This study is being done to determine if an investigational study drug called Omegaven can help to improve liver disease thought to be caused by Total Parenteral Nutrition (TPN). TPN is intravenous feeding (IV feeding tube) that provides patients the nutrients when they are unable to drink fluid or eat food by mouth.

PAtients with direct bilirubin >2gm/dL and short bowel syndrome are eligible for enrollment. Omegaven at 1gm/kg/day will be administered via central venous line instead of standard lipid preparations.

Inclusion Criteria:

1. Patients must be 2 months -10 years of age
2. Patients who are PN dependent (unable to meet nutritional needs solely by enteral nutrition) and are expected to require PN for at least another 30 days
3. Patients considered eligible for study participation must have parenteral nutrition associated liver disease (PNALD) as defined by a direct bilirubin of >2.0mg/dL. Other causes of liver disease should be excluded. A liver biopsy is not necessary for treatment
4. 2 consecutive direct bilirubin test results >2.0mg/dl
5. The patient must have failed standard therapies to prevent the progression of liver disease such as surgical treatment, cyclic TPN, avoiding overfeeding, reduction/removal of copper and manganese from TPN, advancement of enteral feeding, and use of ursodiol (Actigall)
6. Subjects who are currently under treatment for PNALD with Compassionate Use Omegaven and have a direct Bilirubin of< 2, but who remain TPN dependent and require continued therapy with Omegaven

Exclusion Criteria:

1. Other known causes of chronic liver disease (hepatitis C, cystic fibrosis, biliary atresia and alpha 1 anti-trypsin deficiency)
2. Enrollment in any other clinical trial involving an investigational agent (unless approved by the designated physicians on the multidisciplinary team)
3. The parent, guardian, or child is unwilling to provide consent or assent
4. Allergy to any fish product, egg protein, and/or previous allergy to Omegaven
5. Active coagulopathies characterized by on-going bleeding or by a requirement for clotting factor replacement such as fresh frozen plasma or cryoprecipitate to maintain homeostasis
6. Impaired lipid metabolism or severe hyperlipidemia with or without pancreatitis
7. Unstable diabetes mellitus
8. Stroke/embolism
9. Collapse and shock
10. Undefined coma status
11. Active infection at time of initiation of Omegaven® up until such time as child is afebrile with stable vital signs and one negative 48 hour culture