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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)
䊏
Resectability rate: 20%
䊏
Type I and II: Tumor resection with Roux-en-Y hepaticojejunostomy
䊏
Type III: Resection of tumor with left or right hepatectomy, caudate lobectomy and Roux-en-Y
hepaticojejunostomy
Carcinoma of proximal and middle third
䊏
Excision of hepatic and common ducts and Roux-en-Y hepaticojejunostomy
Carcinoma of distal third
䊏
Resectability rate: 50%
䊏
Procedures: Pancreaticoduodenectomy
䊏
5-year survival rate: 20%–60%
Unresectable tumor
䊏
Biliary decompression: Surgical (U-tube) or transhepatic or endoscopic stenting
䊏
Adjuvant therapy: External or local (
䊏
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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