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Key Points

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  1. Omphalocele and gastroschisis are distinct clinical entities.

  2. Associated anomalies and chromosome abnormalities are common in omphalocele, and rare in gastroschisis.

  3. Prenatal diagnosis is common in both entities, which aids in planning for delivery and early postnatal care treatment.

  4. Early delivery and cesarean section delivery methods are not warranted.

  5. The surgical management is highly individualized for each entity, and depends on the overall condition of the newborn, associated anomalies, eviscerated organs, size of abdominal cavity, gestational age, and comorbidities among others.

  6. Overall survival for infants with gastroschisis is over 90%, but is less for infants with omphalocele because of associated anomalies or chromosome abnormalities.

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Abdominal wall defects represent one of the most common congenital surgical problems encountered in neonates. While often discussed as one topic, gastroschisis and omphalocele are different and distinct entities; in this chapter, we will be discussing them separately in terms of management and outcomes.

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Embryology

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The embryologic etiology of congenital abdominal wall defects is not completely understood, and remains a controversial subject. Most authorities currently believe that gastroschisis and omphalocele are embryologically distinct.

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In gastroschisis, the abdominal wall defect is usually located to the right of an intact umbilical cord. The eviscerated contents are not enclosed in a sac. Older hypotheses include rupture of the amniotic membrane at the base of the cord, abnormal regression of the right umbilical vein, and disruption of the vitelline artery resulting in ischemia at the base of the umbilicus. Currently, the ventral body folds theory that suggests failure of migration of the lateral folds (more frequent on the right side) is most accepted. This would make the gastroschisis defect occur early in gestation and prior to development of an omphalocele. Due to the increasing incidence of gastroschisis, there have been a number of possible causative factors that have been considered including tobacco, environmental exposures, lower maternal age, and low socioeconomic status, all suggested by epidemiologic studies but not proven.

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Omphaloceles are not considered to occur as a failure of lateral body wall closure. As these defects will have the umbilical cord coming off the defect itself, it is thought to result from a failure of the intestinal loops to return to the abdomen following the period of physiologic herniation during the 6th to the 10th week of gestation. Etiologically, there are not many teratogens that are implicated in omphalocele causation.

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Associated Anomalies

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Gastroschisis has been considered to be an isolated defect with occasional presence of intestinal atresia (10%-12%). However in a recent review of the literature, a number of anomalies were noted with gastroschisis including cardiac, limb, renal, and occasional chromosomal. These were very few cases, but they suggest that in some patients there may be a genetic component to the defect.

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Infants with omphalocele have a high incidence of associated conditions that can significantly impact ...

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