Disorders of the Biliary Tract :
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Although disorders of the biliary tract don’t garner as much attention as other conditions, they are common. Today’s endoscopy team faces more and more of these disorders every day.
“In terms of biliary endoscopy, we’ve gone from an era a decade ago where endoscopic approaches to the biliary tree were primarily diagnostic — Is there or is there not a stone? Is there or is there not a stricture? — to an era in which the radiology is so good that we can do all of that with either CT (computed tomography) scanning or MRI (magnetic resonance imaging), Over 90 percent of these procedures are now therapeutic, with an overall excellent efficacy and very low risk to the patient with real advances in terms of morbidity and mortality.”
The classic biliary tract diseases can be divided into those that primarily affect children and those that affect adults. The most common indication for liver transplantation in infants and young children is a disease referred to as biliary atresia.
Biliary atresia affects approximately one in every 15,000 live births. Due to bile ducts of the liver not having normal openings, bile becomes trapped in the liver, causing jaundice and cirrhosis. The diagnosis of biliary atresia occurs usually in neonates who remain jaundiced for six weeks or more after birth and have pale stool and dark urine. Some patients will have a jaundice-free period of several weeks before the onset of unremitting jaundice. The condition is fatal if left untreated.
No curative therapy for biliary atresia exists. Surgical treatment, the Kasai portoenterostomy, restores bile flow and clears jaundice, and if successful, achieves a 10-year survival rate. Yet even with early surgery, 60 percent to 80 percent of patients eventually develop end-stage biliary cirrhosis and require liver transplantation.3
Delayed recognition of the disease and delayed referral for specialty care remain major obstacles to optimal timing of the initial surgical intervention. “Recognition is key “The closer you get to [the first 90 days of life], the worse the prospect that you will have a good outcome of the Kasai procedure.”
Primary Sclerosing Cholangitis
Although primary sclerosing cholangitis (PSC) is primarily a disease of the bile ducts of the liver, ducts of the gallbladder and pancreas may also be involved.
Inflamed bile ducts become scarred and hardened, eventually causing liver failure. The disease may be present for several years before symptoms develop, such as fatigue, loss of appetite, weight loss and jaundice. PSC is diagnosed through cholangiography, which involves injecting dye into the bile ducts and taking an X-ray.
About 70 percent of patients who have PSC usually have underlying inflammatory bowel disease, mainly ulcerative colitis.
PSC is more common among men than women. It usually begins between ages 30 and 60, but the disease can also arise during childhood. Due to the difference in clinical presentation of PSC in children, a high degree of suspicion is often required to make the diagnosis of childhood PSC.Most children present with non-specific symptoms, including fatigue and weight loss and, less commonly, itching.8
When you think of the cholestatic diseases in children or adults, technicians and people that may see them for endoscopic reasons should be aware that itch is a very important system that may be related to the disease.
For PSC, there isn’t a lot of effective treatment, You can try endoscopic therapy, which could be attempted to make the patient feel better. Unfortunately, it doesn’t change the natural history of the disease. Most of the patients will undergo cirrhosis and permanent liver damage.
The only cure for PSC will be liver transplantation. This disease has a very worrisome feature. It should be considered a pre-malignant condition because, regardless of how long you have had it, there is a risk for development of cholangiocarcinoma." Cholangiocarcinoma, a malignant tumor of the bile duct, occurs in approximately two out of 100,000 people.
Primary Biliary Cirrhosis
Primary biliary cirrhosis (PBC), a chronic liver disease, is more prevalent than was previously suspected. The disease affects females more often than males, with onset between the ages of 30 and 60 years. PBC, up to the time of a patient developing actual cirrhosis, is a misnomer. It is only the fourth and final stage of the disease that is truly a biliary cirrhosis.
PBC is characterized by progressive destruction of the bile ducts in the liver, with inflammation leading to scarring and cirrhosis. Symptoms can include itching, jaundice, cholesterol deposits on the skin, fluid retention and dry eyes or mouth. Asymptomatic patients usually survive more than 10 years, while patients with symptomatic PBC and jaundice survive approximately seven years.9 Diagnosis is based on physical examination, biochemical tests and liver biopsy.
The new advances in this disease relate to a recognition of its primary auto antibody marker, which is referred to as antimitochondrial antibody, or AMA. AMAs are found in 96 percent of patients with PBC. The presence of AMA is almost diagnostic of the disorder.
Identification of PBC is important because effective treatment with ursodeoxycholic acid has been shown to halt disease progression and improve survival without need for liver transplantation.12
A discussion about diseases of the biliary tract would not be complete without mentioning gallstones. Approximately 16 to 22 million people have or have had gallstones in the United States.13
The management of gallstone disease accounts for 1 percent of annual healthcare expenditures in the United States, approximately $6 billion.
There are two main types of stones. Cholesterol stones arise exclusively in the gallbladder and consist of 50 percent to 100 percent cholesterol.15 Pigment stones may arise anywhere in the biliary tree and are classified as black or brown.
Symptoms often include fever, jaundice and persistent pain. Ultrasonography provides a simple, quick method of diagnosis. It has a 95-percent sensitivity and specificity for stones more than 4 millimeter in diameter.
“Not everybody with gallstone disease needs a cholecystectomy,” Michael says. “About 80 percent of patients remain asymptomatic and only 20 percent of patients run into trouble from their gallbladder disease. It is now recognized that the presence of gallstones in the absence of classic attacks of cholecystitis leads to a recommendation that the gallstones be noted present but no interventions such as surgical removal or cholecystectomy be performed. Thus, the number of cholecystectomies that were previously performed when gallstones were identified — but not necessarily in the context of acute cholecystitis — has decreased in the United States.
With any disease, including gallstones, it is often difficult to foresee advances in diagnosis and treatment. There are current attempts to try to identify genetic changes that are responsible for gallstone formation or the development of biliary cancer. “Once you understand the mechanism, you can try to develop targets for therapy.”
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