Surgical treatment of moyamoya syndrome in patients with sickle cell anemia: outcome following encephaloduroarteriosynangiosis.
OBJECTIVE
Children with sickle cell anemia (SCA) and moyamoya syndrome carry a significant risk of ischemic stroke. Given the success of encephaloduroarteriosynangiosis (EDAS) or pial synangiosis in the treatment of moyamoya disease, the purpose of this study was to examine whether it reliably and durably protected children with SCA and moyamoya syndrome against cerebrovascular complications.


METHODS
The authors retrospectively reviewed a series of 12 patients with SCA who developed clinical and/or radiological evidence of moyamoya syndrome and underwent EDAS.


RESULTS
Eleven patients (92%) presented following a cerebrovascular accident (CVA), transient ischemic attack (TIA), or seizure. Magnetic resonance (MR) imaging or angiography suggested moyamoya vascular changes, and cerebral angiography confirmed the diagnosis in all 12 patients. At the time of surgery, the median age was 12.3 years (range 6.8-19.4 years). Ten (83%) of 12 patients had a history of CVA, and 4 of these patients were compliant with a transfusion protocol at the time of their CVA. Bilateral (7 patients) or unilateral (5 patients) EDAS was performed without complications. The mean follow-up period was 46.8 months (range 8.1-106 months). During the follow-up period, 2 patients (16.7%) suffered cerebrovascular events. One patient, who was stroke-free preoperatively, suffered a CVA 3 weeks after the procedure. The other patient suffered a single left lower-extremity TIA 18 months following right-sided EDAS. She returned to her neurological baseline condition and remains stable 53 months postoperatively. Seven patients underwent follow-up angiography or MR angiography, and evidence of revascularization was noted in all cases. At this time, no patient has developed progressive disease requiring a contralateral procedure after unilateral EDAS.


CONCLUSIONS
The EDAS procedure is a safe and effective treatment option in patients with SCA who develop moyamoya syndrome.
Hankinson
Todd C
TC
Department of Neurosurgery, Division of Hematology and Bone Marrow Transplantation, Columbia University, College of Physicians and Surgeons, New York, NY 10032, USA. tch12@columbia.edu
Bohman
Leif-Erik
LE
Heyer
Geoffrey
G
Licursi
Maureen
M
Ghatan
Saadi
S
Feldstein
Neil A
NA
Anderson
Richard C E
RC
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications*; D002533:Cerebral Angiography; D002548:Cerebral Revascularization / Q000379:methods*; D002560:Cerebrovascular Circulation / Q000502:physiology; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D002546:Ischemic Attack, Transient / Q000517:prevention & control; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D008297:Male; D009072:Moyamoya Disease / Q000150:complications / Q000601:surgery*; D010841:Pia Mater / Q000601:surgery; D012189:Retrospective Studies; D012640:Seizures / Q000517:prevention & control; D020521:Stroke / Q000517:prevention & control; D013524:Surgical Flaps / Q000098:blood supply; D013699:Temporal Arteries / Q000637:transplantation; D016896:Treatment Outcome