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We reviewed the case notes of 110 children with a diagnosis of MS, attending the craniofacial unit in Oxford between 1991 and 2008. Our results showed 38 children (38\/110 or 34.6%) who had at least one additional structural abnormality or had a known syndromic diagnosis were classed as having syndromic MS. Chromosomal abnormalities were noted in 8\/38 (21.4%) children: mosaic marker chromosome 2, 9p deletion (2\/8), 11q deletion, 12pter deletion and duplication of 15q25 with other additional chromosomal abnormalities (3\/8). Other syndromic diagnoses included Silver\u2013Russell syndrome and Greig cephalopolysyndactyly. Prenatal exposure to sodium valproate (VPA) was noted in 8\/110 children (7.8%), with the dose of the VPA being \u22651,000\u2009mg\/day in all cases. Other prenatal exposures reported in this study were: maternal diabetes (6\/110), enoxaparin for hypercoagulable state (1\/110), and thyroxine (1\/110). The majority of patients (72\/110 or 65.4%) had nonsyndromic MS. Speech delay was present in 11 children with nonsyndromic MS (11\/72 or 15.3%) and 10 children with syndromic MS (10\/38 or 26.3%). We conclude that approximately two\u2010thirds of all MS is nonsyndromic. Prenatal exposure to VPA is a common cause of MS. Maternal diabetes, not previously linked to MS, was noted in 5.5% of cases. Chromosomal abnormalities account for about 6% of MS. An increased risk of speech delay is seen with both the syndromic and nonsyndromic forms. \u00a9 2010 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.a.33435","type":"journal-article","created":{"date-parts":[[2010,5,11]],"date-time":"2010-05-11T20:49:50Z","timestamp":1273610990000},"page":"1383-1389","source":"Crossref","is-referenced-by-count":26,"title":["Etiological heterogeneity and clinical characteristics of metopic synostosis: Evidence from a tertiary craniofacial unit"],"prefix":"10.1002","volume":"152A","author":[{"given":"Usha","family":"Kini","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jane A.","family":"Hurst","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jo C.","family":"Byren","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Steven 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