Intestinal Failure Associated Liver Disease (IFALD)

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. (1) This pattern of steatosis is predominantly periportal and helps to define the lesion. Steatohepatitis may occur and result in periportal inflammation, hepatocyte necrosis, and pericellular fibrosis. Extensive fibrosis and progression to cirrhosis can occur with either predominant cholestatic or steatohepatitc lesions, generally occurring 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. (1) 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.

Central Venous Thrombosis:

Usually asymptomatic, but may include swelling in the face, neck, or limbs. You may see prominent superficial veins or pain on starting parenteral nutrition. Central vein thrombosis is confirmed by echocardiography doppler ultrasound, CT scan, and/or venography.

References:

Langnas AN, Goulet O, Quigley EMM, Tappenden KA. Intestinal Failure Diagnosis, Management, and Transplantation. Malden, MA: Blackwell; 2008.

Indications for Transplant

Gastroparesis for Adults

Gastroparesis

Reference History of Transplant

Intestinal Failure Associate Liver Disease (IFALD) Assessment

Central Venous Thrombosis

Malrotation and Mid-gut Volvulus

Gastroschisis

Enteral Support for Children with Intestinal Failure

Total Colonic Hirschsprung Disease

Ischemia for Medical Professionals

Desmoid Tumors for Medical Professionals

Bariatric Surgery Complications