Abstract
Risk factors were studied in 96 children with congenital hydrocephalus (CH) coming from 118,265 consecutive births of known outcome. Hydrocephalus with neural tube defects, intracranial tumors or secondary to brain atrophy were excluded. The prevalence of CH was 0.81 per thousand. Diagnosis was performed prenatally in 41 cases. Forty-three (44.8%) of the cases had hydrocephalus without other malformations (isolated hydrocephalus), 18 (18.7%) infants had recognized chromosomal or non-chromosomal syndromes and 35 children (36.4%) had multiple malformations. Each case was matched to a control. Weight and length at birth of children with hydrocephalus were less than in the controls (p < 0.001). The weight of the placenta was lower than in the controls (p < 0.05). The pregnancy with a hydrocephalic child was more often complicated by threatened abortion, polyhydramnios and oligohydramnios. The mothers of children with hydrocephalus and multiple malformations had used oral contraceptives during the first trimester of pregnancy more often than the mothers of the controls. No differences appeared between the mothers of children with CH and the controls for the other risk factors studied: parental age, parity, previous pregnancies, previous stillbirths, smoking, diabetes, epilepsy, X-rays, hypertension, fever “flu”, medication and occupational exposure. There was an increase of parental consanguinity in the parents of our patients (6.2% v. 1.1%, p < 0.001) and first degree relatives had more non-cerebral malformations than the controls (7.3% v. 3.2%, p < 0.05).
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References
BannisterC.M. (1986): Is the intrauterine treatment of fetal hydrocephalus helpful or harmful? Fetal Therapy 1: 146–149.
ChevenakF.A., BerkowitzR.L., TortoraM. and HobbinsJ.C. (1985): The management of fetal hydrocephalus - Am. J. Obstet. Gynecol. 151: 933–942.
CochraneD.D., MylesS.T., NimrodC., Still.D.K., SugarmanR.G. and WittmannB.K. (1985): Intrauterine hydrocephalus and ventriculomegaly: Associated anomalies and fetal outcome - Can. J. Neurol. Sci. 12: 51–59.
EdwardsJ.H., NormanR.M., RobertsJ.M. (1961): Sexlinked hydrocephalus: Report of a family with 15 affected members - Arch. Dis. Child. 36: 481–485.
EUROCAT Working Group (1989): Surveillance of congenital anomalies, years 1980–1986. Eurocat Report 3, Department of Epidemiology, Catholic University of Louvain, Brussels, pp 29–30.
FernellE., HagbergC. and YonWendtL. (1987): Epidemiology of infantile hydrocephalus in Sweden II. Origin in infants born at term - Acta Paediatr. Scand. 76: 411–417.
International Clearinghouse for Birth Defects Monitoring Systems (ICBDMS) (1991): Congenital malformations worldwide, Elsevier, Amsterdam pp 59–64.
LorberJ. and DeN.C. (1970): Family history of congenital hydrocephalus - Dev. Med. Child. Neurol. Suppl. 22: 94–100.
McKeownT. and RecordR.Y. (1951): Malformations of the central nervous system - Lancet 1: 191–196.
MichejdaM., QueenanJ.T. and McCulloughD. (1986): Present status of intrauterine treatment of hydrocephalus and its future - Am. J. Obstet. Gynecol. 155: 873–882.
NicolaidesK.H., BerryS., SnijdersR.J.M., Thorpe-BeestonJ.G. and GosdenC. (1990): Fetal Lateral Cerebral Ventriculomegaly: Associated Malformations and Chromosomal Defects - Fetal. Diagn. Ther. 5: 5–14.
PalumbosJ.C. and StiermanE.D. (1989): Outcome of 86 cases of prenatally diagnosed hydrocephalus - Am. J. Hum. Genet. 45Suppl A.: 265.
RogersS.C. and Weatherall J.A.C. (1976): Anencephalus, spina bifida and congenital hydrocephalus. England and Wales 1964–72. Studies on medical and population subjects no 32. Office of Population on Censuses and Surveys.
SAS/STAT, TM (1987): Guide for personal computer Version 6 - SAS Institute Inc. pp 773–875.
SteinS., FeldmanJ., ApfelS., KohlS. and CaseyG. (1981): The epidemiology of congenital hydrocephalus. A study in Brooklyn - N.Y. 1968–1976. Childs Brain 8: 253–262.
StollC., RothM.P., DottB. and BigelP. (1984): Etude des malformations congénitales dans le Nord de l'Alsace - Médecine et Hygiène 42: 505–512.
StollC., AlembikY., RothM.P., DottB. and DeGeeterB. (1989): Risk factors in congenital heart disease -Eur. J. Epidemiol. 5: 382–391.
StoneD.H., WomersleyJ., SinclairT. and EvansT. (1989): Declining prevalence of hydrocephalus -Eur. J. Epidemiol. 5: 398–399.
VàradiV., TothZ, TorokO. and PappZ. (1988): Heterogeneity and recurrence risk for congenital hydrocephalus (ventriculomegaly): a prospective study - Amer. J. Med. Genet. 829: 305–310.
WeatherallJ.A.C. (1982): A review of some effects of recent medical practices in reducing the numbers of children born with congenital abnormalities - Health trends Department of Health and Social Security, Welsh Office 14: 85–88.
WilliamsonR.A., SchaubergerC.W., VarnerM.W. and AschenbrennerC.A. (1984): Heterogeneity of prenatal onset hydrocephalus: Management and counseling implications - Am. J. Med. Genet.17: 497–508.
WiswellT.E., TuttleD.J., NorthamR.S. and SimondsG.R. (1990): Major congenital neurologic malformations. A 17 year survey - Am. J. Dis. Child. 144: 61–67.
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Stoll, C., Alembik, Y., Dott, B. et al. An epidemiologic study of environmental and genetic factors in congenital hydrocephalus. Eur J Epidemiol 8, 797–803 (1992). https://doi.org/10.1007/BF00145322
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DOI: https://doi.org/10.1007/BF00145322