Ascending Cholangitis

Ascending Cholangitis Definition

Ascending Cholangitis (AC) is a form of Cholangitis, or bile duct infection, caused by a certain bacteria ascending from the duodenum and bile duct junction. It normally occurs if the bile duct of the patient is already partially blocked by gallstones. It is considered to be a life-threatening condition and a medical emergency.

The disorder is also known as Acute Cholangitis or Acute Ascending Cholangitis.

Ascending Cholangitis ICD-9 Code

The ICD-9 Code for this disease is 576.1.

Ascending Cholangitis Causes

The main cause of AC is bile duct obstruction which occurs commonly due to gallstones. In around 10 to 30% of all cases, bile duct obstruction is caused by other factors like benign stricture, postoperative damage or a change in the structure of the bile ducts such as narrowing at the location of an anastomosis or surgical connection. It can also be caused by various tumors, such as gallbladder cancer, bile duct cancer, cancer of ampulla of Vater, cancer of duodenum or pancreatic cancer. AC also tends to complicate medical procedures like ERCP that involve the bile duct. In order to prevent this, individuals undergoing ERCP should be given prophylactic antibiotics.

Picture of Ascending Cholangitis

Picture 1 – Ascending Cholangitis

Permanent biliary stents, like the ones used in pancreatic cancer, increase the risk of AC occurrence to some degree. However, stents like these are frequently required for keeping the bile ducts active under outside pressure.

Ascending Cholangitis Signs and Symptoms

AC patients may experience problems like:

  • Abdominal pain
  • Rigors
  • Fever
  • Malaise, or a feeling of uneasiness

Jaundice may also be reported in some cases.

A physical examination often reveals signs of jaundice as well as abdominal tenderness in the right upper quadrant area. The Charcot’s triad comprises of the three common symptoms associated with this disorder, namely abdominal pain, fever and jaundice. These symptoms were once assumed to be present in almost 50 to 70% of all cases. However, modern findings have limited the presence of these symptoms to 15 to 20% of all instances. Reynolds’ pentad includes mental confusion and septic shock – two more symptoms apart from the three common with Charcot’s triad. The symptoms, as indicated by Reynolds’ pentad, suggest a worsening of this condition and the eventual development of sepsis, which is seen even rarely.  

The presentation of AC may be atypical in elderly patients. They may collapse directly as a result of septicemia without first exhibiting the typical features. Patients having a stent in their bile duct may not develop the symptoms of jaundice.

Ascending Cholangitis Diagnosis

The diagnosis of AC is carried out by running blood tests and obtaining medical images.

Blood Tests

Regular blood samples obtained from the patients of AC show signs of acute inflammation (elevated C-reactive protein levels and raised count of white blood cells), and abnormalities in the LFTs or liver function tests. In the majority of cases, liver function tests tend to be constant with obstruction, and show raised levels of bilirubin, ?-glutamyl transpeptidase and alkaline phosphatase. However, during the early stages, the main feature may be the pressure on liver cells and test results will be similar to hepatitis, showing elevations in the levels of aspartate transaminase and alanine transaminase.

Blood tests are also often performed in patients having fever and signs of acute infection. The tests reveal the infection-causing bacteria in almost 36% of all cases, generally after 24 to 48 hours of incubation. The bile may also be sent for observation during ERCP. The bacteria which are most commonly linked with Ascending Cholangitis are the gram-negative bacilli, such as Enterobacter (5 to 10%), Klebsiella (15 to 20%) and Escherichia coli (25 to 50%). The gram-positive cocci Enterococcus is responsible for 10 to 20% of all cases of AC. Anaerobic organisms like Clostridium and Bacteroides are responsible for a small number of Cholangitis cases, especially in elderly people as well as those who have previously underwent surgery of biliary system. Parasites such as Clonorchis sinensis and Ascaris lumbricoides are believed to cause Cholangitis in the developing countries. In AIDS patients, various organisms have been known to effectively cause AIDS Cholangiopathy. However, proper AIDS treatment has greatly diminished the risk of such cholangiopathy.

Medical Imaging

Since bile duct obstruction is closely associated with AC, various types of medical imaging techniques are employed to ascertain the nature and site of the obstruction. Generally the first tests that are conducted are the ultrasound examinations, as they are the most easily accessible. Ultrasound may display dilation of bile duct and identify around 38% of the bile duct stones. However, it is difficult to identify stones further down one’s bile duct with the help of ultrasound alone. Ultrasound allows distinguishing between Cholecystitis or inflammations of the gallbladder and Cholangitis, both of which has similar symptoms but has different appearances on ultrasound tests. Another test called the magnetic resonance cholangiopancreatography or MRCP uses MRI or magnetic resonance imaging and has its sensitivity comparable to ERCP. However, the smaller stones can still go undetected on MRCP and accuracy of the results depends on the quality of the diagnostic machinery used by the diagnostician.

The best possible diagnostic option for bile duct obstruction is endoscopic retrograde cholangiopancreatography or ERCP. This method uses endoscopy to pass through a small cannula into one’s bile duct. During this point, a radiocontrast agent is injected to make the duct opaque and then X-rays are obtained which gives the doctor a visual image of the patient’s biliary system. The endoscopic representation of the ampulla sometimes shows a protuberant ampulla emanating from a certain impacted gallstone within the common bile duct. Pus may also be seen coming out from the orifice of the common bile duct. The cholangiograms show the gallstones as non-specified areas within the contour of one’s bile duct. ERCP has now been substituted by MRCP for general diagnostic purposes, except in cases of critically ill patients where delays in diagnostic examinations are not acceptable. If the doctor suspects a high possibility of Cholangitis formation, he will normally recommend an ERCP to achieve the drainage of obstructed common bile duct.

Endoscopic ultrasound or EUS and computed tomography tests are performed when factors other than gallstones are believed to be responsible for Cholangitis, such a tumors. Endoscopic ultrasound is used to obtain a tissue sample or biopsy of the suspicious masses. It may also sometimes replace ERCP for various stone diseases.

Ascending Cholangitis Treatment

AC patients need to be admitted to the hospital and treated with antibiotics and intravenous fluids, especially if the patient’s blood pressure is found to be low. Empirical treatment with the usage of broad-spectrum antibiotics is recommended as long as it is known for sure which pathogen is responsible for the causation of the disease as well as what are the antibiotics it is sensitive to. Combinations of aminoglycosides and penicillins are used widely, even though ciprofloxacin has shown its effectiveness in most cases. Ciprofloxacin also causes fewer side effects and hence preferred more to aminoglycosides. The presence of anaerobic pathogens in critically ill patients or patients who are at a risk of developing anaerobic infections are treated by Metronidazole. The patients are treated with antibiotics for 7 to 10 days. Vasopressors are administered to counter problems created by low blood pressure.

Definitive treatment for AC involves bringing the patient relief from the biliary obstruction. This action is normally postponed for about 24 to 48 hours as it becomes necessary to stabilize and improve the patient’s condition with antibiotics. However, if a patient’s health deteriorates despite adequate treatment, this might need to be executed sooner than that.

An endoscopic retrograde cholangiopancreatography is done to unblock the bile duct. The method incorporates endoscopy which involves passing one fiberoptic rube through one’s stomach into duodenum, identifying the ampulla of Vater and then inserting a small tube into bile duct. Sphincterotomy is commonly done to ease flow of bile from bile duct and allow insertions for removing the gallstones that are blocking the patient’s common bile duct. Alternatively, the orifice of the common bile duct can also be dilated by using a balloon. The stones may be removed by direct suction or with the use of various instruments like balloons or baskets which help in pulling the stones into duodenum. Extracorporeal shock wave lithotripsy helps to manage stones that are larger in size and cannot be removed by standard means or broken by ERCP. Electrohydraulic lithotripsy is another method in which a cholangioscope is used to view the stones and very large stones are broken by using shock waves. Choledochotomy, or surgical treatment of the patient’s common bile duct is done very rarely.

The strictures may be expanded by a stent. Plastic stents can be used in simple gallstone complications, while the permanent self-expanding metallic stents are used if tumors like pancreatic cancer cause pressure and leads to obstruction of the bile ducts. A nasobiliary drain, which is similar to nasogastric tube may also be used to facilitate serial x-ray cholangiograms. The treatment methods that are to be used are generally decided based on intensity of the obstruction, the results of the imaging studies and the patient’s response to the antibiotics. Fresh frozen plasma or vitamin K is administered to reduce the risk of bleeding. Percutaneous transhepatic cholangiography or PTC is done when operating on the obstructions becomes difficult due to their locations.

Surgical removal of a patient’s gallbladder or cholecystectomy is done in case of patients who have undergone treatment for cholangitis caused by gallstone disease. This procedure is generally delayed until all the symptoms have been resolved and MRCP or ERCP have confirmed the fact that the patient’s bile duct is free of gallstones. Patients who have not been treated with cholecystectomy have shown an increased probability of suffering from jaundice, recurrent biliary pain and further cholangitis episodes. They also have a greater risk of death.

Ascending Cholangitis Prognosis

AC often leads to death in most patients, the major cause being an irreversible shock accompanied by multiple organ failure. Improvements in diagnostic and treatment procedures have reduced the mortality rate associated with the disease. Prior to 1980, the rate of mortality was more than 50%, which came down to 10to 30% after 1980. Patients showing symptoms of multiple organ failure are most likely to die unless they are treated with early biliary drainage and systemic antibiotics. Some of the other factors that might lead to death of AC patients include:

  • Pneumonia
  • Heart failure
  • Being of female gender
  • Old age
  • History of liver cirrhosis
  • Narrowing of the bile duct, due to cancer
  • Occurrences of liver abscesses
  • Acute renal failure

Ascending Cholangitis Complications

The following complications can be seen in AC sufferers:

Image of Ascending Cholangitis

Picture 2 – Ascending Cholangitis Image

  • Pneumonia
  • Renal failure
  • Wound infection
  • Cardiac arrythmia
  • Respiratory failure
  • Myocardial ischemia
  • Gastrointestinal bleeding

Ascending Cholangitis is considered to be a medical emergency and the survival rate of its patients is found to be poor. However, immediate medical attention can help to effectively manage the symptoms and increase the chance of survival of sufferers.

References:

http://en.wikipedia.org/wiki/Ascending_cholangitis

http://www.ncbi.nlm.nih.gov/pubmed/10987108

http://emedicine.medscape.com/article/774245-overview

http://bestpractice.bmj.com/best-practice/monograph/345/treatment/step-by-step.html

http://www.gpnotebook.co.uk/simplepage.cfm?ID=1624244226

 

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