Medical Therapy
Ursodeoxycholic Acid
Ursodeoxycholic acid has been shown to improve the abnormal biochemical tests and liver histology but does not appear to affect survival or need for transplantation. Its mechanism of action is unknown but it increases the biliary secretion of bile acids and increases bile flow. Ursodeoxycholic acid prevents damage of liver cell membranes to toxic concentrations of chenodeoxycholic acid in in vitro studies.
Other Drug Therapy
Immunosuppressive and antiinflammatory agents have not been shown to improve the outcome of primary sclerosing cholangitis. These include the use of steroids and penicillamine.
Endoscopic Therapy
The goal of endoscopic therapy is dilation of strictures to a point that bile flow improves. This should result in an improvement in jaundice and decreased episodes of cholangitis . Traditionally, endoscopic therapy has been directed to patients with dominant strictures of the extrahepatic biliary tree. Unfortunately the presence of a dominant stricture occurs in only 10%–15% of patients with primary sclerosing cholangitis. At the Johns Hopkins Hospital, we have utilized the technique of aggressive endoscopic therapy in patients with (or without) diffuse stricturing disease but without cirrhosis. The first step is endoscopic biliary sphincterotomy to facilitate passage of the balloon dilators (Figure 16). | | Figure 16.A-C,Technique of endoscopic biliary sphincterotomy.(Click on the blue letters to view the consecutive images) |
Balloon dilatation of the total extra-hepatic ducts up to and including the common hepatic duct and hepatic bifurcation is then performed . Dilatation is performed from proximal to distal sequentially dilating the whole extrahepatic tree using high-pressure inflation balloons (up to 150psi) (Figure 17). Endoscopic stenting is used only if the strictures are refractory to therapy and is avoided because the low bile flow rate predisposes to early stent occlusion and cholangitis. Irrigation of the bile duct with steroids or saline has not been shown to be beneficial.
| | Figure 17.A-C,Technique of endoscopic balloon dilatation of biliary strictures.(Click on the blue letters to view the consecutive images) |
The main advantages of endoscopic therapy are that it is relatively non-invasive, can be repeated serially if necessary. Endoscopic therapy may decrease jaundice, pruritis and reduce the frequency of acute cholangitis. Although it does not alter the natural history or obviate the need for liver transplantation, endoscopic therapy may significantly improve the quality of life in patients awaiting transplantation.
Percutaneous Therapy
Interventional radiological techniques may be used in conjunction with an endoscopic or surgical approach. It may be useful in accessing the proximal biliary tree when a high-grade stricture precludes endoscopic visualization or when prior surgery makes endoscopic access difficult. Like the endoscopic approach, high-pressure balloon dilatation and stent placement may be performed. The disadvantage of the percutaneous approach is that it is more invasive than the endoscopic approach and requires an indwelling percutaneous catheter for varying lengths of time that may be uncomfortable to the patient (Figure 18).
Surgical Therapy Non-transplant Surgery
Like the endoscopic approach, the goal of surgery is to improve bile flow, reduce jaundice and prevent further attacks of cholangitis. Non-transplant surgical approaches include resection of the extrahepatic bile ducts with biliary-enteric bypass with or without long-term biliary stenting. Another surgical approach is to resect the extrahepatic bile ducts including the bifurcation, dilate the intrahepatic ducts and then permanently stent the bile ducts with polymeric silicone transhepatic biliary stents (Figure 19).
In general these non-transplant surgical approaches may make liver transplantation technically more difficult and increase the morbidity and mortality of transplantation. However this approach may be useful in patients with high-grade strictures that are suspicious for cholangiocarcinoma.
Transplant Surgery For more information about liver transplantation (Johns Hopkins Comprehensive Liver Transplantation Center)
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