Total Hepatic Herniation Associated with Giant Omphalocele: Case Report

Abstract

Omphalocele is a congenital defect of the abdominal wall. The current knowledge of its etiology suggests that this defect is not due to the failure of closure or migration of the body wall. Rather, since the umbilical cord is inserted into the sac, it is considered that the omphalocele appears due to the lack of return of the viscera to the abdominal cavity. Consequently, the abdominal contents are herniated with an incidence of 1.5 to 3 per 10,000 births and are associated with multiple congenital and chromosomal malformations. We present an atypical and rare case of a 37-year-old mother; As a result of his fourth pregnancy, with an ultrasound diagnosis at 21 weeks of omphalocele with herniated fetal liver, with birth without complications by scheduled caesarean section, whose defect was considered a giant omphalocele (> 6cm), and which was treated with the placement of silo since the introduction of the herniated contents (liver) into the abdominal cavity was not achieved. The purpose of this paper is to try to explain the complexity of the management of this type of patient, and the complications related to its management, since, being of rare presentation, it becomes a complex challenge for the multidisciplinary team that must face this type of pathology.