Disorders of the Biliary Tract

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),” says Jean-Pierre Raufman, MD, head of the Division of Gastroenterology and Hepatology at the University of Maryland School of Medicine in Baltimore. “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.”

Biliary Atresia

“The classic biliary tract diseases can be divided into those that primarily affect children and those that affect adults,” says John M. Vierling, MD, director of hepatology and medical director of multi-organ transplantation at Cedars-Sinai Medical Center in Los Angeles, Calif. “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.¹ 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.² 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.⁴ “Recognition is key,” Vierling says. “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.⁵ 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,” says Hazar Michael, MD, director of GI Endoscopy and Endoscopic Ultrasound at Winthrop University Hospital in Minneola, N.Y.

PSC is more common among men than women. It usually begins between ages 30 and 60, but the disease can also arise during childhood.⁶ 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.⁷ 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,” Vierling says.

For PSC, there isn’t a lot of effective treatment, Michael says. “You can try endoscopic therapy, which could be attempted to make the patient feel better,” she says. “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.” Vierling adds, “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.” Vierling says. “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.¹⁰

Vierling says, “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.¹¹ 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

Cholelithiasis (Gallstones)

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.¹⁴

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.¹⁶

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.¹⁷

“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.” Vierling concurs, “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. Raufman, however, mentions that 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.”

Mastering the Procedure of ERCP

Endoscopic retrograde cholangiopancreatography (ERCP) is one of the most challenging and demanding procedures performed in endoscopy units. Many endoscopy staff nurses become overwhelmed by the complexity of patient management and the realm of equipment necessary in providing endoscopic therapy for patients with biliary and pancreatic disease.18 Additionally, skills can be lost if discontinued or reduced in the practice of ERCP. “ERCP is the most technical and most dangerous procedure that we do in the GI unit,” says Gerald Chiappone, BSN, RN, CGRN, technical specialist at the Medical University of South Carolina Digestive Disease Center in Charleston.

Chiappone, a strong proponent of education, recommends the following in order to remain proficient with ERCP techniques:

• Take additional training courses.
• Obtain all available information from the vendor.
• Practice with equipment no longer in use in order to become more comfortable with the procedure.

Risk Factors for Gallstones

Obesity. Obesity is a major risk factor for gallstones, especially in women. A large clinical study showed that being even moderately overweight increases one’s risk for developing gallstones. The most likely reason is that obesity tends to reduce the amount of bile salts in bile, resulting in more cholesterol. Obesity also decreases gallbladder emptying.

Estrogen. Excess estrogen from pregnancy, hormone replacement therapy or birth control pills appears to increase cholesterol levels in bile and decrease gallbladder movement, both of which can lead to gallstones.

Ethnicity. Native Americans have a genetic predisposition to secrete high levels of cholesterol in bile. In fact, they have the highest rate of gallstones in the United States. A majority of Native American men have gallstones by age 60. Among the Pima Indians of Arizona, 70 percent of women have gallstones by age 30. Mexican American men and women of all ages also have high rates of gallstones.

Gender. Women between 20 and 60 years of age are twice as likely to develop gallstones as men.

Age. People over age 60 are more likely to develop gallstones than younger people.

Cholesterol-lowering drugs. Drugs that lower cholesterol levels in blood actually increase the amount of cholesterol secreted in bile. This in turn can increase the risk of gallstones.

Diabetes. People with diabetes generally have high levels of fatty acids called triglycerides. These fatty acids increase the risk of gallstones.

Rapid weight loss. As the body metabolizes fat during rapid weight loss, it causes the liver to secrete extra cholesterol into bile, which can cause gallstones.

Fasting. Fasting decreases gallbladder movement, causing the bile to become over concentrated with cholesterol, which can lead to gallstones.