Intestinal Failure (IF) is a condition in which there is inadequate digestion or absorption of fluid, electrolyte, and nutrients that leads to dehydration or malnutrition. (Matarese et al. 2005)

There are both reversible and irreversible conditions that can lead to intestinal failure. Patients with reversible IF should not be considered for intestinal transplant.

Mucosal Disorders

• Microvillus inclusion*

• Secretory diarrhea

• Autoimmune enteritis

• Intestinal Epithelial Dysplasia* or Tufting Enteropathy

Motility Disorders

• Chronic intestinal pseudo-obstruction (CIPO)

• Hirschsprung’s disease*

• Hollow visceral myopathy and/or neuropathy

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Surgical or Anatomical Disorders

• Volvulus*

• Gastroschisis*

• Necrotizing enterocolitis*

• Ischemia (e.g. arterial embolism, venous occlusion, shock)

• Crohn’s disease (can also be functional)

• Intestinal atresia*

• Bariatric surgery

• Other causes of short bowel

​Obstructive

• Radiation enteritis (results in a progressive fibrosis to an obstructive associated vasculitis)

• Adhesions

• Internal hernia

Gastrointestinal Neoplasm

• Mesenteric desmoid tumor (such as in Gardner’s syndrome, often requiring large resections)

• Diffuse polyposis

• Carcinoid tumors (can elicit a desmoplastic reaction in the mesentery, resulting in wider bowel resection)

• Amyloidosis (systemic disease involving multiple systems, infiltrates the bowel wall)

*Click on the terms with an asterisk to get more details.

Intestinal failure associated liver disease encompasses both acute and chronic derangements of hepatobiliary function, as well as severe complications including fibrosis, cirrhosis, portal hypertension, and hepatocellular failure.

The identification of IFALD relies on persistent elevations (i.e. for >2 weeks) of one or more liver enzymes or bilirubin in the peripheral blood, as liver biopsy is rarely performed in the early stages. The diagnosis can easily be confounded by sepsis, drug toxicity, and biliary obstruction.

Steatosis may occur rapidly upon initiation of parenteral and both macrovesicular and microvesicular fat loading may be apparent. This pattern of steatosis is predominantly periportal and helps to define the lesion. Extensive fibrosis and progression to cirrhosis generally occurs over a prolonged period of time.

Progression to clinically overt liver disease in adults is insidious, and until the onset of jaundice, the only symptom may be upper abdominal discomfort as a result of hepatic enlargement and pruritus (often mild) from cholestasis. Other signs may include hepatosplenomegaly, collateral vessels, ascites, signs of coagulopathy, and/or uncommonly esophageal varices.

Cholelithiasis is frequently reported in adults on long-term parenteral nutrition. It is the practice of some centers to perform cholecystectomy on adults who require long term parenteral nutrition.

Diagnosis is usually based on clinical presentation, liver ultrasound, and liver biopsy. An accurate assessment of the extent of TPN related liver injury is crucial for successful outcome of transplant. The decision to perform simultaneous hepatic replacement is very challenging.

Diagnosis of portal hypertension is based on standard criteria including:

• Low platelet count.

• Enlarged spleen.

• Detection of gastroesophageal varies or portal hypertensive gastropathy.

• Presence of ascites.

Treatment is a multiprotection approach, including trying to wean the patient from parenteral nutrition, reducing the impact of bacterial overgrowth, keeping the patient infection free, trying to reduce lipid exposure, and evaluate the patient for candidacy of intestinal and multivisceral transplant if indicated.

Please refer to (5) for more information.

• The length and site of the resected small intestine.

• Cause of the resection.

• Age of onset of the short bowel syndrome.

• A biological marker, known as citrulline, is sometimes used as a predictor to determine the permanence of intestinal failure.

• Citrulline is a nonessential amino acid that is mostly produced by enterocytes.

• It is correlated with small bowel length and absorptive capacity and is more predictive of permanent intestinal failure than remnant length of small bowel alone.

• Combining the use of citrulline level with length of remaining small bowel offers an approach for identifying parenteral nutrition candidates that may benefit from pharmacologic or surgical gut rehabilitative procedures prior to resorting to intestinal transplant.

• Note: Cannot use this level in the presence of renal insufficiency.