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.
cerebrovascular event
encephalo-duro-arterio-synangiosis
moyamoya syndrome
revascularization surgery
sickle cell disease
stroke
Journal
Pediatric blood & cancer
ISSN: 1545-5017
Titre abrégé: Pediatr Blood Cancer
Pays: United States
ID NLM: 101186624
Informations de publication
Date de publication:
07 2023
07 2023
Historique:
revised:
01
03
2023
received:
09
08
2022
accepted:
04
03
2023
medline:
25
5
2023
pubmed:
15
4
2023
entrez:
14
4
2023
Statut:
ppublish
Résumé
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). 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. 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). 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.
Sections du résumé
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.
Types de publication
Multicenter Study
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
e30336Commentaires et corrections
Type : CommentIn
Type : ErratumIn
Informations de copyright
© 2023 Wiley Periodicals LLC.
Références
Wastnedge E, Waters D, Patel S, et al. The global burden of sickle cell disease in children under five years of age: a systematic review and meta-analysis. J Glob Health. 2018;8(2):021103. doi:10.7189/jogh.08.021103
Hassell KL. Population estimates of sickle cell disease in the U.S. Am J Prev Med. 2010;38(Suppl 4):S512-S521. doi:10.1016/j.amepre.2009.12.022
Baker C, Grant AM, George MG, Grosse SD, Adamkiewicz TV. Contribution of sickle cell disease to the pediatric stroke burden among hospital discharges of African-Americans-United States, 1997-2012. Pediatr Blood Cancer. 2015;62(12):2076-2081. doi:10.1002/pbc.25655
Verduzco LA, Nathan DG. Sickle cell disease and stroke. Blood. 2009;114(25):5117-5125. doi:10.1182/blood-2009-05-220921
Bernaudin F, Verlhac S, Arnaud C, et al. Chronic and acute anemia and extracranial internal carotid stenosis are risk factors for silent cerebral infarcts in sickle cell anemia. Blood. 2015;125(10):1653-1661. doi:10.1182/blood-2014-09-599852
Adams RJ, Brambilla D, Investigators OPSPiSCAST. Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. N Engl J Med. 2005;353(26):2769-2778. doi:10.1056/NEJMoa050460
Adams RJ, McKie VC, Hsu L, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med. 1998;339(1):5-11. doi:10.1056/NEJM199807023390102
Ware RE, Davis BR, Schultz WH, et al. Hydroxycarbamide versus chronic transfusion for maintenance of transcranial doppler flow velocities in children with sickle cell anaemia-TCD with transfusions changing to hydroxyurea (TWiTCH): a multicentre, open-label, phase 3, non-inferiority trial. Lancet. 2016;387(10019):661-670. doi:10.1016/S0140-6736(15)01041-7
Hulbert ML, McKinstry RC, Lacey JL, et al. Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle cell disease. Blood. 2011;117(3):772-779. doi:10.1182/blood-2010-01-261123
Dobson SR, Holden KR, Nietert PJ, et al. Moyamoya syndrome in childhood sickle cell disease: a predictive factor for recurrent cerebrovascular events. Blood. 2002;99(9):3144-3150. doi:10.1182/blood.v99.9.3144
Willis RCotPaToSOotCo, Diseases HLSRGfRoMfI. Guidelines for diagnosis and treatment of moyamoya disease (spontaneous occlusion of the circle of Willis). Neurol Med Chir (Tokyo). 2012;52(5):245-266. doi:10.2176/nmc.52.245
Hall EM, Leonard J, Smith JL, et al. Reduction in overt and silent stroke recurrence rate following cerebral revascularization surgery in children with sickle cell disease and severe cerebral vasculopathy. Pediatr Blood Cancer. 2016;63(8):1431-1437. doi:10.1002/pbc.26022
Yang W, Xu R, Porras JL, et al. Effectiveness of surgical revascularization for stroke prevention in pediatric patients with sickle cell disease and moyamoya syndrome. J Neurosurg Pediatr. 2017;20(3):232-238. doi:10.3171/2017.1.PEDS16576
Vernet O, Montes JL, O'Gorman AM, Baruchel S, Farmer JP. Encephaloduroarterio-synangiosis in a child with sickle cell anemia and moyamoya disease. Pediatr Neurol. 1996;14(3):226-230. doi:10.1016/0887-8994(96)00045-8
Fryer RH, Anderson RC, Chiriboga CA, Feldstein NA. Sickle cell anemia with moyamoya disease: outcomes after EDAS procedure. Pediatr Neurol. 2003;29(2):124-130. doi:10.1016/s0887-8994(03)00047-x
Kennedy BC, McDowell MM, Yang PH, et al. Pial synangiosis for moyamoya syndrome in children with sickle cell anemia: a comprehensive review of reported cases. Neurosurg Focus. 2014;36(1):E12. doi:10.3171/2013.10.FOCUS13405
Alamri A, Hever P, Cheserem J, Gradil C, Bassi S, Tolias CM. Encephaloduroateriosynangiosis (EDAS) in the management of Moyamoya syndrome in children with sickle cell disease. Br J Neurosurg. 2019;33(2):161-164. doi:10.1080/02688697.2017.1339227
Griessenauer CJ, Lebensburger JD, Chua MH, et al. Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease. J Neurosurg Pediatr. 2015;16(1):64-73. doi:10.3171/2014.12.PEDS14522
Winstead M, Sun PP, Martin K, et al. Encephaloduroarteriosynangiosis (EDAS) in young patients with cerebrovascular complications of sickle cell disease: single-institution experience. Pediatr Hematol Oncol. 2017;34(2):100-106. doi:10.1080/08880018.2017.1313917
Suzuki J, Takaku A. Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol. 1969;20(3):288-299. doi:10.1001/archneur.1969.00480090076012
Araki Y, Yokoyama K, Uda K, et al. Postoperative stroke and neurological outcomes in the early phase after revascularization surgeries for moyamoya disease: an age-stratified comparative analysis. Neurosurg Rev. 2021;44(5):2785-2795. doi:10.1007/s10143-020-01459-0
Ko SB. Perioperative stroke: pathophysiology and management. Korean J Anesthesiol. 2018;71(1):3-11. doi:10.4097/kjae.2018.71.1.3
DeBaun MR, Gordon M, McKinstry RC, et al. Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia. N Engl J Med. 2014;371(8):699-710. doi:10.1056/NEJMoa1401731
Arkuszewski M, Krejza J, Chen R, et al. Sickle cell anemia: intracranial stenosis and silent cerebral infarcts in children with low risk of stroke. Adv Med Sci. 2014;59(1):108-113. doi:10.1016/j.advms.2013.09.001
Fasano RM, Meier ER, Hulbert ML. Cerebral vasculopathy in children with sickle cell anemia. Blood Cells Mol Dis. 2015;54(1):17-25. doi:10.1016/j.bcmd.2014.08.007
Kassim AA, DeBaun MR. Sickle cell disease, vasculopathy, and therapeutics. Annu Rev Med. 2013;64:451-466. doi:10.1146/annurev-med-120611-143127
Kato GJ, Hebbel RP, Steinberg MH, Gladwin MT. Vasculopathy in sickle cell disease: biology, pathophysiology, genetics, translational medicine, and new research directions. Am J Hematol. 2009;84(9):618-625. doi:10.1002/ajh.21475
Oshima H, Katayama Y. Discovery of cerebrovascular moyamoya disease: research during the late 1950s and early 1960s. Childs Nerv Syst. 2012;28(4):497-500. doi:10.1007/s00381-012-1708-x
Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91(1):288-294.
Pegelow CH, Adams RJ, McKie V, et al. Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions. J Pediatr. 1995;126(6):896-899. doi:10.1016/s0022-3476(95)70204-0
Majumdar S, Miller M, Khan M, et al. Outcome of overt stroke in sickle cell anaemia, a single institution's experience. Br J Haematol. 2014;165(5):707-713. doi:10.1111/bjh.12795
Smith ER, McClain CD, Heeney M, Scott RM. Pial synangiosis in patients with moyamoya syndrome and sickle cell anemia: perioperative management and surgical outcome. Neurosurg Focus. 2009;26(4):E10. doi:10.3171/2009.01.FOCUS08307
Hankinson TC, Bohman LE, Heyer G, et al. Surgical treatment of moyamoya syndrome in patients with sickle cell anemia: outcome following encephaloduroarteriosynangiosis. J Neurosurg Pediatr. 2008;1(3):211-216. doi:10.3171/PED/2008/1/3/211
Gatti JR, Torriente AG, Sun LR. Clinical presentation and stroke incidence differ by moyamoya etiology. J Child Neurol. 2021;36(4):272-280. doi:10.1177/0883073820967160
Bir S, Kelley RE. Antithrombotic therapy in the prevention of stroke. Biomedicines. 2021;9(12):1906. doi:10.3390/biomedicines9121906
Yang Y, Huang Z, Zhang X. Efficacy and safety of clopidogrel and/or aspirin for ischemic stroke/transient ischemic attack: an overview of systematic reviews and meta-analysis. Medicine (Baltimore). 2021;100(50):e27804. doi:10.1097/MD.0000000000027804
Kleindorfer DO, Towfighi A, Chaturvedi S, et al. 2021 Guideline for the prevention of stroke in patients with stroke and transient ischemic attack: a guideline from the American Heart Association/American Stroke Association. Stroke. 2021;52(7):e364-e467. doi:10.1161/STR.0000000000000375
DeBaun MR, Jordan LC, King AA, et al. American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults. Blood Adv. 2020;4(8):1554-1588. doi:10.1182/bloodadvances.2019001142
Carpenter JL, Nickel RS, Webb J, et al. Low rates of cerebral infarction after hematopoietic stem cell transplantation in patients with sickle cell disease at high risk for stroke. Transplant Cell Ther. 2021;27(12):1018. e1-1018.e9. doi:10.1016/j.jtct.2021.08.026