Bile Duct Diseases and Problems Introduction A bile duct is a tube that carries bile between the liver and gallbladder and the intestine. Bile is a substance made by the liver that helps with digestion. There are many problems that can affect your bile ducts. Treatment for bile duct diseases depends on the cause. Treatment may include surgery, medicines, or other treatments. Liver Gallbladder This reference summary will help you understand diseases and problems that affect the bile ducts. It discusses common diseases and problems of the bile ducts, as well as treatment options. The Biliary System The biliary system is made up of the organs and ducts that make and store bile. This section reviews the anatomy of the biliary system to help you understand bile duct diseases and problems. The gallbladder is a pear-shaped organ that lies just under the liver in the upper right abdomen. Gallbladder Special digestive juices called bile are made in the liver. Bile contains water, cholesterol, fats, bile salts, proteins, and bilirubin. Bile salts break up fat, and bilirubin gives bile and stool a yellowish color. 1
Excess bile is stored in the gallbladder. Bile ducts are small tubes that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Many small ducts in the liver come together to form the right hepatic duct and the left hepatic duct, which lead out of the liver. These two ducts join outside the liver to become the common hepatic duct. The part of the common hepatic duct that is outside the liver is called the extrahepatic bile duct. From this duct, bile may flow through the cystic duct to be stored in the gallbladder. When you eat fatty foods, the gallbladder contracts and pushes the bile into a tube called the cystic duct. Common hepatic duct From the cystic duct, the bile flows to the small intestines through the common bile duct. In the small intestines, bile helps with digestion. General Symptoms Diseases and problems with the bile ducts can cause different symptoms depending on the cause. But, many bile duct diseases and problems have similar symptoms. The main symptom of bile duct diseases or problems causing blockage is jaundice. Jaundice is a yellowing of the skin and whites of the eyes. It is caused by a buildup of bilirubin in the skin and white area of a person s eyes. Bilirubin is a substance formed when red blood cells are broken down. Bilirubin is a part of the bile. Other common symptoms of bile duct diseases and problems include: Fatigue Fever Itchy skin all over the body Pain in the abdomen 2
Some bile duct diseases and problems may also cause: Chills Clay-colored stools Dark urine Dry eyes and mouth Grey or white stools Low-grade fever Weight loss Gallstones may also cause different symptoms, sometimes called a gallstone attack. These include: Nausea or vomiting Pain in the back between the shoulder blades Pain under the right shoulder Steady pain in the upper abdomen that increases rapidly and lasts from 30 minutes to several hours Gallstone attacks often follow fatty meals. They may also cause bloating, gas, and indigestion. If you notice any of these symptoms or other changes, talk to your healthcare provider. Finding a problem early makes treatment easier in most cases. Gallstones Gallstones are a relatively common condition that can cause serious problems, such as a blocked bile duct. Gallstones form when bile stored in the gallbladder hardens into pieces of stone-like material. Bile can harden into stones under certain conditions if it contains too much cholesterol, bile salts, or bilirubin. Gallstones can be as small as a grain of sand or as large as a golf ball. The gallbladder can develop just one large stone, hundreds of tiny stones, or almost any combination. Gallstones 3
Gallstones can block the normal flow of bile if they lodge in any of the bile ducts. Gallstones can block the hepatic ducts, which can cause inflammation of the liver and jaundice. Gallstones can block the cystic duct, which can cause inflammation of the gallbladder. Gallstones can also block the common bile duct, which can cause inflammation of both the gallbladder and liver. Other ducts open into the common bile duct, including the pancreatic duct, which carries digestive enzymes out of the pancreas. If a gallstone blocks the opening to that duct, digestive enzymes can become trapped in the pancreas. This causes an extremely painful inflammation called gallstone pancreatitis. If any of these ducts remain blocked for a significant period of time, severe and life threatening infections can happen. These infections can affect the gallbladder, liver, or pancreas. Surgery to remove the gallbladder is the most common way to treat symptomatic gallstones. The surgery is called cholecystectomy. The liver will still produce bile and it will empty it directly into the small intestines without affecting digestion and health. Nonsurgical approaches are used only in special situations, such as when a patient has a serious medical condition preventing surgery. These approaches use drugs or shock waves to dissolve or break up the stones. Stones usually form again after a nonsurgical treatment. Primary Sclerosing Cholangitis Primary sclerosing cholangitis is a disease that damages and blocks the bile ducts inside and outside the liver. Cholangitis refers to inflammation of the bile ducts. Sclerosing refers to scarring. Sclerosing cholangitis refers to the scarring of the bile ducts caused by inflammation. The disease is also known as PSC for short. In PSC, inflammation of the bile ducts leads to scar formation and narrowing of the ducts over time. As scarring increases, the ducts become blocked. As a result, bile builds up in the liver and damages liver cells. 4
Eventually scar tissues can spread throughout the liver, causing cirrhosis. Cirrhosis can lead to liver failure and death. The causes of PSC are not known. Genes, immune system problems, bacteria, and viruses may play roles in the development of the disease. PSC is linked to inflammatory bowel disease, or IBD. About three out of four people with PSC have a type of IBD called ulcerative colitis. The link between PSC and IBD is not yet understood. Most people with PSC are adults, but the disease also happens in children. The average age at diagnosis is forty. However, PSC progresses slowly, so a person can have the disease for years before symptoms appear. Treatment of PSC aims to relieve symptoms and manage complications. Medical treatment may include: Antibiotics to treat infection Medications to relieve itching Procedures to open blocked bile ducts Vitamin supplements A liver transplant may also be an option if the liver begins to fail. During a liver transplant, the old, damaged liver is surgically removed and a new, donated liver takes its place. Primary Biliary Cirrhosis Primary biliary cirrhosis is a disease that causes the bile ducts in the liver to become inflamed and damaged. Eventually, the bile ducts will become completely destroyed. Without bile ducts in the liver, bile cannot leave the liver. When bile builds up it injures liver tissue. Injured liver tissue from inflammation and the buildup of bile leads to cirrhosis. Cirrhosis causes scarring and may lead to liver failure and death. 5
The cause of primary biliary cirrhosis is unknown. However, it is thought to be an autoimmune disease. Autoimmune diseases are when the immune system attacks the body s own tissues. In primary biliary cirrhosis, the immune system attacks the bile ducts. Primary biliary cirrhosis usually happens between the ages of forty and sixty. Most people are diagnosed early, before the disease progresses. Early treatment can delay the eventual onset of cirrhosis and liver failure. A medicine called ursodiol (Actigall ) assists the liver in moving bile through the ducts to the gallbladder and small intestine. This improves liver function and slows the progression of the disease. Other treatments may include: Calcium supplements Medications to relieve itching Vitamin replacement therapy People with primary biliary cirrhosis should also eat a balanced diet. A healthcare provider or dietician can help create a balanced diet, including which foods to avoid. People with primary biliary cirrhosis should also avoid alcohol and illicit substances. These can cause more damage to the liver. A liver transplant may be an option if the liver begins to fail. During a liver transplant, the old, damaged liver is surgically removed and a new, donated liver takes its place. Biliary Atresia Biliary atresia is a life threatening condition in infants. It happens when the bile ducts inside or outside the liver do not have normal openings. With biliary atresia, bile becomes trapped, builds up, and damages the liver. The damage leads to scarring, loss of liver tissue, and cirrhosis. 6
Biliary atresia likely has many causes, though none are yet proven. It may be caused by: A problem during liver and bile duct development in the womb A viral or bacterial infection after birth An immune system problem Exposure to toxic substances Genetics Biliary atresia must be treated with surgery. Without surgery, infants with biliary atresia are unlikely to live past age two. Children with biliary atresia may also need to be on special diets or vitamin supplements. A healthcare provider can tell you more about these treatments. Cancer Problems with the bile ducts may also be caused by cancer. Cancer begins in your cells, which are the building blocks of your body. Normally, your body forms new cells as you need them, replacing old cells that die. Sometimes this process goes wrong and a tumor forms. If a tumor is cancerous, cells from the tumor can invade other tissues throughout the body. Cancerous cells can spread to different parts of the body through blood vessels and lymph channels. Cancers in the body are given names depending on where the cancer started. Cancer that begins in the bile ducts will always be called bile duct cancer, even if it has spread to other places in the body. Bile duct cancer is a rare type of cancer. Some health conditions raise your risk for bile duct cancer, including: Bile duct structural problems Inflammation and scarring of the bile ducts Liver infection caused by a parasite. A parasite is an animal or plant that gets nutrients by living on or in another organism Ulcerative colitis; ulcerative colitis is chronic inflammation of the colon. This produces ulcers in the lining of the colon. 7
Treatment for bile duct cancer may include surgery, radiation therapy, and chemotherapy. Often some combination of these treatments is used. Radiation therapy uses high-energy rays to kill cancer cells and stop them from growing and spreading. Chemotherapy is the use of drugs to kill cancer cells. Summary A bile duct is a tube that carries bile between the liver and gallbladder and the intestine. Bile is a substance made by the liver that helps with digestion. There are many problems that can affect your bile ducts. Some problems include gallstones, primary sclerosing cholangitis, primary biliary cirrhosis, biliary atresia, and cancer. Treatment for bile duct diseases depends on the cause. Treatment may include surgery, medicines, or other treatments. It may also include radiation therapy or chemotherapy if the cause is cancer. Talk to your healthcare provider if you notice any abnormal changes or symptoms. Finding a problem early makes treatment easier in most cases. 8