Intestinal Failure in Children

Abstract

Intestinal failure (IF) is a condition in which severe intestinal malabsorption mandates artificial nutrition through a parenteral route. Causes of severe protracted IF include short bowel syndrome, congenital diseases of enterocyte development, and severe motility disorders (total or subtotal aganglionosis or chronic intestinal pseudo-obstruction syndrome). IF can result in nutritional failure, defined as the long-term failure to nourish a child by natural or artificial means. Today, IF-associated liver disease is the most common cause of parenteral nutrition (PN) failure, but catheter-related sepsis and extensive vascular thrombosis may also jeopardize the health of those receiving PN. For a child with nutritional failure, intestinal transplantation, often in the form of a composite visceral graft, offers the only chance for long-term survival. The management of IF requires a multidisciplinary approach. There have been a number of recent advances in both medical and surgical treatments of IF. In particular, new intestinal lengthening techniques and the use of PN formulas rich in fish oil both have resulted in decreased rates of severe complications of IF and its treatments. In addition, better awareness of the risks and benefits of intestinal transplantation have resulted in better patient selection, and ultimately in improved patient survival, hence restricting the indication to transplantation only to patients with nutritional failure and no other chance to survive.