Biliary Disorders and Bile Duct Cancer

The biliary duct system is responsible for storing and transporting bile, a yellowish digestive fluid produced in the liver. The bile ducts are the tubes from which bile flows out from the liver. Bile ducts come together like the branches of a tree. The largest duct is called the common bile duct. Before bile is transported to the small intestine to aid in the digestive process, it is collected and concentrated in a storage container called the gallbladder. After a person eats, the gallbladder contracts and forces bile back into the common bile duct and on to the small intestine. Biliary disorders can disrupt the flow of bile, which, in turn, can cause serious health problems. Learn more about the biliary disorders treated at Emory: Biliary Stricture, Biliary Leaks, Primary Biliary Cirrhosis, Primary Sclerosing Cholangitis, and Choledochal Cysts below:

Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a disease that, over time, causes permanent damage to the liver's bile ducts. In people with PSC, inflammation and scarring cause the bile ducts to become blocked. Bile then accumulates in the liver, where it gradually causes cirrhosis (scarring). Backed-up bile can also become infected, causing acute illness. As scar tissue replaces healthy liver tissue, the liver loses its ability to function.

All this happens very slowly, over the course of 10 to 15 years. Most people with PSC are asymptomatic, especially early on. When symptoms do develop, they tend to come and go. Symptoms of primary sclerosing cholangitis include extreme itching, fatigue and jaundice (yellowing of the skin and eyes). Symptoms of a bile infection include fever, chills and abdominal pain. Because people with PSC rarelyt have symptoms, it is often discovered when they undergo routine liver tests. A formal diagnosis is made using cholangiography, a type of X-ray performed under sedation.

There is no cure for PSC. A liver specialist will prescribe medications and diet changes to control symptoms, and in some cases surgery may help improve bile flow. For most people, PSC leads to liver failure, though it can also lead to bile duct cancer. Many people with PSC will need a liver transplant.

Primary Biliary Cirrhosis

In primary biliary cirrhosis, inflammation destroys the bile ducts and prevents bile from escaping the liver. The accumulated bile damages healthy liver tissue, eventually leading to cirrhosis (scarring). As scar tissue replaces healthy liver tissue, the liver loses its ability to function. All this happens very slowly. People with PBC can lead healthy, symptom-free lives for 10 years or more after diagnosis.

Though primary biliary cirrhosis is often asymptomatic, especially early on, the most common symptom is extreme itching, especially in the arms, legs and back. Other symptoms include fluid buildup in the abdomen or legs, jaundice (yellowing of the eyes and skin), or fatty deposits and darkening of the skin under the eyes.

The standard treatment for PBC is a daily dose of a medication called ursodiol. Ursodiol improves liver function and increases life expectancy in people with PBC. Other medications may be indicated for controlling symptoms.

Biliary Stricture

A biliary stricture is a narrowing of the common bile duct. When this happens, bile can back up into the liver, causing abdominal pain, nausea, itching, fever, chills, and jaundice. Strictures can be caused during surgery on nearby tissues, such as the gallbladder. Other conditions, including pancreatitis, gallstones, or primary sclerosing cholangitis, can also cause biliary stricture. Treatment involves surgery  to reopen the duct so that bile flow can return to normal.

Choledochal Cysts

Choledochal Cysts are a congenital abnormality that usually presents in childhood. They are cysts that grow on or around the bile duct and causes abnormal enlargement. Different types of chledochal cysts can be found depending on the location within the bile duct.

Adult presentation is rare and usually exhibits one or more severe complications. Frequently, adults with choledochal cysts complain of vague pain in the upper middle region or right upper quadrant and can develop jaundice or cholangitis. The most common symptom in adults is abdominal pain. A classic triad of abdominal pain, jaundice, and a palpable right upper quadrant abdominal mass are symptoms found in only 10 percent to 20 percent of patients.

Adults with cysts are especially predisposed to the development of cholangiocarcinoma, with the occurrence increasing from 0.7% in the first decade of life to >14% after the age of 20 years. Because of the risk of malignant change in choledochal cysts, the ideal treatment is surgical removal.

Bile Leak

Bile leaks are a rare but serious complication of gallbladder surgery. If a bile duct is damaged during surgery, bile may leak into the abdominal cavity, causing extreme pain. Bile leaks are often corrected by placing a stent (narrow tube) in the duct to keep bile from escaping while the duct heals.

Biliary reconstruction describes a variety of surgical procedures that are performed to rebuild damaged portions of the biliary system. Some conditions that may require biliary reconstruction include bile duct obstructions and bile duct leaks. Such reconstruction can be performed using interventional radiology or ERCP.

It is estimated by the National Cancer Institute (NCI) that...

In the U.S. in 2011 there were 9,250 new diagnosed cases of gallbladder cancer or other biliary cancers and 3,300 deaths as a result of biliary and gallbladder cancer.