{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T15:56:41Z","timestamp":1793807801240,"version":"4.6.1"},"reference-count":24,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["FOC"],"published-print":{"date-parts":[[2008,2]]},"abstract":"\n Object<\/jats:title>\n Progression of vasculopathy associated with moyamoya syndrome is extremely variable. The authors review their experience in patients with unilateral moyamoya syndrome to identify factors predictive of contralateral clinical and imaging-documented disease progression.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n The authors reviewed the clinical and imaging records of all patients with moyamoya syndrome and unilateral disease who underwent cerebral revascularization surgery between January 1985 and June 2006 by using a standardized surgical procedure, pial synangiosis.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n Of 235 surgically treated patients with moyamoya syndrome, 33 (14%) presented with unilateral disease (4 adults and 29 children). There were 16 female and 17 male patients, with an average age of 10.4 years (26.8 years for adults and 8.1 years for children; range 1.5\u201339 years). Twenty patients presented with left-sided disease and 13 with right-sided disease.<\/jats:p>\n The average follow-up after surgery was 5.3 years (3.1 years for adults and 5.6 years for children; range 1\u201316 years). During this period, 10 (30%) of 33 patients progressed to bilateral disease. The mean time until disease progression was 2.2 years (range 0.5\u20138.5 years). Factors associated with progression in this series included contralateral abnormalities on initial angiography, previous history of congenital cardiac anomaly, cranial irradiation, Asian ancestry, and familial moyamoya syndrome. Young age at diagnosis was associated with a more rapid rate of progression (age < 7 years, 0.9 years to progression and age \u2265 7 years, 3.1 years to progression).<\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n Of patients with unilateral moyamoya syndrome, 30% will have progression of arteriopathy during long-term follow-up. In this series, the average time of progression from unilateral to bilateral angiographic disease was 2.2 years. Several factors, including contralateral abnormalities on initial imaging, congenital cardiac anomaly, previous cranial irradiation, Asian ancestry, and familial moyamoya syndrome, were associated with an increased risk of progression. Patients with known unilateral angiographic disease should undergo continued monitoring by using MR imaging and MR angiography at regular intervals. Treatment with pial synangiosis is safe and confers durable protection against stroke in patients with both bilateral and unilateral moyamoya syndrome.<\/jats:p>\n <\/jats:sec>","DOI":"10.3171\/foc\/2008\/24\/2\/e17","type":"journal-article","created":{"date-parts":[[2008,2,14]],"date-time":"2008-02-14T15:12:19Z","timestamp":1203001939000},"page":"E17","source":"Crossref","is-referenced-by-count":114,"title":["Progression of disease in unilateral moyamoya syndrome"],"prefix":"10.3171","volume":"24","author":[{"given":"Edward R.","family":"Smith","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. Michael","family":"Scott","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"issue":"Suppl 2","key":"b1-0240017","series-title":"Clin Neurol Neurosurg","first-page":"S178","article-title":"Long-term follow-up study of patients with unilateral moyamoya disease","volume":"99","author":"Hirotsune","year":"1997"},{"key":"b2-0240017","series-title":"J Neurosurg","first-page":"772","article-title":"Is \u201cunilateral\u201d moyamoya disease different from moyamoya disease?","volume":"85","author":"Houkin","year":"1996"},{"key":"b3-0240017","series-title":"Pediatrics","first-page":"E694","article-title":"Moyamoya syndrome associated with Down syndrome: outcome after surgical revascularization","volume":"116","author":"Jea","year":"2005"},{"key":"b4-0240017","series-title":"Cerebrovasc Dis","first-page":"109","article-title":"Progression of unilateral moyamoya disease: a clinical series","volume":"22","author":"Kelly","year":"2006"},{"key":"b5-0240017","series-title":"Pediatr Neurosurg","first-page":"182","article-title":"Elevation of cerebrospinal fluid levels of basic fibroblast growth factor in moyamoya and central nervous system disorders","volume":"27","author":"Malek","year":"1997"},{"key":"b6-0240017","series-title":"Neurosurgery","first-page":"401","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":"b7-0240017","series-title":"Neurosurgery","first-page":"830","article-title":"Unilaterally symptomatic moyamoya disease in children: long-term follow-up of 20 patients","volume":"59","author":"Nagata","year":"2006"},{"key":"b8-0240017","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":"b9-0240017","series-title":"Childs Nerv Syst","first-page":"145","article-title":"Unilateral (probable) moyamoya disease: long-term follow-up of seven cases","volume":"22","author":"Seol","year":"2006"},{"key":"b10-0240017","series-title":"Skull Base","first-page":"15","article-title":"Surgical management of moyamoya syndrome","volume":"15","author":"Smith","year":"2005"},{"key":"b11-0240017","series-title":"Neurosurgery","first-page":"544","article-title":"Levels of soluble adhesion molecules are elevated in the cerebrospinal fluid of children with moyamoya syndrome","volume":"50","author":"Soriano","year":"2002"},{"key":"b12-0240017","series-title":"Arch Neurol","first-page":"288","article-title":"Cerebrovascular \u201cmoyamoya\u201d disease. 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