Irreversible
intestinal failure in children is predominantly caused by surgical conditions such as
volvulus,
necrotizing enterocolitis, and
gastroschisis. Functional
intestinal failure from motility disorders such as
intestinal pseudo-obstruction or enterocyte dysfunction with
microvillus inclusion disease also may require intestine replacement. Approved indications for intestinal
transplantation include
liver dysfunction, loss of major venous access, frequent central line-related
sepsis, and recurrent episodes of severe
dehydration despite intravenous fluid management. Surgical options include
transplantation of the isolated intestine, combined liver-intestine
transplantation, or multivisceral
transplantation of the stomach, duodenum, pancreas, and small bowel (with or without the liver). Immunosuppression for intestinal
transplantation is based on
tacrolimus therapy, often with induction immunosuppression using
antilymphocyte antibodies (eg, antithymocyte antibody and
alemtuzumab). Experience at centers of excellence demonstrates 1- and 5-year patient survival rates of 95% and 77%, respectively, with ongoing investigations focusing on lowering long-term causes of graft loss such as chronic rejection.