V-GASTROINTESTINAL DISEASE



tlt.iAt and antibiotics and emergency surgery to £2n the biliary tree. The high mortality of 50 per Smt for this disease reflects the age of the patients cenerallv affected, the speed with which sepsis develops, and the frequent failure to identify the biliary tree as the source of sepsis.
Other Disorders of the Biliary Tree. A number of other processes, all of which may present with biliary obstruction, jaundice, or infection, may in-volve’the biliary tree. The approach to evaluating these entities is outlined in Chapter 37.
Benign biliary strictures usually result from sur­gical injury and may cause symptoms days to years later. Early diagnosis is important, as stric­tures that partially obstruct and are clinically asymptomatic may cause secondary biliary cir­rhosis. Biliary stricture should be suspected in anyone with a history of right upper quadrant sur­gery and a persistently elevated serum alkaline phosphatase level. A similar type of benign stric­ture is seen in alcoholics in whom the intrapan-creatic portion of the common bile duct is com­pressed by pancreatic fibrosis. Surgical repair or bypass of these lesions is successful in 75 per cent of patients. Balloon catheter dilation may be use­ful in selected individuals.
Sclerosing cholangitis is an idiopathic condi­tion of nonmalignant, nonbacterial chronic in­flammatory narrowing of the intra- and extrahe-patic bile ducts. It most commonly occurs in males in the third and fourth decades of life, often in association with ulcerative colitis. Patients usually present with pruritus or jaundice, and percutaneous transhepatic cholangiography or endoscopic retrograde cholangiopancreatography shows characteristic changes (”beading”) of the extrahepatic bile ducts. Therapy is supportive.
Structural abnormalities such as choledochal cysts, Caroli’s disease (saccular intrahepatic bile duct dilation), and duodenal diverticuli may also cause bile duct obstruction, often with secondary choledocholithiasis. Hemobilia and intermittent bile duct obstruction by blood clots are caused by hepatic injury, neoplasms, or hepatic artery aneu­rysms.
Biliary neoplasms are rare, but include carci­noma of the gallbladder, scirrhous or papillary ad­enocarcinoma of the bile ducts, and carcinoma of the ampulla of Vater. The latter two neoplasms usually present as unremitting painless jaundice, although necrosis and sloughing of tumor may cause intermittent obstruction and the appearance of occult fecal blood. The term “Klatskin tumor” specifically refers to an adenocarcinoma located at the bifurcation of the common bile duct. Car­cinoma of the gallbladder often presents as ad­vanced disseminated disease, although symptoms also may resemble acute or chronic cholecystitis or bile duct obstruction. Resection of most of these tumors is difficult or impossible and prognosis is poor. For patients with unresectable tumor the goal is palliation, and for those with severe symp­toms due to obstruction, percutaneous or endo­scopic stenting of the biliary tree may be helpful. Stents should not be used in patients with benign disease because complications due to biliary in­fection and plugging of the stent invariably occur; rather, definitive surgical repair or bypass is pre­ferred.
Motility disorders of the biliary tree have not been well recognized in the past. With the use of newer endoscopic techniques for measuring bil­iary pressures and motility, it has become appar­ent that a small group of patients with biliary pain may have symptoms due to hypertension, dys-motility, and/or stenosis of the sphincter of Oddi. However, at this time there is only limited evi­dence to suggest that surgical or endoscopic treat­ment (sphincterotomy) is of value.





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