{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T08:08:19Z","timestamp":1791101299547,"version":"4.1.0"},"reference-count":52,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["FOC"],"published-print":{"date-parts":[[2009,4]]},"abstract":"<jats:sec>\n                    <jats:title>Object<\/jats:title>\n                    <jats:p>Many children with sickle cell anemia (SCA) also have clinical and radiographic findings of an arteriopathy suggestive of moyamoya syndrome. These patients may continue to experience strokes despite optimal medical management. The authors wished to define features of moyamoya syndrome associated with SCA and determine the results of surgical revascularization in these patients at early and late follow-up.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The authors reviewed the clinical and radiographic records of all patients with moyamoya syndrome and SCA who underwent cerebral revascularization surgery using a standardized surgical procedure\u2014pial synangiosis\u2014from 1985 to 2008.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Twelve patients had SCA and moyamoya syndrome. Six patients were female and 6 were male. The average patient age at surgery was 11.3 years (range 3\u201322 years). All patients presented with ischemic symptoms, 11 (92%) with previous transient ischemic attacks, and 10 (83%) with completed strokes. Eleven patients (92%) had radiographic evidence of previous stroke at presentation. None presented with hemorrhage. Surgical treatment included pial synangiosis in all patients. Complications included 1 perioperative stroke, 1 wound infection, and 1 perioperative pneumonia. The average length of hospital stay was 5.7 days (including a 24-hour preoperative admission for hydration) and average blood loss was 92.5 ml\/hemisphere (in a total of 19 hemispheres). Clinical and radiographic follow-up with an average of 49 months (range 9\u2013144 months) demonstrated no worsening in neurological status in any patient. No clinical or radiographic evidence of new infarcts was observed in any patient at late follow-up, despite disease progression in 13 (68%) of 19 hemispheres.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The clinical and radiographic features of moyamoya syndrome associated with SCA appear comparable to primary moyamoya disease. Successful treatment of these patients requires multidisciplinary care involving hematologists, anesthesiologists, and neurosurgeons. Operative treatment of moyamoya syndrome using pial synangiosis appears to be safe and confers long-lasting protection against further stroke in this population, and provides an alternative for failure of optimal medical therapy in patients. This study underscores the potential merit of screening patients with SCA for moyamoya syndrome.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/2009.01.focus08307","type":"journal-article","created":{"date-parts":[[2009,3,31]],"date-time":"2009-03-31T12:12:42Z","timestamp":1238501562000},"page":"E10","source":"Crossref","is-referenced-by-count":72,"title":["Pial synangiosis in patients with moyamoya syndrome and sickle cell anemia: perioperative management and surgical outcome"],"prefix":"10.3171","volume":"26","author":[{"given":"Edward R.","family":"Smith","sequence":"first","affiliation":[{"name":"Departments of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Craig D.","family":"McClain","sequence":"additional","affiliation":[{"name":"Anesthesiology, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew","family":"Heeney","sequence":"additional","affiliation":[{"name":"Hematology\/Oncology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. Michael","family":"Scott","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-0260010","series-title":"Control Clin Trials","first-page":"110","article-title":"Stroke prevention trial in sickle cell anemia","volume":"19","author":"Adams","year":"1998"},{"key":"b2-0260010","series-title":"Ann Neurol","first-page":"699","article-title":"Long-term stroke risk in children with sickle cell disease screened with transcranial Doppler","volume":"42","author":"Adams","year":"1997"},{"key":"b3-0260010","series-title":"N Engl J Med","first-page":"5","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":"b4-0260010","series-title":"Blood","first-page":"3144","article-title":"Moyamoya syndrome in childhood sickle cell disease: a predictive factor for recurrent cerebrovascular events","volume":"99","author":"Dobson","year":"2002"},{"key":"b5-0260010","series-title":"Radiographics","first-page":"483","article-title":"General case of the day. Moyamoya syndrome in a child with sickle cell disease","volume":"13","author":"Drew","year":"1993"},{"key":"b6-0260010","series-title":"Br J Anaesth","first-page":"287","article-title":"Anaesthesia for peculiar cells\u2013a century of sickle cell disease","volume":"95","author":"Firth","year":"2005"},{"key":"b7-0260010","series-title":"Anesthesiology","first-page":"766","article-title":"Sickle cell disease and anesthesia","volume":"101","author":"Firth","year":"2004"},{"key":"b8-0260010","series-title":"Pediatr Neurol","first-page":"124","article-title":"Sickle cell anemia with moyamoya disease: outcomes after EDAS procedure","volume":"29","author":"Fryer","year":"2003"},{"key":"b9-0260010","series-title":"Surg Neurol","first-page":"425","article-title":"Pseudomoyamoya in sickle cell anemia","volume":"18","author":"Garza-Mercado","year":"1982"},{"key":"b10-0260010","series-title":"J Neurosurg Pediatrics","first-page":"211","article-title":"Surgical treatment of moyamoya syndrome in patients with sickle cell anemia: outcome following encephaloduroarteriosynangiosis","volume":"1","author":"Hankinson","year":"2008"},{"key":"b11-0260010","series-title":"Curr Treat Options Neurol","first-page":"357","article-title":"Stroke in children with sickle cell disease","volume":"6","author":"Kirkham","year":"2004"},{"key":"b12-0260010","series-title":"CNS Spectr","first-page":"451","article-title":"Risk factors for arterial ischemic stroke in childhood","volume":"9","author":"Kirkham","year":"2004"},{"key":"b13-0260010","series-title":"Pediatr Radiol","first-page":"409","article-title":"MR angiography in pediatric neurological disorders","volume":"25","author":"Lee","year":"1995"},{"key":"b14-0260010","series-title":"Neurosurgery","first-page":"401","article-title":"Surgical treatment of moyamoya disease in pediatric patients\u2013comparison between the results of indirect and direct revascularization procedures","volume":"31","author":"Matsushima","year":"1992"},{"key":"b15-0260010","series-title":"Stroke","first-page":"45","article-title":"Cerebrovascular disease in sickle cell anemia: a clinical, pathological and radiological correlation","volume":"9","author":"Merkel","year":"1978"},{"key":"b16-0260010","series-title":"J Pediatr","first-page":"896","article-title":"Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions","volume":"126","author":"Pegelow","year":"1995"},{"key":"b17-0260010","series-title":"Stroke","first-page":"2644","article-title":"Management of stroke in infants and children: a scientific statement from a Special Writing Group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young","volume":"39","author":"Roach","year":"2008"},{"key":"b18-0260010","series-title":"J Neurosurg Anesthesiol","first-page":"37","article-title":"Postoperative neurological deterioration following the revascularization surgery in children with moyamoya disease","volume":"10","author":"Sakamoto","year":"1998"},{"key":"b19-0260010","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":"b20-0260010","series-title":"Curr Opin Pediatr","first-page":"657","article-title":"Pediatric stroke: current developments","volume":"19","author":"Seidman","year":"2007"},{"key":"b21-0260010","series-title":"Anesthesiology","first-page":"771","article-title":"Sickle-cell disease: an anesthesiological problem","volume":"16","author":"Shapiro","year":"1955"},{"issue":"2","key":"b22-0260010","series-title":"Neurosurg Focus","first-page":"E17","article-title":"Progression of disease in unilateral moyamoya syndrome","volume":"24","author":"Smith","year":"2008"},{"key":"b23-0260010","series-title":"Skull 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