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The clinical, radiologic and pathologic features of 37 patients diagnosed as having primary sclerosing cholangitis (PSC) were reviewed. Sixty-two per cent were men, and 35% had ulcerative colitis. The patients demonstrated considerable variability in their natural history and pathology. It appeared that they could be divided into four fairly distant groups (1) sclerosing cholangitis affecting primarily the distal common bile duct; (2) sclerosing cholangitis occurring soon after an attack of acute necrotizing cholangitis; (3) chronic diffuse sclerosing cholangitis; and (4) chronic diffuse sclerosing cholangitis associated with inflammatory bowel disease. It is suggested that the patients in these groups may have different etiologies, may respond to different treatment regimes, and may have different prognoses.

During total pancreaticoduodenectomy for chronic pancreatitis, four patients received an intraportal pancreatic mixed-cell autograft prepared by collagenase digestion. The technique of this autotransplantation procedure was successfully developed using a normal canine pancreas, but has proved difficult to apply in the human chronic pancreatitis model. Our four patients became insulin-dependent, with proof of intrahepatic insulin production in only one patient. Three factors have contributed to the lack of graft success: 1) the preoperative endocrine status, 2) systemic hypotension and portal hypertension secondary to graft infusion, and 3) difficulty applying the successful technique in a normal dog pancreas to an extensively scarred human pancreas. The preoperative insulin response during a glucose tolerance test was blunted or delayed in the three patients tested. An immediate decrease in blood pressure and rise in portal pressure occurred in every patient and prevented infusion of the entire graft (30-50%) in three patients. Unfortunately, the patient with the most compromised insulin status was the only patient able to receive the entire graft. Our experience would indicate that further refinements in technique are necessary to prevent the vascular reaction and allow infusion of the entire graft. Furthermore, normal islet cell function is necessary before a successful graft can be expected.

Seventy-four patients underwent operation for chronic pancreatitis during a 22 year period at UCLA Hospital. Follow-up data obtained for 60% of these patients an average of 3.2 years postoperation were analyzed by computer for statistically significant benefit between paired operation combinations and the variables of pain relief, stool habits, alcohol use, readmission for pancreatitis, and narcotic use. The combined group of total and cephalic pancreaticoduodenectomy proved more effective with respect to pain relief and readmission (p less than 0.05) than the group that had pseudocyst drainage. The comparison of groups that underwent resection or ductal drainage showed no statistical differences for the above variables. Regardless of type of operation, if the patient had evidence of pancreatic calcifications and had abstained from alcohol postoperatively, the likelihood of a return to normal activity was more favorable (p less than 0.05).

A computerized analysis of prognostic variables was performed in 96 proven cases of extrahepatic bile duct carcinoma treated over a 24-year period at UCLA. Forty-nine percent of the lesions were in the upper third of the bile ducts and 47% of these were resected, for an operative mortality rate of 23% and a maximum survival rate of 4.5 years. Palliative procedures in this region were associated with a 16% mortality rate and maximum survival rate of three years. The patients whose lesions were in the middle third suffered no operative mortality rate for resection or palliation and had a 12% five-year survival rate, with the longest survivor lasting 11 years. In the lower third lesions, 67% were resected by Whipple's procedures, for an 8% mortality rate and a five-year survival rate of 28% extending to nine years. Resection of these difficult carcinomas offers the best hope of survival but must be weighed against the high operative mortality risk in those lesions located in the hilar region.

Traditional management of patients with primary sclerosing cholangitis (PSC) has included steroids 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.