{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T15:39:18Z","timestamp":1790091558226,"version":"4.0.1"},"reference-count":35,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["PED"],"published-print":{"date-parts":[[2015,7]]},"abstract":"<jats:sec>\n<jats:title>OBJECT<\/jats:title>\n<jats:p>Pediatric patients with sickle cell disease (SCD) and moyamoya syndrome (MMS) are at significant risk for cerebrovascular accidents despite chronic transfusion therapy. Encephaloduroarteriosynangiosis (EDAS) and encephalomyoarteriosynangiosis (EMAS) are additional therapeutic options for these patients. To date, the incidence of complications after and efficacy of EDAS and EMAS in stroke prevention in this population have been described in several institutional case series reports, but no randomized prospective trials have been reported.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>METHODS<\/jats:title>\n<jats:p>The authors retrospectively reviewed the cases of all pediatric patients at the University of Alabama at Birmingham with a history of homozygous hemoglobin S (HbS) and sickle cell\/\u03b2-thalassemia (SB0 thalassemia) and on chronic transfusion therapy, including 14 patients with MMS who underwent EDAS or EMAS.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>RESULTS<\/jats:title>\n<jats:p>Sixty-two patients with SCD and on chronic transfusion therapy were identified. After exclusion of patients on chronic transfusion therapy for indications other than stroke prevention, 48 patients (77.4%) remained. Of those patients, 14 (29.1%) underwent EDAS or EMAS. Nine (18.8%) and 25 (52.1%) patients were on chronic transfusion therapy for primary or secondary stroke prevention, respectively, but did not undergo EDAS or EMAS. The 14 patients with SCD and radiological evidence of MMS and on chronic transfusion therapy for primary or secondary stroke prevention underwent 21 EDAS or EMAS procedures for progressive vascular disease (92.9% of patients), stroke (71.4%), and\/or seizure (7.1%). The mean (\u00b1 SD) time from initiation of chronic transfusion therapy to EDAS or EMAS was 76.8 \u00b1 58.8 months. Complications included 1 perioperative stroke, 1 symptomatic subdural hygroma, 1 postoperative seizure, and 1 case of intraoperative cerebral edema that required subsequent cranioplasty. Before EDAS or EMAS, the stroke rate was calculated to be 1 stroke per 7.8 patient-years. One additional stroke occurred during the follow-up period (mean follow-up time 33.7 \u00b1 19.6 months), resulting in a post-EDAS\/EMAS stroke rate of 1 stroke per 39.3 patient-years, a 5-fold reduction compared with that in the pre-EDAS\/EMAS period. The patients\u2019 mean pre-EDAS\/EMAS HbS level of 29.5% \u00b1 6.4% was comparable to the mean post-EDAS\/EMAS HbS level of 25.5% \u00b1 6.1% (p = 0.104).<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>CONCLUSIONS<\/jats:title>\n<jats:p>The results of this retrospective case series in a large cohort of pediatric patients with SCD and MMS suggest that EDAS\/EMAS provides a stroke-prevention benefit with an acceptably low morbidity rate. Given the combined experience with EDAS and EMAS for this indication at this and other institutions, a prospective clinical trial to assess their efficacy compared with that of chronic transfusion therapy alone is warranted.<\/jats:p><\/jats:sec>","DOI":"10.3171\/2014.12.peds14522","type":"journal-article","created":{"date-parts":[[2015,4,3]],"date-time":"2015-04-03T12:57:21Z","timestamp":1428065841000},"page":"64-73","source":"Crossref","is-referenced-by-count":41,"title":["Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease"],"prefix":"10.3171","volume":"16","author":[{"given":"Christoph J.","family":"Griessenauer","sequence":"first","affiliation":[{"name":"Departments of Neurosurgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey D.","family":"Lebensburger","sequence":"additional","affiliation":[{"name":"Pediatric Hematology Oncology, University of Alabama at Birmingham, Alabama; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle H.","family":"Chua","sequence":"additional","affiliation":[{"name":"Harvard Medical School, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Winfield S.","family":"Fisher","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lee","family":"Hilliard","sequence":"additional","affiliation":[{"name":"Pediatric Hematology Oncology, University of Alabama at Birmingham, Alabama; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina J.","family":"Bemrich-Stolz","sequence":"additional","affiliation":[{"name":"Pediatric Hematology Oncology, University of Alabama at Birmingham, Alabama; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas H.","family":"Howard","sequence":"additional","affiliation":[{"name":"Pediatric Hematology Oncology, University of Alabama at Birmingham, Alabama; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James M.","family":"Johnston","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-peds14522","series-title":"Control Clin Trials","doi-asserted-by":"publisher","first-page":"110","DOI":"10.1016\/S0197-2456(97)00099-8","article-title":"Stroke prevention trial in sickle cell anemia","volume":"19","author":"Adams","year":"1998"},{"key":"b2-peds14522","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-peds14522","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":"b4-peds14522","series-title":"Pediatr Neurosurg","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1159\/000120932","article-title":"Pial synangiosis for moyamoya syndrome in children","volume":"23","author":"Adelson","year":"1995"},{"key":"b5-peds14522","series-title":"Adv Med Sci","doi-asserted-by":"publisher","first-page":"108","DOI":"10.1016\/j.advms.2013.09.001","article-title":"Sickle cell anemia: intracranial stenosis and silent cerebral infarcts in children with low risk of stroke","volume":"59","author":"Arkuszewski","year":"2014"},{"key":"b6-peds14522","series-title":"Br J Haematol","doi-asserted-by":"publisher","first-page":"524","DOI":"10.1111\/j.1365-2141.2009.07630.x","article-title":"Chronic transfusion practice for children with sickle cell anaemia and stroke","volume":"145","author":"Aygun","year":"2009"},{"key":"b7-peds14522","series-title":"Blood Cells Mol Dis","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1016\/j.bcmd.2011.06.002","article-title":"Effect of chronic transfusion therapy on progression of neurovascular pathology in pediatric patients with sickle cell anemia","volume":"47","author":"Bishop","year":"2011"},{"key":"b8-peds14522","series-title":"Ann Hematol","doi-asserted-by":"publisher","first-page":"785","DOI":"10.1007\/s00277-008-0670-x","article-title":"Does regular blood transfusion prevent progression of cerebrovascular lesions in children with sickle cell disease?","volume":"88","author":"Brousse","year":"2009"},{"key":"b9-peds14522","series-title":"Sickle Cell Disease (SCD): Data & Statistics","author":"Centers for Disease Control and Prevention"},{"key":"b10-peds14522","series-title":"Health Technol Assess","first-page":"1","article-title":"The clinical effectiveness and cost-effectiveness of primary stroke prevention in children with sickle cell disease: a systematic review and economic evaluation","volume":"16","author":"Cherry","year":"2012"},{"key":"b11-peds14522","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"719","DOI":"10.3171\/jns.1997.86.4.0719","article-title":"Dural inversion procedure for moyamoya disease. Technical note","volume":"86","author":"Dauser","year":"1997"},{"key":"b12-peds14522","series-title":"N Engl J Med","doi-asserted-by":"publisher","first-page":"699","DOI":"10.1056\/NEJMoa1401731","article-title":"Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia","volume":"371","author":"DeBaun","year":"2014"},{"key":"b13-peds14522","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":"b14-peds14522","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1227\/NEU.0b013e3181fc5ec2","article-title":"Clinical and angiographic outcomes from indirect revascularization surgery for Moyamoya disease in adults and children: a review of 63 procedures","volume":"68","author":"Dusick","year":"2011"},{"key":"b15-peds14522","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":"b16-peds14522","series-title":"Neurosurgery","first-page":"957","article-title":"Significance of focal cerebral hyperperfusion as a cause of transient neurologic deterioration after extracranial-intracranial bypass for moyamoya disease: comparative study with non-moyamoya patients using N-isopropyl-p-[(123)I]iodoamphetamine single-photon emission computed tomography","volume":"68","author":"Fujimura","year":"2011"},{"key":"b17-peds14522","series-title":"J Neurosurg Pediatr","doi-asserted-by":"publisher","first-page":"211","DOI":"10.3171\/PED\/2008\/1\/3\/211","article-title":"Surgical treatment of moyamoya syndrome in patients with sickle cell anemia: outcome following encephaloduroarteriosynangiosis","volume":"1","author":"Hankinson","year":"2008"},{"key":"b18-peds14522","series-title":"Childs Nerv Syst","first-page":"734","article-title":"How does angiogenesis develop in pediatric moyamoya disease after surgery? A prospective study with MR angiography","volume":"20","author":"Houkin","year":"2004"},{"key":"b19-peds14522","series-title":"Blood","doi-asserted-by":"publisher","first-page":"772","DOI":"10.1182\/blood-2010-01-261123","article-title":"Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle cell disease","volume":"117","author":"Hulbert","year":"2011"},{"key":"b20-peds14522","series-title":"J Pediatr Neurosci","doi-asserted-by":"publisher","first-page":"115","DOI":"10.4103\/1817-1745.76102","article-title":"Multiple burr hole surgery as a treatment modality for pediatric moyamoya disease","volume":"5","author":"Kapu","year":"2010"},{"issue":"1","key":"b21-peds14522","series-title":"Neurosurg Focus","doi-asserted-by":"publisher","first-page":"E12","DOI":"10.3171\/2013.10.FOCUS13405","article-title":"Pial synangiosis for moyamoya syndrome in children with sickle cell anemia: a comprehensive review of reported cases","volume":"36","author":"Kennedy","year":"2014"},{"key":"b22-peds14522","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":"b23-peds14522","series-title":"Br J Haematol","doi-asserted-by":"publisher","first-page":"707","DOI":"10.1111\/bjh.12795","article-title":"Outcome of overt stroke in sickle cell anaemia, a single institution\u2019s experience","volume":"165","author":"Majumdar","year":"2014"},{"key":"b24-peds14522","series-title":"Surg Neurol","doi-asserted-by":"publisher","first-page":"313","DOI":"10.1016\/S0090-3019(81)80017-1","article-title":"A new surgical treatment of moyamoya disease in children: a preliminary report","volume":"15","author":"Matsushima","year":"1981"},{"issue":"Suppl","key":"b25-peds14522","series-title":"Neurol Med Chir (Tokyo)","doi-asserted-by":"publisher","first-page":"297","DOI":"10.2176\/nmc.38.suppl_297","article-title":"An indirect revascularization method in the surgical treatment of moyamoya disease\u2014various kinds of indirect procedures and a multiple combined indirect procedure","volume":"38","author":"Matsushima","year":"1998"},{"key":"b26-peds14522","series-title":"Pediatr Blood Cancer","doi-asserted-by":"publisher","first-page":"823","DOI":"10.1002\/pbc.24392","article-title":"National trends in incidence rates of hospitalization for stroke in children with sickle cell disease","volume":"60","author":"McCavit","year":"2013"},{"key":"b27-peds14522","series-title":"Radiology","doi-asserted-by":"publisher","first-page":"311","DOI":"10.1148\/radiology.206.2.9457180","article-title":"Sickle cell disease: imaging of cerebrovascular complications","volume":"206","author":"Moran","year":"1998"},{"key":"b28-peds14522","series-title":"Clin Imaging","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1016\/S0899-7071(03)00121-9","article-title":"Sickle cell cerebrovascular disease: usual and unusual findings on MR imaging and MR angiography","volume":"28","author":"Moritani","year":"2004"},{"key":"b29-peds14522","series-title":"Blood","first-page":"288","article-title":"Cerebrovascular accidents in sickle cell disease: rates and risk factors","volume":"91","author":"Ohene-Frempong","year":"1998"},{"key":"b30-peds14522","series-title":"J Pediatr","doi-asserted-by":"publisher","first-page":"896","DOI":"10.1016\/S0022-3476(95)70204-0","article-title":"Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions","volume":"126","author":"Pegelow","year":"1995"},{"key":"b31-peds14522","series-title":"Blood","doi-asserted-by":"publisher","first-page":"162","DOI":"10.1182\/blood.V63.1.162.162","article-title":"Effect of transfusion therapy on arteriographic abnormalities and on recurrence of stroke in sickle cell disease","volume":"63","author":"Russell","year":"1984"},{"issue":"4","key":"b32-peds14522","series-title":"Neurosurg Focus","doi-asserted-by":"publisher","first-page":"E10","DOI":"10.3171\/2009.01.FOCUS08307","article-title":"Pial synangiosis in patients with moyamoya syndrome and sickle cell anemia: perioperative management and surgical outcome","volume":"26","author":"Smith","year":"2009"},{"key":"b33-peds14522","series-title":"Arch Neurol","doi-asserted-by":"publisher","first-page":"288","DOI":"10.1001\/archneur.1969.00480090076012","article-title":"Cerebrovascular \u201cmoyamoya\u201d disease. Disease showing abnormal net-like vessels in base of brain","volume":"20","author":"Suzuki","year":"1969"},{"key":"b34-peds14522","series-title":"Pediatr Neurol","doi-asserted-by":"publisher","first-page":"226","DOI":"10.1016\/0887-8994(96)00045-8","article-title":"Encephaloduroarterio-synangiosis in a child with sickle cell anemia and moyamoya disease","volume":"14","author":"Vernet","year":"1996"},{"key":"b35-peds14522","series-title":"Blood","doi-asserted-by":"publisher","first-page":"3925","DOI":"10.1182\/blood-2011-11-392340","article-title":"Stroke With Transfusions Changing to Hydroxyurea (SWiTCH)","volume":"119","author":"Ware","year":"2012"}],"container-title":["Journal of Neurosurgery: Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/16\/1\/article-p64.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/16\/1\/article-p64.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/16\/1\/article-p64.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,15]],"date-time":"2023-04-15T11:55:53Z","timestamp":1681559753000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/16\/1\/article-p64.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,7]]},"references-count":35,"journal-issue":{"issue":"1"},"URL":"https:\/\/doi.org\/10.3171\/2014.12.peds14522","relation":{},"ISSN":["1933-0707","1933-0715"],"issn-type":[{"value":"1933-0707","type":"print"},{"value":"1933-0715","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,7]]}}}