Ann Surg 1998 Mar;227(3):412-23

Primary sclerosing cholangitis: resect, dilate, or transplant

Ahrendt SA, Pitt HA, Kalloo AN, Venbrux AC, Klein AS, Herlong HF, Coleman J,
Lillemoe KD, Cameron JL
Department of Surgery, The Johns Hopkins Medical Institutions, Baltimore,
Maryland, USA
Abstract
OBJECTIVE: The current study examines the results of extrahepatic biliary
resection, nonoperative endoscopic biliary dilation with or without percutaneous
stenting, and liver transplantation in the management of patients with primary
sclerosing cholangitis (PSC). SUMMARY BACKGROUND DATA: Primary sclerosing
cholangitis is a progressive inflammatory disease leading to secondary biliary
cirrhosis. The most effective management of sclerosing cholangitis before the
onset of cirrhosis remains unclear. METHODS: From 1980 to 1994, 146 patients
with PSC were managed with either resection of the extrahepatic bile ducts and
long-term transhepatic stenting (50 patients), nonoperative endoscopic biliary
dilation with or without percutaneous stenting (54 patients), medical therapy
(28 patients), and/or liver transplantation (21 patients). RESULTS:
Procedure-related morbidity and mortality rates were similar between surgically
resected and nonoperatively managed patients. In noncirrhotic patients, the
serum bilirubin level was significantly (p < 0.05) reduced from preoperative
levels (8.3+/-1.5 mg/dL) 1 (1.7+/-0.4 mg/dL) and 3 (2.7+/-0.9 mg/ dL) years
after resection, but not after endoscopic or percutaneous management. For
noncirrhotic PSC patients, overall 5-year survival (85% vs. 59%) and survival
until death or transplantation (82% vs. 46%) were significantly longer (p <
0.05) after resection than after nonoperative dilation with or without stenting.
For cirrhotic patients, survival after liver transplantation was longer than
after resection or nonoperative dilation with or without stenting. Five patients
developed cholangiocarcinoma, including three (6%) of the nonoperatively managed
patients but none of the resected patients. CONCLUSIONS: In carefully selected
noncirrhotic patients with PSC, resection and long-term stenting remains a good
option. Patients with cirrhosis should undergo liver transplantation