACKGROUND: Central nervous system tumors encompass the leading cause of cancer-related death in the pediatric population. Neuroendoscopic techniques have been optimized over the years, becoming an important tool for the management of brain tumors. Our study aims to review the indications for neuroendoscopic procedures and the feasibility of combined interventions. METHODS: This is a single-center, self-adjudicated, retrospective experience. Inclusion criteria consisted of pediatric patients (≤18 years old) who underwent management of brain tumor or related diseases with the employment of neuroendoscopy. RESULTS: A total of 47 patients undergoing 51 procedures met inclusion criteria. The mean age was 9.8 ± 4.6 years, and the majority were female (55.3%). Common indications for endoscopic intervention were hydrocephalus management (n = 24; 16 endoscopic third ventriculostomies and 9 septostomies), tumor biopsy (n = 19), cyst fenestration (n = 16), and tumor resection (n = 9). In one third of the cases, combined interventions occurred during a single operative session. Hydrocephalus was successfully managed in 74.4% of cases; tumor biopsy confirmed the diagnosis in 95.8% of cases, and gross total resection was achieved in 88.9% of cases. Cyst fenestration required reintervention in 3 cases: one case associated with initial cyst enlargement and 2 cases with the development of new tumor cysts separated from the originally fenestrated cyst. The overall complication rate was 6.3%, with only one major safety event, which was successfully managed. CONCLUSIONS: Neuroendoscopy is an important minimally invasive tool for diagnosing and treating pediatric patients with brain tumors, permitting to address multiple problems in a single surgery.