Biliary Atresia: Causes, Symptoms, Risk Factors, Diagnosis

In Newborns, biliary atresia is the medical term for the condition where bile cannot flow from the liver to the small intestine.

Bile is a fluid that the liver both creates and excretes. It moves from the liver to the small intestine through a network of organs that resemble tubes and is known as the bile duct system, where it helps the body digest and absorb food.

Children with biliary atresia have clogged bile ducts as a result of trauma and scarring. Therefore, bile cannot enter the small intestine. Instead, it accumulates in the liver and damages it.

Read this blog further to get a quick overview of this important topic for SURGERY to ace your NEET PG exam preparation.

Causes of Biliary Atresia

Although the exact cause of biliary atresia is unknown, it can be possibly due to:

• Inflammation (swelling) and scarring brought on by immune system issues
• The presence of a virus
• Exposure to dangerous substances
• Alterations (mutations) in the genes. The baby's parents do not pass on biliary atresia.

Signs and Symptoms of Biliary Atresia

Following are mentioned some of the signs of biliary atresia:

• Jaundice, a disease in which there is too much bilirubin in the blood, or the appearance of yellow skin and eyes, is the first indicator of biliary atresia. A chemical called bilirubin is created when red blood cells degrade. Jaundice is a common condition in the first week of life for babies, but if it persists after two weeks, a doctor should be consulted.

Other biliary atresia signs and symptoms include:

• Pale-colored stools in Infants, typically have yellow, green, or brown stools.
• Brown urine in color
• A bloated stomach (as the liver and spleen expand)
• Having trouble gaining weight
• Ascites (abdominal fluid)
• If the biliary atresia is not treated, there will be a liver failure after a few months.

Risk Factors of Biliary Atresia

• Bleeding Cholangitis (liver and bile duct infection)
• A hole in the intestine that could allow the intestinal contents to spill into the stomach
• Scarring-related intestinal obstruction

Diagnosis of Biliary Atresia

The doctor will gather the infant's medical history and do a physical examination in order to identify biliary atresia. A pediatric hepatologist, a medical professional who focuses on treating kids with liver disorders, may also request a number of tests, such as:

• Blood tests to measure bilirubin levels and seek for symptoms of liver impairment
• Using ultrasound, which sends high-frequency sound waves through the body's tissues. The echoes are captured and converted into pictures of the body's internal organs.
• Performing a liver biopsy (the removal of liver cells or tissue for testing)
• A radioactive material (tracer) is introduced into circulation during a hepatobiliary scan. The patient may have biliary atresia if the tracer is not eliminated by the liver.
• During a cholangiogram, a dye is injected into the gallbladder to determine whether or not it has a primary bile duct. The doctor can identify biliary atresia if it doesn't.

Treatment of Biliary Atresia

Biliary atresia has no known treatment. The Kasai technique, a type of surgery, is the primary treatment. In this procedure, the surgeon substitutes the patient's small intestine with the damaged bile ducts that are located outside the liver. The liver will subsequently be immediately drained into the small intestine at that point.

Most biliary atresia cases that undergo this procedure are successful. But if treatment fails, the young patient most likely needs a liver transplant. Depending on when the procedure is done, the child might require a liver transplant even if the surgery is successful.

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Complications of Biliary Atresia

Immediate complications following surgery are rare. The majority of issues arise as the liver condition progresses.

It is typical to develop a bile duct infection following the Kasai surgery. Antibiotics given intravenously are used to treat this. Oral antibiotics could be used to continue the treatment.

Itching or jaundice could appear. These are frequently successfully treated with drugs like ursodeoxycholic acid.

Changes in the liver and intestines' blood flow are common in cirrhosis patients. These alterations may result in issues including easily bruised skin, nosebleeds, fluid retention, and swollen veins (varices) in the stomach and esophagus.

A rapid and significant quantity of bleeding in the stomach and intestines may result from increased pressure in these veins.

This might be a major problem. Bleeding can typically be halted with prompt, skilled medical attention. Sometimes, specialized treatments involving the injection of a vessel-hardening (sclerosing) agent are required for treatment.

Diuretics, a class of drugs that aid in flushing out excess water from the body, can be used to treat fluid retention in the body. 

Other cirrhosis consequences could develop as the condition worsens.

Prognosis of Biliary Atresia

The child will typically not live past the age of 2 without a liver transplant if surgery is not performed. The timing of the Kasai procedure and the liver's health at the time of surgery determine how long a child will survive after the procedure without a liver transplant. Many people will eventually require a liver transplant, even after a successful procedure.

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