{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:02:57Z","timestamp":1791295377666,"version":"4.1.0"},"reference-count":60,"publisher":"American Academy of Pediatrics (AAP)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2005,11,1]]},"abstract":"<jats:p>Objectives. This study was undertaken to describe the clinical, radiologic, and angiographic features of moyamoya syndrome in a surgical series of children and adults with Down syndrome. We wished to define the features of moyamoya syndrome associated with Down syndrome and to determine the results of surgical revascularization among these patients at early and late follow-up times.<\/jats:p>\n               <jats:p>Methods. We reviewed the clinical, radiologic, and angiographic records of all patients with moyamoya syndrome associated with Down syndrome, as a subset of a previously reported, consecutive series of patients who underwent cerebral revascularization surgery with a standardized surgical procedure, pial synangiosis, between January 1, 1985, and June 30, 2004.<\/jats:p>\n               <jats:p>Results. Of 181 patients with moyamoya syndrome from the initial series who were treated surgically during the study period, 16 patients had Down syndrome (10 female patients and 6 male patients). The average age at onset was 9.3 years (range: 1\u201329 years); the average age at the time of surgery was 9.8 years (range: 2\u201329 years). Although the presenting symptoms were transient ischemic attacks for 10 patients and strokes for 6 patients, computed tomographic and\/or MRI scans demonstrated bilateral infarctions for 9 patients and unilateral infarctions for 6, with only 1 patient having no imaging evidence of a previous stroke. No cases presented with intracerebral hemorrhage. Preoperative angiography showed the presence of bilateral moyamoya syndrome changes for all patients, including posterior circulation involvement for 8 patients. Surgical treatment included pial synangiosis for all patients, although 1 patient underwent a superficial temporal artery-middle cerebral artery bypass in the contralateral hemisphere. Surgical complications included symptomatic subdural hematomas requiring evacuation, at 48 days and 54 days postoperatively (2 cases), seizures (2 cases), and strokes within 30 days after surgery, at 1 day and 7 days postoperatively (2 cases). Late clinical and radiologic follow-up data (average: 67.6 months; range: 6\u2013146 months) demonstrated no worsening in neurologic status for any patient except for 1 patient who developed a seizure disorder with associated chronic hypocalcemia; she was totally dependent at the 10-year follow-up evaluation, despite no evidence of new infarction since her surgery. There was no clinical or radiologic evidence of new infarction for any patient in late follow-up evaluations. Postoperative angiography, conducted 1 year after surgery for 11 patients, revealed radiologic evidence of good to excellent cerebral revascularization in 85% of the surgically treated hemispheres. Patients were maintained on lifetime aspirin therapy.<\/jats:p>\n               <jats:p>Conclusions. The clinical, radiologic, and angiographic features of moyamoya syndrome associated with Down syndrome seem comparable to those of primary moyamoya disease. Cerebral revascularization surgery with the pial synangiosis technique seems to confer long-lasting protection against additional strokes in this patient population. The presence of moyamoya syndrome should be considered in the evaluation of patients with Down syndrome who present with transient ischemic attack-like symptoms.<\/jats:p>","DOI":"10.1542\/peds.2005-0568","type":"journal-article","created":{"date-parts":[[2005,11,1]],"date-time":"2005-11-01T18:08:50Z","timestamp":1130868530000},"page":"e694-e701","source":"Crossref","is-referenced-by-count":116,"title":["Moyamoya Syndrome Associated With Down Syndrome: Outcome After Surgical Revascularization"],"prefix":"10.1542","volume":"116","author":[{"given":"Andrew","family":"Jea","sequence":"first","affiliation":[{"name":"Departments of Neurosurgery"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward R.","family":"Smith","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Robertson","sequence":"additional","affiliation":[{"name":"Radiology, Children\u2019s 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":"417","published-online":{"date-parts":[[2005,11,1]]},"reference":[{"key":"2021120819494023000_R1","doi-asserted-by":"crossref","unstructured":"Scott RM, Smith JL, Robertson RL, Madsen JR, Soriano SG, Rockoff MA. Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial syangiosis. J Neurosurg.\u20082004;100:142\u2013149","DOI":"10.3171\/ped.2004.100.2.0142"},{"key":"2021120819494023000_R2","doi-asserted-by":"crossref","unstructured":"Matsushima Y, Inaha Y. Moyamoya disease in children and its surgical treatment: the introduction of a new surgical procedure and its follow-up angiograms. Childs Brain.\u20081984;11:155\u2013170","DOI":"10.1159\/000120172"},{"key":"2021120819494023000_R3","doi-asserted-by":"crossref","unstructured":"Adelson PD, Scott RM. Pial synangiosis for moyamoya syndrome in children. Pediatr Neurosurg.\u20081995;23:26\u201333","DOI":"10.1159\/000120932"},{"key":"2021120819494023000_R4","unstructured":"Takeuchi K, Shimizu K. Hypoplasia of the bilateral internal carotid arteries. Brain Nerve.\u20081957;9:37\u201343"},{"key":"2021120819494023000_R5","doi-asserted-by":"crossref","unstructured":"Suzuki J, Takaku A. Cerebrovascular \u201cmoyamoya\u201d disease: a disease showing abnormal net-like vessels in base of brain. Arch Neurol.\u20081969;20:288\u2013299","DOI":"10.1001\/archneur.1969.00480090076012"},{"key":"2021120819494023000_R6","doi-asserted-by":"crossref","unstructured":"Matsushima T, Inoue T, Natori Y. Children with unilateral occlusion or stenosis of the ICA associated with surrounding moyamoya vessels: \u201cunilateral\u201d moyamoya disease. Acta Neurochir (Wien).\u20081994;131:196\u2013202","DOI":"10.1007\/BF01808612"},{"key":"2021120819494023000_R7","doi-asserted-by":"crossref","unstructured":"Olds MV, Griebel RW, Hoffman HJ, Craven M, Chuang S, Schutz H. The surgical treatment of childhood moyamoya disease. J Neurosurg.\u20081987;66:675\u2013680","DOI":"10.3171\/jns.1987.66.5.0675"},{"key":"2021120819494023000_R8","unstructured":"Arevalo CE, Marchezzotti A, Haessler A, Lanosa R. Moyamoya disease and Down syndrome [in Spanish]. Medicina (B Aires).\u20081993;53:473"},{"key":"2021120819494023000_R9","doi-asserted-by":"crossref","unstructured":"Aylett SE, Britton JA, De Sousa CM. Down syndrome and moyamoya disease: presentation with subarachnoid hemorrhage. Pediatr Neurol.\u20081996;14:259\u2013261","DOI":"10.1016\/0887-8994(96)00022-7"},{"key":"2021120819494023000_R10","doi-asserted-by":"crossref","unstructured":"Berg JM, Armstrong D. On the association of moyamoya disease with Down syndrome. J Ment Defic Res.\u20081991;35:398\u2013403","DOI":"10.1111\/j.1365-2788.1991.tb00412.x"},{"key":"2021120819494023000_R11","doi-asserted-by":"crossref","unstructured":"Bitzer M, Topka H. Progressive cerebral occlusive disease after radiation therapy. Stroke.\u20081995;26:131\u2013136","DOI":"10.1161\/01.STR.26.1.131"},{"key":"2021120819494023000_R12","unstructured":"Cornelio-Nieto JO, Davila-Gutierrez G, Ferreyro-Irigoyen R, Alcala H. Acute hemiplegia in childhood and alternating hemiconvulsions secondary to moya-moya disease: report of a case associated with Down\u2019s syndrome [in Spanish]. Bol Med Hosp Infant Mex.\u20081990;47:39\u201342"},{"key":"2021120819494023000_R13","doi-asserted-by":"crossref","unstructured":"Cramer SC, Robertson RL, Dooling EC, Scott RM. Moyamoya and Down syndrome: clinical and radiological features. Stroke.\u20081996;27:2131\u20132135","DOI":"10.1161\/01.STR.27.11.2131"},{"key":"2021120819494023000_R14","doi-asserted-by":"crossref","unstructured":"Dai AI, Shaikh ZA, Cohen ME. Early-onset moyamoya syndrome in a patient with Down syndrome: case report and review of the literature. J Child Neurol.\u20082000;15:696\u2013699","DOI":"10.1177\/088307380001501012"},{"key":"2021120819494023000_R15","unstructured":"de Borchgrave V, Saussu F, Depre A, de Barsy T. Moyamoya disease and Down syndrome: case report and review of the literature. Acta Neurol Belg.\u20082002;102:63\u201366"},{"key":"2021120819494023000_R16","doi-asserted-by":"crossref","unstructured":"Fukushima Y, Kondo Y, Kuroki Y. Are Down syndrome patients predisposed to moyamoya disease?Eur J Pediatr.\u20081986;144:516\u2013517","DOI":"10.1007\/BF00441756"},{"key":"2021120819494023000_R17","doi-asserted-by":"crossref","unstructured":"Fukuyama Y, Osawa M, Kanai N. Moyamoya disease (syndrome) and the Down syndrome. Brain Dev.\u20081992;14:254\u2013256","DOI":"10.1016\/S0387-7604(12)80242-7"},{"key":"2021120819494023000_R18","unstructured":"Fung CW, Kwong KL, Tsui EY, Wong SN. Moyamoya syndrome in a child with Down syndrome. Hong Kong Med J.\u20082003;9:63\u201366"},{"key":"2021120819494023000_R19","doi-asserted-by":"crossref","unstructured":"Gaggero R, Donati PT, Curia R, De Negri M. Occlusion of unilateral carotid artery in Down syndrome. Brain Dev.\u20081996;18:81\u201383","DOI":"10.1016\/0387-7604(95)00114-X"},{"key":"2021120819494023000_R20","doi-asserted-by":"crossref","unstructured":"Goldstein EM, Singer HM. Moyamoya-like disease in Down\u2019s syndrome. Pediatr Neurosurg.\u20081990-1991;16:14\u201316","DOI":"10.1159\/000120496"},{"key":"2021120819494023000_R21","doi-asserted-by":"crossref","unstructured":"Gordon N, Isler W. Childhood moyamoya disease. Dev Med Child Neurol.\u20081989;31:103\u2013107","DOI":"10.1111\/j.1469-8749.1989.tb08418.x"},{"key":"2021120819494023000_R22","unstructured":"Ichiba N, Murakawa S, Ohtahara S. A case of Down syndrome with \u201cmoyamoya\u201d disease: a consideration on fibromuscular dysplasia [in Japanese]. No To Hattatsu.\u20081984;16:487\u2013491"},{"key":"2021120819494023000_R23","unstructured":"Inoue R, Katayama S, Kasai N, Hori S. Middle cerebral artery occlusion with unilateral moyamoya like vessels and with ruptured anterior cerebral artery aneurysm: its relationship to the antiphospholipid syndrome [in Japanese]. No To Shinkei.\u20081994;46:995\u2013998"},{"key":"2021120819494023000_R24","unstructured":"Inoue T, Matsushima T, Fujii K, Fukui M, Hasuo K, Matsuo H. Akin moyamoya disease in children [in Japanese]. No Shinkei Geka.\u20081993;21:59\u201365"},{"key":"2021120819494023000_R25","doi-asserted-by":"crossref","unstructured":"Junqueira PA, Moura-Ribeiro MV. Moyamoya and Down syndrome: study conducted by meta-analysis [in Portuguese]. Arq Neuropsiquiatr.\u20082002;60:274\u2013280","DOI":"10.1590\/S0004-282X2002000200017"},{"key":"2021120819494023000_R26","doi-asserted-by":"crossref","unstructured":"Kestle J, Hoffman H, Mock A. Moyamoya phenomenon after radiation for optic glioma. J Neurosurg.\u20081993;72:32\u201335","DOI":"10.3171\/jns.1993.79.1.0032"},{"key":"2021120819494023000_R27","doi-asserted-by":"crossref","unstructured":"Kitahara T, Okumura K, Semba A, Yamaura A, Makino H. Genetic and immunologic analysis on moya-moya. J Neurol Neurosurg Psychiatry.\u20081982;45:1048\u20131052","DOI":"10.1136\/jnnp.45.11.1048"},{"key":"2021120819494023000_R28","doi-asserted-by":"crossref","unstructured":"Kushima K, Satoh Y, Ban Y, et al. Graves thyrotoxicosis and moyamoya disease. Can J Neurol Sci.\u20081991;18:140\u2013142","DOI":"10.1017\/S0317167100031607"},{"key":"2021120819494023000_R29","doi-asserted-by":"crossref","unstructured":"Leno C, Mateo I, Cid C, Berciano J, Sedano C. Autoimmunity in Down syndrome: another possible mechanism of moyamoya disease. Stroke.\u20081998;29:868\u2013869","DOI":"10.1161\/01.STR.29.4.868"},{"key":"2021120819494023000_R30","doi-asserted-by":"crossref","unstructured":"Mito T, Becker LE. Vascular dysplasia in Down syndrome: a possible relationship to moyamoya disease. Brain Dev.\u20081992;14:248\u2013251","DOI":"10.1016\/S0387-7604(12)80239-7"},{"key":"2021120819494023000_R31","unstructured":"Nagaska T, Shiozawa Z, Kobayashi M, Shindo K, Tsunoda S, Amino A. Autopsy findings in Down syndrome with cerebrovascular disorder. Clin Neuropathol.\u20081996;15:145\u2013149"},{"key":"2021120819494023000_R32","unstructured":"Nishimura M, Takakura H, Ieshima A, Eda I, Ohno K, Takashima S. A case of Down syndrome with moyamoya disease [in Japanese]. No To Hattatsu.\u20081985;17:71\u201375"},{"key":"2021120819494023000_R33","doi-asserted-by":"crossref","unstructured":"Okuno T, Prensky A, Gado M. The moyamoya syndrome association with irradiation of an optic glioma in children: report of two cases and review of the literature. Pediatr Neurol.\u20081985;1:311\u2013316","DOI":"10.1016\/0887-8994(85)90036-0"},{"key":"2021120819494023000_R34","unstructured":"Outwater EK, Platenberg RC, Wolpert SM. Moyamoya disease in Down syndrome. AJNR Am J Neuroradiol.\u20081989;10(suppl):S23\u2013S24"},{"key":"2021120819494023000_R35","doi-asserted-by":"crossref","unstructured":"Park M, Raila FA, Russell WF. Moyamoya disease in an adult with Down syndrome: comparison of magnetic resonance angiography and conventional angiography. South Med J.\u20081996;89:89\u201392","DOI":"10.1097\/00007611-199601000-00018"},{"key":"2021120819494023000_R36","doi-asserted-by":"crossref","unstructured":"Pearson E, Lenn NJ, Cail WS. Moyamoya and other causes of stroke in patients with Down syndrome. Pediatr Neurol.\u20081985;1:174\u2013179","DOI":"10.1016\/0887-8994(85)90060-8"},{"key":"2021120819494023000_R37","doi-asserted-by":"crossref","unstructured":"Rajakulasingam K, Cerullo L, Raimondi A. Childhood moyamoya syndrome: postirradiation pathogenesis. Childs Brain.\u20081975;5:467\u2013475","DOI":"10.1159\/000119842"},{"key":"2021120819494023000_R38","doi-asserted-by":"crossref","unstructured":"Schulz IS, Jahara F, Pompeu F, Figueira F, de Oliveira MI. Progressive occlusive disease of the cerebral arteries associated with Down syndrome: considerations on the etiopathogenesis [in Portuguese]. Arq Neuropsiquiatr.\u20081981;39:237\u2013243","DOI":"10.1590\/S0004-282X1981000200013"},{"key":"2021120819494023000_R39","unstructured":"Soto-Ares G, Hamon-Kerautret M, Leclerc X, Vallee L, Pruvo JP. Moyamoya associated with Down syndrome. J Radiol.\u20081996;77:441\u2013444"},{"key":"2021120819494023000_R40","unstructured":"Suzuki J. Moyamoya Disease. New York, NY: Springer; 1983:131\u2013143"},{"key":"2021120819494023000_R41","doi-asserted-by":"crossref","unstructured":"Takanashi J, Sugita K, Honda A, Niimi H. Moyamoya syndrome in a patient with Down syndrome presenting with chorea. Pediatr Neurol.\u20081993;9:396\u2013398","DOI":"10.1016\/0887-8994(93)90111-O"},{"key":"2021120819494023000_R42","unstructured":"Takanashi J, Sugita K, Ishii M. A girl with Down syndrome complicated by moyamoya disease and symptomatic atlanto-axial instability [in Japanese]. No To Hattatsu.\u20081993;25:248\u2013252"},{"key":"2021120819494023000_R43","unstructured":"Takasugi H, Maemoto T, Kitazawa K, Honda A. A case of Down syndrome with moyamoya syndrome presenting extensive multiple cerebral infarction during measles infection [in Japanese]. No To Hattatsu.\u20082000;32:39\u201343"},{"key":"2021120819494023000_R44","doi-asserted-by":"crossref","unstructured":"van Erven PM, Gabreels FJ, Thijssen HO, Renier WO. The moya-moya syndrome: a report of two children. Clin Neurol Neurosurg.\u20081982;84:179\u2013184","DOI":"10.1016\/0303-8467(82)90041-5"},{"key":"2021120819494023000_R45","doi-asserted-by":"crossref","unstructured":"Vicari S, Albertini G. Moyamoya disease in Down syndrome: a report of two cases. J Ment Defic Res.\u20081991;35:392\u2013397","DOI":"10.1111\/j.1365-2788.1991.tb00411.x"},{"key":"2021120819494023000_R46","unstructured":"Wanifuchi H, Kagawa M, Takeshita M, et al. Autoimmune antibody in moyamoya disease [in Japanese]. No Shinkei Geka.\u20081986;14:31\u201335"},{"key":"2021120819494023000_R47","doi-asserted-by":"crossref","unstructured":"Matsushima Y, Aoyagi M, Niimi Y, et al. Symptoms and their pattern of progression in childhood moyamoya disease. Brain Dev.\u20081990;12:784\u2013789","DOI":"10.1016\/S0387-7604(12)80007-6"},{"key":"2021120819494023000_R48","doi-asserted-by":"crossref","unstructured":"Gordon N. Alternating hemiplegia of childhood. Dev Med Child Neurol.\u20081995;37:464\u2013468","DOI":"10.1111\/j.1469-8749.1995.tb12030.x"},{"key":"2021120819494023000_R49","unstructured":"Ohaegbulam C, Magge S, Scott RM. Moyamoya syndrome. In: McLone DG, ed. Pediatric Neurosurgery: Surgery of the Developing Nervous System. New York, NY: WB Saunders; 2001:1077\u20131092"},{"key":"2021120819494023000_R50","doi-asserted-by":"crossref","unstructured":"Imaizumi F, Hayashi K, Saito K, Osawa M, Fukuyama Y. Long-term outcomes of pediatric moyamoya disease monitored to adulthood. Pediatr Neurol.\u20081998;18:321\u2013325","DOI":"10.1016\/S0887-8994(97)00209-9"},{"key":"2021120819494023000_R51","unstructured":"Yonekawa Y, Goto Y, Ogata N. Moyamoya disease: diagnosis, treatment, and recent achievement. In: Barnett HJM, Mohr JP, Stein BM, Yatsu FM, eds. Stroke: Pathophysiology, Diagnosis, and Management. New York, NY: Churchill Livingstone; 1992:721\u2013748"},{"key":"2021120819494023000_R52","doi-asserted-by":"crossref","unstructured":"Nishimoto A. Moyamoya disease. Neurol Med Chir.\u20081979;19:221\u2013228","DOI":"10.2176\/nmc.19.221"},{"key":"2021120819494023000_R53","doi-asserted-by":"crossref","unstructured":"Matsushima Y, Fukai N, Tanaka K. A new surgical treatment of moyamoya disease in children: a preliminary report. Surg Neurol.\u20081981;15:313","DOI":"10.1016\/S0090-3019(81)80017-1"},{"key":"2021120819494023000_R54","unstructured":"Moritake K, Handa H, Yonekawa Y, Taki W, Okuno T. Follow-up study on the relationship between age at onset of illness and outcome in patients with \u201cmoyamoya disease.\u201d Neurol Surg.\u20081986;14:957\u2013963"},{"key":"2021120819494023000_R55","doi-asserted-by":"crossref","unstructured":"Gadoth N. On the problem of essential and secondary moyamoya and vascular dysplasia in Down syndrome. Brain Dev.\u20081993;15:317\u2013318","DOI":"10.1016\/0387-7604(93)90034-6"},{"key":"2021120819494023000_R56","doi-asserted-by":"crossref","unstructured":"Jansen JN, Donker AJ, Luth WJ, Smit LM. Moyamoya disease associated with renovascular hypertension. Neuropediatrics.\u20081990;21:44\u201347","DOI":"10.1055\/s-2008-1071457"},{"key":"2021120819494023000_R57","doi-asserted-by":"crossref","unstructured":"Gururaj A, Hardy D, Al-Gazali LI, Sztriha L, Roos A, Nork M. Are the strokes in moyamoya syndrome associated with Down syndrome due to protein C deficiency?Brain Dev.\u20082002;24:719\u2013722","DOI":"10.1016\/S0387-7604(02)00075-X"},{"key":"2021120819494023000_R58","doi-asserted-by":"crossref","unstructured":"Fong CT, Brodeur GM. Down\u2019s syndrome and leukemia: epidemiology, genetics, cytogenetics and mechanisms of leukemogenesis. Cancer Genet Cytogenet.\u20081987;28:55\u201376","DOI":"10.1016\/0165-4608(87)90354-2"},{"key":"2021120819494023000_R59","doi-asserted-by":"crossref","unstructured":"Zipursky A, Poon A, Doyle J. Leukemia in Down syndrome: a review. Pediatr Hematol Oncol.\u20081992;9:139\u2013149","DOI":"10.3109\/08880019209018329"},{"key":"2021120819494023000_R60","doi-asserted-by":"crossref","unstructured":"Chimowitz MI, Lynn MJ, Howlett-Smith H, et al. Comparison of warfarin and aspirin for symptomatic intracranial arterial stenosis. N Engl J Med.\u20082005;352:1305\u20131316","DOI":"10.1056\/NEJMoa043033"}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/116\/5\/e694\/1025116\/zpe0110500e694.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/116\/5\/e694\/1025116\/zpe0110500e694.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,8]],"date-time":"2021-12-08T22:52:42Z","timestamp":1639003962000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/116\/5\/e694\/68081\/Moyamoya-Syndrome-Associated-With-Down-Syndrome"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,11,1]]},"references-count":60,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2005,11,1]]},"published-print":{"date-parts":[[2005,11,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.2005-0568","relation":{},"ISSN":["0031-4005","1098-4275"],"issn-type":[{"value":"0031-4005","type":"print"},{"value":"1098-4275","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005,11,1]]}}}