Effectiveness of surgical revascularization for stroke prevention in pediatric patients with sickle cell disease and moyamoya syndrome

Abstract

<jats:sec><jats:title>OBJECTIVE</jats:title><jats:p>Sickle cell disease (SCD) in combination with moyamoya syndrome (MMS) represents a rare complication of SCD, with potentially devastating neurological outcomes. The effectiveness of surgical revascularization in this patient population is currently unclear. The authors’ aim was to determine the effectiveness of surgical intervention in their series of SCD-MMS patients by comparing stroke recurrence in those undergoing revascularization and those undergoing conservative transfusion therapy.</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>The authors performed a retrospective chart review of patients with MMS who were seen at the Johns Hopkins Medical Institution between 1990 and 2013. Pediatric patients (age &lt; 18 years) with confirmed diagnoses of SCD and MMS were included. Intracranial stroke occurrence during the follow-up period was compared between surgically and conservatively managed patients.</jats:p></jats:sec><jats:sec><jats:title>RESULTS</jats:title><jats:p>A total of 15 pediatric SCD-MMS patients (28 affected hemispheres) were included in this study, and all were African American. Seven patients (12 hemispheres) were treated with indirect surgical revascularization. The average age at MMS diagnosis was 9.0 ± 4.0 years, and 9 patients (60.0%) were female. Fourteen patients (93.3%) had strokes before diagnosis of MMS, with an average age at first stroke of 6.6 ± 3.9 years. During an average follow-up period of 11.6 years, 4 patients in the conservative treatment group experienced strokes in 5 hemispheres, whereas no patient undergoing the revascularization procedure had any strokes at follow-up (p = 0.029). Three patients experienced immediate postoperative transient ischemic attacks, but all recovered without subsequent strokes.</jats:p></jats:sec><jats:sec><jats:title>CONCLUSIONS</jats:title><jats:p>Indirect revascularization is suggested as a safe and effective alternative to the best medical therapy alone in patients with SCD-MMS. High-risk patients managed on a regimen of chronic transfusion should be considered for indirect revascularization to maximize the effect of stroke prevention.</jats:p></jats:sec>

Management of Stroke in Neonates and Children: A Scientific Statement From the American Heart Association/American Stroke Association

“…Radiographically, revascularization reverses white matter changes, improves measures of cerebral oxygenation, and increases cerebral blood flow while stabilizing stroke burden, despite progressive arteriopathy. 191,265,[270][271][272][273][274] Clinically, surgery decreases ischemic symptoms, headache, and risk of hemorrhage and markedly reduces stroke rates (without surgery, stroke risk is 32% at 1 year and 66%-90% at 5 years; after surgery, stroke risk drops to <5% for most populations at both the 1-and 5-year time points) while concomitantly improving functional and cognitive outcomes. 20,190,265,269,272,273,[275][276][277] As for many surgical procedures, an important predictor of surgical outcome is whether the child is treated at a highvolume center with a dedicated pediatric cerebrovascular team.…”

“…Other less established therapies for secondary stroke prevention include surgical revascularization procedures 273,274,290 and hematopoietic stem cell transplantation. 291,292 No study to date has used a randomized controlled trial design or a rigorous before-and-after evaluation to assess neurological injury, including serial long-term assessment of neurological and neuroimaging changes with a central adjudication committee.…”

2017 Yearbook of Neurorestoratology

“…Neurorestorative surgery may generally be divided into three categories: (i) neural reconstruction [35], such as bridging peripheral nerves between, above, and below the injury site, end-to-side facial-hypoglossal anastomosis [36]; (ii) neural decompression, such as cordotomy or myelotomy [25], and decompression for compression impairment of peripheral nerve fibers [37]; (iii) surgical revascularization [38]. Further evidence in support of functional recovery through neurorestorative surgery appeared this year [39,40].…”

The management of childhood cancer survivors at risk for stroke: A Delphi survey of regional experts

“…Similar to the general population, stroke risk for survivors is also associated with medical comorbidities including hypertension, diabetes, and health behaviors such as smoking . The clinical management of survivors at risk for stroke may include education about stroke symptoms, aggressive management of all cardiovascular risk factors (hypertension, obesity, hyperlipidemia, and diabetes), primary stroke prevention with aspirin, and in some cases neurovascular surgery . It is also recommended that consideration be given to screening asymptomatic survivors treated with cranial radiation for CVD with neuroimaging (http://www.surviorshipguidelines.org).…”

“…5 The clinical management of survivors at risk for stroke may include education about stroke symptoms, aggressive management of all cardiovascular risk factors (hypertension, obesity, hyperlipidemia, and diabetes), primary stroke prevention with aspirin, and in some cases neurovascular surgery. [11][12][13] It is also recommended that consideration be given to screening asymptomatic survivors treated with cranial radiation for CVD with neuroimaging (www.surviorshipguidelines.org). 14 A systematic review of the medical literature, however, did not identify scientific evidence to support the benefit of these clinical approaches to reduce morbidity or morality for childhood cancer survivors (CCS) at risk for stroke.…”