Bile Duct Diseases
What Is It?
Bile ducts are small drainage “pipes” that carry bile (a mix of cholesterol, acids, salts and waste products) from the liver to the gallbladder and from the gallbladder to the small intestine. Your gallbladder stores bile until you eat, then releases bile into your small intestine to help digest food. A variety of diseases can affect your bile ducts. All cause some form of blockage, which is why the various diseases cause similar symptoms.
Gallstones are the most common cause of blocked bile ducts. Stones typically form inside the gallbladder and can block the common bile duct, a large drainpipe at the base of the liver. If the duct remains blocked, waste can accumulate in the bile duct system and in the bloodstream. Also, if bacteria above the blockage accumulates and backs up into the liver, a severe infection called ascending cholangitis may result. If a gallstone stops in between the gallbladder and the common bile duct, an infection called cholecystitis can occur.
Less common causes of blockages include cancers of the bile duct (cholangiocarcinomas) and strictures (scars that narrow the ducts after infection, surgery or inflammation).
Other bile duct diseases are uncommon, and include primary sclerosing cholangitis and primary biliary cirrhosis. Typically diagnosed in mid-adulthood, these conditions create ongoing inflammation in the bile duct walls, which can narrow and scar the walls. These conditions have an inherited (genetic) component, although other factors seem necessary to trigger the disease. Primary sclerosing cholangitis is more common in men, and 75 percent of the time it is seen in people with inflammatory bowel disease (ulcerative colitis or Crohn’s disease). Primary biliary cirrhosis is more common in women, and is sometimes associated with autoimmune diseases such as Sjogren’s syndrome, thyroiditis, scleroderma or rheumatoid arthritis.
Biliary atresia is a rare form of bile duct blockage that occurs in some infants two weeks to six weeks after birth, a time when the bile ducts have not completed their development normally.
The chronic conditions of primary sclerosing cholangitis, primary biliary cirrhosis and Biliary atresia can result in inflammation and scarring of the liver, a condition known as cirrhosis.
Symptoms of a blocked bile duct may be abrupt and severe (for example, when a gallstone blocks the whole drainage system all at once), or they may appear slowly many years after bile duct inflammation has begun. Bile duct diseases cause some symptoms when waste products build up in the body. Other symptoms result from the bile ducts’ failure to deliver certain digestive juices (bile salts) to the intestines, preventing the absorption of some fats and vitamins. Symptoms of a blocked bile duct include:
- Yellowing of the skin (jaundice) or eyes (icterus), from the buildup of a waste product called bilirubin
- Itching (not limited to one area; may be worse at night or in warm weather)
- Dark, cola colored urine
- Weight loss
- Fever or night sweats
- Abdominal pain, especially common on the right side under the rib cage
- Greasy or clay colored stools
- A diminished appetite
Your doctor may suspect that you have a bile duct problem if you have any of the classic symptoms or if a blood test shows that you have a high level of bilirubin. Your doctor will take your medical history and conduct a physical exam to look for clues that could explain damage to the bile ducts and liver. Because liver inflammation (hepatitis) and liver scarring (cirrhosis) can cause similar symptoms, your doctor will ask about your alcohol use, drug use and sexual practices, all of which can result in liver disease. If you have gallstones, have had pancreatitis or abdominal surgery, or have symptoms of an autoimmune condition (such as arthritis pain, dry mouth or eyes, skin rashes or bloody diarrhea), tell your doctor. Because some medicines can slow drainage through the bile ducts, your medicines should be reviewed.
You may need blood tests to measure your levels of alkaline phosphatase, bilirubin or gamma-glutamyltransferase. These are markers of bile duct obstruction. Other blood tests may suggest liver inflammation or cirrhosis. Occasionally, specialized blood tests may be helpful, such as antibody tests to diagnose primary biliary cirrhosis or primary sclerosing cholangitis, and a blood test that measures CA 19-9, which could indicate cholangiocarcinoma.
If your doctor suspects a bile duct problem, additional tests will depend on the suspected cause of the disease. Commonly used tests include:
- Ultrasound of the liver, which can show enlargement of the ducts above a blockage
- Computed tomography (CT) scan or magnetic resonance imaging (MRI) scan of the liver
- Endoscopic retrograde cholangiopancreatography, an examination in which a small camera on a flexible cord is inserted through your mouth and down past your stomach to the opening where the common bile duct empties into your stomach. A dye can be injected into the common bile duct that will appear on X-rays. How the bile ducts look on the X-rays can provide clues to the problem. Sample cells from the bile duct walls can be examined under a microscope for evidence of cancer. Treatments to relieve blockages can be performed during this examination.
- Magnetic resonance cholangiopancreatography, an examination similar to the endoscopic exam above, but MRI images can be obtained without passing an endoscope into the stomach. The best information is obtained from this test when radiologists have good experience reading the results. The disadvantage of this test is that tissue for a biopsy (laboratory examination) cannot be obtained.
- Cholangiography (X-rays of the bile ducts), which can also be done after dye is injected into the liver. This enables doctors to watch the flow of bile as it drains from the liver. Tissue for biopsy can be obtained during this procedure and any blockages or narrowing can be relieved.
- A liver biopsy sample, obtained using a needle through the skin. The tissue is examined for evidence of inflammation or cancer.
If you have a chronic form of bile duct disease, your doctor may check you for cholesterol abnormalities or osteoporosis. Both of these conditions are more common in someone with longstanding bile duct drainage abnormalities.
To treat a gallstone blockage and infection, doctors first prescribe antibiotics. After the infection subsides, a surgeon removes the gallbladder. Symptoms caused by a scar (stricture) may improve rapidly after treatment restores the duct’s drainage.
Symptoms of primary biliary cirrhosis and primary sclerosing cholangitis may steadily get worse and lead to cirrhosis and liver failure after years of damage. When liver failure develops, a liver transplant can improve survival. However, primary sclerosing cholangitis and primary biliary cirrhosis can return after transplant.
If you have gallstones, you can prevent bile duct blockage and serious infection (ascending cholangitis or cholecystitis) by having your gallbladder removed. This is done using small incisions in the abdomen, in a surgery called laparoscopic cholecystectomy.
If you are overweight or have High cholesterol, you are at higher risk for developing gallstone. To avoid trouble, work toward a healthy weight through diet and exercise. Women who take birth control pills or hormone replacement therapy are at higher risk for gallstones and may factor this risk in their decision to use these medications.
Although cholangiocarcinoma is uncommon, risks associated with developing it include smoking, eating a high carbohydrate diet and having gallstones.
Certain parasite infections (Clonorchis sinensis and Opisthorchis viverrini, also known as Chinese liver fluke) can increase the risk for bile duct infections and cancers. If you travel to Southeast Asia, eat fish only if it is well cooked. If you do eat undercooked fish while traveling in this area, ask your doctor for a stool parasite test, especially if you have symptoms of weight loss or diarrhea.
To treat a gallstone blockage accompanied by signs of persistent pain or infection, a gastroenterologist or surgeon can remove stones in the bile duct using endoscopic retrograde cholangiopancreatography. The endoscope cuts through the base of the common bile duct, allowing a stone to pass through. In some cases the endoscope may be used to remove the stone using wire loops. Doctors recommend that anyone with a bile duct blockage from a gallstone have his or her gallbladder removed, so that another blockage will not occur.
This same procedure can widen an area of scarred bile duct (a stricture) by inserting and expanding a wire coil (called a stent) within the duct.
It’s rare to find bile duct cancer early, but if it is found early, it can be treated with surgery. When cancer is more advanced, surgery cannot totally remove the tumor. Surgical procedures can help cancer patients feel better, even if they cannot provide a cure. Surgery can reroute the bile duct to allow better drainage. Radiation treatments can help to shrink (but not cure) a bile duct tumor.
Biliary atresia, the failure to develop normal bile ducts in infants, can be treated by surgery. One method uses a portion of the baby’s intestines to substitute for missing segments of the bile duct. Another method requires rerouting the bile drainage and additional intestinal surgery. Unfortunately, most babies with this condition continue to have inflammation due to poor drainage, and will eventually develop scarring (cirrhosis) and require a liver transplant.
Because both primary biliary cirrhosis and primary sclerosing cholangitis cause the liver to get worse, a liver transplant is eventually needed for long term survival. Several treatments may reduce symptoms or delay the progression of the disease. In primary biliary cirrhosis, the medicines ursodiol (Actigall), colchicine and methotrexate (Folex, Rheumatrex) can be effective.
The most bothersome symptom in chronic bile duct disease, itching, can be reduced with medicine — cholestyramine (Questran) or colestipol (Colestid) — that prevents irritants in the gut from being absorbed. Another medicine, naloxone (Narcan), might neutralize irritants that cause itching. If greasy stools are a problem, a low fat diet may be helpful. Doctors recommend multivitamin supplements to improve nutrition.
When To Call A Professional
If you develop yellowing of the skin or eyes, contact your doctor. If you also have fever or abdominal pain, call for professional advice immediately.
Infections related to gallstone blockage have excellent results when treated. The most severe infection, ascending cholangitis, has a death rate of less than 1 percent if treated promptly.
A majority of people with primary sclerosing survive only nine to 12 years after they’ve been diagnosed if they don’t get a liver transplant. People with primary biliary cirrhosis typically survive between 10 and 17 years after they’ve been diagnosed (if they have no symptoms at the time of diagnosis), and close to seven years once symptoms develop. Because these diseases are so predictable, and because they occur in young adults, people with these conditions are given high priority for liver transplants. Because there is a high risk of complications and death during a liver transplant, transplants are only recommended when more severe symptoms have developed. There is also the possibility of the disease returning in the new liver.
The survival rate for people with cholangiocarcinoma is poor because most people are not diagnosed until the cancer has reached an advanced stage. The five year survival rate for bile duct cancers overall is only between 5 percent and 10 percent. If the cancer has not deeply invaded the body, however, surgery can produce excellent results and allow long term survival (at least five years) for 75 percent to 80 percent of very early cancers.
by Martin A. Harms, M.D.
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