eMedicine Specialties > Gastroenterology > Biliary

Acalculous Cholecystopathy

Author: Alan BR Thomson, MD, Professor of Medicine, Division of Gastroenterology, University of Alberta, Canada
Coauthor(s): Walter E Pofahl, MD, Chief, Division of General Surgery, Associate Professor, Department of Surgery, Brody School of Medicine at East Carolina University; Jack A Di Palma, MD, Director, Division of Gastroenterology, Professor, Department of Internal Medicine, University of South Alabama College of Medicine
Contributor Information and Disclosures

Updated: Mar 5, 2010

Introduction

Background

The hallmark of acalculous cholecystopathy, frequently called biliary dyskinesia, is recurrent right upper quadrant pain in the absence of gallstones. Acalculous cholecystitis refers to cholecystitis without gallstones. Patients frequently undergo extensive, often invasive and expensive, testing prior to receiving definitive therapy. Although the treatment of choice is laparoscopic cholecystectomy, the rates of symptomatic improvement are not as favorable as in patients with biliary colic and gallstones.

Surgeons typically label acalculous cholecystopathy as biliary dyskinesia. For gastroenterologists, biliary dyskinesia is a synonym for sphincter of Oddi dysfunction, which is a distinct disease process. This article discusses only acalculous cholecystopathy.

Hepatobiliary (HIDA) scan showing persistent gall...

Hepatobiliary (HIDA) scan showing persistent gallbladder activity despite washout of radioisotope from liver and remainder of the biliary tree, which is suggestive of acalculous cholecystopathy.

Hepatobiliary (HIDA) scan showing persistent gall...

Hepatobiliary (HIDA) scan showing persistent gallbladder activity despite washout of radioisotope from liver and remainder of the biliary tree, which is suggestive of acalculous cholecystopathy.


Pathophysiology

Acalculous cholecystopathy is a clinical condition characterized by biliary colic-type pain in the absence of gallstones. The exact pathophysiology is unknown but likely is due to an abnormal gallbladder motility that possibly causes a relative obstruction of the cystic duct.

Frequency

United States

The true incidence of acalculous cholecystopathy is unknown. With the advent of laparoscopic cholecystectomy, data suggesting an increased rate of cholecystectomy exist. In general, 10-15% of patients undergoing laparoscopic cholecystectomy have biliary dyskinesia. Acute acalculous cholecystitis (AAC) can occur in young and middle-aged otherwise healthy outpatients.1

International

Almost 10% of persons with severe multiple injuries treated in a Western European trauma unit developed cholecystitis requiring cholecystectomy. This included AAC in about 40%, chronic acalculous cholecystitis (CAC) in 40% and cholecystitis with cholecystolithiasis in 20%.2 In other series, CAC is about 9 times more common than AAC.3

Mortality/Morbidity

The mortality and morbidity of acalculous cholecystopathy are related to the invasive diagnostic tests that frequently are performed and to the treatment of the condition (ie, cholecystectomy). Biliary dyskinesia does not progress to more serious conditions, such as acute cholecystitis.

Race

No data regarding racial distribution exist.

Sex

As with calculous biliary disease, acalculous cholecystopathy occurs more frequently in females than in males.

Age

As with calculous disease, most patients with acalculous cholecystopathy are aged 40-60 years.

Clinical

History

  • The presentation of AAC may be similar to that of acute calculous cholecystitis (ACC).
  • The presentation of AAC is highly variable, with or without right upper quadrant pain and tenderness, fever, leukocytosis, elevated liver enzymes and lipase or amylase.4
  • Pain
    • Characteristically occurs in the right upper quadrant 30-60 minutes after meals
    • Usually lasts 1-4 hours and typically does not radiate
    • Often exacerbated by greasy and spicy foods
  • Nausea is the most commonly associated symptom.
  • Vomiting is unusual.
  • Other gastrointestinal symptoms suggest diagnoses other than acalculous cholecystopathy.
  • Acute cholecystitis may occur as a complication of critical illness, such as sepsis or cardiovascular disease.

Physical

  • The physical examination is directed toward ruling out other possible etiologies of the pain.
  • No abnormal physical examination findings usually are associated with acalculous cholecystopathy.
  • Mild right upper quadrant tenderness may occur.

Causes

Associations of AAC/CAC

Open table in new window

Table
 
ACC
ACC
Occludin


Claudin-1


Claudin-2, -3, -4
 

20-1
 

 
ACC
ACC
Occludin


Claudin-1


Claudin-2, -3, -4
 

20-1
 

  • Impaired smooth muscle contractility may be important in the pathophysiology of acalculous cholecystitis. Studies of calcium homeostasis in gallbladder smooth muscle of guinea pigs with experimental cholecystitis show reduced Ca2+ influx and release, as well as impaired muscle responsiveness to CCK and to caffeine.68
  • The increased incidence of calculous and acalculous biliary disease in females suggests an association with female sex hormones.
    • Abnormalities in gallbladder emptying are especially pronounced during pregnancy.
    • Although estrogen and progesterone receptors have been found in the gallbladder, no direct effects of these hormones on gallbladder contractility have been elucidated.
  • The hepatic branches of the left vagus innervate the gallbladder. Division of these branches or of the anterior vagus during operations on the foregut is associated with an increased incidence of gallstones, presumably because of diminished gallbladder contractility.
  • Cell proliferation and apoptosis may be increased in the gallbladder epithelium of patients with acute acalculous cholecystitis.

Histopathology

There are overlaps in the histopathology of AAC and ACC in critically ill patients,69 and it has been suggested that "...AAC is largely a manifestation of systemic critical illness, whereas ACC is a local disease of the gallbladder." This is based on the comparison of AAC and ACC. In AAC, the following are found:

  • Bile infiltration in the gallbladder muscle layer is more common, deeper, and wider.
  • Epithelial degeneration is prominent.
  • Inflammatory cells are widespread.
  • Necrosis in the muscle layer is more common and wider and deeper. 

More on Acalculous Cholecystopathy

Overview: Acalculous Cholecystopathy
Differential Diagnoses & Workup: Acalculous Cholecystopathy
Treatment & Medication: Acalculous Cholecystopathy
Follow-up: Acalculous Cholecystopathy
Multimedia: Acalculous Cholecystopathy
References
Further Reading

References

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Further Reading

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Keywords

acalculous cholecystopathy, biliary dyskinesia, acalculous cholecystitis, laparoscopic cholecystectomy, sphincter of Oddi dysfunction

Contributor Information and Disclosures

Author

Alan BR Thomson, MD, Professor of Medicine, Division of Gastroenterology, University of Alberta, Canada
Alan BR Thomson, MD is a member of the following medical societies: Alberta Medical Association, American College of Gastroenterology, American Gastroenterological Association, Canadian Association of Gastroenterology, Canadian Medical Association, College of Physicians and Surgeons of Alberta, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Coauthor(s)

Walter E Pofahl, MD, Chief, Division of General Surgery, Associate Professor, Department of Surgery, Brody School of Medicine at East Carolina University
Walter E Pofahl, MD is a member of the following medical societies: American College of Surgeons, Association for Academic Surgery, Association for Surgical Education, Medical Association of the State of Alabama, Society of American Gastrointestinal and Endoscopic Surgeons, and Southeastern Surgical Congress
Disclosure: Nothing to disclose.

Jack A Di Palma, MD, Director, Division of Gastroenterology, Professor, Department of Internal Medicine, University of South Alabama College of Medicine
Jack A Di Palma, MD is a member of the following medical societies: American College of Gastroenterology and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Medical Editor

Marco G Patti, MD, Professor of Surgery, Director, Center for Esophageal Diseases, University of Chicago Pritzker School of Medicine
Marco G Patti, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Surgeons, American Gastroenterological Association, American Medical Association, American Surgical Association, Association for Academic Surgery, Pan-Pacific Surgical Association, Society for Surgery of the Alimentary Tract, Society of American Gastrointestinal and Endoscopic Surgeons, Southwestern Surgical Congress, and Western Surgical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Michael A Grosso, MD, Consulting Staff, Department of Cardiothoracic Surgery, St Francis Hospital
Michael A Grosso, MD is a member of the following medical societies: American College of Surgeons, Society of Thoracic Surgeons, and Society of University Surgeons
Disclosure: Nothing to disclose.

CME Editor

Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy
Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences
Disclosure: Nothing to disclose.

Chief Editor

John Geibel, MD, DSc, MA, Vice Chairman, Professor, Department of Surgery, Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology, Yale University School of Medicine; Director of Surgical Research, Department of Surgery, Yale-New Haven Hospital
John Geibel, MD, DSc, MA is a member of the following medical societies: American Gastroenterological Association, American Physiological Society, American Society of Nephrology, Association for Academic Surgery, International Society of Nephrology, New York Academy of Sciences, and Society for Surgery of the Alimentary Tract
Disclosure: AMGEN Royalty Other

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