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Clinical and radiographic characteristics were analyzed, along with surgical outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS<\/jats:title><jats:p>A total of 537 patients underwent surgery for moyamoya during the study period. Eighteen of those patients (3.4% of the total series) had moyamoya and a family history of moyamoya and were included in this study. Of these 18 patients, 14 were non-twin siblings, and the remaining 4 represented 2 pairs of identical (affected) twins.<\/jats:p><jats:p>The presentation was predominantly ischemic (72%), but 4 patients (33%) were asymptomatic when they were found to have moyamoya. Bilateral disease was present in 13 patients (72%). Radiographic stroke prevalence (67%), Suzuki grade (3.3), and angiographic findings were comparable to findings in nonfamilial moyamoya patients. Thirty revascularization procedures were performed, with a 3.3% operative stroke rate per hemisphere and no new strokes in an average follow-up period of 4.5 years.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSIONS<\/jats:title><jats:p>In a North American surgical series, familial moyamoya existed in 3.4% of cases, and was distinguished by manifesting in a broad range of ethnic groups, with a higher proportion of male patients and increased rates of asymptomatic and unilateral disease in comparison to nonfamilial moyamoya. Screening indications remain controversial and the current data are used to suggest guidelines. Surgical therapy is warranted, effective, and durable in these patients, but patients should be carefully selected.<\/jats:p><\/jats:sec>","DOI":"10.3171\/2016.12.peds16468","type":"journal-article","created":{"date-parts":[[2017,3,10]],"date-time":"2017-03-10T14:58:48Z","timestamp":1489157928000},"page":"553-559","source":"Crossref","is-referenced-by-count":23,"title":["Incidence, clinical features, and treatment of familial moyamoya in pediatric patients: a single-institution series"],"prefix":"10.3171","volume":"19","author":[{"given":"Jonathan","family":"Gaillard","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Yale University, New Haven, Connecticut; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Klein","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Duran","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Yale University, New Haven, Connecticut; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Armide","family":"Storey","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. 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