Management and course

Small intraduodenal choledochoceles are best treated by endoscopic sphincterotomy but all other biliary cysts require surgical therapy. For extrahepatic cysts, excision and drainage is preferable to drainage alone because of the risks of recurrent cholangitis and malignant transformation. Resection is the preferred treatment for localized intrahepatic cysts The patient with diffuse intrahepatic cysts may require hepatic transplantation if hepatic failure or portal hypertension develops. Chronic antibiotic therapy may reduce the risk of recurrent cholangitis, particularly in Caroli disease.

In addition to cholangitis, pancreatitis (type III), biliary cirrhosis, and liver abscesses may complicate the course of disease in patients with biliary cysts. Cyst rupture during pregnancy or labor has prompted the recommendation that pregnant women with symptomatic cysts deliver by cesarean section. The most feared complication is malignant degeneration. This risk is particularly high for adult patients, as 15% develop carcinoma. Carcinoma may occur throughout the biliary tract and pancreas, including the gallbladder and sites uninvolved by the cysts. The prognosis of these tumors is dismal. Almost all patients die soon after diagnosis.

Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a disorder of the bile ducts involving chronic inflammation leading to structuring of bile ducts and liver fibrosis. Patients with PSC have a high prevalence of ulcerative colitis (up to 90%). PSC may eventually lead to end-stage liver disease.

Clinical presentation

The onset of PSC is insidious, and it can be diagnosed when asymptomatic elevations in serum liver biochemistry values are detected. Alternatively, symptomatic patients may have progressive fatigue, pruritus, weight loss, and jaundice for an average of 2 years before a diagnosis is made. Cholangitis is uncommon and often indicates the presence of superimposed choledocholithiasis or bile duct carcinoma. Patients with associated inflammatory bowel disease are more likely to have both intrahepatic and extrahepatic ductal disease. Isolated involvement of the extrahepatic ducts is more common in patients without inflammatory bowel disease (38%) than in patients with inflammatory bowel disease (7%). A small percentage of patients with PSC have other associated immune disorders (e.g. Sjögren syndrome, hereditary acquired immunodeficiency syndromes, or the antiphospholipid antibody syndrome) (Table 34.1). PSC should be differentiated from other causes of chronic cholestasis, including primary biliary cirrhosis, autoimmune hepatitis, and recurrent pyogenic cholangitis (oriental cholangiohepatitis).

Table 34.1 Disorders associated with primary sclerosing cholangitis

Disorder	Prevalence (%)
Inflammatory bowel disease
Chronic ulcerative colitis	50–75
Crohn’s disease	<5
Immunodeficiency syndromes
Angio-immunoblastic lymphadenopathy	Rare
Acquired immunodeficiency syndrome	Rare
Familial immunodeficiency syndromes	Rare
Miscellaneous
Recurrent pancreatitis	4–25
Antiphospholipid antibody syndrome	Rare
Sjögren syndrome	Rare
Rheumatoid arthritis	Rare
Retroperitoneal fibrosis	Rare