Major omphalocele with liver protrusion. Liver dysmorphism. A case study.

In the Renaissance (1634), Ambroise Paré described for the first time the omphalocele, highlighting the serious prognosis of this malformation. The incidence of omphalocele varies between 1/2000-1/6000 births, both sexes being equally affected. We present the case of a male infant, born at term after a pregnancy that was not followed by the family doctor or by the specialist obstetrician and who died after five days. On the anterior abdominal wall in the umbilical region, there was a formation of about 10 cm in diameter, covered by a membrane apparently avascular through whose transparency there could be observed a blue-reddish tumor, of discreet firm consistency that while breathing compressed the apparently avascular membrane that covers it. Intraoperative there was noticed liver herniation by parietal defect, operating the liver reintegration in umbilical cavity, subsequently the child dying from a cardio-respiratory arrest. The particularity of this case is on one hand the liver herniation without intestinal loops, the most common liver hernia being with intestinal loops and, on the other hand the presence of hepatic dysmorphism. We also mention the trilaminar structure of omphalocele membrane sac, which makes us think that the herniation was secondary, after the tenth week, through a defect of the anterior abdominal wall closure in the umbilicus region. The combination of these elements creates the uniqueness of this case.