Aim of the Study
To describe one-year outcomes from a population-based cohort of infants with exomphalos and explore factors associated with mortality.
Following ethics approval (ref:12/SC/0416), a prospective population-based cohort study of live-born infants with exomphalos was undertaken over a two-year period(2014-2016) in the UK and Ireland using the BAPS-CASS methodology. Data were collected at twenty-eight days and one-year. Outcomes assessed included: infant death, length of initial hospital stay in days (LOS) and need for ongoing respiratory support (including oxygen therapy). Possible factors related to mortality were explored using univariable logistic-regression. Data are presented as odds ratios (OR) with 95% confidence intervals, n(%) or median(interquartile range).
Complete data were obtained for 122 infants. Sixteen (13%) died before one-year. Significant factors associated with mortality were: prematurity (OR 0.70(0.60-0.83) per week increase in gestational age); birthweight (OR 0.99(0.99-0.99) per 500g increase); intrauterine growth-restriction (OR 7.5(1.96-28.65)); other anomalies (OR 5.38(0.68-42.67)) and defect diameter(cm)/weight(kg) ratio (OR 2.93 per unit increase(1.47-5.81)). There was no statistically significant association between mortality and sex, ethnicity, sac-rupture or presence of extracorporeal liver. Figure 1 shows the relationship between prematurity and survival.
15/106 infants who survived to one-year (14%) were receiving respiratory support at 28 days. 12/15 of these infants were preterm, three were still receiving support ar one-year.
LOS for all surviving infants was 13(8-40); 10(5-13), 31(18-71) and 78(40-153) for those undergoing primary-closure, conservative management and silo-reduction respectively.
Comorbidity, in particular preterm birth, has a major impact on survival and ongoing morbidity in infants with exomphalos. Where this is absent, the outlook is excellent. Future work should aim to understand the causes of preterm birth in fetuses with exomphalos.
Figure 1. One-year survival of exomphalos infants according to gestational-age