Chapter 100

### Key Points

1. Isolated intestinal transplantation is indicated for patients with permanent intestinal failure whose liver, including portal pressures and other systemic organs are normal. Outcomes are very good with 1-year patient and graft survivals approaching 90% and 80%, respectively.

2. Combined intestine–liver transplantation is indicated if intestinal failure is accompanied by end-stage liver disease or severe portal hypertension (cholestasis, hypersplenism, coagulopathy). Outcomes remain favorable with about a 70% patient and graft 1-year survivals.

3. A discouraging and as yet unexplained outcome is the fall-off of patient and graft survival at 10 years for both isolated intestine as well as intestine–liver transplants.

4. The progressive development of newer improved immunosuppressive strategies has diminished the frequency of organ rejection and graft versus host disease which may lower long-term complication rates such as vascular, gastrointestinal, infectious, and lymphoproliferative disorders.

The intestine has been the last solid organ to be successfully transplanted, a journey spanning over 60 years which had seen the development of clinical kidney, liver, heart, and lung transplantation. During this time, life-saving support of patients losing function of their intestine came in the form of Total Parenteral Nutrition (TPN) similar to hemodialysis for kidney failure, as well as the development of several forms of corrective surgery for short gut; however, a comprehensive vision of outcomes, challenges, and future direction was lacking. Indeed, for the most part it was the individual patient's failing due to TPN-induced liver failure and the searching care at established liver transplant centers which promoted the continued research and clinical development of intestine transplantation. This process had been a series of trial and error case studies which paralleled immunosuppressive milestones in other organs and that was reported in historical summaries as “pre and post cyclosporine eras.” Except for one pediatric recipient in Paris, these pioneer cases failed quickly due to acute rejection, sepsis, and multisystem organ failure. By the late 1980s the successful outcomes with cyclosporine and the transplantation of other organs, advances in organ preservation and procurement techniques, and improvements in perioperative care and prevention of infection provided the clinical platform for the introduction of a new immunosuppressive drug FK 506 (now known as Tacrolimus). These factors formed the essential components for the initiation in 1990 of an experimental trial of intestine transplantation in children under FK 506, the success of which fostered the development of multidisciplinary care teams managing a heretofore poorly defined malady now known as intestinal failure. In October 2000, intestinal transplantation was recognized by the Center for Medicare and Medicaid Services (CMS) as an established treatment for patients with intestinal failure; since then there has been an increasing number of patients referred for intestinal transplantation. There are now nearly 700 patients alive with a functioning graft in the United States as of December 2007.

Interestingly, the development of the field of intestinal failure management has been one of the most important successes of intestine transplantation and is based on the work of multidisciplinary ...

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