Internal biliary diversion to avoid liver transplantation in an adult with intractable pruritus due to idiopathic intrahepatic cholestasis

Cholestasis is due to the obstruction at any level of the excretory pathway of bile. One particularly troublesome symptom of cholestasis is pruritus which leads to a profound effect on a patient’s quality of life. In children with progressive familial intrahepatic cholestasis (PFIC), medical treatment often fails. An alternative surgical procedure using biliary diversion offers significant relief for intractable pruritus in non-responders. A 43-year-old male presented with a history of persistent jaundice and intractable pruritus for a 2-month duration. His liver enzymes were markedly elevated. However, his liver synthetic function was preserved. After extensive evaluation, a cause for cholestasis was not identified. A multi-disciplinary decision was to consider liver transplantation, but as his liver synthetic functions were remarkably preserved and the intractable pruritus was the sole indication for a transplantation, it was finally decided that internal biliary diversion should be done for symptomatic relief. His pruritus dramatically improved at 6 weeks post-operative. His liver enzymes and bilirubin levels also decreased compared to his pre-operative status. Currently, his liver functions are being closely monitored. Though it is not used in adults, the experience of biliary diversion in children with PFIC shows that there is a considerable improvement of symptoms and postpone the need for a transplant. In our patient, liver function and bilirubin as expected did not show a major improvement. But the dramatic improvement of the symptoms gave us the time to postpone the liver transplantation. A biliary diversion is a reasonable option that needs to be considered even in adults with refectory pruritus due to cholestasis.