[Incidence and survival in children with selected types of congenital defects in the Czech Republic from 1994 to 2001. (Part 1)].

OBJECTIVE

Analysis of prenatal and postnatal incidence of selected types of birth defects in the Czech Republic in the period of 1994-2001. In the postnatally diagnosed cases the survival in the course of the first year of life was analyzed. Selected birth defects--anencephaly, spina bifida, encephalocele, neural tube defects, congenital hydrocephalus, Down syndrome, were investigated.

TYPE OF STUDY: Retrospective analysis from registry of birth defects in the UZIS Czech Republic in the period of 1994-2001.

METHOD: The authors analyzed frequency of prenatally and postnatally diagnosed cases of 6 selected types of birth defects in the period of 1994-2001 in the Czech Republic. In cases where the diagnosis was established after birth, analysis of survival and death rate during the first year of life was performed for selected birth defects.

RESULTS

In the period of 1994-2001, 745,410 children were born in the Czech Republic. There were 3,572 children with one or more birth defects diagnosed in this cohort during the first year of life. The mean incidence of all cases in this period of observation was 304.52 per 10,000 live born children. The percentage of prenatally diagnosed cases, ended for this diagnosis in the above mentioned period of observation, was on the average 72.5% in neural tube and the highest in anencephaly--more than 96%. In cases of congenital hydrocephalus it was higher than 45% and in Down syndrome it represented 56.5%. The survival in children with neural tube defects was 82.5% on the average, being obviously zero in anencephaly. The children with congenital hydrocephalus survive in more than 75% and those with Down syndrome survive in almost 95%.

CONCLUSION

Incidence of these types of birth defects in newborns in the Czech Republic is presently lower than in previous years particularly due to successful prenatal diagnostics. Cases, where the diagnosis is established after delivery, represent an important part of perinatal, neonatal and post-neonatal mortality and morbidity.