Background: Omphalocele is a midline abdominal hernia that conditions important rates of mortality and morbidity. Infants born with omphalocele present an increased risk of having structural and chromosomal anomalies or syndromes. It is a defect that requires surgical treatment with multiple postoperative comorbidities.
Purpose: To identify and characterize the cases of omphalocele of a Level III Neonatal Intensive Care Unit and analyse the impact of clinical and demographic characteristics of both infant and mother in patients’ outcome.
Methods: This is a retrospective study based on the record analysis of infants that have been hospitalized in the Neonatal Intensive Care Unit of “Centro Hospitalar de São João” with the diagnosis of omphalocele between 2003 and 2012. Sixteen patients fulfilled these criteria. For each one, data about pregnancy, maternal history, prenatal diagnosis, delivery, newborn, treatment and follow-up was collected and analysed.
Results: The mean birth weight was 2,761 grams and the mean gestational age was 37 weeks. Prenatal diagnosis was performed in 12 (75%) of the cases; mean gestational week of diagnosis was lower in patients that died. Overall, 50% (80% of deceased patients) had large defects. Major malformations were seen in 25% of cases most often in deceased infants. Low 1st minute Apgar score, need of inotropic support, more days of parenteral nutrition and lower birth weight had statistically significant impact on mortality. After surgical correction 31.3% died, 18.8% had a residual hernia and 12.5% a gastroesophageal reflux, respectively.
Conclusion: The mortality rates of patients with the diagnosis of omphalocele are not negligible because of associated anomalies or postoperative complications. We have been able to correlate some clinical features with mortality and found out that patients who survive can have other comorbidities mainly in the first years of life. However the majority of infants are expected to have a good long-term development and quality of life.