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a.Clinical presentation

1.Symptoms and signs include right upper quadrant pain or painless jaundice, usually without fever or chills, pruritis, fatigue, nausea, and symptoms of hepatic failure.

2.Other inflammatory conditions, particularly ulcerative colitis, may be present.

b.Histology. The bile ducts show edema and areas of inflammation and fibrosis.

c.Diagnosis

1.The diagnosis is usually made by ERCP or a transhepatic cholangiogram and occasionally by intraoperative cholangiography.

2.Criteria needed to fulfill the diagnosis are:

a.Thickening and stenosis of a major portion of the biliary ductal system

b.Absence of prior surgery, choledocholithiasis, malignancy, or congenital biliary anomalies

c.No evidence of primary liver disease, particularly primary biliary cirrhosis

d.Treatment. Operative management is dependent on the level of bile duct involvement and the amount of fibrosis present. Restoration of adequate and permanent biliary drainage is the goal of operative management.

1.Internal biliary drainage, via either a hepaticoenteric or choledochoenteric anastomosis, is the preferred method of management. This is successful only when the major area of involvement is the extrahepatic bile ducts.

2.External biliary drainage, using a T tube or other percutaneous stent, establishes adequate drainage initially, but inevitably it becomes contaminated, and the patient may contract bacterial cholangitis.

3.Cholecystectomy is performed only when gallbladder disease requires it.

e.Postoperative treatment is strongly dependent on the presence of preoperative sepsis and the adequacy of drainage. Steroids are not beneficial and could potentially complicate the postoperative course.

f.Prognosis is poorly defined at present. If the liver parenchyma has been damaged or if the intrahepatic ducts are significantly involved, only hepatic transplantation offers a real chance of longevity, and this procedure is only possible when the patient is free of sepsis.

4.Fibrosis of the sphincter of Oddi is a disorder of uncertain etiology that causes colicky right upper quadrant pain, nausea, vomiting, and frequently recurrent pancreatitis. Treatment is by endoscopic papillotomy of transduodenal sphincteroplasty.

GNeoplasms

1.Benign tumors of the gallbladder are rare. They include papilloma, adenomyoma, fibroma, lipoma, myoma, myxoma, and carcinoid.

2.Carcinoma of the gallbladder accounts for 4% of all carcinomas. It is the most common cancer of the biliary tract and occurs in 1% of all patients undergoing biliary tract surgery.

a.Etiology. Although the cause is not known, 90% of the patients have cholelithiasis. About 80% of the tumors are adenocarcinomas. Metastases occur by lymphatic spread to the pancreatic, duodenal, and choledochal nodes and by direct extension to the liver.

b.Clinical presentation. The most common complaint is right upper quadrant pain. This is often associated with nausea and vomiting. The diagnosis is rarely made preoperatively. Patients with calcification of the wall of the gallbladder (porcelain gallbladder) that is seen on plain radiograph of the abdomen have a carcinoma of the gallbladder in appromimately one half of cases.

c.Treatment. The only truly curable cases are those in which the tumor is found incidentally at cholecystectomy for other reasons. If there is microscopic invasion of the gallbladder, cholecystectomy with wedge resection of the

liver and regional lymphadenectomy may improve the survival.

d.Prognosis is poor: The 5-year survival rate ranges from 0%–10%.

3.Common bile duct malignant tumors are rare and difficult to cure.

a.Clinical presentation

1.The patient usually complains of pruritus, anorexia, weight loss, and an aching right upper quadrant pain. Jaundice is usually severe.

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2.The following diseases may be associated with this malignancy:

a.Sclerosing cholangitis

b.Chronic parasitic infection of the bile ducts

c.Gallstones (present in 18%–65% of cases)

d.Prior exposure to Thorotrast

b.Diagnosis may be made by percutaneous transhepatic cholangiography or ERCP. Both procedures are capable of biopsy for pathologic examination.

c.Pathology. These tumors are called cholangiocarcinoma.

1.The gross pathologic finding is a mass involving a portion of the bile ducts. The microscopic appearance is that of adenocarcinoma, although the distinction from sclerosing cholangitis may be difficult.

2.The tumor may be located in the distal common bile duct, the common hepatic duct or cystic duct, or the right or left hepatic duct (most common location). When the confluence of the hepatic ducts is involved, the tumor is termed a Klatskin tumor.

3.The tumor initially metastasizes to the regional lymph nodes (16% of cases), spreads by direct extension into the liver (14%), or metastasizes to the liver (10%).

d.Treatment. The management of common bile duct tumors is generally surgical, although fewer than 10% are resectable at the time of the initial diagnosis.

1.Tumors in the distal duct may be resected by pancreaticoduodenectomy (Whipple procedure) with biliary and gastrointestinal reconstruction. More proximal lesions can sometimes be locally resected with biliary reconstruction. The average length of survival after resection is 23 months. Postoperative radiation may improve the life expectancy.

2.Unresectable lesions should have rigid stents placed to provide palliation of the biliary obstructive symptoms.

a.Laparotomy with no bypass is associated with an average survival time of less than 6 months.

b.With stenting, the average survival time is 19 months.

i.In this procedure, either a transhepatic stent is placed percutaneously or a U tube is placed surgically.

ii.The U tube passes from the skin, through the liver, through the tumor, into the common bile duct, and then out through the abdominal wall (Fig. 14-16).

e.Prognosis

1.Metastatic spread of the tumor is usually slow and is not responsible for death.

2.The usual cause of death is related to the following:

a.Progressive biliary cirrhosis due to inadequate biliary drainage

b.Persistent intrahepatic infection and abscess formation

c.General debility

FIGURE 14-17 Biliary cysts. Longmire's modification of Alonso-Lej's classification.

(Reprinted with permission from Longmire W. Congenital cystic disease of the liver and biliary system. Ann Surg. 1971;174:721.)

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5.Treatment. Due to the risk of malignancy, cyst excision (rather than bypass) is the cornerstone of surgery.

a.Type I patients are treated with cholecystectomy, cyst excision, and a Roux-en-Y choledochojejunostomy.

b.Type II patients are treated by excision of the common bile duct diverticulum.

c.Type III patients are treated by cyst excision and choledochoduodenostomy or by transduodenal sphincteroplasty.

d.The type IV anomaly may be fatal. Patients require liver transplantation.

I Congenital biliary atresia

(see Chapter 29, IX B)

JTrauma

1.Gallbladder injuries are uncommon but are seen after both penetrating and nonpenetrating trauma. Associated visceral injuries are common and most frequently (72%) involve the liver.

a.Types of injuries to the gallbladder include contusions, avulsion, rupture, and traumatic cholecystitis.

b.Clinical presentation. Right upper quadrant pain, right chest pain, biliary leakage through a penetrating wound, and shock are the most common presenting symptoms.

c.Diagnosis is most frequently made at laparotomy. A peritoneal tap may be negative.

2.Extrahepatic bile duct injuries

a.Operative injury. Most extrahepatic bile duct injuries are iatrogenic, occurring during cholecystectomy.

1.Clinical presentation. Only 15% of intraoperative injuries are diagnosed at the time of surgery, and 85% present days to years later with progressive jaundice, cholangitis, or cirrhosis and its complications.

2.Diagnosis is by transhepatic cholangiography or ERCP.

3.Treatment. End-to-end (duct-to-duct) anastomosis may be done at the time of initial injury; otherwise, a Roux-en-Y choledochojejunostomy is necessary.

4.The mortality rate after repair of a chronic biliary stricture is 8%–10%, and death is usually secondary to

liver failure.

b.Other extrahepatic bile duct injuries almost always accompany other visceral injuries and result from trauma, such as gunshot wounds. Isolated bile duct injuries are rare.

1.Clinical presentation is the same as those in gallbladder injuries.

2.Diagnosis is made at laparotomy.

3.Treatment, aside from administration of antibiotics, involves meticulous exploration of the ducts. Either a primary end-to-end (duct-to-duct) anastomosis, or, more commonly, a Roux-en-Y choledochojejunostomy is appropriate. If the patient is unstable, drainage with a T tube is expedient.

c.Intraperitoneal extravasation of bile

1.The extravasation of sterile bile results in chemical peritonitis.

a.This may be a mild peritonitis, producing ascites, or a localized collection.

b.Continuous outpouring of sterile bile may produce an extensive chemical peritonitis and shock.

2.Infected intraperitoneal bile induces a fulminant and frequently fatal peritonitis.