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Giant omphaloceles: surgical management and perinatal outcomes
Adesola C Akinkuotu1, Fariha Sheikh1, Oluyinka O Olutoye2
1Texas Children's Fetal Center, Texas Children's Hospital, Houston, Texas.
Insights
Infants with omphalocele and associated anomalies face higher mortality. For giant omphalocele (GO), delayed surgical closure shows promise, but further research is needed to confirm optimal repair timing.
Area of Science:
- Pediatric Surgery
- Neonatal Care
- Congenital Abnormalities
Background:
- Omphalocele is a congenital abdominal wall defect.
- Management and outcomes of affected infants require detailed description.
Purpose of the Study:
- To describe the current management and outcomes of infants diagnosed with omphalocele.
- To analyze the impact of associated anomalies on omphalocele patient outcomes.
Main Methods:
- Retrospective review of 95 omphalocele patients (2003-2014).
- Classification based on isolated, minor, or major associated anomalies.
- Definition of Giant Omphalocele (GO) as >50% liver in the sac; assessment of fetal lung volumes.
Main Results:
- Mortality was significantly higher in infants with major (41%) and minor (17%) anomalies compared to isolated omphalocele (0%).
- Infants with major anomalies experienced longer intubation and hospital stays.
- For Giant Omphalocele (GO), 85% underwent delayed closure with 80% 6-month survival; delayed repair had fewer revisions than early repair.
Conclusions:
- Associated anomalies are the primary predictor of morbidity and mortality in omphalocele.
- Delayed closure appears effective for Giant Omphalocele (GO) management.
- Prospective studies are necessary to determine the optimal surgical timing for GO repair.
Background:
The purpose of this study was to describe the current management and outcomes of infants with omphalocele.
Methods:
The medical records of all patients treated for omphalocele at a large children's hospital from January, 2003-February, 2014 were reviewed. Patients were classified as having an isolated omphalocele or omphalocele with minor or major associated anomalies. Prenatal data collected included fetal magnetic resonance imaging-based observed-to-expected total fetal lung volumes. Giant omphalocele (GO) was defined as >50% of liver in the omphalocele sac.
Results:
Of 95 patients, 59 presented prenatally and had comprehensive fetal center evaluation. Of 82 live-born infants, 21 had chromosomal and 25 had major associated anomalies. No live-born baby with an isolated defect (n = 19) died, whereas mortality was 41% and 17% for those with major and minor anomalies, respectively (P = 0.006). Infants with major anomalies had significantly longer median length of intubation (36 versus 0 versus 0 d; P = 0.04) and hospital stay (157 versus 28.5 versus 18 d; P < 0.001) compared with those with minor or no anomalies. Of 40 infants with GO, the majority (85%) were managed surgically by delayed closure with a median age at repair of 10 mo (range, 3.4-23.6 mo). Six-month survival was 80%. None of the delayed repair patients required a later operative revision, whereas 2 of 5 with early repair did.
Conclusions:
The presence of associated anomalies is the strongest predictor of morbidity and mortality in fetuses or neonates with omphalocele. In patients with GO, delayed closure is associated with good outcomes, but larger, prospective studies comparing delayed to early closure are needed to determine the optimal timing of repair.
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