Intestinal Transplantation

Abstract

Intestinal failure (IF) is characterized by the inability of the digestive tract to absorb nutrients in order to cover metabolic needs. The main etiologies in children are short bowel (mainly after intestinal atresia, gastroschisis, necrotizing enterocolitis, and midgut volvulus), intestinal motility disorders (Long segment Hirschsprung’s disease and intestinal pseudo-obstruction), and congenital enterocyte disorders causing intractable diarrhea (tufting enteropathy and microvillus inclusion disease). The first-line treatment of intestinal failure is parenteral nutrition (PN), which provides excellent long-term results (90% patient survival, with close to a normal quality of life on home PN). In short bowel syndromes, intestinal rehabilitation programs, including intestinal lengthening techniques, may help to partially or totally reverse the dependency on PN. Intestinal transplantation is indicated in cases of irreversible intestinal failure and severe complications of PN: loss of venous access due to large vessel thrombosis, lifethreatening line infections, liver disease, and poor quality of life in some patients, due to chronic intestinal obstruction and/or water and electrolyte losses. Other rare indications include retransplantations and (exceptionally) tumors. Intestinal transplantation is classified into four surgical subtypes, according to the organs needed together with the small bowel +/- right colon: isolated intestinal transplantation, liver and intestinal transplantation, modified multivisceral transplantation (all digestive organs without the liver), and multivisceral transplantation (all digestive organs with the liver). A renal transplantation may be added if needed. Adequate preparation for the procedure is very important, since the short-term results correlate with the general condition of the child at surgery. The intestine is a highly immunogenic organ, requiring a high level of immunosuppression. After intestinal transplantation, the child is exposed to rejection and early to late graft loss and to complications of immunosuppression, including infections, tumors, and drug toxicity (mainly renal function impairment). Current research aims at improving graft survival and reducing the complications of immunosuppression, the transplant Graal being induced tolerance.