Long-term outcome of intractable ulcerating enterocolitis of infancy.

BACKGROUND: Chronic enterocolitis is rare in infancy and accounts for less than 0.5% of all newly diagnosed inflammatory bowel disease (IBD) in the UK. Presentation at this young age is usually indicative of underlying immunodeficiency/immunodysregulation. A group of such infants suffer intractable ulcerating enterocolitis of infancy (IE) in which there is a pan-enteritis with marked oro-anal involvement and deep flask like mucosal ulcers throughout the colon.

METHODS: Retrospective review of presenting features, treatment and long-term outcome in a series of 8 children with typical IE.

RESULTS: The 8 children were aged between 1 and 4 weeks at onset (median 2 weeks, mean 2.3 weeks), of which 7 were followed up for 2-22 years (median 7.5 years, mean 11 years). All 8 children had an intractable disease course requiring a colectomy for control of symptoms. The median age at colectomy was 1.7 years (range 4 months-4 years). Three children developed a generalised lymphadenopathy due to uncontrolled EBV-related lymphoid proliferations (ages 4, 12, 18). These comprised a monomorphous B-lymphoycte lympho-proliferative disorder, a large pleomorphic follicular lymphoma, and a high grade pleomorphic B cell non-Hodgkin's lymphoma.

CONCLUSIONS: Infants with IE have a high risk of developing lymphomatous proliferations that appears to be related to the underlying immunodysregulation. Use of aggressive immunosuppression and acquisition of EBV infection appears to accelerate this process; hence we advocate early colectomy in confirmed cases. In children with IE screening for EBV and vigilance for abnormal lymphoid proliferations is paramount.