Not long ago, it was customary to close the abdomen of an infant with necrotic bowel, without performing a resection, and explain to the parents that there was no alternative but to let the child die. Today, it would be considered more appropriate to at least have discussed the option of intestinal transplantation with them before an irreversible decision had been made. Nevertheless, it is not yet an automatic decision or one to be taken lightly – the evaluation, pre-operative testing, wait for an organ to become available, the operation itself, the postoperative medical regimen, and the intensive follow-up care all combine to create a huge undertaking that is not a realistic option for every child or every family: it requires an enormous commitment, invariably causes tremendous emotional and financial strain, and disrupts nearly every aspect of home life. These patients and their families need to be supported in many ways while awaiting the transplant, with as much as possible of the work up preferably being performed close to home.
For some patients, small intestinal transplantation is the only alternative to long-term parenteral nutrition. This includes patients who have essentially no intestinal length and those with an irreversible motility or functional disorder of the gut. Children with short bowel can sometimes be treated effectively with a bowel lengthening operation, such as the STEP procedure, which in most cases is a reasonable consideration even if it is felt likely to only delay the eventual need for intestinal transplantation. Some of the visceral myopathy disorders that occur in adolescence will improve over time or are segmental, allowing palliation with some type of an enterostomy. Many children on long-term parenteral nutrition inevitably develop cirrhosis and thus will require liver transplantation in addition to small intestine transplantation. Finally, it is also important to do everything possible to preserve vascular access sites in patients with intestinal failure: using the smallest catheters compatible with therapy, not ligating veins, and taking all precautions to prevent central-line associated blood stream infections.