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Improved survival of infants with omphalocele
1Division of Pediatric Surgery, UCLA School of Medicine, Los Angeles, California 90095-1749, USA.
Insights
Surgical repair of omphalocele (a congenital abdominal wall defect) shows improved outcomes. Staged repair with controlled intra-abdominal pressure leads to low operative mortality and good long-term quality of life for affected infants.
Area of Science:
- Pediatric Surgery
- Neonatal Care
- Congenital Anomalies
Background:
- Omphalocele management has evolved over 40 years.
- Historically, omphalocele surgical management has had over 10% mortality.
- Significant improvements in care have been made.
Purpose of the Study:
- To review the characteristics, management, and outcomes of omphalocele patients.
- To evaluate surgical outcomes in a single-center cohort.
- To assess the impact of different surgical approaches.
Main Methods:
- Retrospective review of 31 omphalocele patients.
- Surgical care provided between 1980 and 1995.
- Analysis of patient characteristics, management strategies, and outcomes.
Main Results:
- 25 patients had associated congenital anomalies.
- Most patients underwent operative repair, with silo construction for large defects.
- Operative mortality was low (1 patient), with another death due to comorbidities.
Conclusions:
- Omphalocele repair outcomes depend on visceroabdominal disproportion and anomaly severity.
- Staged repair with limited intra-abdominal pressure elevation yields low mortality.
- Good long-term quality of life is achievable for these patients.
Background:
The surgical management of patients with omphalocele has evolved over the past 4 decades. Despite many improvements in care, the reported mortality has been over 10%.
Methods:
This study reviewed the characteristics, management, and outcome of 31 patients with omphalocele who underwent surgical care between 1980 and 1995 at a single hospital.
Results:
Twenty-five patients had additional congenital anomalies. All but 1 patient underwent operative repair; 13 of these patients with large defects had a silo chimney constructed initially. Fourteen patients underwent primary fascial and skin closure at the initial operation. Only 1 patient died perioperatively; another patient died without operation due to other major malformations.
Conclusions:
The results following surgical repair of omphalocele defects depend on the degree of visceroabdominal disproportion and on the severity of associated anomalies. The operative mortality for staged omphalocele repair with limited elevation of intraabdominal pressure is low, and the long-term quality of life of these patients is good.