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Bile Duct Diseases
Medically reviewed on May 14, 2018
What Is It?
Your gallbladder stores bile until you eat, then releases bile into your small intestine to help digest food. Bile is made in the liver. It contains a mix of products such as bilirubin, cholesterol, and bile acids and salts. Bile ducts are drainage “pipes” that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine.
A variety of diseases can affect your bile ducts. All block the bile ducts in some way, 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, the drainpipe at the base of the liver. If the duct remains blocked, bilirubin backs up and enters the blood stream. If bacteria above the blockage accumulates and backs up into the liver, it may cause a severe infection called ascending cholangitis. If a gallstone stops in between the gallbladder and the common bile duct, an infection called cholecystitis may 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. Primary sclerosing cholangitis is more common in people with inflammatory bowel disease (ulcerative colitis or Crohn’s disease). Primary biliary cirrhosis is more common in women. It is sometimes associated with autoimmune diseases such as Sjögren’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 started. Bile duct diseases often cause symptoms related to liver products backing up and leaking into the blood stream. 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)
Light brown urine
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 examine you 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 will need blood tests to measure your levels of alkaline phosphatase, bilirubin and/or gamma-glutamyltransferase (GGT). 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. A blood test with a high level of CA 19-9 may suggest a diagnosis of cholangiocarcinoma.
If your doctor suspects a bile duct problem, additional tests will depend on the suspected cause of the disease. Commonly used tests include:
Right upper quadrant ultrasound. This provides pictures of the liver, gallbladder and common bile duct. For example, it 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. The advantage: MRI images can be obtained without passing an endoscope into the stomach. 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 (cholecystitis), 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 are long-lasting diseases. They 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 are overweight or have high cholesterol, you are at higher risk of developing gallstone. To avoid trouble, work toward a healthy weight through diet and exercise. Also, a near-starvation diet aimed at rapid weight loss can also result in gallstone formation.
Although cholangiocarcinoma is uncommon, smoking appears to increase the risk.
Certain parasite infections (Clonorchis sinensis and Opisthorchis viverrini, also known as Chinese liver fluke) are associated with a higher risk of bile duct diseases. 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 endoscopist may insert various devices into the bile duct to extract the stone. 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. Doctors generally recommend that anyone with a bile duct blockage from a gallstone have his or her gallbladder removed to prevent another blockage.
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. However, most babies with this condition continue to have inflammation due to poor drainage, and eventually develop scarring (cirrhosis) and require a liver transplant.
Because both primary biliary cirrhosis and primary sclerosing cholangitis can cause severe liver failure, a liver transplant may be needed for long-term survival. Treatment may reduce symptoms or delay the progression of the disease. In primary biliary cirrhosis, the medicine used most often is ursodiol ( Actigall ).
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 , 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. Even the most severe infection, ascending cholangitis, has a low death rate if treated promptly.
The prognosis for primary sclerosing cholangitis and primary biliary cirrhosis has improved with better medical treatment and the potential for liver transplantation.
The prognosis for people with cholangiocarcinoma is better if the cancer is discovered while it is still confined to the duct. It can then be treated surgically. Once the cancer has spread, the survival rate is much lower.
Learn more about Bile Duct Diseases
Symptoms and treatments
- Gallbladder and bile duct cancer
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- World J Surg Oncol
- v.13; 2015
Dermatomyositis as the first manifestation of gallbladder adenocarcinoma: case report and literature overview
Dermatomyositis (DM) is characterized by pathognomic cutaneous manifestations (heliotrope rash, periorbital edema, Gottron’s papules) and proximal muscle weakness. In this paper, I will present the case of a 48-year-old female patient whose dermatomyositis was initially diagnosed as vasculitis. Following the patient’s inadequate response to corticosteroid treatment, clinical and radiologic examinations were performed, showing inoperable gallbladder adenocarcinoma. Although initial chemotherapy led to regression, the dermatomyositis developed an independent course with new pathological changes leading to the progression of the disease. I will also present an overview of case reports in English published so far. Gallbladder carcinoma should be added to the list of malignancies with dermatomyositis and has to be excluded by relevant investigation in women.
Dermatomyositis (DM) is an uncommon inflammatory myopathy characterized by pain and weakness in the proximal muscles and cutaneous manifestations. The skin findings of DM include scaly violaceous papules and plaques overlaying the bony prominences of the hands (Gottron’s papules), violaceous patches on the periorbital skin (heliotrope eruption), photodistributed poikilodermatous patches and plaques, scaly plaques on the scalp and lateral thighs, periungual telangiectasia, and ragged cuticles [ 1 ]. Malignancies may occur before, simultaneously with or after the onset of DM [ 2 ]. The most common malignancies associated with DM are ovarian and lung cancer [ 3 ]. The pathogenesis of paraneoplastic DM is still relatively unknown, although there is some evidence to support the involvement of humoral and cell-mediated immune systems and the presence of tumour antigens provoking an autoimmune response [ 3 ]. Treatment of DM is empirical, often involving a combination of steroids and immunomodulatory drugs [ 4 ]. Where DM is associated with underlying malignancy, tumour therapy may lead to the resolution of DM. Interestingly, a reverse relationship also exists, whereby a flare of DM occurs with tumour recurrence – an association that is demonstrated in this report [ 5 ].
A 48-year-old female patient with history of skull fracture suffered in a traffic accident 25 years ago presented in mid-January 2014 in the infectology clinic of her local hospital for facial, neck and ear erythema; butterfly-shaped rash; pain in the upper arm muscles; stiffness of hands and dysphagia. Considering the sedimentation (SE) 30 creatine kinase (CK) 308 result, as well as leukocyte (L) 11.4 (white blood cells (WBC) differential percent neutrophils (% neu) 87, percent lymphocytes (% lymphs) 7.2, percent monocytes (% monos) 5.9, percent eosinophils (% eos) 0), the patient was referred to immunology tests (anti-antinuclear antibodies verified by indirect immunofluorescence (ANA IIF) slightly positive and anti-double-strained DNA (anti-dsDNA), anti-Ro/SSA antibodies (anti-SS-A52), anti-Ro/SSA antibodies (anti-SS-A60), SSB/La antibody (anti-SSB), anti-Sm antibodies directed against 7 proteins (anti-Sm), autoantibodies directed against the RNP/Sm ribonucleoprotein complex (anti-Sm/RNP), anti-DNA topoisomerase 1 antibody (anti-DNA-topo 1), antibody directed against Jo-1 protein (anti-Jo-1), CENP-B specific anti-centromere autoantibodies (anti-CENP-B), autoantibodies to ribosomal P (anti-RIBO P) negative). Since the administered corticosteroid treatment yielded no improvement, a multi-slice computed tomography (MSCT) of the abdomen (Figure 1 ) was performed, showing a pronounced thickening of the cholecyst wall ranging from 11 to 17 millimeters (mm), scattered and eccentric in appearance; two intraluminally present choleliths, one within the fundus 18 × 16 mm and the other infundibular 22 × 16 mm; and no evident pericholecystic edema. The tumour markers are carcinoembryonic antigen (CEA) 0.7 μg/l and carbohydrate antigen 19-9 (CA 19-9) 964 U/ml. Laparoscopically visualized gallbladder showed malign alterations. Urgent cytological analysis showed malign cells, after which the procedure was reverted to laparotomy which verified omentum carcinomatosis, as well as infiltration of the fifth liver segment. Palpable lymph nodes were in the direction of the hepatoduodenal ligament. The procedure was finished with exploration. Final histopathology report of the omentum was adenocarcinoma (diffuse solid clusters of polymorphous atypical hyperchromatic tumour cells and gland formations with atypical mucous-cylindrical epithelium).
Preoperative MSCT of the abdomen and the pelvis.
Oncologist suggested treatment with cisplatin 25 mg/m2 (per square meter of body-surface area) and gemcitabine 1,000 mg/m2, each administered on days 1 and 8 and repeated every 21 days for up to 6 cycles, unless disease progression or unacceptable toxicity occurs. Prior to starting chemotherapy in March 2014, the dermatologist increased the corticosteroid dosage (methylprednisolone 60 mg/day) due to newly manifested periorbital edema and macular exanthem in the regions of the trunk, neck and dorsum of the hand (Figures 2 and and3).3 ). The patient refused the recommended skin biopsy. Still, there was some improvement in muscular strength.
Periorbital edema and macular exanthem of the face.
Gottron’s papules on the metacarpophalangeal and interphalangeal joints.
Prior to the first cycle of chemotherapy, the patient was given two doses of erythrocyte concentrate for normocytic anaemia (haemoglobin (Hgb) 95 g/l, haematocrit (Ht) 0.345 l/l, mean corpuscular volume (MCV) 82.8 fl). After 10 days of high-dose corticosteroid therapy, the skin eruptions faded. During and after the second cycle of chemotherapy, the patient complained of general weariness and fatigue.
After 3 cycles of chemotherapy, new skin changes developed on the right upper arm and both thighs, with periorbital edema more pronounced on the left side, despite the 32 mg/day maintenance dose of methylprednisolone. A follow-up MSCT of the abdomen showed a 12-mm thickening of the cholecyst wall – a regression, compared to the initial MSCT result – and increased tumour marker levels (CEA 1.6 μg/l, CA 19-9 2,525 U/ml). During the next three chemotherapy cycles, additional skin changes manifested in the armpit and on the scalp; some marker levels also increased (CA 19-9 3,259 U/ml; the gamma-glutamyl transferase (GGT) doubled). Final MSCT of the thorax, abdomen and pelvis was performed after treatment completion in August 2014, showing a progression of hepatic changes and ascites, after which the patient was referred to palliative care.
The patient presented in the case report was treated using the standard care of gall bladder carcinoma. The Helsinki Declaration was obeyed. Ethical board of Hospital Sisters of Charity gave the approval.
Gallbladder carcinoma is the most common and the most malignant tumour of the biliary tract. It occurs mostly between the ages of 60 and 70 and is 2 to 6 times more common in women. Histologically, 95% of gallbladder carcinoma patients have adenocarcinoma; this was also the case with my patient, as the final histopathology report of the omentum showed. Interestingly, the patient was not aware of her long-standing cholelithiasis and had no pathological condition connected to increased risk.
Gallbladder carcinoma has an extremely bleak prognosis; only 5% to 10% of patients who undergo surgery are likely to survive the following 5 years [ 6 ]. Also, one third of patients whose surgery is finished with exploration show peritoneal and liver metastases that were not visible on MSCT or MRI. In cases of suspected disseminated malignant disease, diagnostic laparoscopy is recommended [ 7 ]. My patient’s preoperative CT of the thorax, abdomen and pelvis showed no sign of liver or peritoneal metastases.
Although skin biopsy was not performed on my patient and although she did not test positive for autoantibodies usually present in patients suffering from dermatomyositis, it is my opinion that her clinical presentation is consistent with paraneoplastic dermatomyositis. The patient underwent chemotherapy with cisplatin and gemcitabine, which was in accordance with the results of the study of phase III of patients with advanced or metastatic carcinoma. Adding cisplatin to gemcitabine, that is its interaction between gemcitabine and cisplatin afforded significant progression-free survival (median, 8.4 versus 6.5 months; hazard ratio (HR), 0.72; 95% confidence interval (CI), 0.57 to 0.90; p = .003) and overall survival benefits (median, 11.7 versus 8.3 months; HR, 0.70; 95% CI, 0.54 to 0.89; p = .002). The most common side effects include decreased WBC count and fatigue [ 8 ]. My patient never exhibited decreased WBC count, which was partly due to the effects of corticosteroid therapy. Since receiving the second cycle of chemotherapy, she complained of increased general weariness and fatigue, which could be due to the chemotherapy treatment.
In January 2015, I searched PubMed for terms gallbladder cancer, gallbladder carcinoma and dermatomyositis and found a total of six case reports in English describing dermatomyositis in patients suffering from gallbladder carcinoma [ 9 – 13 ]. All of the cases featured female patients over 44 years of age, some with present risk factors (previously verified gallstones). The time interval between the appearance of dermatomyositis and malignant disease ranged from 2 weeks to 2 years. Considering the patient was initially treated in a relatively small facility and the administered treatment did not lead to improvement of her condition, it was soon suspected she had a malignant disease.
In the cases I studied, malignant disease was diagnosed within 2 to 7 months.
My patient initially had elevated CK, slightly positive ANA IIF and negative anti-Jo-1, as described in other patients. The tumour markers were initially determined to be CEA and CA 19-9. CEA was within range, whereas CA 19-9 was 24 times above normal. Elevated serum CEA and CA 19-9 levels could be suggestive of gallbladder cancer, bearing in mind that CA 19-9 has higher specificity, and CEA a higher sensitivity [ 14 ]. Although some authors describe a complete regression of skin changes following adequate treatment, in the case of my patient, the dermatomyositis and the malignant disease progressed independently of one another [ 13 ]. Following high-dose corticosteroid therapy, the skin changes faded within the same period as described in the case report of another patient; however, after 3 cycles of maintenance therapy, they progressed, although the radiology report verified the regression of the disease.
Gall bladder carcinoma should be added to the list of malignancies with dermatomyositis and has to be excluded by relevant investigation in women, especially since an inexpensive, simple and easily available initial testing exists – abdomen sonography.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
I would like to express my sincere gratitude to my PhD mentor, Professor Bozo Kruslin, for his perceptive questions which made me look deeper into the core of the topic. I would also like to thank Mrs. Anica Vrdoljak for technical support and Mrs. Iva Martina Bura for providing language help.
|% eos||percent eosinophils|
|% lymphs||percent lymphocytes|
|% monos||percent monocytes|
|% neu||percent neutrophils|
|ANA IIF||antinuclear antibodies verified by indirect immunofluorescence|
|anti-CENP-B||CENP-B specific anti-centromere autoantibodies|
|anti-DNA-topo 1||anti-DNA topoisomerase 1 antibody|
|anti-Jo-1||antibody directed against Jo-1 protein|
|anti-RIBO P||autoantibodies to ribosomal P|
|anti-Sm||anti-Sm antibodies directed against 7 proteins|
|anti-Sm/RNP||autoantibodies directed against the RNP/Sm ribonucleoprotein complex|
|CA 19-9||carbohydrate antigen 19-9|
|MCV||mean corpuscular volume|
|MSCT||multi-slice computed tomography|
|WBC||white blood cells|
The author declares no competing interests.
PJ wrote the initial draft and composed the manuscript of this paper. The author has read and approved the final manuscript.
Articles from World Journal of Surgical Oncology are provided here courtesy of BioMed Central
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