Intestinal rehabilitation is the science that exhibits the amazing ability of the intestine to adapt to different and unexpected situations. It encompasses pharmacological, dietary, and surgical options that can reconstitute the intestine to normal function. Intestinal failure occurs when the intestine cannot sustain the nutritional needs of an individual and maintain adequate fluid balance. Parenteral nutrition (PN) is required in these cases in order to provide daily nutrition and hydration independent of the GI tract. Patients most likely to exhibit permanent intestinal failure or require long term parenteral nutrition include those with an inadequate remaining bowel length, an absent ileocecal valve and/or colon, active mucosal disease, bowel dysmotility, or infectious enteritis.
Bowel Length and Long-term PN Requirement in SBS
When the intestine is irreversibly affected and rehabilitation is not possible, PN may be required indefinitely. Parenteral nutrition saves thousands of lives each year but is not tolerated by everyone. Patients with a very short length of bowel, those who develop recurrent catheter related blood stream infections (CRBSI), or those who experience multiple central vein access thromboses may be at a higher risk of developing life threatening complications from the use of long-term parenteral nutrition. Indications for intestinal transplantation vary in the adult and pediatric populations.
In the pediatric population, intestinal failure is usually the result of one of the following:
- Short Bowel Syndrome (SBS). This is a malabsorption disorder caused by the surgical removal (resection) of large sections of intestine. Most cases are acquired due to removal of diseased bowel due to congenital malformation (gastroschisis, intestinal atresia) of the intestine, necrotizing enterocolitis (seen in some premature babies), or loss of blood supply to the gut (volvulus). The degree to which patients suffer the consequences of SBS depends largely on the remaining intestinal anatomy. For example, a large jejunal resection should not disturb absorption substantially because of the ability of the remaining ileum and colon to absorb increased fluid and electrolytes, maintain bile salts, and prolong movement of food and fluid through the intestine. A large ileal resection, on the other hand, leads to significant fat malabsorption, and if the colon also is resected, fluid and electrolyte balance can be severely impaired.
- Dysmotility disorders may also be referred to as Chronic Intestinal Pseudo-obstruction (CIPO). The anatomy and length of the bowel may be preserved, but the function (the way the small bowel moves) is impaired. Symptoms may be similar to a bowel obstruction and can include severe abdominal pain and distension, severe bloating, nausea, vomiting and the inability to eat.
- Intractable diarrhea of Infancy: This includes conditions such as microvillus inclusion disease and tufted enteropathy. These conditions are associated with severe diarrhea where nutrition and growth can only be maintained by parenteral nutrition.
A list of the most common diagnoses preceding intestinal transplantation in the pediatric population is described below.
Pediatric Diagnoses Predicting Intestinal Transplant:
- Necrotizing enterocolitis
- Intestinal atresia
- Intestinal pseudo-obstruction
- Microvillus inclusion disease
- Intractable diarrhea of infancy
- Autoimmune enteritis
- Intestinal polyposis
Does Your Child Need an Intestinal Transplant?
If your child’s medical condition falls into one of the following categories, he/she may be a candidate for an intestinal transplant.
1) TPN Related Complications
- Parenteral nutrition-induced liver disease
Liver failure is the worst complication induced by PN. An increase in bilirubin, ALT, AST and alkaline phosphatase may represent the first signs of liver failure. Liver failure is responsible for the majority of mortality caused by intestinal failure. We generally work hard to avoid this complication.
- Central venous catheter (CVC) related thrombosis of two or more central veins
If you lose one or more central vein accesses from thrombosis of a PN line, you may be at risk of losing your remaining available access for nutrition and hydration (central veins).
- Frequent episodes of central line sepsis
This could include two or more episodes per year of systemic sepsis secondary to line infections requiring hospitalization or a single episode of line-related fungemia (infection caused by fungus).
- Frequent episodes of severe dehydration despite intravenous fluid administration in addition to parenteral nutrition
2) Underlying Disease with an Increased Risk of Morbidity
- Congenital mucosal disorders (i.e. microvillus inclusion disease, tufting enteropathy).
- Ultra short bowel syndrome (gastrostomy, duodenostomy, residual small bowel <10 cm in infants and <40 cm in older children).
- Desmoid tumors associated with familial adenomatous polyposis.
3) Intestinal Failure with an Intolerance to Parenteral Nutrition
- Intestinal failure with high morbidity (frequent hospitalizations, narcotic dependency) or inability to function (i.e. pseudo-obstruction, high output stoma).