Correlation between the presence of liver herniation and perinatal outcome in prenatally diagnosed fetal omphalocele

Aims: To investigate the association between the presence of liver herniation and perinatal course and outcome of fetal omphalocele. Methods: Cases of fetal omphalocele managed at our hospital between 1990 and 2006 were retrospectively reviewed and grouped according to the location of the liver. Results: Thirty-three fetal omphalocele cases were diagnosed. The chromosomal status of 29 of 33 fetuses was determined. The rate of chromosomal abnormalities in cases with an extracorporeal liver was significantly lower (2/18) than in the intracorporeal group (6/11) (P=0.028). In chromosomally normal cases, four with extracorporeal liver resulted in early neonatal death compared to none with intracorporeal liver. Five of the 21 chromosomally normal fetuses showed an abnormal volume of amniotic fluid. All five cases had extracorporeal liver and two of them resulted in neonatal death. Conclusions: Fetuses with an extracorporeal liver had a lower rate of chromosomal abnormalities than those in the intracorporeal liver group. However, in chromosomally normal cases, it appeared that extracorporeal livers might be associated with more life-threatening anomalies, amniotic fluid volume abnormalities, and a higher rate of mortality than in the group with an intracorporeal liver. Upon diagnosis of fetal omphalocele, a careful search for liver location should be conducted before counseling.