Traditional management of patients with primary sclerosing cholangitis (PSC) has included steroid and/or choledochotomy plus prolonged T-tube drainage. The authors have not been convinced, however, that either of these therapies significantly influence the course of the disease. Therefore, a more aggressive surgical approach was adopted that included performance of a choledochoenteric anastomosis in all patients with PSC who had either (1) a major area of extrahepatic blockage, or (2) primary involvement of the extrahepatic bile ducts. Using this approach, 17 of 22 patients (77%) managed surgically at the UCLA Medical Center from 1974 through 1980 have undergone a choledochoenteric anastomosis. Of these 17 patients, 13 (77%) have had an excellent or good result following surgery. Four patients whose disease was confined to the common bile duct have all had excellent results (mean follow-up 49.3 months since surgery). In addition, 18 of the entire group of 22 patients (82%) are still alive, a mean of 52.2 months after operation and 64.7 months following establishment of the diagnosis. These results in 22 patients encourage the authors to continue to pursue an aggressive surgical approach for patients with primary sclerosing cholangitis.
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