History
Biliary disease presents with some diversity, from no symptoms to a constellation of signs and symptoms of varying severity and combination. An accurate diagnosis, therefore, begins with listening closely to the patient. Reaching an accurate diagnosis is aided by clinical experience and often involves imaging studies.
When abdominal pain is the chief symptom, seek to determine when it began and the subsequent events. Clarify what the pain feels like to the patient; visceral pain is perceived as a vague, dull, gnawing, burning, or aching sensation, whereas parietal pain is sharper in quality and better localized. Psychological conditions (eg, anxiety, worry) may enhance pain perception, while impaired consciousness tends to blunt pain perception.
Biliary-type pain
Biliary disease often presents with upper abdominal pain. The pain quality is a penetrating, aching or tightness, typically severe and located in the epigastrium. The sensation usually is difficult to describe; it may develop suddenly, last for 15 minutes to several hours, and then resolve suddenly. Although the term biliary colic is used commonly, it is a misnomer because the pattern of pain is constant. The pain is caused by an obstruction to the flow of bile, with distension of the biliary lumen, and is clinically similar to when the obstruction occurs at the cystic duct or at another level of the common bile duct. As noxious visceral stimuli become more intense, referred pain may be experienced in the posterior scapula or right shoulder area and may be accompanied by nausea and vomiting.
Jaundice
Bilirubin metabolism and transport principally are handled by the hepatobiliary tract. A yellow discoloration of the skin begins to appear when the serum bilirubin rises above 3 mg/dL, and the yellow discoloration is termed jaundice. Abnormalities leading to jaundice may occur in various phases of the process.
Jaundice and abdominal pain
The combination of jaundice and abdominal pain suggests a subacute obstruction of the biliary ductal system. In elderly patients, however, biliary tract obstruction may be painless. Rarely, acute viral hepatitis can be confused with biliary-type pain.
Painless jaundice
The development of jaundice in the absence of abdominal pain is suggestive of a malignant obstruction of the bile duct. Here, the onset of jaundice is gradual and may be associated with anorexia; weight loss; and acholic, soft or loose stools. Nonbiliary causes should be considered, including increased bilirubin production (eg, from hemolysis, blood transfusions, or ineffective erythropoieses) and decreased bilirubin clearance due to hereditary defects (eg, unconjugated hyperbilirubinemia in Gilbert syndrome and Crigler-Najjar syndrome types I and II, conjugated hyperbilirubinemia in Dubin-Johnson syndrome and Rotor syndrome).
Pruritus
Itching is an unpleasant sensation in the skin associated with a strong desire to scratch. While several causes exist, itching is associated with cholestasis and may become the patient's most bothersome symptom. Itching may appear at first in the hands and feet, but it usually becomes generalized and typically is worse at night. Itching does not distinguish the cause of cholestasis as hepatic or biliary.
Fatigue
The insidious onset of fatigue, followed by pruritus and then jaundice, is observed to varying degrees in diseases of the intrahepatic bile ducts, such as primary biliary cholangitis, primary sclerosing cholangitis, and vanishing bile duct syndrome.
Weight loss
A history of weight loss is associated with more serious diseases of the biliary tract. The weight loss may be caused by inadequate nutrient intake (eg, anorexia) or malabsorption of fats (eg, a paucity of bile in cholestatic diseases or prolonged biliary obstruction).
Miscellaneous
Other symptoms, including fatty food intolerance, gas, bloating, and dyspepsia, do not reliably indicate the presence of biliary tract disease.
Physical Examination
The patient with acute biliary-type pain often is restless, anxious, and frustrated by unsuccessful attempts to find a comfortable position. Severe pain of acute onset usually is associated with facial grimacing. Writhing, diaphoretic patients usually are acutely and seriously ill; however, some patients with peritonitis may lie still, with a worried facial expression, and avoid being touched or jostled.
Vital signs
Vital signs may be normal. The presence of fever suggests the presence of inflammation or infection. Tachycardia and hypertension occasionally accompany pain. Tachycardia and hypotension suggests hypovolemia or the presence of sepsis.
Skin
In people with light skin, the skin color may suggest not only jaundice but also provide clues to the etiology; a yellow discoloration is associated with indirect hyperbilirubinemia, a more orange hue can be observed with hepatocellular jaundice, and a dark green tint may develop with prolonged biliary obstruction. Evidence of easy bruisability may indicate a coagulopathy associated with cirrhosis. Patients with cholestasis classically exhibit excoriation of the skin (from scratching, typically sparing the mid back), melanin pigmentation, and xanthomas of the eyelids and extensor surfaces.
Eyes
Scleral icterus, a yellow discoloration of the whites of the eyes, results from hyperbilirubinemia. Although this term is in common use, it actually is a misnomer. The sclerae are relatively impervious to most compounds; the covering conjunctiva becomes permeated with unconjugated bilirubin, causing the yellow appearance. Approximately 58% of examiners are able to detect scleral icterus when the serum bilirubin rises above 2.5 mg/dL.
Abdomen
The abdomen should first be observed to determine if it is scaphoid, flat, distended, or asymmetric. Auscultation may reveal absent bowel sounds, suggesting an ileus, hyperactive bowel sounds (borborygmi), or high-pitched tinkling suggesting intestinal obstruction. The elicitation of pain and involuntary guarding during gentle palpation or jostling of the abdomen suggests peritonitis. Palpation may reveal a mass or fullness in the right upper quadrant.
In the patient with jaundice, an enlarged gallbladder is suggestive of malignant obstruction of the bile duct. In the absence of jaundice, the patient with a palpable mass in the right upper quadrant may have a gallbladder tumor or chronic obstruction of the cystic duct.
Gallbladder hydrops is a rare condition resulting from chronic common duct obstruction or mucosal inflammation, in which the gallbladder becomes grossly distended by an uninfected clear mucoid fluid. Although it usually requires cholecystectomy, when the condition is associated with a mucocutaneous lymph node syndrome (Kawasaki disease), it tends to be self-limited and resolve spontaneously.
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A normal postcholecystectomy cholangiogram.
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Biliary disease. In a patient with persistent elevation of liver-associated enzymes, the contrast medium entering the biliary ductal system preferentially enters the cystic duct.
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Biliary disease. Even when the catheter is advanced to the proximal common hepatic duct, contrast dye preferentially fills the cystic duct and gallbladder rather than allowing visualization of the intrahepatic ductal system (same patient as in previous image).
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Biliary disease. In this image, the common bile duct is occluded with a balloon-tipped catheter. Contrast material fills the intrahepatic ductal system to reveal diffuse intrahepatic sclerosing cholangitis.
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Biliary disease. Common bile duct stones are among the most frequent problems occurring in the biliary system. In this cholangiogram, the stones line up like peas in a pod.
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Biliary disease. After a biliary sphincterotomy, a balloon-tipped catheter is used to remove the stones one by one.
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Biliary disease. This clearing cholangiogram shows a common bile duct free of filling defects and good flow into the duodenum. The stones are visible as filling defects in the duodenal bulb.
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Biliary disease. A patient with pancreatic cancer developed jaundice during his treatment. The cholangiogram shows a stricture in the distal common bile duct.
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Biliary disease. A patient with pancreatic cancer developed jaundice during his treatment (same patient as in previous image). To palliate the jaundice, the biliary stricture is dilated and stented with a 10F plastic stent. Note the contrast dye flowing down the stent.
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Biliary disease. This computed tomography scan of the abdomen shows a large tumor mass in the head of the pancreas. The brightly colored object within the mass is the biliary stent placed by endoscopic retrograde cholangiopancreatography.
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Biliary disease. This abdominal computed tomography scan shows mild intrahepatic biliary ductal dilatation.
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Biliary disease. Abdominal computed tomography scanning in a patient with jaundice revealed polycystic liver disease.
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Biliary disease. Findings on this endoscopic retrograde cholangiopancreatogram exclude extrahepatic biliary obstruction but demonstrate that the intrahepatic biliary ductal system is splayed by multiple hepatic cysts.
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Biliary disease. This cholangiogram shows a choledochal cyst. Fusiform dilatation of the entire extrahepatic bile duct is present.
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A 92-year-old woman had recurrent abdominal pain and jaundice. A right upper quadrant ultrasonogram showed a dilated biliary duct with no stones. She had a previous Roux-en-Y surgery that made endoscopic retrograde cholangiopancreatography impossible. Critical aortic stenosis increased the risk of most interventions. This percutaneous cholangiogram, performed under conscious sedation in the operating room, revealed a large stone missed by the ultrasonogram. It was removed successfully with percutaneous choledochoscopy and electrohydraulic lithotripsy.
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Biliary disease. This cholangiogram shows a stone too large to deliver through a standard biliary sphincterotomy.
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Biliary disease. Here, a mechanical lithotripter is used to grab a stone too large to deliver through a standard biliary sphincterotomy and crush it into small pieces (same patient as in previous image). The smaller pieces then are removed with a balloon-tipped catheter.
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Biliary disease. A patient had malignant strictures of the biliary system palliated with metal mesh stents. Unfortunately, the tumor grew through the metal mesh to reobstruct the biliary system.
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Biliary disease. A patient had malignant strictures of the biliary system that were palliated with metal mesh stents, but the tumor grew through the metal mesh to reobstruct the biliary system (same patient as in previous image). In this image, after a wire was passed through the lumen, a balloon-dilating catheter was passed into the metal mesh stents and inflated to enlarge the lumen.
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Biliary disease. A patient had malignant strictures of the biliary system that were palliated with metal mesh stents, but the tumor grew through the metal mesh to reobstruct the biliary system (same patient as in previous image). After a wire was passed through the lumen, a balloon-dilating catheter was passed into the metal mesh stents and inflated to enlarge the lumen. In this image, two plastic stents were passed into the intrahepatic ductal system to again palliate the obstruction.
