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

Philipp R Aldana¹, Ricardo A Hanel², Joseph Piatt³, Sabrina H Han⁴, Manisha M Bansal⁵, Corinna Schultz⁶, Cynthia Gauger⁵, John M Pederson^{7

8}, John C Wellons Iii⁹, Monica L Hulbert¹⁰, Lori C Jordan¹¹, Adnan Qureshi¹², Kelsey Garrity¹, Adam P Robert¹, Asmaa Hatem¹, Jennifer Stein⁴, Emily Beydler⁴, P David Adelson¹³, Stephanie Greene¹⁴, Paul Grabb¹⁵, James Johnston¹⁶, Shih-Shan Lang¹⁷, Jeffrey Leonard¹⁸, Suresh N Magge¹⁹, Alex Scott²⁰, Sanjay Shah²¹, Edward R Smith²², Jodi Smith²³, Jennifer Strahle²⁰, Sudhakar Vadivelu²⁴, Jennifer Webb²⁵, David Wrubel²⁶

Affiliations

¹ Department of Neurosurgery, University of Florida College of Medicine - Jacksonville and Wolfson Children's Hospital, Jacksonville, Florida, USA.
² Lyerly Neurosurgery, Baptist Neurological Institute, Jacksonville, Florida, USA.
³ Division of Neurosurgery, Nemours Neuroscience Center, A.I. duPont Hospital for Children, Wilmington, Delaware, USA.
⁴ University of Florida College of Medicine, Gainesville, Florida, USA.
⁵ Department of Pediatric Hematology/Oncology, Nemours Children's Health System and Wolfson Children's Hospital, Jacksonville, Florida, USA.
⁶ Department of Pediatrics, Nemours Center for Cancer and Blood Disorders, Nemours Children's Hospital, Wilmington, Delaware, USA.
⁷ Superior Medical Experts, St. Paul, Minnesota, USA.
⁸ Nested Knowledge, St. Paul, Minnesota, USA.
⁹ Division of Pediatric Neurological Surgery, Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
¹⁰ Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri, USA.
¹¹ Division of Pediatric Neurology, Department of Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
¹² Department of Neurology, Zeenat Qureshi Stroke Institute, University of Missouri, Columbia, Missouri, USA.
¹³ Barrow Neurological Institute, Phoenix Children's Hospital, Phoenix, Arizona, USA.
¹⁴ Department of Neurosurgery, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
¹⁵ Department of Neurosurgery, Children's Mercy Hospital, Kansas, Missouri, USA.
¹⁶ Department of Neurosurgery, Children's Hospital of Alabama, Birmingham, Alabama, USA.
¹⁷ Department of Neurosurgery and Pediatric Neurosurgery, University of Pennsylvania School of Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
¹⁸ Department of Neurosurgery, Nationwide Children's Hospital, Columbus, Ohio, USA.
¹⁹ Department of Neurosurgery, CHOC Neuroscience Institute, Children's Health of Orange County, Orange, California, USA.
²⁰ Department of Neurosurgery, Washington University School of Medicine, Washington University in Saint Louis, St Louis, Missouri, USA.
²¹ Department of Pediatric Hematology/Oncology, Phoenix Children's Hospital, Phoenix, Arizona, USA.
²² Department of Neurosurgery, Children's Hospital Boston, and Harvard Medical School, Boston, Massachusetts, USA.
²³ Department of Pediatric Neurosurgery, Goodman Campbell Brain and Spine, Peyton Manning Children's Hospital, Indianapolis, Indiana, USA.
²⁴ Division of Neurosurgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
²⁵ Department of Hematology/Oncology, Children's National Hospital, Washington, District of Columbia, USA.
²⁶ Department of Neurosurgery, Children's Healthcare of Atlanta, Egleston Hospital, Atlanta, Georgia, USA.

PMID: 37057741
DOI: 10.1002/pbc.30336

Multicenter Study

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

Philipp R Aldana et al. Pediatr Blood Cancer. 2023 Jul.

. 2023 Jul;70(7):e30336.

doi: 10.1002/pbc.30336. Epub 2023 Apr 14.

Authors

Affiliations

¹ Department of Neurosurgery, University of Florida College of Medicine - Jacksonville and Wolfson Children's Hospital, Jacksonville, Florida, USA.
² Lyerly Neurosurgery, Baptist Neurological Institute, Jacksonville, Florida, USA.
³ Division of Neurosurgery, Nemours Neuroscience Center, A.I. duPont Hospital for Children, Wilmington, Delaware, USA.
⁴ University of Florida College of Medicine, Gainesville, Florida, USA.
⁵ Department of Pediatric Hematology/Oncology, Nemours Children's Health System and Wolfson Children's Hospital, Jacksonville, Florida, USA.
⁶ Department of Pediatrics, Nemours Center for Cancer and Blood Disorders, Nemours Children's Hospital, Wilmington, Delaware, USA.
⁷ Superior Medical Experts, St. Paul, Minnesota, USA.
⁸ Nested Knowledge, St. Paul, Minnesota, USA.
⁹ Division of Pediatric Neurological Surgery, Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
¹⁰ Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri, USA.
¹¹ Division of Pediatric Neurology, Department of Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
¹² Department of Neurology, Zeenat Qureshi Stroke Institute, University of Missouri, Columbia, Missouri, USA.
¹³ Barrow Neurological Institute, Phoenix Children's Hospital, Phoenix, Arizona, USA.
¹⁴ Department of Neurosurgery, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
¹⁵ Department of Neurosurgery, Children's Mercy Hospital, Kansas, Missouri, USA.
¹⁶ Department of Neurosurgery, Children's Hospital of Alabama, Birmingham, Alabama, USA.
¹⁷ Department of Neurosurgery and Pediatric Neurosurgery, University of Pennsylvania School of Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
¹⁸ Department of Neurosurgery, Nationwide Children's Hospital, Columbus, Ohio, USA.
¹⁹ Department of Neurosurgery, CHOC Neuroscience Institute, Children's Health of Orange County, Orange, California, USA.
²⁰ Department of Neurosurgery, Washington University School of Medicine, Washington University in Saint Louis, St Louis, Missouri, USA.
²¹ Department of Pediatric Hematology/Oncology, Phoenix Children's Hospital, Phoenix, Arizona, USA.
²² Department of Neurosurgery, Children's Hospital Boston, and Harvard Medical School, Boston, Massachusetts, USA.
²³ Department of Pediatric Neurosurgery, Goodman Campbell Brain and Spine, Peyton Manning Children's Hospital, Indianapolis, Indiana, USA.
²⁴ Division of Neurosurgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
²⁵ Department of Hematology/Oncology, Children's National Hospital, Washington, District of Columbia, USA.
²⁶ Department of Neurosurgery, Children's Healthcare of Atlanta, Egleston Hospital, Atlanta, Georgia, USA.

PMID: 37057741
DOI: 10.1002/pbc.30336

Erratum in

Corrigendum for: ''Highlight: Is smoke the signal for surgery? Should the moyamoya syndrome "puff of smoke" trigger cerebral revascularization surgery in children with sickle cell disease?".
Hsu LL. Hsu LL. Pediatr Blood Cancer. 2023 Oct;70(10):e30558. doi: 10.1002/pbc.30558. Epub 2023 Jul 13. Pediatr Blood Cancer. 2023. PMID: 37439549 No abstract available.

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.

Keywords: cerebrovascular event; encephalo-duro-arterio-synangiosis; moyamoya syndrome; revascularization surgery; sickle cell disease; stroke.

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Comment in

Highlight: Is smoke the signal for surgery? Should the moyamoya syndrome "puff of smoke" trigger cerebral revascularization surgery in children with sickle cell disease?
Hsu LL. Hsu LL. Pediatr Blood Cancer. 2023 Jul;70(7):e30371. doi: 10.1002/pbc.30371. Epub 2023 May 2. Pediatr Blood Cancer. 2023. PMID: 37132151 No abstract available.

References

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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

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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

Authors

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