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Definition

What is congenital biliary atresia?

Biliary atresia is an illness of the bile ducts that affects only infants. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the small intestine, where it helps digest fats.

In patients who suffer biliary atresia, the bile ducts become inflamed and blocked soon after birth. This causes bile to remain in the liver, where it starts to destroy liver cells rapidly and cause cirrhosis, or scarring of the liver.

The classification of biliary atresia is according to the site of atresia in the extrahepatic biliary system:

  • Type I: common bile duct atresia with patent proximal ducts.
  • Type II: common hepatic duct atresia with cystic structures in the porta hepatis.
  • Type III: right and left hepatic duct atresia to the level of the porta hepatis (most common).

How common is congenital biliary atresia?

Biliary atresia is a rare disease. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of congenital biliary atresia?

Infants and children with biliary atresia develop progressive cholestasis. Cholestasis is a condition in which bile doesn’t have ability to leave the liver and builds up inside of it. Until the liver is unable to excrete bilirubin through the bile ducts in the form of bile, bilirubin begins to accumulate in the blood, causing symptoms.

Symptoms of biliary atresia usually appear between two and six weeks after birth. The baby will appear

  • Jaundiced (yellowing of the skin)
  • Itchiness
  • Poor absorption of nutrients (causing delays in growth)
  • Pale stools, dark urine, and a swollen abdomen. Eventually
  • Cirrhosis with portal hypertension will develop.
  • If left untreated, biliary atresia can lead to liver failure.

Unlike other forms of jaundice, biliary-atresia-related cholestasis mostly does not result in kernicterus( a form of brain damage resulting from liver dysfunction). Because the liver in biliary atresia is still able to conjugate bilirubin, and conjugated bilirubin is unable to cross the blood–brain barrier.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above orhave any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes congenital biliary atresia?

The cause of this disease is unknown. In some infants, the condition is most likely congenital, meaning present from birth. The causes of this disease are likely that a number of factors may play a role.

A defect in early bile duct development (particularly those with other abnormalities)

Arising in the perinatal period due to an external cause such as an hepatotropic virus reovirus 3 infection, congenital cytomegalovirus infection and autoimmunity.

However, experimental evidence is insufficient to confirm any of these theories

Risk factors

What increases my risk for congenital biliary atresia?

There are many risk factors for congenital biliary atresia, such as:

International

The incidence of biliary atresia is highest in Asian populations, and it may be more common in Chinese infants compared with Japanese infants.

Sex

Extrahepatic biliary atresia is more common in females than in males.

Age

Biliary atresia is a disorder unique to the neonatal period.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

 

How is congenital biliary atresia diagnosed?

Since other conditions lead to symptoms similar to those of biliary atresia, doctors must perform many tests before a conclusive diagnosis can be made. These tests may include

  • Blood and liver tests
  • An ultrasound examination
  • X-rays
  • A liver biopsy. This is  a small amount of liver tissue is removed with a needle for examination in a laboratory.

How is congenital biliary atresia treated?

Unfortunately, there is no cure for biliary atresia. A surgical procedure is the main treatment. This surgery will help the blocked bile ducts outside the liver be replaced with a length of the baby’s own intestine, which acts as a new duct. This surgery is called the Kasai procedure after Dr. Morio Kasai, the Japanese surgeon who developed it.

The aim of the Kasai procedure is to allow drainage of bile from the liver into the intestine through the new duct. If this surgery is performed early (before 3 months of age), the operation is completely or partially successful about 80 percent of the time. In babies who respond well, jaundice and other symptoms usually disappear after several weeks. Besides, the Kasai procedure is most successful in babies younger than 3-months-old, so early diagnosis is extremely important to save children’s life.

If the Kasai procedure is not successful, the only other option is a liver transplant. However, a suitable donor organ must be found quickly, before damage to the liver from the backed-up bile becomes deadly.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage congenital biliary atresia?

The following lifestyles and home remedies might help you cope with congenital biliary atresia:

  • This is a congenital disease but scientists do know that biliary atresia is not hereditary; parents do not pass it on to their child. It is also not contagious, and it is not preventable. It is also not caused by anything an expectant mother did or did not do.
  • Does not accept the surgical treatment of biliary atresia, only 1 percent survive to the age of 4, but the surgery should make a lot of determination, for babies and families have a profound impact on the early developmental delay, the first year to be repeated hospitalization, after there reoperation and other complex issues.
  • Over the years think Kasai surgery biliary atresia can be applied as a first step of treatment until after the baby growth and development, then the implementation of liver transplantation, in order to achieve a permanent cure.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: August 10, 2017 | Last Modified: August 10, 2017

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