were retrieved through the database and analyzed through chart review.Results: There were 127 cases of omphalocele and 121 cases of gastroschisis. Ninety-three of the 127 probands with omphalocele had karyotype determinations and 20% were abnormal; for gastroschisis, 37 had cytogenetic studies and none had chromosomal anomalies. Seventy-six percent of the probands with omphalocele had associated abnormalities; twenty-three percent of the probands with gastroschisis, none of which were syndromic, had associated anomalies. The prematurity rate for omphalocele was 42% and mortality was 22%; for gastroschisis, the figures were 57% and 9%, respectively. There were no significant differences in teratogen exposure between the two groups. Familial cases of both defects occurred in Ͻ 4%, and the prevalence of dizygous twinning was increased in both groups. Mean maternal age was 21.8 years for the gastroschisis group, 27.2 years for the omphalocele group. Conclusions: In this study, the number of cases of omphalocele and gastroschisis were similar, compared to the expected 3:2 ratio, suggesting an increase in the occurrence of gastroschisis. Cases with omphalocele had more syndromic and nonsyndromic anomalies, more chromosomal anomalies, a higher mortality rate, and older mothers. Genet Med 2004:6(4):232-236.