The Changing Role of Surgery for Sclerosing Cholangitis

Abstract

Primary sclerosing cholangitis is a chronic cholestatic disease that may ultimately progress to hepatic parenchymal dysfunction and death from premature liver failure. Symptomatic patients should undergo radiological evaluation to exclude secondary causes of cholangitis and to evaluate the extent and location of biliary ductal disease. A percutaneous liver biopsy is advisable in any patient with clinical or biochemical evidence of cirrhosis. Patients with diffuse ductal involvement should initially receive medical treatment, preferably in a center conducting prospective clinical trials. Liver transplantation should be considered the initial procedure in primary sclerosing cholangitis patients with diffuse ductal involvement, complications of cirrhosis or deteriorating liver function. Nontransplant procedures should be restricted to symptomatic patients with a dominant extrahepatic stricture. In properly selected patients, effective and durable palliation of symptoms can be anticipated with biliary enteric drainage. Many currently available techniques obviate the need for long-term transanastomotic stenting, thus minimizing the risk of recurrent cholangitis and the need for repeated tube changes. These patients may also be candidates for percutaneous or endoscopic balloon cholangioplasty. Liver transplantation should also be considered in patients with a dominant stricture and histologic evidence of biliary cirrhosis or hepatic deterioration following a nontransplant procedure.

Predictors of Successful Clinical and Laboratory Outcomes in Patients with Primary Sclerosing Cholangitis Undergoing Endoscopic Retrograde Cholangiopancreatography

“…PSC is a progressive disorder for which liver transplantation is the final therapeutic option in select patients (6,12,13,30). The role of ERCP as a diagnostic tool is well established, but its use for therapy should be selective because not all patients will benefit.…”

Primary sclerosing cholangitis: diagnostic and management challenges

“…PSC may also be managed surgically, and options include biliary reconstructive procedures, proctocolectomy (in patients with ulcerative colitis), and LT. 76 …”

Measurement of parenchymal function and bile duct flow in primary sclerosing cholangitis using dynamic 99mTc‐HIDA SPECT

“…An association with inflammatory bowel disease, usually ulcerative colitis, has been reported 3–7 . The median survival from time of diagnosis is 9–12 years, and in untreated patients will lead to premature death in 30–40% 8–10 . Five to 20% of patients can be expected to develop cholangiocarcinoma in the course of the disease 11–14 .…”

“…There is currently no effective or specific medical treatment for PSC, the only definitive treatment being liver transplantation. There are, however, many controversies regarding patient selection for, and timing of, transplantation 8,15 …”