Successful heterotopic segmental liver transplantation from a live donor to a patient with Alagille syndrome

Alagille syndrome is characterized by a paucity of bile ducts in the liver. The syndrome is associated with some or all the features of chronic choles tasis, cardiac disease, skeletal abnormalities, ocular defects and a distin ctive facial appearance. The most common finding is chronic cholestasis, wh ich causes intractable pruritus, xanthoma, deficiency of certain metabolic nutrients and growth retardation. Cardiac abnormalities are the most common cause of death in these patients. It is unusual to see the clinical pictur e of hepatic failure resulting in cirrhosis and requiring transplantation, but liver transplantation is indicated in Alagille syndrome patients who ha ve chronic cholestasis. If the disease is diagnosed in childhood, transplan tation can improve significantly the patient's prognosis and the quality of life. In recent years, auxiliary liver transplantation has gained populari ty for treating both acute and chronic liver disease. Heterotopic segmental liver transplantation is an alternative treatment modality for patients wh o do not require native liver removal. Individuals with Alagille syndrome a re good candidates for this type of treatment, Copyright (C) 2001 by W.B. S aunders Company.