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FIGURE 7.26. Management algorithm for biliary tract injury. Abbreviations: ERCP, endoscopic retro- grade cholangiopancreatography; GB, gallbladder; RUQ, right upper quadrant.
FIGURE 7.27. Microscopic appearance of chronic sclerosing cholangitis, in which dense scarring replaces bile ducts, sometimes referred to as the disappearing duct syndrome. (Courtesy of Linda D. Ferrell, MD.)
RECURRENT PYOGENIC CHOLANGITIS
Over 85% of patients with recurrent pyogenic cholangitis are under 50 years of age, and associated malnutrition and low socioeconomic status are common. Approximately 25% of patients have Clonorchis sinensis ova in their stool. Another 13% to 20% have ascariasis. The most common clinical presentation is that of Charcot’s triad: RUQ pain, fever and chills, and jaundice.Attacks are intermittent and may occur from every week to every several months. Severe attacks can lead to septic shock and death. Approx­imately 20% of patients have hepatomegaly and 9% a palpable gallbladder. Chronic acalculous cholecystitis has been reported in as many as 40% of patients.
Investigations
Laboratory Studies
The findings are those of obstructive jaundice with high alkaline phosphatase. Leukocytosis and eosinophilia may be present, and blood culture may be positive for gram­negative organisms and anaerobes.
Imaging Studies
Ultrasound is useful if there are dilated ducts but not as useful as ERCP or THC. CT scan may demonstrate duct dilatation, hepatic atrophy, and intraductal stones (Figure
7.28). About 80% of cases have stones, both intrahepati­cally and extrahepatically. These stones are the pigment
type. The most distinctive features are biliary strictures, most commonly hilar stricture involving the left hepatic duct, followed by common duct stricture. Typically, stric­tures occur at several levels, with some dilatation of the duct between levels, giving a beaded appearance. Intra­hepatic biliary stricture is common, particularly in the left system. Hepatic abscess, hepatic fibrosis, and atrophy may also be present.
Treatment
The principles of surgical treatment are to achieve ade­quate drainage, remove as many stones as possible, and create biliary-enteric anastomoses to allow other stones to empty out and provide ready future access to the biliary tract.
The surgical techniques used include Roux-en-Y hepaticojejunostomy, with the proximal end of the Roux limb brought out in the RUQ as a fistula or a wide chole­dochoduodenostomy to allow endoscopic intubation of the CBD when needed (Figure 7.29). Occasionally, the left hepatic lobe may be severely abscessed or severely atro­phied enough to require resection.
Reoperation is required in approximately 75% of patients unless the biliary tree can be accessed through a jejunal fistula or choledochoduodenostomy to facilitate removal of recurrent stones endoscopically. The mortality rate, which is related to duration of disease and number of reoperations, is 5% in 5 to 10 years and 12.5% in 10 to 20 years.
9
FIGURE 7.28. Recurrent pyogenic cholangitis. This CT scan illustrates some of the common features, including dilated intrahepatic ducts and stones or debris in several ducts (arrows). (Courtesy of Henry I. Goldberg, MD.)
230 .................................................................................................................................... Biliary T ract
FIGURE 7.29. Roux-en-Y hepaticojejunostomy for oriental cholangiohepatitis. The end of the Roux limb is brought out to the abdominal wall to provide subsequent access in the event of recurrent stones.
Clinical Picture
Most gallbladder cancers are found incidentally at the time of cholecystectomy for gallstones. The tumor may represent a polypoid mass or diffuse thickening of the gallbladder wall (Figure 7.30), or it may have spread extensively to lymph nodes or invaded adjacent organs. If symptoms are present preoperatively, they are indistin­guishable from those due to gallstones, although weight loss, anorexia, and jaundice in the absence of choledo­cholithiasis may be suggestive of the diagnosis.
Tr e at m e n t
When the lesion is resectable, cholecystectomy is the treat­ment of choice, with or without regional lymph node dis­section and wedge excision of the liver at the GB fossa. More radical operations have been recommended, but there are no data to justify them. Often the tumor is advanced at the time of surgery and resection is not possible. Occasionally, the presence of cancer is detected postoperatively by the pathologist in the gallbladder speci­men. What should be done? No data exist to defini­tively indicate that reoperation is indicated to perform lymphadenectomy and limited hepatic resection. The approach, however, appears reasonable unless the tumor is only intramucosal, in which case the patient has been ade­quately treated with cholecystectomy alone. The progno­sis is grim, with a 5-year survival rate of less than 15%.
11
NEOPLASMS OF THE BILIARY TRACT
Neoplasms of the intrahepatic ducts and of the hilum of the liver were discussed in Chapter 6. This section dis­cusses carcinomas of the gallbladder and the extrahepatic bile ducts.
Carcinoma of the Gallbladder
Carcinoma of the GB is the fifth most common malig­nancy in the gastrointestinal tract and has a dismal prog­nosis. The incidence is highest in Israel, Bolivia, Chile, and in native Americans in the sourthwestern United States. The disease occurs most commonly in people over the age of 65 years. The etiology is unknown, but the pattern of distribution among native Indians in both North and South America suggests a strong genetic cause. Gallstones, closely associated with gallbladder cancer, are found in 70% to 90% of patients with cancer. But gallbladder cancer is seen in only 0.5% to 3.0% of patients with gallstones, while the incidence increases to 20% when the gallbladder is calcified or porcelain. The incidence of GB cancer in cholelithiasis is not high enough to justify prophylactic cholecystectomy in asymptomatic patients. Histologically, the tumor is adenocarcinoma, most often scirrhous. In 15% of patients, the tumor is papillary. The spread is locoregional to the lymphatics. Direct invasion of the liver, duodenum, or stomach occurs frequently.
Extrahepatic Bile Duct Tumor or Cholangiocarcinoma
Primary bile duct tumors are usually adenocarcinomas involving the common hepatic or common bile ducts. They may involve the bifurcation of the hepatic duct (hilar); the proximal, middle or distal third; or the ampullary region (Figure 7.31). These tumors are more common in patients with ulcerative colitis and sclerosing cholangitis. Tumors at the hepatic hilum present a greater surgical challenge and are often unresectable.
10
The classic presentation is progressive, painless jaun­dice, pruritus, anorexia, and weight loss. RUQ or deep epi­gastric discomfort is often present. Hepatomegaly may be present, but the tumor itself is rarely palpable. When the tumor involves the distal common bile duct with a patent cystic duct, an enlarged gallbladder may be palpable (Courvoisier’s sign). Malignant bile duct obstruction may be complicated by cholangitis, but the incidence is not high except following ERCP or THC when prophylactic antibiotic coverage has not been used.
Investigations
L
ABORATORY FINDINGS
The serum bilirubin level is
generally markedly elevated (>10mg/dl), as is the alkaline phosphatase. Hepatocellular dysfunction, if present, is minimal.
C linical D isorders.............................................................................................................................. 231
FIGURE 7.30. Carcinoma of the gallbladder. The CT scan shows a calcified stone in the gallbladder (arrowhead) and a soft tissue mass in the gallbladder wall (small arrows) due to gallbladder carcinoma. (Courtesy of Henry I. Goldberg, MD.)
FIGURE 7.31. Distribution of cholangiocarcinoma in the extrahepatic biliary tract. (Adapted from Blumgart LH. Surgery of the Liver and Biliary Tract, 2nd ed. New York: Churchill Livingstone, 1994.)
232 .................................................................................................................................... Biliary T ract
FIGURE 7.32. The ERCP shows a cholangiocarcinoma at the junction of the right and left hepatic ducts, known as a Klatskin tumor. Notice marked enlargement of intrahepatic ducts, particularly of the left system. (Courtesy of John P. Cello, MD.)
I
MAGING STUDIES
Ultrasound, usually performed as the first imaging study, shows dilated intra- and extrahep­atic ducts depending on tumor location. Ultrasound exam­ination, however, rarely provides adequate information about the primary pathology. MRC has emerged as the best noninvasive imaging technique (Figure 7.14) and sup­plants THC. On the other hand, ERCP has the advantage of enabling histological diagnosis from biopsy or brush­ing. Generally, ERCP is most useful for distal bile duct tumors, while THC is preferred over CT scan for proximal tumors. Preoperative celiac angiography is useful in determining operability by showing whether the portal vein is involved.
Types of Bile Duct Tumors
H
ILAR CHOLANGIOCARCINOMA OR KLATSKIN TUMOR
In hilar cholangiocarcinoma, also known as Klatskin tumor, the resectability rate is less than 20% (Figure 7.32). Nonetheless, all patients should undergo surgical explo­ration unless inoperability has been established by imaging studies. At exploration, the tumor is not resectable if any of the following circumstances are found: (1) presence of peritoneal metastasis; (2) invasion of adjacent structures; (3) invasion of portal vein, left and right portal veins, or hepatic arteries; and (4) presence of tumor within second­order biliary radicles of both hepatic lobes.
ROXIMAL AND MIDDLE THIRD EXTRAHEPATIC BILE
P
D
When resectable, these tumors are amenable to
UCT
excision of the hepatic and common ducts and Roux-en-Y hepaticojejunostomy (Figure 7.33).
ISTAL THIRD BILE DUCT AND PERIAMPULLARY LESIONS
D
The resectability rate for these lesions is greater than 50%, and the surgical treatment of choice is pancreaticoduo­denectomy, either of the pylorus-sparing type or the classic Whipple resection. Five-year survival rates of 30% to 49% have been reported for periampullary cholangiocarcinoma after curative pancreaticoduodenectomy.
12
Tr e at m e n t
The essentials of treatment are listed in Table 7.12. Treat­ment varies by type of tumor, and surgical procedure depends on tumor location. Hilar lesions present the greatest surgical challenge and the worst outcome.
ESECTION FOR CURE Extent of resection depends on
R
the portion of the proximal extrahepatic biliary system involved. The modified Bismuth–Corlett classification of hilar tumors provides a useful anatomic guide for the required resection (Figure 7.34). Type I and II tumors may be removed without the need to perform hepatic resection. Biliary-enteric anastomosis can be accomplished between either the hepatic duct (Type I) or the right and left hepatic ducts (Type II) and a Roux limb of the jejunum. Type III lesion, if resectable, requires either right (IIIA) or left (IIIB)
C linical D isorders.............................................................................................................................. 233
A
B
FIGURE 7.33. (A) Gross appearance of a large intraductal papillary cholangiocarcinoma of the common bile duct. (B) Microscopically, papillary projection of the tumor is evident. The appearance contrasts with the usual manifestation of cholangiocarcinoma, in which extensive fibrosis is seen with only islands of adenocarcinoma cells. In addition, an intraductal papillary carcinoma may appear large but be attached to the CBD wall through a relatively small pedicle. (Courtesy of Linda D. Ferrell, MD.)
234 .................................................................................................................................... Biliary T ract
TABLE 7.12. Essentials: Treatment of Bile Duct Tumors
Hilar cholangiocarcinoma (Klatskin tumor)
Resectability rate: 20%
Type I and II: Tumor resection with Roux-en-Y hepaticojejunostomy
Type III: Resection of tumor with left or right hepatectomy, caudate lobectomy and Roux-en-Y hepaticojejunostomy
Carcinoma of proximal and middle third
Excision of hepatic and common ducts and Roux-en-Y hepaticojejunostomy
Carcinoma of distal third
Resectability rate: 50%
Procedures: Pancreaticoduodenectomy
5-year survival rate: 20%–60%
Unresectable tumor
Biliary decompression: Surgical (U-tube) or transhepatic or endoscopic stenting
Adjuvant therapy: External or local (
Chemotherapy of little use
192
Ir wire) radiation
hepatic lobectomy for cure (Figure 7.35). Caudate lobe resection may also be required. Some surgeons have per­formed total hepatectomy and orthotopic liver transplan­tation after chemoradiation in selected cases. this treatment is unproven.
13
The value of
NRESECTABLE TUMORS If the tumor cannot be
U
resected, decompression of the biliary tree is required. Several surgical procedures were in use prior to the advent of percutaneous stenting. At the time of surgery, U-tube stenting can be performed. The procedure requires identi­fication of the obstructed left or right hepatic duct system. A silastic tube is placed through the abdominal wall, through the dome of the liver, across the tumor, out of the common bile duct, and through the abdominal wall. The segment of the tube within the bile ducts has multiple per­forations. Such tubes tend to be obstructed with sludge but are easily replaceable without operation. Other alternatives, now more commonly used, are placement of stents endo­scopically or transhepatically. Endoscopic or transhepatic stenting are equally effective, and selection of one over the other usually depends on the type of expertise available at the institution.
UTCOME The best 5-year survival rates after curative
O
resection of hilar cholangiocarcinoma have been 30% with an operative mortality of 4%.
5
The 5-year survival after stenting alone is about 5%. Although considerable debate exists about the relative value of surgical and non­surgical treatment, the only chance of a cure is surgical resection.
DJUVANT THERAPY Cholangiocarcinoma is resistant
A
FIGURE 7.34. Bismuth–Corlette classification of hilar tumors. (Adapted with permission from Bismuth H, Nakache R, Diamond T. Management strategies in resection for hilar cholangiocarci­noma. Ann Surg 1992;215:31–38.
C linical D isorders.............................................................................................................................. 235
to both radio- and chemotherapy. External radiation and local radiation with
192
Ir wire have been reported to be of some benefit, and postoperative radiation may reduce recurrence rates.
A
B
FIGURE 7.35. (A) Gross appearance of hilar cholangiocarcinoma at the confluence of the right and left ducts. (B) Microscopically, there is dense stroma, little inflammation, and few isolated tumor cells. (Courtesy of Linda D. Ferrell, MD.)
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