Chapter 131

Intestine transplantation is now an established treatment for irreversible intestinal failure, allowing affected children the possibility to eat and drink normally while having an improved survival with good growth, development, and quality of life.

The first ventures into intestine transplantation were experiments in animals, demonstrating the technical feasibility of this surgical innovation.1 Technical achievements aside, clinical success remained elusive, primarily because of the inability to control immunologic issues leading to rejection and infection. Even during the 1980s, in which the use of cyclosporin A was introduced, survival following intestinal transplantation was extremely rare. The introduction of tacrolimus heralded in the modern era for intestinal transplantation with improved long-term graft and patient survival.

To understand the challenges faced in achieving outcomes comparable to other forms of solid organ transplantation, it is important to recognize some fundamental differences between the gut and the more familiar transplant organs: the kidney, heart, and liver. The gastrointestinal tract is in direct and continual contact with billions of microbes in an intensely immunologically complex environment. The mass of intestinal tissue transplanted is large and carries a vast number of lymphocytes both in the lamina propria and Peyer patches as part of the gut’s mucosal immune system. The intestinal allograft is not sterile and comes with it own sizable microbial populations. Immunosuppression is critical to preventing cellular rejection and yet diminishes immunoprotective mechanisms. In the face of mucosal injury as a result of rejection, barrier functions is impaired allowing bacterial translocation at a time when immunosuppressive medication is being increased. The balancing act between too much and too little immunosuppression is a common theme in transplant medicine but for intestinal transplantation the margin for error is especially narrow.

Over the past 20 years, as the success of the procedure has improved, the number of intestine transplants has increased steadily to the current figure of about 200 intestine transplants per year worldwide. More widespread application is limited by: (1) the shortage of suitable size-matched, deceased donor organs and (2) the continued high rates of late graft and patient loss as a result of infection and rejection.2-6 The first of these factors mean that even if listed the mortality on the waiting list for intestine transplantation is the highest for any group of patients awaiting solid organ transplantation and is especially significant for the young children in need of composite intestine and liver allografts where pretransplant mortality has been reported as high as 60% of candidates. Competition for organs, especially from the larger group of patients awaiting isolated liver transplantation, as well as the size constraints for intestine and liver donors because of the small peritoneal volume in infant recipients with short gut syndrome, limits organ availability. Although reducing intestinal length and removing the right hepatic lobe can achieve reduction of allograft volume, outcome data for this procedure are limited. There are no widely available living related options but these have been successful at the ...

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