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Gastroschisis and omphalocele. An eight-year review
Insights
Gastroschisis and omphalocele are distinct abdominal wall defects with different clinical features and outcomes. Gastroschisis has a lower mortality rate, while omphalocele is associated with more severe anomalies.
Area of Science:
- Pediatric Surgery
- Neonatal Care
- Congenital Anomalies
Background:
- Gastroschisis and omphalocele are congenital abdominal wall defects with historically unclear distinctions.
- Recent data suggests significant differences in their clinical presentation and management.
Purpose of the Study:
- To compare the clinical features, associated anomalies, and outcomes of gastroschisis and omphalocele.
- To identify factors influencing mortality and survival rates in infants with these conditions.
Main Methods:
- Retrospective analysis of 75 infants treated for gastroschisis or omphalocele since 1971.
- Comparison of incidence, prematurity rates, associated malformations, and mortality between the two groups.
Main Results:
- Gastroschisis occurred twice as frequently as omphalocele and is increasing. Prematurity was common in gastroschisis (65%), with jejunoileal/colonic atresias being the main associated anomaly. Gastroschisis mortality was 12.7%.
- Omphalocele was associated with 23% prematurity, but 66% had anomalies, predominantly cardiac (52%) and chromosomal (40%). Omphalocele mortality was 34%, largely due to these anomalies. Survival was 94% in omphalocele patients without cardiac or chromosomal defects.
Conclusions:
- Gastroschisis and omphalocele are significantly different conditions requiring distinct management strategies.
- Omphalocele's high mortality is primarily linked to severe cardiac and chromosomal defects; survival is high when these are absent.
- Preventable deaths in gastroschisis suggest potential for improved outcomes through optimized surgical treatment and neonatal care.
Abstract:
Until recently confusion has existed concerning the clinical features and surgical treatment of gastroschisis and omphalocele. Since 1971 75 infants with these abdominal wall defects have been treated at our institution. Significant differences (p equal to 0.001 in all instances) were noted between the two diseases. Gastroschisis occurred twice as often as omphalocele and is increasing in frequency. Prematurity was commonly seen with gastroschisis (65%). While the overall incidence of malformations associated with gastroschisis was low (23%), the vast majority of the additional malformations were jejunoileal or colonic atresias. The mortality rate was 12.7% among gastroschisis patients, with only one death attributable to prematurity. All other deaths were preventable, indicating that even lower mortality rates are feasible. Omphalocele was associated with a 23% incidence of premature birth but associated anomalies were present in 66% of the patients. Major cardiac (52%) and chromosomal defects (40%) predominated. In addition, 17% of omphalocele patients had either Cantrell's pentalogy or cloacal/bladder exstrophy. The mortality rate in omphalocele (34%) was nearly three times that of gastroschisis. Nine of ten patients who died from omphalocele died either from major cardiac or chromosomal disease. However, in patients without cardiac or chromosomal defects the survival rate was 94%.