Introduction: Intestinal failure-associated liver disease (IFALD) refers to hepatic dysfunction that results from prolonged parenteral nutrition (PN) use. IFALD is multifactorial in origin and remains a major cause of morbidity and mortality. Prior to 2004, IFALD was associated with mortality as high as 90% in infants who remained on PN greater than 1 year. The advent of new strategies for intravenous lipid emulsion (ILE) administration and improved catheter care now allow many patients to remain on PN and recover from this once fatal condition. Several additional treatment modalities are often used to further improve outcomes for IFALD patients and they are reviewed here.Areas covered: The etiology of IFALD is presented, as well as the rationale behind the use of ILEs that contain fish oil. Other management strategies are addressed, including the effects of several pharmacologic and nutritional interventions.Expert opinion: Like its etiology, the management of IFALD is multifactorial. Prompt recognition of patients at risk, avoiding macronutrient excess, and preventing central line associated bloodstream infections will improve outcomes. In patients who develop IFALD, the use of fish oil monotherapy seems to be efficacious. The most effective intervention, however, continues to be discontinuation of PN and achieving full enteral feedings.
Keywords: Intestinal failure-associated liver disease; fish oil; intravenous lipid emulsion; metronidazole; phenobarbital; ursodiol.