Biliary Disease Clinical Presentation

  • Author: Annie T Chemmanur, MD; Chief Editor: Julian Katz, MD   more...
 
Updated: Jun 24, 2011
 

History

Biliary disease presents with some diversity, from no symptoms to a constellation of signs and symptoms of varying severity and combination. 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 whether 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 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 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 first in the hands and feet* but 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 cirrhosis, 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 indicate the presence of biliary tract disease reliably.
Next

Physical

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 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 color, 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.
  • 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 (hydrops) 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.
Previous
Next

Causes

  • Gallstones: In about 80% of patients, gallstones are clinically silent. Approximately 20% of patients develop symptoms over 15-20 years, that is, about 1% per year, and almost all become symptomatic before complications develop. Biliary-type pain, the typical clinical presentation, is due to obstruction of the bile duct lumen. The predictive value of other complaints (eg, intolerance to fatty food, indigestion) is too low to be clinically helpful. The incidence of gallbladder cancer developing in the setting of cholelithiasis is low, about 0.1% per year. Two main types of gallstones exist.
    • Cholesterol stones (85%): These are divided into 2 subtypes—pure (90-100% cholesterol) or mixed (50-90% cholesterol).
      • Pure stones often are solitary, whitish, and larger than 2.5 cm in diameter. Mixed stones usually are smaller, multiple in number, and occur in various shapes and colors. They tend to be arranged in laminated layers of an alternating thicker whitish cholesterol and a thinner dark pigment in a concentric pattern around a pigmented center (similar to the rings visible on the cross section of a tree). These stones tend to occur in residents of Western countries, and they usually are found in the gallbladder.
      • The risk factors associated with the development of cholesterol gallstones include obesity, a high-calorie diet, clofibrate therapy, gastrointestinal disorders involving major malabsorption of bile acids, cystic fibrosis with pancreatic insufficiency, and female sex and the use of oral contraceptives and other estrogenic medications. Coffee, ascorbic acid, has been shown to reduce the risk of symptomatic cholesterol gallstones.
    • Pigment stones (15%) occur in 2 subtypes—brown and black.
      • Brown stones are made up of calcium bilirubinate and calcium-soaps. Bacteria are involved in their formation via secretion of beta glucuronidase and phospholipase. The bacterial glycocalyx aggregates with the bile pigment and precipitates out of solution. These stones are more common in Asia and tend to form within the bile ducts. They frequently are associated with periampullary duodenal diverticula.
      • Black stones typically form in the gallbladder and result when excess bilirubin enters the bile and polymerizes into calcium bilirubinate. These stones are more common in patients with chronic hemolysis, alcoholic cirrhosis, and advanced age.
  • Acute calculus cholecystitis is an inflammation of the gallbladder that develops in the setting of an obstructed cystic or bile duct. It usually develops after 5 hours of biliary-type pain. The initial inflammation is caused by chemical irritation, and bacterial infection probably is a secondary event. A change in the perception of pain, classically a migration to the right upper quadrant, suggests transmural inflammation of the gallbladder, with involvement of the parietal peritoneum. Nausea and vomiting are common associated symptoms, and most patients are afebrile early in the course of the disease.
  • Mirizzi syndrome refers to common hepatic duct obstruction caused by an extrinsic compression from an impacted stone in the cystic duct.[1] It has been estimated to occur in 0.7-1.4% of all cholecystectomies. It is often not recognized preoperatively, which can lead to significant morbidity and biliary injury, particularly with laparoscopic surgery.
  • Acute acalculous cholecystitis is the presence of an inflamed gallbladder in the absence of an obstructed cystic or common bile duct. It typically occurs in the setting of a critically ill patient (eg, severe burns, multiple traumas, lengthy postoperative care, prolonged intensive care) and accounts for 5% of cholecystectomies. Because abdominal pain, fever, and leukocytosis are relatively common in these patients and the signs and symptoms are not specific for acalculous cholecystitis, the physician must have a high index of suspicion to make the diagnosis. The etiology is thought to have an ischemic basis, and a gangrenous gallbladder may result. This condition has an increased rate of complications and mortality. An uncommon subtype known as acute emphysematous cholecystitis generally is caused by infection with clostridial organisms and occlusion of the cystic artery associated with atherosclerotic vascular disease and, often, diabetes.
  • Chronic cholecystitis is a common disorder that frequently is associated with gallstones. The clinical features are nonspecific, and cholescintigraphy initially may suggest the diagnosis. The pathogenesis is poorly understood but may be due to abnormal bile composition leading to chemical injury of the gallbladder mucosa. Histologic evidence of a mononuclear infiltrate, fibrosis, and epithelial metaplasia confirm the diagnosis. A subset of patients develops dystrophic calcifications within the fibrosis, leading to a porcelain gallbladder, which is a risk factor for gallbladder carcinoma.
  • Cholangitis is an infection of the biliary system, complicating benign and malignant obstruction of the biliary tract. The clinical presentation is quite variable depending on the nature of the illness, patient age, and condition of the patient. Charcot triad (ie, fever, right upper quadrant pain, jaundice) occurs in only 20-70% of cases. Hypotension and mental status changes also may accompany severe infection, a pentad described by Reynolds in 1959.[2] The organisms typically identified are enteric in origin, notably Escherichia coli, Streptococcus faecalis, Clostridium species, Klebsiella species, Enterobacter species, Pseudomonas species, and Proteus species. They probably enter the biliary system via portal bacteremia. No correlation exists between the severity of clinical manifestations and the presence or absence of pus in the biliary system; however, suppurative cholangitis is associated with a higher mortality rate.
  • Recurrent pyogenic cholangitis, also known as "oriental cholangiohepatitis," is prevalent in several parts of Asia and the Pacific Rim countries. It is limited to Asian immigrants in America, occurs in the second to fourth decades of life, and is associated with a lower socioeconomic class. It is initiated by parasitic infestation of the biliary ducts by Opisthorchis sinensis (formerly Clonorchis sinensis), in which the adult fluke may impair bile flow. In the setting of bile stasis and secondary bacterial infection, pigment stones form around ova and sets the stage for the intermittent obstruction leading to recurrent pyogenic cholangitis. Pathologic changes principally affect the intrahepatic bile ducts (curiously, more often the left duct).
  • PSC is a chronic cholestatic biliary disease characterized by nonsuppurative inflammation and fibrosis of the biliary ductal system. The cause is unknown but is associated with autoimmune inflammatory diseases, such as chronic ulcerative colitis and Crohn colitis (less commonly), and rare conditions, such as Riedel thyroiditis and retroperitoneal fibrosis. Most patients present with fatigue and pruritus and, occasionally, jaundice. The natural history is variable but involves progressive destruction of the bile ducts, leading to cirrhosis and liver failure. The clinical features of cholangitis (ie, fever, right upper quadrant pain, jaundice) are uncommon unless the biliary system has been instrumented.
  • Primary biliary cirrhosis
    • PBC is a progressive cholestatic biliary disease that presents with fatigue and itching or asymptomatic elevation of the alkaline phosphatase. Jaundice develops with progressive destruction of bile ductules that eventually leads to liver cirrhosis and hepatic failure. This autoimmune illness has a familial predisposition, in which even unaffected family members may have immunologic abnormalities, especially an increased serum immunoglobulin M (IgM) and an association with human leucocyte antigen (HLA)-DR8.[3]
    • While numerous autoantibodies have been identified, antimitochondrial antibodies (AMA) are present in 95% of patients. AMA is a family of antibodies; those directed against the inner mitochondrial membrane antigen M2 in the 2-oxo-acid dehydrogenase complex are most specific for PBC.[4] Circulating immune complexes also have been identified but are unlikely to play a pathogenic role. Circulating T lymphocyte levels initially are within the reference range and decline as the disease progresses. The histologic appearance of the bile duct destruction resembles hepatic allograft rejection and graft-versus-host disease of the liver and appears to be mediated by cytotoxic T lymphocytes.
  • Autoimmune cholangitis represents a rare, distinct disease entity. While it shares some features with PBC, the results of tests for AMA are negative, the levels of gamma globulin and IgM are lower, and the results of tests for fluorescent antinuclear antibody (FANA) and anti–smooth muscle antibody (ASMA) are positive more commonly.
  • Neoplasms of the biliary tract: Carcinoma of the biliary system manifests with clinical symptoms of weight loss (77%), nausea (60%), anorexia (56%), abdominal pain (56%), fatigue (63%), pruritus (51%), fever (21%), malaise (19%), diarrhea (19%), constipation (16%), and abdominal fullness (16%). Symptomatic patients usually have advanced disease, with spread to hilar lymph nodes before obstructive jaundice occurs. It is associated with a poor prognosis.
    • Gallbladder cancer: This uncommon malignancy affects 2.5 individuals per 100,000 population. It represents 54% of biliary tract cancers, and more than 6500 patients die from this disease in the United States each year. Cancer that develops in the infundibulum can produce hydrops of the gallbladder that is clinically indistinguishable from an obstructing stone.
    • Cholangiocarcinoma is an adenocarcinoma of the bile ducts.[5] It may occur without associated risk factors, but it is associated more commonly with chronic cholestatic liver disease such as PSC, choledochal cysts, oriental cholangiohepatitis, and work-related handling of asbestos. Cholangiocarcinoma accounts for 25% of biliary tract cancers. Patients usually present with jaundice, a vague upper or right upper quadrant abdominal pain associated with anorexia, weight loss, and pruritus.
    • Ampullary cancer accounts for 8% of biliary tract cancers. It most commonly presents with painless jaundice or acute pancreatitis.
  • Biliary tract cysts: Cystic dilatation of the biliary tree is an uncommon abnormality. About half of the patients present with some combination of jaundice, abdominal pain, and an abdominal mass. The presence of these cysts often is associated with anomalous union of the pancreatic and biliary ductal system. This suggests that pancreatic juice enters the bile, causes a proteolytic and inflammatory injury to the duct wall, and leads to biliary cyst formation. The most commonly used classification scheme was proposed by Todani, which defines 5 cyst types, with groups I and IV having subtypes.
    • Type I involves a cystic dilatation of the extrahepatic biliary system. In subtype 1a (most common), the entire extrahepatic duct is diffusely involved. In subtype 1b (rare), a localized portion of the common bile duct is segmentally cystic. In subtype 1c (uncommon), the common bile duct is diffusely dilated.
    • Type II (rare) is a diverticulum of the extrahepatic bile duct.
    • Type III (uncommon) is a cystic dilatation of the intraduodenal portion of the common bile duct (sometimes referred to as a choledochocele).
    • Type IV has multiple cysts. Subtype IVa (uncommon) involves both the intrahepatic and extrahepatic biliary system, while subtype IVb (rare) has multiple cysts confined to the extrahepatic system.
    • Type V (rare) is characterized by single or multiple cysts involving the intrahepatic bile ducts (usually referred to as Caroli disease). Clinical symptoms usually are the result of associated complications such as cholangitis, choledocholithiasis, pancreatitis, hepatic abscess, cirrhosis, and biliary malignancy.
Previous
 
 
Contributor Information and Disclosures
Author

Annie T Chemmanur, MD  Attending Physician, Metrowest Medical Center and University of Massachusetts Memorial Hospital, Marlborough Campus

Annie T Chemmanur, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Gastroenterological Association, American Medical Association, and Massachusetts Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

Jeanette G Smith, MD  Fellow, Department of Gastroenterology-Hepatology, University of Connecticut School of Medicine

Jeanette G Smith, MD is a member of the following medical societies: American College of Physicians, American Gastroenterological Association, and American Public Health Association

Disclosure: Nothing to disclose.

George Y Wu, MD, PhD  Professor, Department of Medicine, Director, Hepatology Section, Herman Lopata Chair in Hepatitis Research, University of Connecticut School of Medicine

George Y Wu, MD, PhD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, American Medical Association, American Society for Clinical Investigation, and Association of American Physicians

Disclosure: Springer Consulting fee Consulting; Gilead Consulting fee Review panel membership; Gilead Honoraria Speaking and teaching; Bristol-Myers Squibb Honoraria Speaking and teaching; Springer Royalty Review panel membership

Specialty Editor Board

Ronnie Fass, MD  Chief of Gastroenterology, Southern Arizona VA Health Care System; Professor of Medicine, Division of Gastroenterology, University of Arizona School of Medicine

Ronnie Fass, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians-American Society of Internal Medicine, American Gastroenterological Association, American Motility Society, American Society for Gastrointestinal Endoscopy, and Israel Medical Association

Disclosure: Takeda Pharmaceuticals Grant/research funds Conducting research; Takeda Pharmaceuticals Consulting fee Consulting; Takeda Pharmaceuticals Honoraria Speaking and teaching; Vecta Consulting fee Consulting; XenoPort Consulting fee Consulting; Eisai Honoraria Speaking and teaching; Wyeth Pharmaceuticals Conducting research; AstraZeneca Grant/research funds Conducting research; Eisai Consulting fee Consulting

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

James L Achord, MD  Professor Emeritus, Department of Medicine, Division of Digestive Diseases, University of Mississippi School of Medicine

James L Achord, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Medical Association, American Society for Gastrointestinal Endoscopy, Mississippi State Medical Association, New York Academy of Sciences, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Paul Yakshe, MD, to the development and writing of this article.

References
  1. Yun EJ, Choi CS, Yoon DY, Seo YL, Chang SK, Kim JS, et al. Combination of magnetic resonance cholangiopancreatography and computed tomography for preoperative diagnosis of the Mirizzi syndrome. J Comput Assist Tomogr. Jul-Aug 2009;33(4):636-40. [Medline].

  2. REYNOLDS BM, DARGAN EL. Acute obstructive cholangitis; a distinct clinical syndrome. Ann Surg. Aug 1959;150(2):299-303. [Medline]. [Full Text].

  3. Solis Herruzo JA, Solis Munoz P, Munoz Yague T. The pathogenesis of primary biliary cirrhosis. Rev Esp Enferm Dig. Jun 2009;101(6):413-423. [Medline].

  4. Berg CP, Kannan TR, Klein R, et al. Mycoplasma antigens as a possible trigger for the induction of antimitochondrial antibodies in primary biliary cirrhosis. Liver Int. Jul 2009;29(6):797-809. [Medline].

  5. Cavallaro A, Cavallaro V, Di Vita M, Cappellani A. Main bile duct carcinoma management. Our experience on 38 cases. Ann Ital Chir. Mar-Apr 2009;80(2):107-11. [Medline].

  6. Drebber U, Mueller JJ, Klein E, et al. Liver biopsy in primary biliary cirrhosis: clinicopathological data and stage. Pathol Int. Aug 2009;59(8):546-54. [Medline].

  7. Kageoka M, Watanabe F, Maruyama Y, et al. Long-term prognosis of patients after endoscopic sphincterotomy for choledocholithiasis. Dig Endosc. Jul 2009;21(3):170-5. [Medline].

  8. Ohashi A, Tamada K, Wada S, et al. Risk factors for recurrent bile duct stones after endoscopic papillary balloon dilation: long-term follow-up study. Dig Endosc. Apr 2009;21(2):73-7. [Medline].

  9. Gurusamy K, Sahay SJ, Burroughs AK, Davidson BR. Systematic review and meta-analysis of intraoperative versus preoperative endoscopic sphincterotomy in patients with gallbladder and suspected common bile duct stones. Br J Surg. Jul 2011;98(7):908-16. [Medline].

  10. Castaing D, Vibert E, Bhangui P, et al. Results of percutaneous manoeuvres in biliary disease: The Paul Brousse experience. Surg Endosc. Jun 2011;25(6):1858-65. [Medline].

  11. Catena F, Ansaloni L, Di Saverio S, et al. Prospective analysis of 101 consecutive cases of laparoscopic cholecystectomy for acute cholecystitis operated with harmonic scalpel. Surg Laparosc Endosc Percutan Tech. Aug 2009;19(4):312-6. [Medline].

  12. Murphy MM, Shah SA, Simons JP, et al. Predicting major complications after laparoscopic cholecystectomy: a simple risk score. J Gastrointest Surg. Aug 12 2009;epub ahead of print. [Medline].

  13. Moody FG, Vecchio R, Calabuig R. Transduodenal sphincteroplasty with transampullary septectomy for stenosing papillitis. Am J Surg. Feb 1991;161(2):213-8. [Medline].

  14. Allison RR, Zervos E, Sibata CH. Cholangiocarcinoma: an emerging indication for photodynamic therapy. Photodiagnosis Photodyn Ther. Jun 2009;6(2):84-92. [Medline].

  15. Belli G, Limongelli P, Fantini C, et al. Laparoscopic and open treatment of hepatocellular carcinoma in patients with cirrhosis. Br J Surg. Sep 2009;96(9):1041-8. [Medline].

  16. Acosta JM, Civantos F, Nardi GL. Fibrosis of the papilla of Vater. Surg Gynecol Obstet. Apr 1967;124(4):787-94. [Medline].

  17. Afdhal NH, Smith BF. Cholesterol crystal nucleation: a decade-long search for the missing link in gallstone pathogenesis. Hepatology. Apr 1990;11(4):699-702. [Medline].

  18. Bennion LJ, Grundy SM. Risk factors for the development of cholelithiasis in man (second of two parts). N Engl J Med. Nov 30 1978;299(22):1221-7. [Medline].

  19. Boey JH, Way LW. Acute cholangitis. Ann Surg. Mar 1980;191(3):264-70. [Medline].

  20. Bohacek L, Pace DE. Advanced laparoscopic training and outcomes in laparoscopic cholecystectomy. Can J Surg. Aug 2009;52(4):291-294. [Medline]. [Full Text].

  21. Bolondi L, Gaiani S, Testa S. Gall bladder sludge formation during prolonged fasting after gastrointestinal tract surgery. Gut. Jul 1985;26(7):734-8. [Medline].

  22. Broome U, Olsson R, Loof L. Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis. Gut. Apr 1996;38(4):610-5.

  23. Burnstein MJ, Ilson RG, Petrunka CN. Evidence for a potent nucleating factor in the gallbladder bile of patients with cholesterol gallstones. Gastroenterology. Oct 1983;85(4):801-7. [Medline].

  24. Carey MC, Small DM. The physical chemistry of cholesterol solubility in bile. Relationship to gallstone formation and dissolution in man. J Clin Invest. Apr 1978;61(4):998-1026. [Medline].

  25. Castiella A, Iribarren JA, Lopez P. Ursodeoxycholic acid in the treatment of AIDS-associated cholangiopathy. Am J Med. Aug 1997;103(2):170-1.

  26. Csendes A, Burdiles P, Maluenda F. Simultaneous bacteriologic assessment of bile from gallbladder and common bile duct in control subjects and patients with gallstones and common duct stones. Arch Surg. Apr 1996;131(4):389-94.

  27. Denman ST. A review of pruritus. J Am Acad Dermatol. Mar 1986;14(3):375-92. [Medline].

  28. Everhart JE, Khare M, Hill M. Prevalence and ethnic differences in gallbladder disease in the United States. Gastroenterology. Sep 1999;117(3):632-9.

  29. Gallinger S, Taylor RD, Harvey PR. Effect of mucous glycoprotein on nucleation time of human bile. Gastroenterology. Sep 1985;89(3):648-58. [Medline].

  30. Gerecht WB, Henry NK, Hoffman WW. Prospective randomized comparison of mezlocillin therapy alone with combined ampicillin and gentamicin therapy for patients with cholangitis. Arch Intern Med. Jun 1989;149(6):1279-84.

  31. Graziadei IW, Wiesner RH, Batts KP. Recurrence of primary sclerosing cholangitis following liver transplantation. Hepatology. Apr 1999;29(4):1050-6. [Medline].

  32. Harnois DM, Angulo P, Jorgensen RA. High-dose ursodeoxycholic acid as a therapy for patients with primary sclerosing cholangitis. Am J Gastroenterol. May 2001;96(5):1558-62.

  33. Harnois DM, Lindor KD. Primary sclerosing cholangitis: evolving concepts in diagnosis and treatment. Dig Dis. Jan-Apr 1997;15(1-2):23-41.

  34. Heathcote EJ. Management of primary biliary cirrhosis. The American Association for the Study of Liver Diseases practice guidelines. Hepatology. Apr 2000;31(4):1005-13.

  35. Hendrickse MT, Rigney E, Giaffer MH. Low-dose methotrexate is ineffective in primary biliary cirrhosis: long-term results of a placebo-controlled trial. Gastroenterology. Aug 1999;117(2):400-7.

  36. Holzbach RT. Recent progress in understanding cholesterol crystal nucleation as a precursor to human gallstone formation. Hepatology. Nov-Dec 1986;6(6):1403-6. [Medline].

  37. Izumi Y, Teramoto K, Ohshima M. Endoscopic resection of duodenal ampulla with a transparent plastic cap. Surgery. Jan 1998;123(1):109-10. [Medline].

  38. Jones DE, Gray JC, Newton J. Perceived fatigue is comparable between different disease groups. QJM. Sep 2009;102(9):617-24. [Medline].

  39. Kaplan MM. Primary biliary cirrhosis. N Engl J Med. Nov 21 1996;335(21):1570-80.

  40. Krishnamurthy GT, Turner FE. Pharmacokinetics and clinical application of technetium 99m-labeled hepatobiliary agents. Semin Nucl Med. Apr 1990;20(2):130-49. [Medline].

  41. Lam SK, Wong KP, Chan PK. Recurrent pyogenic cholangitis: a study by endoscopic retrograde cholangiography. Gastroenterology. Jun 1978;74(6):1196-1203. [Medline].

  42. Levy PF, Smith BF, LaMont JT. Human gallbladder mucin accelerates nucleation of cholesterol in artificial bile. Gastroenterology. Aug 1984;87(2):270-5. [Medline].

  43. Liermann Garcia RF, Evangelista Garcia C, McMaster P. Transplantation for primary biliary cirrhosis: retrospective analysis of 400 patients in a single center. Hepatology. Jan 2001;33(1):22-7. [Medline].

  44. Lim JH. Oriental cholangiohepatitis: pathologic, clinical, and radiologic features. Am J Roentgenol. Jul 1991;157(1):1-8. [Medline].

  45. Mazer NA, Carey MC. Quasi-elastic light-scattering studies of aqueous biliary lipid systems. Cholesterol solubilization and precipitation in model bile solutions. Biochemistry. Jan 18 1983;22(2):426-42. [Medline].

  46. Moody FG. Pathogenesis and treatment of inflammatory lesions of the papilla of Vater. Jpn J Surg. Sep 1985;15(5):341-7. [Medline].

  47. Nijhawan PK, Therneau TM, Dickson ER. Incidence of cancer in primary biliary cirrhosis: the Mayo experience. Hepatology. May 1999;29(5):1396-8.

  48. O'Connor MJ, Schwartz ML, McQuarrie DG, Sumer HW. Acute bacterial cholangitis: an analysis of clinical manifestation. Arch Surg. Apr 1982;117(4):437-41. [Medline].

  49. O'Connor MJ, Sumner HW, Schwartz ML. The clinical and pathologic correlations in mechanical biliary obstruction and acute cholangitis. Ann Surg. Apr 1982;195(4):419-23. [Medline].

  50. Palazzo L, Girollet PP, Salmeron M. Value of endoscopic ultrasonography in the diagnosis of common bile duct stones: comparison with surgical exploration and ERCP. Gastrointest Endosc. Sep 1995;42(3):225-31. [Medline].

  51. Pomeranz IS, Shaffer EA. Abnormal gallbladder emptying in a subgroup of patients with gallstones. Gastroenterology. 1985;88:801. [Medline].

  52. Quirk DM, Rattner DW, Fernandez-del Castillo C. The use of endoscopic ultrasonography to reduce the cost of treating ampullary tumors. Gastrointest Endosc. Oct 1997;46(4):334-7. [Medline].

  53. Ransohoff DF, Gracie WA, Wolfenson LB. Prophylactic cholecystectomy or expectant management for silent gallstones. A decision analysis to assess survival. Ann Intern Med. Aug 1983;99(2):199-204. [Medline].

  54. Ros E, Zambon D. Postcholecystectomy symptoms. A prospective study of gall stone patients before and two years after surgery. Gut. Nov 1987;28(11):1500-4. [Medline].

  55. Ruffolo TA, Sherman S, Lehman GA. Gallbladder ejection fraction and its relationship to sphincter of Oddi dysfunction. Dig Dis Sci. Feb 1994;39(2):289-92. [Medline].

  56. Sampliner RE, Bennett PH, Comess LJ. Gallbladder disease in Pima Indians. Demonstration of high prevalence and early onset by cholecystography. N Engl J Med. Dec 17 1970;283(25):1358-64. [Medline].

  57. Schoenfield LJ, Carey MC, Marks JW. Gallstones: an update. Am J Gastroenterol. Sep 1989;84(9):999-1007. [Medline].

  58. Sievers MS, Marquis JR. The Southwest American Indian's burden: biliary disease. Journal of the American Medical Association. 1962;182:570-572.

  59. Strauch GO. Primary carcinoma of the gallbladder: presentation of seventy cases from the Rhode Island Hospital and a cumulative review of the last ten years. American Literature of Surgery. 1960;47:368.

  60. Strom BL, Soloway RD, Rios-Dalenz JL. Risk factors for gallbladder cancer. An international collaborative case-control study. Cancer. Nov 15 1995;76(10):1747-56.

  61. Thistle JL, Cleary PA, Lachin JM. The natural history of cholelithiasis: the National Cooperative Gallstone Study. Ann Intern Med. Aug 1984;101(2):171-5.

  62. Wienser RH, Porayko MK, LaRusso NF, et al. In: Schiff L, Schiff ER, eds. Diseases of the Liver. 7th ed. Philadelphia, Pa:. JB Lippincott;1993:411-426.

  63. Wolfhagen FH, Sternieri E, Hop WC. Oral naltrexone treatment for cholestatic pruritus: a double-blind, placebo-controlled study. Gastroenterology. Oct 1997;113(4):1264-9. [Medline].

  64. Yap L, Wycherley AG, Morphett AD. Acalculous biliary pain: cholecystectomy alleviates symptoms in patients with abnormal cholescintigraphy. Gastroenterology. Sep 1991;101(3):786-93. [Medline].

  65. Yoshida J, Chijuwa K. Practical classification of the branching types of the biliary tree: an analysis of 1094 consecutive direct cholangiograms. J Am Coll Surg. 1997;185:274-82. [Medline].

  66. Zeman RK. Cholelithiasis and cholecystitis. In: Gore RM, Levine MS, Laufer I, eds. Text of Gastrointestinal Radiology. Philadelphia, Pa:. WB Saunders;1994:1654-55.

Previous
Next
 
A normal postcholecystectomy cholangiogram.
Biliary disease. In this patient with persistent elevation of liver-associated enzymes, the contrast entering the biliary ductal system preferentially enters the cystic duct.
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.
Biliary disease. In this image, the common bile duct is occluded with a balloon-tipped catheter. Contrast fills the intrahepatic ductal system to reveal diffuse intrahepatic sclerosing cholangitis.
Biliary disease. Common bile duct stones are among the most common problems occurring in the biliary system. In this cholangiogram, the stones line up like peas in a pod.
Biliary disease. After a biliary sphincterotomy, a balloon-tipped catheter is used to remove the stones one by one.
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.
Biliary disease. This patient with pancreatic cancer has developed jaundice during his treatment. The cholangiogram shows a stricture in the distal common bile duct.
Biliary disease. A patient with pancreatic cancer has developed jaundice during his treatment. To palliate the jaundice, the biliary stricture is dilated and stented with a 10F plastic stent. Note the contrast flowing down the stent.
Biliary disease. The CT 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 (ERCP).
Biliary disease. This abdominal CT scan shows mild intrahepatic biliary ductal dilation.
Biliary disease. This patient with jaundice has polycystic liver disease on abdominal CT scan.
Biliary disease. Findings on an endoscopic retrograde cholangiopancreatography (ERCP) exclude extrahepatic biliary obstruction but demonstrate that the intrahepatic biliary ductal system is splayed by multiple hepatic cysts.
Biliary disease. This cholangiogram shows a choledochal cyst. Fusiform dilation of the entire extrahepatic bile duct is present.
This 92-year-old woman had recurrent abdominal pain and jaundice. A right upper quadrant ultrasound showed a dilated biliary duct with no stones. She had a previous Roux-en-Y surgery that made endoscopic retrograde cholangiopancreatography (ERCP) impossible. Critical aortic stenosis that increased the risk of most interventions. This percutaneous cholangiogram, performed under conscious sedation in the operating room, revealed a large stone missed by the ultrasound. It was removed successfully with percutaneous choledochoscopy and electrohydraulic lithotripsy.
Biliary disease. This cholangiogram shows a stone too large to deliver through a standard biliary sphincterotomy.
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. The smaller pieces then are removed with a balloon-tipped catheter.
Biliary disease. This patient had malignant strictures of the biliary system that were palliated with metal mesh stents. Unfortunately, the tumor has grown through the metal mesh to reobstruct the biliary system.
Biliary disease. This patient had malignant strictures of the biliary system that were palliated with metal mesh stents. Unfortunately, the tumor has grown through the metal mesh to reobstruct the biliary system. After a wire is passed through the lumen, a balloon-dilating catheter is passed into the metal mesh stents and inflated to enlarge the lumen.
Biliary disease. This patient had malignant strictures of the biliary system that were palliated with metal mesh stents. The tumor has grown through the metal mesh to reobstruct the biliary system. 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, 2 plastic stents were passed into the intrahepatic ductal system to again palliate the obstruction.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.