BAPS-CASS: A population-based national study of the surgical outcomes of infants born with Hirschsprung's disease
Hirschsprung's Disease (HD) is a common cause of intestinal obstruction in the newborn period, characterized by an absence of ganglia in the enteric nervous system of the distal bowel (aganglionosis). The estimated incidence of HD is 1 in 5000 live births,with 90% of cases diagnosed in the newborn period. However, there is limited information about the incidence and mode of presentation of HD in the UK. Information regarding operative outcome is limited to case series collected over prolonged periods at single referral centres, rather than population-based data, which give a full and unbiased picture. A recent survey of UK and Irish paediatric surgeons highlighted recent changes in the preferred management of HD. To date, there are no prospective, population-based observational studies of HD to define current surgical management strategies and provide truly representative outcome data in a cohort of children born during the same (short) time period. Long-term outcome is of paramount importance, and defines the success of the early surgical management of HD. Most children with HD do not develop complications during the first 30 post-operative days, emphasizing the importance of assessment of later outcome.
Between 1st October 2010 and 30th September 2012, 305 infants with HD were identified in the UK and Ireland. Over the same time-period, there were 1,729,854 live births in the UK and Ireland, giving an estimated incidence of 1.7 infants with HD per 10,000 live births [95% CI 1.5-1.9].Early complications, including stoma formation, were common. Analysis of outcomes at one year is ongoing.