Omegaven is an intravenous fat emulsion (IFE) comprised of omega-3 fatty acids derived from fish oil. It will be used in an open label compassionate use treatment protocol, as an alternative to soybean oil (omega-6), as the sole IFE source of parenteral nutrition in an effort to reduce and/or reverse parenteral nutrition associated liver disease. The study will evaluate the safety and efficacy of Omegaven use in pediatric patients with PN dependence and PNALD.

Inclusion Criteria:

• Patients will be PN dependent (unable to meet nutritional needs solely by enteral nutrition) and are expected to require PN for at least another 30 days
• Parenteral nutrition associated liver disease (PNALD) as defined as a direct bilirubin of ≥ 2 mg/dL on the last two consecutive bilirubins while on PN. Other causes of liver disease should be excluded. A liver biopsy is not necessary for treatment.
• Failed standard/conventional therapies to prevent progression of PNALD.
• Age newborn to 17 years of age
• Signed informed consent.

Exclusion Criteria:

• Allergy to eggs and/or shellfish
• Female who is pregnant or lactating
• Severe hemorrhagic disorder
• Other causes of chronic liver disease (Hepatitis C, Cystic fibrosis, biliary atresia, and alpha 1 anti-trypsin deficiency,)
• 18 years of age or older
• Parent/legally authorized representative is unwilling to consent.