Intestinal Failure (IF) can develop when your child's small intestine does not provide the necessary long-term nutrition needed to sustain a normal life. This leads to the need for additional long-term nutrition to be delivered through large veins in the body. This is called "Parenteral Nutrition" or "PN."

To read more extensively about short bowel syndrome and pediatric gastrointestinal disorders, please visit our page here.

Disorders or Conditions That Can Lead to Intestinal Failure:

Short Bowel Syndrome, resulting from:

• Intestinal atresia (lack of development of the intestine).

• Apple peel syndrome (a rare form of intestinal atresia).

• Volvulus (twisting of the intestine).

• Necrotizing enterocolitis (NEC) (inflammation and loss of blood flow to the intestine, leading to severe damage).

• Gastroschisis (the intestine develops outside of the body).

• Crohn’s disease (intestine becomes inflamed and scarred, requiring extensive resection, leading to short bowel).

• Complicated intussusception (part of the intestine is folded into another part and compromises the blood flow to the involved portion of the intestine).

• Vascular thrombosis (blood clots) or ischemia (lack of blood flow) to the intestine.

• Tumors, requiring extensive resection of the bowel.
  

Absorption Disorders:

• Microvillous Atrophy (MVA)

• Intestinal Epithelial Dysplasia (IED)

Motility Disorders

• Chronic Intestinal Pseudo-Obstruction (CIPO)

• Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS)

• Extensive Hirschsprung’s disease (Total colonic aganglionosis)

If your child's small bowel has permanently failed, also known as intestinal failure, he or she may require an intestinal transplant. Intestinal failure is when the intestine is not able to digest and absorb enough nutrients to support your child's growth and daily function.

Children with intestinal failure require the use of parenteral nutrition, or PN, which is the delivery of nutrients through the large veins in his or her body.

Intestinal failure alone is not an indicator for intestinal transplant, rather, your child must also have a complication from parenteral nutrition (PN), as noted below. It is usually from long-term use of PN in which these issues occur.

In some cases, other factors, such as quality of life, are taken into account when considering intestinal transplant. Each case is individualized and insurance and transplant center approval will vary. Please discuss your child's situation with their team.

These indications are often the result of long-term PN use because of intestinal failure caused by an underlying disease, as noted below.

Please note: It is at the discretion of each individual transplant center and team to ultimately determine a patient's candidacy for transplant.

The contraindications will ultimately be determined by your transplant team, but in most cases the following will exclude you from receiving an intestinal transplant:

• Cardiopulmonary deficiency

• Aggressive malignancy: Your transplant center may consider your cancer aggressive if it forms, grows, or spreads quickly or is unresponsive to conventional cancer treatments, such as chemotherapy and radiation.

• Some forms of autoimmune disease with poor prognosis as deemed by the transplant center.

• Acquired Immunodeficiency Syndrome (AIDS).

• Existence of life-threatening intra-abdominal infections or sepsis.

• Inadequate social or financial support.

1. Isolated intestinal transplant: This is a transplant in which only the small intestine (jejunum and ileum) is transplanted.

2. Modified Multivisceral Transplant: This is a transplant in which the stomach, pancreas, duodenum, and small intestine (jejunum and ileum) are transplanted. In some cases, the colon may be included.

3. Multivisceral Transplant: This is a transplant in which the stomach, pancreas, duodenum, small intestine (jejunum and ileum), and liver are transplanted. In some cases, the colon may be included. Please note: Multivisceral transplants can also include other organs, such as a kidney, if indicated.

Please see below for a visual and more information.