OMPHALOCELE AND GASTROSCHISIS IN EUROPE - A SURVEY OF 3-MILLION BIRTHS 1980-1990

A total of 732 cases of omphalocele and 274 cases of gastroschisis was registered in 21 regional registers in Europe (EUROCAT registers) dur ing the period 1980-1990. The total prevalence rates were 2.52 pes 10, 000 for omphalocele and 0.94 per 10,000 for gastroschisis. There was s ignificant heterogeneity in total prevalence rates among regions for o mphalocele. Consistently higher than average total prevalence rates of omphalocele were found in the five centers of the British Isles. This was in large part linked to the association between omphalocele and n eural tube defects. A significant female excess among the cases of omp halocele associated with neural tube defects, in comparison with an in significant male excess for other cases of omphalocele, was observed. Geographical differences in the total prevalence of gastroschisis are partly explained by differences in maternal age distributions in the p opulations surveyed. Omphalocele was an isolated malformation in 46% o f cases; gastroschisis was isolated in 79% of cases. The average birth weight and gestational age of both isolated and multiply malformed cas es of both omphalocele and gastroschisis were low, especially for mult iply malformed cases, and to a greater extent for isolated gastroschis is than for isolated omphalocele. Prenatal diagnosis leading to termin ation of pregnancy was reported in 33.2% of omphalocele and in 26.5% o f gastroschisis cases, demonstrating the considerable impact of curren t prenatal screening programs. On the basis of clinical manifestations , epidemiologic characteristics, and the presence and type of addition al malformations, omphalocele and gastroschisis can be considered hete rogeneous conditions. (C) 1995 Wiley-Liss, Inc.