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The incidence and distribution of 934 reported cases diagnosed prior to age 6 months, and born to Utah residents from 1940 to 1979, were analyzed. Data were ascertained by examination of multiple sources, e.g., 982,066 birth, 11,161 fetal death, and 248,208 death certificates, and selected hospital and clinic records. Of the 934 reported cases, 700 met our selection criteria for congenital hydrocephalus, which results in a crude incidence of 0.70 per 1,000 live and stillbirths. Seventy\u2010one cases (10.1%) had additional, multiple congenital anomalies. The male\/female sex ratios of the 619 cases of isolated congenital hydrocephalus (occurring as a single entity or in the absence of other reported or known birth defects) and those with multiple congenital anomalies (71 cases) were virtually identical, being 1.45 and 1.48, respectively. A significant 85% increase in the rate of reported cases was observed for the period 1966 to 1970. However, examination of patients' records from 1966 to 1975 in the hospital responsible for almost all of this increase suggests that this was an iatrogenically related \u201cepidemic\u201d caused by several factors: the introduction and possible misinterpretation of pneumoencephalograms (PEG) in the diagnosis of hydrocephalus (PEG was replaced by CAT scanning in the early 1970s), inappropriate diagnosis, and incorrect recording of age at time of diagnosis. \u00a9 1994 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.1320520202","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T17:16:50Z","timestamp":1118510210000},"page":"123-129","source":"Crossref","is-referenced-by-count":36,"title":["Epidemiology of congenital hydrocephalus in Utah, 1940\u20131979: Report of an iatrogenically related \u201cepidemic\u201d"],"prefix":"10.1002","volume":"52","author":[{"given":"Brent L.","family":"Blackburn","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert M.","family":"Fineman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,6,7]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1962.04210050047005"},{"key":"e_1_2_1_3_1","first-page":"1","article-title":"Hydrocephalus: A twenty year survey of hydrocephalic births","volume":"76","author":"Banner EA","year":"1956","journal-title":"Lancet"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/72.2.246"},{"key":"e_1_2_1_5_1","first-page":"289","article-title":"The incidence of congenital diseases and defects in a South Swedish population","volume":"2","author":"Book JA","year":"1951","journal-title":"Acta Genet"},{"key":"e_1_2_1_6_1","first-page":"168","article-title":"Sex\u2010linked hydrocephalus with severe mental defect","volume":"1","author":"British Medical Journal Editorial","year":"1962","journal-title":"Acta Genet"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.5.2.81"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.1965.tb10885.x"},{"key":"e_1_2_1_9_1","first-page":"401","article-title":"Temporal trends in the incidence of birth defects\u2010United States","volume":"28","author":"Centers for Disease Control","year":"1979","journal-title":"MMWR"},{"key":"e_1_2_1_10_1","series-title":"Birth Defects Original Article Series, The National Foundation\u2010March of Dimes","first-page":"1","volume-title":"Analysis of epidemiological factors in congenital malformations, report from the Collaborative Perinatal Project","author":"Chung CS","year":"1975"},{"key":"e_1_2_1_11_1","first-page":"205","article-title":"Major malformations of the central nervous system in Hungary","volume":"24","author":"Czeizel A","year":"1970","journal-title":"Br J Prev Soc Med"},{"key":"e_1_2_1_12_1","first-page":"115","article-title":"Congenital malformations of the central nervous system in Scotland","volume":"12","author":"Edwards JH","year":"1958","journal-title":"Br J Prev Soc Med"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.36.189.486"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.36.189.481"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.1972.tb03316.x"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02950032"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/tera.1420050206"},{"key":"e_1_2_1_18_1","first-page":"226","article-title":"X\u2010linked hydrocephalus, with aqueductal stenosis, mental retardation, and adduction\u2010flexion deformity of the thumbs","volume":"2","author":"Faivre J","year":"1976","journal-title":"Childs Brain"},{"key":"e_1_2_1_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-0528.1954.tb07701.x"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.2105\/AJPH.49.4.497"},{"key":"e_1_2_1_21_1","first-page":"153","article-title":"Declining incidence of central nervous system anomalies in New York State","volume":"16","author":"Gittelsohn AM","year":"1962","journal-title":"Br J Prev Soc Med"},{"key":"e_1_2_1_22_1","series-title":"US Public Health Service Publ 1163","first-page":"305","volume-title":"Genetics and the Epidemiology of Chronic Diseases","author":"Gittelsohn AM","year":"1965"},{"key":"e_1_2_1_23_1","first-page":"164","article-title":"Defects of the central nervous system in Finland. 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