{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,11]],"date-time":"2026-07-11T13:35:23Z","timestamp":1783776923495,"version":"3.55.0"},"reference-count":38,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["PED"],"published-print":{"date-parts":[[2008,3]]},"abstract":"<jats:sec>\n<jats:title>Object<\/jats:title>\n<jats:p>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.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>The authors retrospectively reviewed a series of 12 patients with SCA who developed clinical and\/or radiological evidence of moyamoya syndrome and underwent EDAS.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>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\u201319.4 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\u2013106 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.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>The EDAS procedure is a safe and effective treatment option in patients with SCA who develop moyamoya syndrome.<\/jats:p><\/jats:sec>","DOI":"10.3171\/ped\/2008\/1\/3\/211","type":"journal-article","created":{"date-parts":[[2008,2,29]],"date-time":"2008-02-29T18:54:07Z","timestamp":1204311247000},"page":"211-216","source":"Crossref","is-referenced-by-count":64,"title":["Surgical treatment of moyamoya syndrome in patients with sickle cell anemia: outcome following encephaloduroarteriosynangiosis"],"prefix":"10.3171","volume":"1","author":[{"given":"Todd C.","family":"Hankinson","sequence":"first","affiliation":[{"name":"Departments of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leif-Erik","family":"Bohman","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geoffrey","family":"Heyer","sequence":"additional","affiliation":[{"name":"Neurology, and"},{"name":"Pediatrics; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maureen","family":"Licursi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Hematology and Bone Marrow Transplantation, Columbia University, College of Physicians and Surgeons, New York, New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saadi","family":"Ghatan","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neil A.","family":"Feldstein","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard C. E.","family":"Anderson","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-0010211","series-title":"N Engl J Med","doi-asserted-by":"publisher","first-page":"2769","DOI":"10.1056\/NEJMoa050460","article-title":"Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease","volume":"353","author":"Adams","year":"2005"},{"key":"b2-0010211","series-title":"N Engl J Med","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1056\/NEJM199807023390102","article-title":"Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography","volume":"339","author":"Adams","year":"1998"},{"key":"b3-0010211","series-title":"Surg Neurol","first-page":"3","article-title":"Bilateral temporal-middle cerebral artery anastomosis for Moyamoya syndrome","volume":"8","author":"Amine","year":"1977"},{"key":"b4-0010211","series-title":"Child Neuropsychol","doi-asserted-by":"publisher","first-page":"120","DOI":"10.1080\/09297040600800956","article-title":"Neurocognitive sequelae of pediatric sickle cell disease: a review of the literature","volume":"13","author":"Berkelhammer","year":"2007"},{"key":"b5-0010211","series-title":"Blood","doi-asserted-by":"publisher","first-page":"1657","DOI":"10.1182\/blood.V79.7.1657.1657","article-title":"A modified transfusion program for prevention of stroke in sickle cell disease","volume":"79","author":"Cohen","year":"1992"},{"key":"b6-0010211","series-title":"Pediatrics","doi-asserted-by":"publisher","first-page":"804","DOI":"10.1542\/peds.65.4.804","article-title":"Moyamoya and Fanconi's anemia","volume":"65","author":"Cohen","year":"1980"},{"key":"b7-0010211","series-title":"J Child Neurol","doi-asserted-by":"publisher","first-page":"696","DOI":"10.1177\/088307380001501012","article-title":"Early-onset Moyamoya syndrome in a patient with Down syndrome: case report and review of the literature","volume":"15","author":"Dai","year":"2000"},{"key":"b8-0010211","series-title":"Blood","doi-asserted-by":"publisher","first-page":"3144","DOI":"10.1182\/blood.V99.9.3144","article-title":"Moyamoya syndrome in childhood sickle cell disease: a predictive factor for recurrent cerebrovascular events","volume":"99","author":"Dobson","year":"2002"},{"key":"b9-0010211","series-title":"Radiographics","doi-asserted-by":"publisher","first-page":"483","DOI":"10.1148\/radiographics.13.2.8460234","article-title":"General case of the day. Moyamoya syndrome in a child with sickle cell disease","volume":"13","author":"Drew","year":"1993"},{"issue":"47 Suppl","key":"b10-0010211","series-title":"Ann Clin Res","first-page":"21","article-title":"The treatment of Moyamoya disease by superficial temporal-middle cerebral artery (STA-MCA) anastomosis","volume":"18","author":"Erickson","year":"1986"},{"key":"b11-0010211","series-title":"Pediatr Neurol","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1016\/S0887-8994(03)00047-X","article-title":"Sickle cell anemia with moyamoya disease: outcomes after EDAS procedure","volume":"29","author":"Fryer","year":"2003"},{"key":"b12-0010211","series-title":"Surg Neurol","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1016\/S0090-3019(96)00358-8","article-title":"Current state of study on moyamoya disease in Japan","volume":"47","author":"Fukui","year":"1997"},{"key":"b13-0010211","series-title":"Surg Neurol","doi-asserted-by":"publisher","first-page":"425","DOI":"10.1016\/0090-3019(82)90178-1","article-title":"Pseudomoyamoya in sickle cell anemia","volume":"18","author":"Garza-Mercado","year":"1982"},{"key":"b14-0010211","series-title":"Pediatr Neurosurg","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1159\/000120496","article-title":"Moyamoya-like disease in Down's syndrome","volume":"16","author":"Goldstein","year":"1990"},{"key":"b15-0010211","series-title":"Stroke","article-title":"The cerebral vasculopathy of PHACES syndrome","author":"Heyer","year":"2008"},{"key":"b16-0010211","series-title":"Dev Med Child Neurol","doi-asserted-by":"publisher","first-page":"824","DOI":"10.1017\/S001216220500174X","article-title":"Intellectual decline in children with moyamoya and sickle cell anaemia","volume":"47","author":"Hogan","year":"2005"},{"key":"b17-0010211","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"679","DOI":"10.3171\/jns.1978.49.5.0679","article-title":"Treatment of moyamoya disease with STA-MCA anastomosis","volume":"49","author":"Karasawa","year":"1978"},{"key":"b18-0010211","series-title":"Clin Neurol Neurosurg","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1016\/j.clineuro.2006.06.004","article-title":"Surgical results in pediatric moyamoya disease: angiographic revascularization and the clinical results","volume":"109","author":"Kim","year":"2007"},{"key":"b19-0010211","series-title":"Neurosurgery","first-page":"88","article-title":"Combined encephaloduroarteriosynangiosis and bifrontal encephalogaleo(periosteal)synangiosis in pediatric moyamoya disease","volume":"50","author":"Kim","year":"2002"},{"key":"b20-0010211","series-title":"Surg Neurol","first-page":"353","article-title":"The Moyamoya syndrome and the neurosurgeon","volume":"4","author":"Krayenb\u00fchl","year":"1975"},{"key":"b21-0010211","series-title":"Blood","doi-asserted-by":"publisher","first-page":"847","DOI":"10.1182\/blood-2005-10-009506","article-title":"Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results","volume":"108","author":"Lee","year":"2006"},{"key":"b22-0010211","series-title":"Neurology","doi-asserted-by":"publisher","first-page":"1893","DOI":"10.1212\/WNL.41.12.1893","article-title":"Accelerated intracranial occlusive disease, oral contraceptives, and cigarette use","volume":"41","author":"Levine","year":"1991"},{"key":"b23-0010211","series-title":"Acta Neurochir (Wien)","doi-asserted-by":"publisher","first-page":"135","DOI":"10.1007\/BF01407339","article-title":"Surgical treatment for paediatric patients with moyamoya disease by indirect revascularization procedures (EDAS, EMS, EMAS)","volume":"98","author":"Matsushima","year":"1989"},{"key":"b24-0010211","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"401","DOI":"10.1227\/00006123-199209000-00003","article-title":"Surgical treatment of moyamoya disease in pediatric patients\u2014comparison between the results of indirect and direct revascularization procedures","volume":"31","author":"Matsushima","year":"1992"},{"key":"b25-0010211","series-title":"Neurol Res","doi-asserted-by":"publisher","first-page":"66","DOI":"10.1080\/01616412.1997.11740774","article-title":"EC-IC bypass improves chronic ischemia in a patient with moyamoya disease secondary to sickle cell disease: an in vivo microdialysis study","volume":"19","author":"Mendelowitsch","year":"1997"},{"key":"b26-0010211","series-title":"Stroke","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1161\/01.STR.9.1.45","article-title":"Cerebrovascular disease in sickle cell anemia: a clinical, pathological and radiological correlation","volume":"9","author":"Merkel","year":"1978"},{"key":"b27-0010211","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"830","DOI":"10.1227\/01.NEU.0000227527.69766.43","article-title":"Unilaterally symptomatic moyamoya disease in children: long-term follow-up of 20 patients","volume":"59","author":"Nagata","year":"2006"},{"issue":"2 Suppl","key":"b28-0010211","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"238","DOI":"10.3171\/jns.2005.102.2.0238","article-title":"Development of a de novo cerebral arteriovenous malformation in a child with sickle cell disease and moyamoya arteriopathy. Case report","volume":"102","author":"O'Shaughnessy","year":"2005"},{"key":"b29-0010211","series-title":"Pediatric Neurosurgery: Surgery of the Developing Nervous System","first-page":"1077","article-title":"Moyamoya syndrome","author":"Ohaegbulam","year":"2001"},{"key":"b30-0010211","series-title":"Adv Pediatr","first-page":"247","article-title":"Neurologic complications of sickle cell disease","volume":"36","author":"Pavlakis","year":"1989"},{"key":"b31-0010211","series-title":"Tex Rep Biol Med","first-page":"293","article-title":"Cerebral vascular accidents in sickle cell anemia","volume":"40","author":"Powars","year":"1980"},{"key":"b32-0010211","series-title":"J Can Assoc Radiol","first-page":"332","article-title":"Moyamoya in sickle cell disease demonstrated by DSA and Hexabrix","volume":"36","author":"Ramsewak","year":"1985"},{"issue":"2 Suppl Pediatrics","key":"b33-0010211","series-title":"J Neurosurg","first-page":"142","article-title":"Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis","volume":"100","author":"Scott","year":"2004"},{"key":"b34-0010211","series-title":"Skull Base","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1055\/s-2005-868160","article-title":"Surgical management of moyamoya syndrome","volume":"15","author":"Smith","year":"2005"},{"key":"b35-0010211","series-title":"Brain","doi-asserted-by":"publisher","first-page":"107","DOI":"10.1093\/brain\/93.1.107","article-title":"Natural history of acute hemiplegia of childhood","volume":"93","author":"Solomon","year":"1970"},{"key":"b36-0010211","series-title":"Anesth Analg","first-page":"1066","article-title":"Anesthetic management of children with moyamoya syndrome","volume":"77","author":"Soriano","year":"1993"},{"key":"b37-0010211","series-title":"N Engl J Med","doi-asserted-by":"publisher","first-page":"846","DOI":"10.1056\/NEJM197210262871703","article-title":"Occlusion of large cerebral vessels in sickle-cell anemia","volume":"287","author":"Stockman","year":"1972"},{"key":"b38-0010211","series-title":"Pediatr Neurol","doi-asserted-by":"publisher","first-page":"226","DOI":"10.1016\/0887-8994(96)00045-8","article-title":"Encephaloduroarteriosynangiosis in a child with sickle cell anemia and moyamoya disease","volume":"14","author":"Vernet","year":"1996"}],"container-title":["Journal of Neurosurgery: Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/1\/3\/article-p211.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/1\/3\/article-p211.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/1\/3\/article-p211.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,15]],"date-time":"2023-04-15T14:46:23Z","timestamp":1681569983000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/1\/3\/article-p211.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,3]]},"references-count":38,"journal-issue":{"issue":"3"},"URL":"https:\/\/doi.org\/10.3171\/ped\/2008\/1\/3\/211","relation":{},"ISSN":["1933-0707","1933-0715"],"issn-type":[{"value":"1933-0707","type":"print"},{"value":"1933-0715","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,3]]}}}