NMS. Surgery

207

Chapter 10 ♦ Liver, Gallbladder, and Biliary Tree Disorders

PRIMARY SCLEROSING CHOLANGITIS I. Etiology:

A. Progressive inflammation and stenosis of the biliary tree. B. Leads to biliary cirrhosis. C. Associated with ulcerative colitis. D. Exact cause unknown. II. Clinical presentation: Jaundice, pruritis, fatigue, liver failure. III. Diagnosis: A. LFTs B. ERCP and MRCP C. Liver biopsy IV. Treatment: A. Symptom management B. Only treatment is liver transplant, however, PSC can recur. V. Prognosis: A. Disease can be progressive. B. Increased risk of cholangiocarcinoma. PRIMARY BILIARY CHOLANGITIS I. Etiology: A. Autoimmune disease. B. Progressive bile duct destruction and liver failure. II. Clinical presentation: A. Fatigue, pruritis, xanthomata, jaundice, osteoporosis. B. Females > males. III. Diagnosis: A. Labs: LFTs and anti-mitochondrial antibody. B. Liver biopsy IV. Treatment: A. Medical: 1. Symptom management. 2. Some potential survival benefit with ursodeoxycholic acid. B. Surgery: If liver failure develops, consider liver transplant. V. Prognosis: A. Depends on disease progression, usually reflected in bilirubin levels. B. Disease can recur after transplant. C. Patients are also at increased risk of HCC. CHOLEDOCHAL CYSTS I. Etiology: Congenital dilations of the biliary tree. II. Classification: A. Type I 1. Most common. 2. Dilation of the extrahepatic bile ducts. 3. Can be cystic, focal, or fusiform.