Sickle cell anemia with moyamoya disease: outcomes after EDAS procedure

Fryer, Robert H.; Anderson, Richard C.; Chiriboga, Claudia A.; Feldstein, Neil A.

doi:10.1016/S0887-8994(03)00047-X

« Previous Next »Pediatric Neurology
Volume 29, Issue 2 , Pages 124-130, August 2003

Sickle cell anemia with moyamoya disease: outcomes after EDAS procedure

Robert H. Fryer, MD, PhD
- Division of Pediatric Neurology, Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, USA
- Communications should be addressed to:Dr. Fryer; The Neurologic Institute; Box 93, 710 West 168th Street; New York, NY 10032, USA.
Richard C. Anderson, MD
- Department of Neurosurgery, College of Physicians and Surgeons, Columbia University, New York, NY, USA
Claudia A. Chiriboga, MD, MPH
- Harlem Hospital Center, New York, New York, USA.
Neil A. Feldstein, MD
- Division of Pediatric Neurology, Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, USA
- Department of Neurosurgery, College of Physicians and Surgeons, Columbia University, New York, NY, USA

Received 29 October 2002; accepted 21 January 2003.

Abstract

Moyamoya disease is a relatively uncommon neurovascular complication of sickle cell anemia. We report a case series of six patients with sickle cell anemia who developed moyamoya disease and underwent encephaloduroarteriosynangiosis procedures. These six patients presented with either cerebrovascular accidents, transient ischemic attacks, or seizures, and subsequent magnetic resonance imaging scans were suggestive of moyamoya-like changes in the cerebral vasculature. Conventional cerebral angiography was used to confirm the diagnosis in all six patients. Four of six patients manifested a cerebrovascular accident before surgery, and two of these patients were compliant on a transfusion protocol at the time of their cerebrovascular accident. Bilateral (n = 4) or unilateral (n = 2) encephaloduroarteriosynangiosis procedures were performed without any complications. The patient who was stroke-free preoperatively had a cerebrovascular accident 2 weeks after the procedure; otherwise, all patients have remained free of neurovascular complications with an average follow-up of 33 months. Collateral anastomoses between external and internal carotid arteries were established by magnetic resonance angiography in three patients. The encephaloduroarteriosynangiosis procedure is a safe and effective treatment option in patients with sickle cell anemia who develop moyamoya disease.

Keywords: Sickle cell anemia, moyamoya, EDAS, stroke, ischemia