Abstract:
BACKGROUND: 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. CASE PRESENTATION: 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. CONCLUSION: 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.
