Outcomes of exomphalos: an institutional experience.

OBJECTIVE: Abdominal wall defects, particularly exomphalos, in newborn infants are associated with significant morbidity and mortality. The objective of the present study was to review the outcomes of neonates with exomphalos in our neonatal intensive care unit during the last 12 years. STUDY
DESIGN: In this retrospective study 52 neonates with exomphalos were identified from 1996 to 2007. Exomphalos were stratified by the type of defect [exomphalos minor versus major (major defined as defect size more than 5 cm and/or liver in the sac)]. Clinical data, demographic data, and outcome measures of mortality, length of stay (LOS), duration of mechanical ventilation and age at full enteral feeds were studied. Associated anomalies were compared between the two groups.
RESULTS: Of the 52 neonates, 1 was transferred back to referring hospital after surgical repair of the defect and was not analyzed. Exomphalos minor accounted for 24 cases and exomphalos major in 27 cases. Mortality was higher in infants with exomphalos major (n = 9, 33%) compared to infants with exomphalos minor (n = 2, 8%). The median LOS (10 vs. 47 days, P = 0.023), median age at full enteral feeds (5 vs. 23 days, P = 0.004) and median duration of mechanical ventilation (7 vs. 23 days, P = 0.001) were shorter for exomphalos minor compared to exomphalos major. Bacteremia was present in 4 (15%) of neonates with exomphalos major. Syndromic associations were present in 8 neonates (33%) with exomphalos minor compared to 2 neonates (7%) with exomphalos major. Beckwith Wiedemann syndrome was most frequently noted in neonates with syndromic exomphalos minor. Trisomy 13 was the only chromosomal abnormality in the entire cohort (1/51 = 2%) and was seen in a single neonate (1/27 = 3.7%) with exomphalos major. Non-syndromic anomalies were seen in 12 (50%) and 14 neonates (52%) with the minor and major defects, respectively. Only four neonates with exomphalos minor (16%) and ten neonates with exomphalos major (37%) had no associated anomalies. Pulmonary hypoplasia and pulmonary hypertension were identified on either lung biopsy or autopsy (n = 5) as causes of mortality.
CONCLUSION: Neonates with exomphalos minor have better survival, decreased LOS, time to full enteral feeds and shorter duration of mechanical ventilation. Syndromic associations were more common in exomphalos minor. Respiratory failure was the major cause of mortality in infants with exomphalos major.