Transplantation (Tx) of the right colon, together with the small bowel, provides patients with a non functional colon, as in congenital diarrheas or motility disorders, with a better quality of life, without more early infections in our experience. However, colitis may arise later on, as we describe here, and be difficult to control.

Patients and results. From 1994 on, 99 children received 107 transplants, and 70 also a colon. Among the 57 who survived more than 3 months with a functional graft, 9 patients (16%) developed a colitis. Family history of inflammatory bowel disease (IBD) or allergies was negative. Indication for Tx was Hirschsprung (4), tufting enteropathy (2), microvillous inclusion disease (1), short bowel syndrome (1), chronic intestinal pseudoobstruction (1). Mean age at Tx was 5.4 yrs (2.2-8.5), 4 patients had a combined liver-small bowel Tx (L-SBTx). Immunosuppression was basiliximab, tacrolimus, steroids. Mean delay between Tx and ostomy closure was 8 months (1-21).

Symptoms of colitis appeared after a mean of 4.8 yrs (1-12). Inflammation (elevated CRP) was always present. In 7/9 patients, a microorganism was found at onset of symptoms (2 Campylobacter, 2 EBV, 2 norovirus, 1 cryptosporidium). Endoscopy showed ulcers of variable severity. At pathology, mixed inflammatory infiltrates were present, with frequent eosinophils (4/9). There were no signs of rejection. Stenosis of the ileocaecal valve developed in 3 patients, of whom 2 had norovirus at onset. Treatment was parenteral nutrition (5), steroids (4), antiviral agents (3), infliximab (3), mesalazine (1), surgery (2). At last follow-up, only 3 patients with L-SBTx survived with a functional graft, 2 with complete recovery, 1 with a stenosis of the ileocaecal valve with a proximal fistula, 2 isolated small bowel grafts had been removed, and 3 patients had died, 2 after bowel graft removal and 1 (with stenosis of the valve) of a cardiac arrest (hypokaliemia ?).

Conclusion. The transplanted colon gives a functional advantage to patients with colonic disease. However a minority developed a colitis akin to IBD, with often an infectious trigger, that could progress to severe chronic colitis or stenosis of the valve. They were no signs of acute rejection at the time of diagnosis, however many small bowel grafts were lost later on of acute or chronic rejection. This colitis could be a particular manifestation of chronic rejection, or a “de novo” IBD. The treatment followed the guidelines for IBD, with a variable efficiency. We are trying to study more closely the pathology, and the risk factors (such as NOD2), in order to better understand and control this complication.