Management should occur through a multidisciplinary intestinal failure service whenever available.
The immediate priorities are correction of dehydration and electrolyte deficits, control of sepsis, treatment of the underlying gastrointestinal disease and provision of sufficient nutrition.
Use the gut whenever it is functional. Oral and enteral feeding help preserve intestinal adaptation and should be maximised without causing uncontrolled fluid losses.
Parenteral nutrition or intravenous fluid support is life saving therapy for patients whose intestine cannot independently maintain hydration or nutrition. ESPEN recommends home parenteral nutrition as primary therapy for chronic intestinal failure when the underlying problem is not immediately reversible.
Parenteral prescriptions must be individualised according to energy requirements, enteral absorption, renal function, stoma losses and biochemical monitoring. A fixed universal electrolyte formula is inappropriate.
Central venous access must be treated as an organ preserving resource. Prevent catheter infection using meticulous aseptic technique and investigate fever during infusion promptly for bloodstream infection.
Correct the mechanism whenever possible. This may include fistula closure, relief of obstruction, restoration of intestinal continuity or treatment of inflammatory bowel disease.
Patients with potentially reversible Type II disease should undergo nutritional rehabilitation before major reconstructive surgery.
Intestinal rehabilitation seeks to progressively reduce intravenous support through dietary optimisation, antimotility and antisecretory therapy, restoration of bowel continuity and, in selected short bowel syndrome, treatment with GLP 2 analogue therapy.
Intestinal transplantation is reserved for selected patients with irreversible intestinal failure and life threatening complications of parenteral support, such as progressive intestinal failure associated liver disease, loss of central venous access or recurrent severe catheter related sepsis.