BAPS-CASS: Surveillance of the surgical outcomes of infants with Meconium Ileus

Summary

Meconium Ileus (MI) is defined as small bowel obstruction in the newborn period caused by inspissated meconium within the terminal ileum. There is a well-recognised association of MI with cystic fibrosis (CF); 10% - 15% of infants with CF will present with MI. There is currently limited information about the incidence and mode of presentation of MI in the UK and Ireland. The British Association of Paediatric Surgeons Congenital Anomalies Surveillance System (BAPS-CASS) was established in 2006 to address such issues and has improved case ascertainment to up to 95% in specific anomaly review. However, MI is not a congenital anomaly and as such is not reported in any UK registry, however by limiting the study to CF patients with MI we will be able to utilise CF registries to cross reference the cases and ensure high levels of case ascertainment. This study will collect accurate data on the incidence, mode of presentation, genotypes, clinical characteristics and outcomes for this group of infants and will provide valuable information which will aid in the counselling of parents with infants diagnosed with MI and CF.