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Congenital defects of the abdominal wall
Insights
Congenital abdominal wall defects like omphalocele, cord hernia, and gastroschisis have distinct embryogenesis and outcomes. Survival rates vary, with associated anomalies and low birthweight impacting mortality in these newborn conditions.
Area of Science:
- Pediatric Surgery
- Developmental Biology
- Neonatology
Background:
- Congenital abdominal wall defects encompass omphalocele, cord hernia, and gastroschisis.
- These conditions present unique anatomical features and embryological origins.
- Understanding these differences is crucial for effective management and improving infant outcomes.
Purpose of the Study:
- To review a 22-year experience with 138 newborn infants diagnosed with congenital evisceration.
- To differentiate between omphalocele, cord hernia, and gastroschisis based on defect characteristics, embryogenesis, and associated anomalies.
- To analyze survival rates and factors influencing mortality in these conditions.
Main Methods:
- Retrospective review of 138 newborn infants with congenital abdominal wall defects over 22 years.
- Classification of defects based on sac presence, rectus muscle insertion, and associated anomalies.
- Analysis of survival data and identification of mortality-associated factors.
Main Results:
- Omphalocele: Large defect with sac, lateral rectus insertion, common anomalies. Survival: 31/51.
- Cord hernia: Small defect with sac, xiphoid rectus insertion, common anomalies. Survival: 22/28.
- Gastroschisis: Small defect without sac, xiphoid rectus insertion, few anomalies, frequent prematurity. Survival: 40/59.
Conclusions:
- Gastroschisis likely results from umbilical coelom failure and midgut rupture.
- Omphalocele arises from primary body fold closure failure.
- Cord hernia stems from failure of midgut return from the umbilical coelom.
- Mortality is linked to anomalies, low birthweight, and tension during surgical closure.
Abstract:
We reviewed a 22 year experience with 138 newborn infants with congenital evisceration through defects of the abdominal wall. Omphalocele is a large defect which always has a sac, in which the rectus muscles insert laterally on the costal margins and which usually has associated anomalies. Cord hernia is a small defect which always has a sac, in which the rectus muscles insert at the xiphoid and which commonly has associated anomalies. Gastroschisis is a small defect which never has a sac, in which the rectus muscles insert at the xiphoid and has few associated anomalies, though prematurity is frequent. We hypothesize that gastroschisis develops because the umbilical coelom fails to form, which forces the elongating midgut to rupture into the amniotic cavity. This differs from the embryogenesis of omphalocele, which is failure of closure of a primary body fold, and from that of cord hernia, which is failure of the midgut to return from the umbilical coelom. The number of infants in this series who survived after surgical repair of an omphalocele was 31 of 51 patients; of a cord hernia, 22 of 28 patients, and of gastroschisis, 40 of 59 patients. Factors contributing to mortality were associated anomalies, low birthweight and surgical closure under excessive tension.