Contemporary Outcomes of Neonatal Exomphalos: A 12‐Year Single‐Centre Experience
Agana Gnanakuruparan, Chandrasen K. Sinha, Sandeep ShettyABSTRACT
Aim
Exomphalos is a congenital abdominal wall defect associated with significant neonatal morbidity and mortality. Contemporary UK outcome data remain sparse. This study describes outcomes, anomalies, and management strategies for live‐born infants with exomphalos over a 12‐year period at a UK tertiary neonatal surgical centre.
Methods
A retrospective audit of 36 live‐born infants admitted between 2011 and 2023 was performed. Infants were classified as exomphalos major (ExoMaj; defect ≥ 5 cm and/or containing the liver) or exomphalos minor (ExoMin; defect < 5 cm). Outcomes included mortality, hospital stay, ventilation duration, age to full enteral feeds, and complication rates.
Results
Of 36 infants, 53% ( n = 19) had ExoMaj and 47% ( n = 17) had ExoMin. Overall mortality was 11% ( n = 4), all deaths in the ExoMaj group (21% vs. 0%; p = 0.04). ExoMaj was associated with longer hospital stay (26 vs. 8 days; p < 0.001), longer ventilation (2 vs. 1 days; p = 0.03), higher complication rates (70% vs. 0%; p = 0.004), and delayed full enteral feeds (20 vs. 7 days; p = 0.003). All deaths were attributable to respiratory failure secondary to pulmonary hypertension and/or pulmonary hypoplasia. All ExoMaj infants with PPHN and/or pulmonary hypoplasia died, whilst the infant with pulmonary stenosis survived. Pulmonary defects predominated in ExoMaj (37%); Beckwith‐Wiedemann syndrome in ExoMin (41%).
Conclusions
All mortality occurred in ExoMaj and was attributable exclusively to PPHN and/or pulmonary hypoplasia. Anomaly type may be more clinically relevant than anomaly presence alone. These hypothesis‐generating findings have relevance to antenatal counseling. Larger multicentre studies are required to define independent predictors of outcome.