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Major omphalocele with liver protrusion. Liver dysmorphism. A case study
V Ardeleanu1, Costinela Georgescu, I Fulga
1Department of Morphologic Sciences, Faculty of Medicine and Pharmacy, "Lower Danube" University, Galati, Romania.
Insights
This case report details a rare omphalocele with liver herniation and hepatic dysmorphism in a male infant. The infant
Area of Science:
- Pediatric Surgery
- Neonatal Malformations
- Congenital Abnormalities
Background:
- Omphalocele, a congenital abdominal wall defect, has a variable incidence and affects both sexes equally.
- Historically described in 1634 by Ambroise Paré, omphalocele carries a serious prognosis.
- This report focuses on a unique case presentation of omphalocele in a neonate.
Observation:
- A male infant presented with a large omphalocele containing herniated liver without intestinal loops.
- The omphalocele sac exhibited a trilaminar structure, suggesting secondary herniation after the tenth week of gestation.
- Hepatic dysmorphism was noted in conjunction with the liver herniation.
Findings:
- The case is unique due to liver herniation without intestinal loops and the presence of hepatic dysmorphism.
- Intraoperative liver reintegration was performed, but the infant succumbed to cardio-respiratory arrest.
- The trilaminar membrane structure suggests a defect in anterior abdominal wall closure at the umbilical region.
Implications:
- This case highlights the diverse presentations of omphalocele and the challenges in managing complex congenital malformations.
- Understanding the embryological basis of such defects is crucial for accurate diagnosis and potential interventions.
- Further research into the etiology and management of omphalocele with atypical herniations is warranted.
Abstract:
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.
