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Gastroenterology > Biliary
Acalculous Cholecystopathy
Article Last Updated: Jun 12, 2008
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Alan BR Thomson, MD, MSc, PhD, Professor, Department of Medicine, Division of Gastroenterology, University of Alberta Faculty of Medicine
Alan BR Thomson is a member of the following medical societies: American Federation for Aging Research, American Federation for Clinical Research, American Gastroenterological Association, American Geriatrics Society, American Physiological Society, Canadian Association of Gastroenterology, Gastroenterology Research Group, New York Academy of Sciences, and Royal Society of Medicine
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
Editors: Marco G Patti, MD, Professor of Surgery, Director, Center for Esophageal Diseases, University of Chicago Pritzker School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Michael A Grosso, MD, Consulting Staff, Department of Cardiothoracic Surgery, St Francis Hospital; Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy; 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
Author and Editor Disclosure
Synonyms and related keywords:
biliary dyskinesia, acalculous cholecystitis, laparoscopic cholecystectomy, sphincter of Oddi dysfunction
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 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.
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.
International
No specific data on the incidence of biliary dyskinesia outside of the United States exist.
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.
History
- 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
- A number of hormones have prokinetic or inhibitory effects on gallbladder motility.
- The hormones stimulating gallbladder contraction include cholecystokinin (CCK), gastrin, secretin, and motilin.
- Vasoactive inhibitory peptide, somatostatin, and pancreatic polypeptide inhibit gallbladder motility.
- Histamine stimulates contraction via histamine 1 (H1) receptors and relaxation via histamine 2 (H2) receptors.
- Of these hormones, only somatostatin is used as a therapeutic agent. The actions of somatostatin appear to be due to the inhibition of CCK-mediated gallbladder emptying.
- 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.
Bile Duct Strictures
Biliary Colic
Biliary Disease
Biliary Obstruction
Cholecystitis
Choledocholithiasis
Cholelithiasis
Duodenal Ulcers
Gallbladder Cancer
Gastric Ulcers
Gastritis, Acute
Gastritis, Chronic
Helicobacter Pylori Infection
Hepatitis, Viral
Irritable Bowel Syndrome
Pancreatitis, Acute
Pancreatitis, Chronic
Lab Studies
- No laboratory studies specific for acalculous cholecystopathy exist. Instead, the studies help rule out other conditions that are part of the differential diagnosis.
- Liver profile – To rule out hepatitis, acute cholecystitis, and choledocholithiasis
- CBC – To rule out acute inflammation as observed in cases of hepatitis or acute cholecystitis
- Helicobacter pylori serology or breath testing – Helpful in ruling out causes of gastritis and peptic ulcer disease
Imaging Studies
- Ultrasound of the right upper quadrant
- Ultrasound is most useful to rule out conditions in the differential diagnosis.
- Ultrasound detects abnormalities of the gallbladder, including the presence (or absence) of stones, wall thickening, or pericholecystic fluid.
- Dilatation of the intrahepatic and extrahepatic biliary tree suggests biliary obstruction due to stone, stricture, or malignancy.
- The ultrasound results are usually normal in patients with acalculous cholecystopathy.
- Ultrasound is also useful in detecting hepatic masses.
- Hepatobiliary scanning
- Hepatobiliary (hydroxy iminodiacetic acid [HIDA]) scanning may have some use in determining which patients with right upper quadrant pain, a lack of gallstones, and no other etiology may benefit from cholecystectomy.
- After the gallbladder fills with the radioisotope, a cholecystokinin analog is administered. This analog stimulates emptying of the gallbladder, allowing an ejection fraction to be calculated. A gallbladder ejection fraction of less than 35% is considered abnormal.
- However, there are discrepant results. In one study, the sensitivity is less than 60%, and the specificity is less than 70%, with a positive predictive value (PPV) of 91.3% and a negative predictive value (NPV) of only 22.5%.1
- This controversy may be because no consensus exists regarding the dose and the infusion rate of cholecystokinin or regarding the definition of an abnormal ejection fraction. Some patients with a normal ejection fraction may respond to surgery. It remains unknown if there is a causal overlap from a low ejection fraction and sphincter of Oddi spasm. It is controversial whether an ejection fraction of less than 35% in a patient with the appropriate symptom complex may benefit from laparoscopic cholecystectomy. Administration of the analog also may recreate the pain experienced by the patient.
- HIDA scanning has high sensitivity and specificity to help establish the diagnosis of acute cholecystitis.
- Computed tomography scanning
- CT scanning rarely is useful in diagnosing biliary dyskinesia.
- Instead, CT scan helps rule out (when indicated) other conditions in the differential diagnosis.
Procedures
- Other procedures are used, when indicated, to rule out other conditions in the differential diagnosis.
- Upper endoscopy is the most commonly performed procedure.
- Upper endoscopy findings are normal in patients with biliary dyskinesia.
- Endoscopy is very accurate in detecting gastritis and peptic ulcer disease, which should be treated appropriately prior to considering cholecystectomy for acalculous cholecystopathy.
Medical Care
No effective medical treatment of acalculous cholecystopathy exists. Some authors suggest that in patients with acute acalculous cholecystitis without overt peritonitis, antibiotics may be useful before cholecystectomy.
Surgical Care
- Laparoscopic cholecystectomy is indicated for the treatment of biliary dyskinesia after all of the other conditions in the differential diagnosis have been ruled out with a reasonable degree of certainty. The procedure usually is performed as an outpatient operation.
- Intraoperative cholangiography is performed at the discretion of the operating surgeon.
- In poor surgical candidates, cholecystostomy or nasobiliary drainage and lavage may be treatment options.
Consultations
Gastroenterologists and surgeons are the consultants usually involved in the care of these patients. Most patients are evaluated and treated as outpatients.
Diet
No specific dietary restrictions are applicable. Avoiding foods that exacerbate symptoms, typically fatty or spicy foods, may temporarily diminish some of the symptoms.
Activity
No restrictions on activity are applicable.
No effective medical treatment of acalculous cholecystopathy exists.
Further Outpatient Care
- Patients undergoing surgical therapy were 2.8 times more likely to have symptomatic relief versus nonoperative therapy.
- Laparoscopic cholecystectomy usually is performed as an outpatient procedure.
- In the absence of complications, the patient usually is seen 2-4 weeks after the operation.
In/Out Patient Meds
- Postoperative pain medications are provided but usually are only required for 7-10 days in progressively decreasing amounts.
Complications
- Patients with acalculous gallbladder disease have approximately double the mortality rate (0.8%) compared with patients with calculous disease.
- The complications are those related to cholecystectomy.
- In addition to the morbidities inherent in any procedure (eg, bleeding, infection, damage to adjacent structures), several morbidities are unique to laparoscopic cholecystectomy.
- Complications related to carbon dioxide pneumoperitoneum, such as acidosis and shoulder pain, also occur. These complications usually resolve rapidly in most healthy patients.
- The rate of bile duct injury is approximately 3 cases per 1000 procedures.
Prognosis
- Laparoscopic cholecystectomy provides symptomatic relief in 90-95% of cases of biliary colic associated with gallstones. In contrast, with appropriate patient selection, only approximately 80% of patients with acalculous cholecystopathy experience resolution of their symptoms.
Medical/Legal Pitfalls
- The main pitfall is failure to consider and evaluate the patient for other conditions in the differential diagnosis, especially malignancy (see Differentials).
- The other potential source of problems and patient dissatisfaction is the failure to inform patients of the possibility of persistent symptoms following the operation. Although most patients will experience relief or improvement in their symptoms, approximately 15-20% will continue to experience the same pain they felt preoperatively.
| Media file 1:
Hepatobiliary (HIDA) scan showing liver uptake of the radioisotope. |
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Media type: X-RAY
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| Media file 2:
Hepatobiliary (HIDA) scan showing biliary excretion of the radioisotope. |
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Media type: X-RAY
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| Media file 3:
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. |
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Media type: X-RAY
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| Media file 4:
Impaired gallbladder emptying despite injection of cholecystokinin analog. |
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Media type: X-RAY
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Acalculous Cholecystopathy excerpt Article Last Updated: Jun 12, 2008
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