Management of intestinal failure.

Intestinal failure (IF) occurs when the body is unable to sustain its energy and fluid requirements without support, due to loss of functional small bowel. Prolonged IF is seen after large intestinal resection and described as short bowel syndrome (SBS). The hallmark of the management is parental nutrition (PN), which is costly and may be associated with the well-recognized problems of parental nutrition associated liver disease (PNALD) and line related sepsis. Cessation of PN at the earliest possible stage is desirable but for this enteral autonomy has to be achieved first. Intestinal adaptation occurs when the remaining gut goes through morphological changes increasing its absorptive capacity. Factors such as intraluminal nutrients, gastrointestinal secretions and hormones facilitate adaptation. Enteral feeds are a potent stimulant to adaptation and should be started as soon as the clinical situation permits. Some drugs are thought to increase intestinal adaptation. These include glutamine, growth hormone and glucagon like peptide- 2, but there is a paucity of pediatric data to guide their use. In some cases surgical bowel lengthening procedures can be performed to increase the absorptive surface area. An isolated liver transplantation may be required if the liver has sustained irreversible damage but intestinal autonomy seems achievable. When prolonged PN is either unsustainable or associated with unacceptable side effects, small bowel transplantation should be considered as a treatment option.