title: Primary sclerosing cholangitis: role of extrahepatic biliary resection.
abstract: BACKGROUND
  Most centers advocate orthotopic liver transplantation (OLT) for patients with primary sclerosing cholangitis (PSC) and cirrhosis. Management of PSC patients without cirrhosis remains controversial. We examined the results of extrahepatic biliary resection (EHBR) for PSC.
  
  
  METHODS
  Between 1981 and 2006, 126 patients with PSC underwent EHBR (n = 77) or OLT (n = 49). Data on biliary drainage procedures, perioperative morbidity, and longterm survival were collected and analyzed.
  
  
  RESULTS
  Of 77 patients undergoing EHBR, mean preoperative bilirubin level was 5.6 mg/dL. Nine (11.7%) patients had cirrhosis. Most patients had preoperative biliary drainage (ERCP, 61.0%; PTC, 67.5%). At operation, 73 (94.8%) patients underwent EHBR, including hepatic duct bifurcation. Most patients also had insertion of bilateral transhepatic silicone elastomer biliary stents; 4 (5.2%) underwent EHBR with stent insertion plus hepatectomy. For EHBR patients, perioperative complication rate was 38.7% and 30-day mortality was 3.9%. Bilirubin levels significantly decreased postoperatively (mean drop 3.8 mg/dL; p < 0.01). At 3 years, 57.1% of patients had no PSC-related readmissions, and 16.2% had more than 3. At a median followup of 10.5 years, 5- and 10-year survival was 76.4% and 52.7%, respectively. Cholangiocarcinoma did not develop in any patients, and only seven required OLT. Factors associated with worse survival included postoperative bilirubin >or= 2 mg/dL and history of cirrhosis (both p < 0.001). In patients undergoing EHBR, noncirrhotic patients had significantly better longterm outcomes versus cirrhotic patients (10-year survival, 60.2% versus 12.0%; p < 0.001). In contrast, 10-year survival of OLT patients with cirrhosis was 57.0%.
  
  
  CONCLUSIONS
  Noncirrhotic patients with PSC can be successfully managed with EHBR. EHBR for noncirrhotic patients is associated with low perioperative morbidity, few readmissions, no new cholangiocarcinomas, and 10-year survival > 60%. OLT should be reserved for patients with PSC and associated hepatic cirrhosis.
authors.affiliation: Department of Surgery, Johns Hopkins University Hospital, Baltimore, MD 21287, USA. tpawlik1@jhmi.edu
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Pawlik
authors.forename: Vanessa A
authors.initials: VA
authors.lastname: Olbrecht
authors.forename: Henry A
authors.initials: HA
authors.lastname: Pitt
authors.forename: Ana L
authors.initials: AL
authors.lastname: Gleisner
authors.forename: Michael A
authors.initials: MA
authors.lastname: Choti
authors.forename: Richard D
authors.initials: RD
authors.lastname: Schulick
authors.forename: John L
authors.initials: JL
authors.lastname: Cameron
mesh_terms: D000328:Adult; D017734:Bile Ducts, Extrahepatic / Q000601:surgery*; D001662:Biliary Tract Surgical Procedures / Q000706:statistics & numerical data*; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008103:Liver Cirrhosis / Q000209:etiology; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D009017:Morbidity; D016019:Survival Analysis