Ch. 54 – Biliary System

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1578 Section X Abdomen abdominal mass. Only 10% of patients present with this triad. Adults have a slightly different presentation (abdominal pain and jaundice) than children because of a higher incidence of bile calculi or sludge and pancreatic-biliary ductal malformation. Severe hepatobiliary complications may result from long-standing biliary cysts if left untreated. Portal hypertension may develop as a result of portal vein compression by the adjacent cyst or cirrhosis secondary to long-term biliary obstruction. Some patients may present with variceal hemorrhage as an initial manifestation. Very rarely, patients may present with bilious ascites and peritonitis as a result of rupture of a choledochal cyst, and pseudocysts may appear surrounding the common bile duct. Those patients also have a high incidence of developing sludge, cholelithiasis, or choledocholithiasis and have commonly had a prior cholecystectomy. The incidence of carcinoma (bile duct, hepatic, or gallbladder in origin) in the choledochal cyst ranges from 2.5% to 26%, which is well above the rate of less than 1% for the general population. Many patients have biliary cancer at the time of initial presentation. Chronic infl ammation caused by bile stagnation has been suggested as a possible mechanism for the development of cancer by causing metaplasia of the epithelium of the cystic wall. Laboratory evaluation may demonstrate liver dysfunction in 60% of adult patients but is not specifi c. The diagnosis can be established with ultrasound or CT scan (Fig. 54-30A). Cholangiography is required to determine the type of choledochal cyst and plan operative treatment (see Fig. 54-30B). ERCP is more useful in defi ning the distal ductal anatomy and the presence of APBDJ, whereas PTC is useful in defi ning the proximal ductal anatomy and the presence of intrahepatic disease. Management The goals of management are to relieve symptoms and prevent long-term complications of biliary cysts such as cholangitis, portal hypertension, cirrhosis, and potential carcinoma. Cholecystectomy, resection of the extrahepatic biliary tract including the choledochal cyst, with Roux-en-Y hepaticojejunostomy is the appropriate treatment for type I and II choledochal cysts. The goal of cyst excision is to completely remove the lining mucosa of the bile duct cysts (Fig. 54-31), with oversewing of the distal duct. Simple enteric drainage (choledochocystojejunosotomy) of the bile duct cyst is associated with recurrent biliary stasis and infection 24 and does not prevent development of biliary malignancy. Todani and colleagues have reported a 10-year mean interval between internal drainage and detection of carcinoma. 41 Cholangiocarcinoma is uncommon in children with choledochal cysts, but the risk in adults may be as high as 30%. Resection of the extrahepatic cyst is also recommended for type IV cysts; if the intrahepatic cysts are confi ned to one side of the liver, hepatic resection of the involved liver is recommended. With long-standing Caroli’s disease (Fig. 54-32), hepatic parenchymal damage may ensue from diffuse biliary dilation and periportal fi brosis and eventually result in cirrhosis and liver failure. Liver trans- A B Figure 54-30 A, Computed tomography scan demonstrating type I choledochal cyst. B, Endoscopic retrograde cholangiopancreatography visualizing anatomy and relationship to nearby structures of choledochal cyst is important in operative planning. (From Shah SA, Alsoufi B, Callery MP: Bile duct cysts. Available online: http://www.unboundsurgery.com.) plantation offers a potential cure without long-term complications of recurrences of Caroli’s disease. Polyploid Lesions of the Gallbladder Benign masses classifi ed as polyploid lesions of the gallbladder include benign pseudotumors, such as cholesterol polyps and adenomyomatosis, and adenomas, and appear in 3% to 7% of normal subjects undergoing abdominal ultrasound and in 2% to 12% of cholecystectomy specimens. Cholesterol polyps are the most common
Figure 54-31 Operative photography demonstrating neck of biliary cyst during excision of a type I choledochal cyst. Figure 54-32 Computed tomography demonstrating Caroli’s disease. benign masses of the gallbladder and are usually smaller than 10 mm, have a characteristic echogenic pedunculated appearance on ultrasound, and are often multiple (30% of cases). Adenomyomatosis appears as a sessile polyp with characteristic microcysts on ultrasound and is often larger than 10 mm. Adenoma may be diffi cult to distinguish from adenocarcinoma of the gallbladder; the main differentiating feature is a lack of transmural invasion on ultrasound, which is sometimes diffi cult to accurately assess. Risk factors associated with malignancy are age older than 60 years, coexistence of gallstones, a documented increase in size, and size larger than 10 mm. All patients with symptomatic polyploid lesions of the Chapter 54 Biliary System 1579 gallbladder should undergo laparoscopic cholecystectomy. Any patient with risk factors or a suspicion of in situ or frank adenocarcinoma should undergo open cholecystectomy. Lesions smaller than 10 mm that are asymptomatic and without ultrasound features of neoplasia may be safely observed with follow-up imaging. Benign Biliary Masses The most common benign tumor of the extrahepatic biliary tree is the papilloma or adenoma, which arises from the glandular epithelium lining the ducts. Most of these tumors are found in or close to the ampulla (47%). Jaundice is the presenting symptom in most patients but can also be accompanied by right upper quadrant pain. A benign adenomatous tumor should be included in the differential in all secondary operations performed for obstruction of the biliary system. It is a soft lesion, is diffi cult to palpate, and presents very little resistance to ductal probes. Treatment should consist of total resection of symptomatic lesions with some duct wall as well. High recurrence rates have been reported after simple curettage of the polyps. Lesions situated at the ampulla can usually be managed by transduodenal papillotomy or wide local excision. Benign infl ammatory tumors of the bile duct, known as pseudotumors and benign fi brosing disease can mimic biliary tract tumors. Their histology consists of only infl ammatory cells and fi brosis. Pseudotumors appear to occur most frequently above the biliary bifurcation but are still usually extrahepatic. About 10% of resected cholangiocarcinomas are pseudotumors in origin. MALIGNANT BILIARY DISEASE Gallbladder Cancer Cancer of the gallbladder is an aggressive malignancy that occurs predominantly in elderly people. Besides the exceptional cases detected incidentally at the time of cholecystectomy for gallstone disease, which are usually early stage, the prognosis for most patients is poor. Series from the Western hemisphere have reported 5-year survival rates of only 5% to 38%. 42-44 Unfortunately, many of these tumors are unresectable at presentation, and most must be managed nonoperatively. Recently, an aggressive surgical approach for patients with localized gallbladder cancer has produced encouraging results with an acceptable morbidity. Incidence Gallbladder cancer is the fi fth most common gastrointestinal malignancy, with about 5000 new cases diagnosed annually in the United States. Cancer of the gallbladder is two to three times more common in women than men, in part because of the higher incidence of gallstones in women. More than 75% of patients with this malignancy are older than 65 years. The incidence of gallbladder cancer varies considerably with both ethnic background and geographic location. In the United States, gallbladder cancer is more common in Native Americans. Similarly,
Page 1 and 2: Biliary System Ravi S. Chari, MD an
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Ch. 54 – Biliary System

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