level of obstruction were comparable in both groups. Surgical therapy consisted of Roux-en-Y

hepaticojejunostomy, and endoscopic therapy consisted of placement of an endoprosthesis with

trimonthly elective exchange for 1 year. Successful stent placement was accomplished in 94% of

patients managed endoscopically. Six of the 66 endoscopic patients, however, underwent surgical

reconstruction either for failed stent placement or for other reasons. Early complications occurred more

frequently in the surgically treated group (26% vs. 8%; p < 0.03). However, the only procedure-related

death occurred in a patient in whom severe pancreatitis developed after endoscopic stent placement.

Late complications, which included primarily episodes of cholangitis, occurred only in the endoscopic

group (27%). The overall complication rates, therefore, were similar at 26% for surgical patients and

35% for endoscopic patients. The mean follow-up and definition of success were similar to those in the

aforementioned study. After surgery, excellent results were observed in 83% of patients, with a

recurrent stricture developing in six patients at a mean of 40 months after the initial operation. After

endoscopic stenting, excellent results were observed in 72% of patients, with restricture developing in

18% of patients at a mean of 3 months after stent removal. The investigators concluded that endoscopic

stenting should be considered for the initial attempt at definitive management in suitable patients in the

hope of avoiding reoperation.

The final and most recent comparative study describing 528 patients over 18 years has confirmed a

number of interesting observations.23 More specifically, patients with all types of bile duct injuries were

most commonly treated by endoscopists (40%), followed by surgeons (36%) and interventional

radiologists (24%). Success rates however were higher for surgery (88%) compared to either endoscopy

(76%) or interventional radiology (50%). This observation was accomplished with an overall morbidity

amongst the surgical cohort of 24% with no 90-day mortality. Not surprisingly, outcomes also improved

dramatically over time amongst all approaches. Although the reason for this progress is clearly

multifactorial, issues such as improved selection of patients for each approach, increased experience in

reconstruction at the surgeon level (and therefore, fewer surgeons performing repairs overall), and

perhaps pursuit of more proximal biliary-enteric anastamoses and/or more selective use of transhepatic

stents may each play a role. Taken as a whole, this modern analysis of bile duct injuries confirms the

importance of ensuring significant clinician experience with this complication, a multidisciplinary team

comprised of HPB surgeons, interventional endoscopists and interventional radiologists, and thoughtful

selection of a given patient for the appropriate therapeutic option with the highest chance of long-term

success.

Table 62-5 Diseases Associated with Primary Sclerosing Cholangitis

PRIMARY SCLEROSING CHOLANGITIS

Primary sclerosing cholangitis is an idiopathic disease characterized by intrahepatic and extrahepatic

inflammatory strictures of the bile ducts that cannot be attributed to other specific causes. The cause of

primary sclerosing cholangitis is unknown. Many experts consider primary sclerosing cholangitis to be

an autoimmune reaction because it is associated with other autoimmune diseases, such as ulcerative

colitis, retroperitoneal fibrosis, and Riedel thyroiditis (Table 62-5). It is likely that a number of causes,

including viral or bacterial infections, toxic drug reactions, and congenital anomalies, can all result in

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the same end-stage injury that is recognized as primary sclerosing cholangitis.

The usual clinical presentation of patients with primary sclerosing cholangitis involves intermittent

jaundice, which begins insidiously in the fourth or fifth decade of life. Right upper quadrant pain,

pruritus, fever, weight loss, and fatigue can also occur. The disease is characterized by cyclic remissions

and exacerbations. Despite the nomenclature, acute cholangitis is uncommon without previous biliary

manipulation or surgery. The diagnosis is suggested by clinical presentation associated with cholestatic

liver function test abnormalities. The levels of bilirubin often fluctuate with respect to the remissions

and exacerbations of the disease and the extent of hepatic injury. Alkaline phosphatase is usually

elevated out of proportion to the serum bilirubin, and is a more persistent finding. The diagnosis,

however, usually is confirmed by cholangiography, which reveals multiple dilatations and strictures

(beading) of the intrahepatic and extrahepatic bile ducts (Fig. 62-13). MRCP has become the preferred

procedure as it is noninvasive. ERC, however, remains an important tool for both diagnostic and

therapeutic procedures. An important distinction must be made as cholangiocarcinoma must be

considered in the differential diagnosis of a dominant stricture. Furthermore, cholangiocarcinoma may

develop after presentation in up to 30% of patients with primary sclerosing cholangitis. Therefore,

endoscopic brushings and biopsies are frequently required. The disease should be followed closely by

cholangiography and liver biopsy to provide appropriate management before the development of biliary

cirrhosis.

Figure 62-13. A: Cholangiogram of a patient with primary sclerosing cholangitis. Multiple irregular strictures and dilatation

(beading) of intrahepatic bile ducts can be seen. B: Endoscopic retrograde cholangiogram showing extensive involvement of

extrahepatic bile duct (BD) with primary sclerosing cholangitis. (B reproduced with permission from Lillemoe KD, Pitt HA,

Cameron JL. Primary sclerosing cholangitis. Surg Clin North Am 1990;70:1390.)

No known specific medical therapy is effective for primary sclerosing cholangitis. The most

encouraging results, from a prospective, randomized, placebo-controlled trial, suggest that

ursodeoxycholic acid significantly improves serum liver function tests and liver histologic appearance.

Unfortunately, there were no significant differences in clinical outcome between the two groups at up to

6 years of follow-up.36 Nonoperative dilation therapy by the endoscopic route has been used for

dominant strictures with favorable results.37

8 An aggressive surgical approach is advocated for selected symptomatic patients with primary

sclerosing cholangitis because of the lack of effective medical therapy. One surgical approach, in

patients with a dominant stricture at the hepatic duct bifurcation, uses resection of the bifurcation and

long-term transhepatic stenting with Silastic stents. This mode of therapy was recently reported in 77

patients with resection of the hepatic duct bifurcation, with hepatic lobectomy performed in another

four patients.38 The perioperative complication rate was 39% and 30-day mortality was 3.9%. Bilirubin

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improved and 57% of patients had no primary sclerosing cholangitis–related readmissions. At median

follow-up of 10.5 years, the 5- and 10-year survival rates were 76.4% and 52.7%, respectively.

Cholangiocarcinoma did not develop in any patients and only seven required liver transplant. Over the

same period, liver transplant was performed in 49 patients with cirrhosis with a 10-year survival of

57%.

The role of biliary surgery in primary sclerosing cholangitis, however, has decreased considerably

with the growing success of liver transplantation. Primary sclerosing cholangitis has become one of the

most common indications for liver transplantation in the United States, with 5-year actuarial survival

and graft survival rates of 85% and 72%, respectively.39 Liver transplantation should be considered

before the disease is too advanced. The development of a poor quality of life as a result of disabling

fatigue, intractable pruritus, severe muscle wasting, and chronic or recurrent bacterial cholangitis or

persistent elevations in serum bilirubin are primary indications for referral for liver transplantation.

Preoperative recognition of cholangiocarcinoma is extremely important in this population in that the

development of this complication significantly worsens the result after liver transplantation. The

presence of known malignancy results in patients being refused transplantation. The microscopic

identification of intrahepatic cholangiocarcinoma in the absence of lymph node involvement, often

demonstrated in the explanted liver specimen, however, does not usually portend a poor prognosis.

Although it can be extremely difficult to confidently confirm or refute the presence of

cholangiocarcinoma in this setting, both MRCP and more recently SpyGlass cholangioscopy during

ERCP can be extremely helpful. SpyGlass cholangioscopy in particular provides the advantage of direct

visualization of the biliary mucosa/lesion, as well as an opportunity for biopsy.

Patients with primary sclerosing cholangitis have a significantly higher rate of development of

nonanastomotic biliary strictures after liver transplantation, with histologic features on

posttransplantation biopsy consistent with recurrence of the disease. Other causes of stricture, such as

hepatic artery thrombosis, preservation-related ischemia, cytomegalovirus infection, and chronic

ductopenic rejection, can cause similar lesions. Recurrent primary sclerosing cholangitis usually does not

have an aggressive course.

Resection of the hepatic duct bifurcation and long-term transanastomotic stenting in selected patients

can preclude or delay the need for hepatic transplantation. Moreover, this operation does not eliminate

or influence the results of hepatic transplantation. Resection of the hepatic bifurcation and long-term

transhepatic stenting can be recommended for selected patients with primary sclerosing cholangitis with

severe strictures at or distal to the hepatic duct bifurcation but without established biliary cirrhosis. In

patients with biliary cirrhosis, hepatic transplantation is recommended.

BILE DUCT STRICTURES SECONDARY TO CHRONIC PANCREATITIS

9 Chronic pancreatitis is an uncommon cause of benign bile duct strictures, resulting in less than 10% of

such cases. Transient partial obstruction of the distal common bile duct caused by inflammation and

edema frequently occurs in patients with acute pancreatitis. With chronic pancreatitis, however, the

clinical problem is distal bile duct obstruction caused by inflammation and parenchymal fibrosis of the

gland. These strictures classically involve the entire intrapancreatic segment of the common bile duct

and are associated with dilatation of the entire proximal biliary tree (Fig. 62-14). In most cases, the

cause of the chronic pancreatitis is alcoholism. Often, advanced disease is present in that the incidence

of pancreatic calcification, diabetes, and malabsorption is increased at the time of presentation with

jaundice compared with patients with chronic pancreatitis without jaundice. Common bile duct

strictures have been reported to occur in 3% to 29% of patients with chronic alcoholic pancreatitis. In a

review of a number of clinical series, the overall incidence of common bile duct strictures in patients

with chronic pancreatitis was 5.7%.40 The exact incidence of common bile duct strictures is not known,

however, because cholangiography is not routinely performed in patients with chronic pancreatitis.

The clinical presentation of patients with common bile duct strictures secondary to chronic

pancreatitis is variable. Some patients have no symptoms, with the diagnosis of bile duct strictures

suggested only by abnormal liver function test results. The serum alkaline phosphatase appears to be

the most sensitive laboratory finding and is elevated in more than 80% of patients. Abdominal pain with

or without jaundice is another common presentation. In some cases, the abdominal pain can be difficult

to distinguish from the pain associated with chronic pancreatitis. Failure to recognize and address a bile

duct stricture, however, can lead to ultimate failure of operative procedures performed for chronic pain

in patients with chronic pancreatitis. Finally, the development of jaundice in patients with chronic

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