An Atypical Presentation of a Large Congenital Umbilical Cord Hernia Antenatally Diagnosed as an Omphalocele With the Sole Left Lobe of the Liver: A Case Report

We report an infrequent case of a full-term male neonate presenting with a large congenital hernia of the umbilical cord (6 cm × 6 cm) containing only the entire left lobe of the liver. Prenatal ultrasonography suggested an omphalocele; however, the definitive postnatal diagnosis was established based on the presence of a narrow rim of normal skin encircling the umbilical ring and the proximal umbilical cord. Primary closure of the fascial defect was achieved successfully, with no intraoperative or postoperative complications observed. The patient recovered well postoperatively and was discharged in stable condition. Accurate differentiation from an omphalocele is crucial for appropriate management. Unlike omphaloceles, congenital hernias of the umbilical cord (CHUC) typically do not require extensive genetic or cardiac evaluation, and their surgical repair is generally less complex. However, awareness of atypical presentations of CHUC can aid in timely diagnosis, guide surgical planning, and improve clinical outcomes.

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Publication Details

Journal
Pediatric and Developmental Pathology
Published
2025-12-12
DOI
https://doi.org/10.1177/10935266251399455
Primary Topic
Congenital Anomalies and Fetal Surgery
Type
article
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article

An Atypical Presentation of a Large Congenital Umbilical Cord Hernia Antenatally Diagnosed as an Omphalocele With the Sole Left Lobe of the Liver: A Case Report

Zlatan Zvizdić, Azra Karamustafic, Semir Vranić, Edin Medjedović et al.
Pediatric and Developmental Pathology
Congenital Anomalies and Fetal Surgery
article

An Atypical Presentation of a Large Congenital Umbilical Cord Hernia Antenatally Diagnosed as an Omphalocele With the Sole Left Lobe of the Liver: A Case Report

Zlatan Zvizdić, Azra Karamustafic, Semir Vranić, Edin Medjedović, Asmir Jonuzi
article en

Abstract

We report an infrequent case of a full-term male neonate presenting with a large congenital hernia of the umbilical cord (6 cm × 6 cm) containing only the entire left lobe of the liver. Prenatal ultrasonography suggested an omphalocele; however, the definitive postnatal diagnosis was established based on the presence of a narrow rim of normal skin encircling the umbilical ring and the proximal umbilical cord. Primary closure of the fascial defect was achieved successfully, with no intraoperative or postoperative complications observed. The patient recovered well postoperatively and was discharged in stable condition. Accurate differentiation from an omphalocele is crucial for appropriate management. Unlike omphaloceles, congenital hernias of the umbilical cord (CHUC) typically do not require extensive genetic or cardiac evaluation, and their surgical repair is generally less complex. However, awareness of atypical presentations of CHUC can aid in timely diagnosis, guide surgical planning, and improve clinical outcomes.

Pediatric and Developmental PathologyVol. 29(2)
University of Sarajevo (BA), Qatar University (QA)
Openalex Percentile: Top 40%
Congenital Anomalies and Fetal Surgery
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