Cholestasis – Bile Stasis, Symptoms, Jaundice, Treatment, Bile Ducts, and Bile Stasis in Liver Diseases

Cholestasis, or bile stasis, refers to the backup of bile within the bile ducts. Bile consists of bilirubin, bile acids, and other bile components. In cholestasis, bile is retained in the bile ducts and cannot flow into the intestines.

The cause of cholestasis can be

• a disorder of bile production in the liver (bile production disorder) or
• a disturbance in bile flow (bile flow disorder)

.

In a bile flow disorder, there is reduced or absent flow of bile into the intestines. This is caused by mechanical factors, meaning the bile ducts are not fully patent. For example, gallstones or tumors can block the ducts. A disorder of bile production is usually attributable to chronic liver disease. On the one hand, cholestasis causes the accumulated substances in the bile to enter the bloodstream. On the other hand, cholestasis disrupts the digestion of fat and fat-soluble vitamins in the intestines.

These mechanisms lead to the three characteristic symptoms of cholestasis:

• yellowing of the skin (jaundice or icterus),
• a change in the color of urine and stool (dark urine and pale stool), and
• itching (pruritus) all over the body.

In cases of cholestasis, it is important to distinguish whether the causes of bile stasis lie within or outside the liver. Based on the causes, two different forms of cholestasis are distinguished:

• intrahepatic (non-obstructive) cholestasis and
• extrahepatic (obstructive) cholestasis.

Intrahepatic cholestasis results from a disturbance in bile production within the liver cells (hepatocytes).

In extrahepatic cholestasis, mechanical obstructions in the bile ducts prevent the flow of bile.

Illustration of the bile duct leading to the intestine © lom123 | AdobeStock

Possible causes of intrahepatic (non-obstructive) cholestasis include

• various forms of liver inflammation, such as viral hepatitis, as well as
• alcohol-related liver diseases such as cirrhosis or alcoholic fatty liver disease.

In addition, other causes may include

• liver tumors,
• side effects of medications,
• hormonal changes during pregnancy, and
• a lack of oxygen to the liver

can lead to intrahepatic cholestasis.

Extrahepatic (obstructive) cholestasis is caused by mechanical obstructions in the bile ducts. These include, for example,

• gallstones,
• tumors such as bile duct and pancreatic carcinomas,
• bile duct strictures, and
• parasites.

Other possible causes of extrahepatic cholestasis include

• narrowing of a bile duct,
• cicatricial strictures, and
• inflammation of the bile ducts or the pancreas.

Cholestasis typically manifests as

• yellowing of the skin and eyes (jaundice),
• brown-colored urine (dark urine),
• pale stools, and
• itching (pruritus) all over the body.

The severity of these symptoms can vary depending on the duration, extent, and type of bile stasis.

The yellowing of the skin and eyes occurs because excess bile acids and bile pigments enter the bloodstream. Normally, they are excreted through the intestines. The excess bilirubin then accumulates in the skin and in all internal organs.

Cholestasis causes yellowing of the eyes (jaundice) © Alessandro Grandini | AdobeStock

The brown discoloration of the urine is also due to the bilirubin released into the bloodstream during cholestasis. It reaches the kidneys via the blood and is excreted in the urine.

The discoloration of the stool is a result of a lack of bilirubin in the intestines. Bile acids are normally required to break down fats in the intestines. Without bile acids in the intestines, fats and fat-soluble vitamins from food are not properly broken down and absorbed. As a result, the stool becomes discolored and light or pale in color.

The itching all over the body is caused by the increased concentration and accumulation of bile acids in the blood and skin. This activates certain tissue hormones that trigger a mild inflammatory reaction in the skin. The itching leads to increased scratching and, as a result, may also cause skin damage.

Depending on the underlying causes of cholestasis, additional symptoms may also occur. These may include, among other things,

• colic-like abdominal pain,
• nausea,
• vomiting,
• loss of appetite, and
• fever

may occur.

Cholestasis is diagnosed based on symptoms and physical examination findings, as well as through

• a blood test,
• an ultrasound examination (sonography),
• an ERCP, and
• a cholangiography.

Based on the symptoms, the doctor determines whether the underlying causes of cholestasis are within or outside the liver. The laboratory blood test shows the severity of the cholestasis. To do this, liver function tests and the bilirubin level in the blood are measured.

If the blood test results indicate cholestasis, an ultrasound examination is usually performed. It is used for further diagnosis and to determine the cause. This can reveal, for example,

• dilatation of the bile ducts—as seen with gallstones or other obstructions—as well as
• a distended gallbladder

be detected.

An ultrasound image is usually part of the diagnosis of cholestasis © familylifestyle | AdobeStock

Depending on the cause of cholestasis, additional imaging methods may be used. Cholangiography, for example, allows for X-ray visualization of the bile ducts using a contrast agent. ERCP is an endoscopic X-ray contrast imaging procedure of the bile ducts.

Treatment for cholestasis depends on whether it is intrahepatic or extrahepatic cholestasis.

In the case of intrahepatic cholestasis, treatment involves addressing the underlying cause—that is, the primary disease. If, for example, the cholestasis was caused by liver cirrhosis, the cirrhosis must be treated. In such cases, a liver transplant may be considered, among other options. If certain medications are the underlying cause of the cholestasis, they must be discontinued.

In the case of extrahepatic cholestasis, it is necessary to remove the mechanical obstructions to bile flow. This is usually done through surgery, which can be performed either open or endoscopically. Depending on the cause of the extrahepatic cholestasis, this may involve

• stone removal,
• the placement of a stent or a biliary endoprosthesis in the bile duct, or
• dilatation (widening) of the bile ducts

.

Regardless of the cause, other treatment methods are also used. Their goal is to treat and alleviate the symptoms and complications of cholestasis.

Long-term cholestasis can lead to vitamin deficiencies such as vitamin K deficiency, vitamin D deficiency, and calcium deficiency. Therefore, dietary measures and the intake of vitamins K and D as well as calcium are recommended to prevent bone diseases. Colestyramine can also be taken to relieve the itching typical of cholestasis.

What is cholestasis?

Cholestasis is a blockage of bile flow or a reduction or stoppage of bile flow. This results in bile accumulation in the liver and bile ducts. The consequence is an increase in bilirubin in the liver and blood, leading to jaundice.

What are the possible causes of cholestasis?

Cholestasis can be caused by intrahepatic cholestasis due to a loss of liver cell function or by extrahepatic cholestasis due to a blockage of the bile ducts. Tumors, cysts, inflammation, or cholangitis can trigger cholestasis. Hormonal changes or medications can also lead to bile stasis.

What are the symptoms of cholestasis?

Typical symptoms of cholestasis include jaundice, itching, dark urine, and pale stools. Jaundice is caused by elevated bilirubin levels, which originate from old or damaged red blood cells. Pale stools and itching are classic signs of impaired bile flow.

How is it diagnosed and treated?

Diagnosis and treatment involve blood tests measuring alkaline phosphatase and gamma-glutamyl transpeptidase, as well as imaging procedures such as MRI. In some cases, a liver biopsy is necessary. Treatment for cholestasis depends on the cause and may involve medication or endoscopic procedures such as ERCP.

Is cholestasis dangerous?

If left untreated, cholestasis can lead to liver failure or cirrhosis. The severity of the cholestasis determines the risk of further complications. A balanced and healthy diet can be supportive but is no substitute for targeted treatment.