Long-term outcome after partial external biliary diversion for intractablepruritus in patients with intrahepatic cholestasis

Background: Chronic intrahepatic cholestasis is associated with severe prur itus that is often refractory to maximal medical management and leads to si gnificantly impaired quality of life. The hypothesis in this study was that partial external biliary diversion (PEBD) can substantially improve intrac table pruritus secondary to intrahepatic cholestasis with subsequent improv ement of functional quality of life. Methods: Parents' and/or patients' clinical rating of pruritus, growth perc entiles, biochemical parameters, and liver biopsies performed before and af ter surgery were compared in a retrospective medical record review. Results: Eight children underwent PEBD from 1990 through 1997. Complete fol low-up data were available for seven patients. Before surgery, all patients had intense pruritus, which was not responsive to maximal medical therapy. Specimens obtained in preoperative liver biopsies showed moderate (n = 1), minimal (n = 6), or no (n = 1) portal fibrosis. After PEBD, all patients r eceived ursodeoxycholic acid (10-15 mg/kg/dose two to three times daily) un til resolution of pruritus. Of the seven patients with complete follow-up d ata, six had complete resolution of pruritus and sustained resolution up to 8 years after surgery. The patient with mild to moderate residual pruritus was the youngest to undergo PEBD. Growth improved from below the 5th perce ntile before surgery to the 5th through the 25th percentiles for five of si x patients with more than 6 years' follow-up. All families reported improve d quality of life, defined by school attendance and ability to resume norma l activity with peers. There has been no clinical evidence of progression o f liver disease. Conclusion: Partial external biliary diversion is effective in the, long-te rm treatment of pruritus refractory to medical therapy and provides a favor able outcome in a select group of patients with chronic intrahepatic choles tasis without cirrhosis.