# Extremely Rare Co-occurrence of Left Gastroschisis-Like Abdominal Wall Defect and an Omphalocele in a Very Low Birth Weight Infant: A Case Report

## TL;DR

This case report describes a rare condition in a premature infant with two unusual abdominal wall defects, requiring complex surgical treatment.

## Contribution

The paper presents the second documented case of co-occurring left-sided gastroschisis-like defect and omphalocele in a very low birth weight infant.

## Key findings

- The infant had a large left-sided abdominal wall defect and an omphalocele with liver prolapse.
- Treatment involved staged surgical techniques including artificial patches and component separation.
- Imaging suggested hypoplasia of the upper left abdominal wall and possible dysfunction of the left superior epigastric artery.

## Abstract

Gastroschisis is almost always a small, right-sided, periumbilical, abdominal wall defect, and its occurrence on the left side of the umbilicus is extremely rare. Furthermore, omphaloceles and gastroschisis usually do not co-occur. The present report is the 2nd worldwide to describe the co-occurrence of an omphalocele and left gastroschisis-like abdominal wall defect.

A 32-year-old pregnant woman (gravidity 2 and parity 1) was referred to our center because gastroschisis was detected in her fetus at 15 weeks and 3 days of gestational age. At 33 weeks and 3 days of gestational age, cesarean section was performed to deliver the female infant after a premature rupture of membranes. Her birth weight was 1368 g, and her Apgar scores were 8 at 1 min and 9 at 5 min. At birth, a macroscopic examination revealed an omphalocele with liver prolapse and a large, left-sided, epigastric, abdominal defect with an associated prolapse of the liver, spleen, stomach, and intestine. The patient had 2 very challenging conditions, namely, an early delivery with immature lung function and 2 large abdominal defects, for which a staged operation was performed using a combination of techniques involving the application of an artificial patch to close the abdominal wall temporarily, a hydro-fiber dressing to promote epithelization, and component separation to close the rather large defect permanently. Contrast CT revealed a defect in the lower costal cartilage and hypoplasia of the upper left abdominal wall. The peripheral part of the left superior epigastric artery was unclear in the imaging study.

The abdominal wall defect might have differed from other cases of left-sided gastroschisis in terms of its pathogenesis in the upper left abdomen. The presence of lower left hypoplastic thorax strongly suggested that dysfunction of the left superior epigastric artery had caused the abdominal wall defect. There is no standard treatment for this extremely rare, congenital malformation. Thus, its treatment requires the application of several techniques, each to address different aspects of the condition at the corresponding, surgical stage.