Reduction in Overt and Silent Stroke Recurrence Rate Following Cerebral Revascularization Surgery in Children with Sickle Cell Disease and Severe Cerebral Vasculopathy

Abstract

Background Children with sickle cell disease (SCD) and moyamoya may benefit from indirect cerebral revascularization surgery in addition to chronic blood transfusion therapy for infarct prevention. We sought to compare overt and silent infarct recurrence rates in children with SCD undergoing revascularization. Methods This was a retrospective cohort study of all children with SCD and moyamoya treated at two children’s hospitals. Clinical events and imaging studies were reviewed. Results Twenty-seven children with SCD and confirmed moyamoya receiving chronic transfusion therapy were identified, of whom 12 underwent indirect cerebral revascularization. Two subjects had post-operative transient ischemic attacks and another had a subarachnoid blood collection, none of which caused permanent consequences. Two subjects had surgical wound infections. Among these 12 children, the rate of overt and silent infarct recurrence decreased from 13.4 infarcts/100 patient-years before revascularization to 0 infarcts/100 patient-years after revascularization (p=0.0057); the post-revascularization infarct recurrence rate was also significantly lower than the overall infarct recurrence of 8.87 infarcts/100 patient-years in 15 children without cerebral revascularization (p=0.025). Conclusion The rate of overt and silent infarct recurrence was significantly lower following indirect cerebral revascularization. A prospective study of cerebral revascularization in children with SCD is needed.

Risk Factors for Recurrent Arterial Ischemic Stroke in Children and Young Adults

“…Both Moyamoya syndrome and progressive vasculopathy in children with SCD are risk factors for recurrent silent and clinically overt ischemic strokes; the risk was estimated to be 12.7/100 patient-years. The indirect revascularization procedure is a proven method for reducing recurrent ischemic brain incidents in these patients [63].…”

Aspirin in childhood acute ischemic stroke: The evidence for treatment and efficacy testing

“…For pediatrics, it often presents with ischemia during the first decade of life and accounts for 6% of childhood AIS in North America . Moyamoya management includes ASA and/or surgical revascularization, as the latter is not available at all centers . Rarity and heterogeneity of both presentation and management have limited the strength of pediatric moyamoya data.…”

“…[38][39][40] Moyamoya management includes ASA and/or surgical revascularization, as the latter is not available at all centers. 41 Rarity and heterogeneity of both presentation and management have limited the strength of pediatric moyamoya data. Most publications are surgically focused but report similar long-term stroke outcomes for neurosurgical and medical approaches, especially long term.…”

How I manage sickle cell patients with high transcranial doppler results

“…In a small case series of patients on chronic transfusion therapy without available BMT donors, the addition of hydroxycarbamide to transfusion resulted in disease stabilisation in 2 out of 7 patients (Brousse et al , ), but this therapeutic strategy may require further investigation in a larger cohort of patients. Surgical revascularisation approaches are also used in children with progressive cerebrovascular disease and Moyamoya syndrome on chronic transfusion programmes with improved stroke‐prevention benefit compared to chronic transfusion therapy alone (Hall et al , ).…”