Biliary Atresia: Symptoms, Treatment

Overview

Biliary atresia is a rare condition only in infants. It happens when the bile ducts are scarred and blocked. With this condition, bile can’t flow into the intestine as normal, causing it to build up in the liver and damage it. Over time, this may lead to scarring, loss of liver tissue and function, and cirrhosis.

In most cases, symptoms occur between 2 and
4 weeks after birth though some babies may get it in the womb.

Biliary atresia can be life-threatening, but
most babies survive to their adulthood with effective treatment.

In the United States, this condition
affects about 1 out of every 12,000 infants.

Types

Biliary atresia can be divided into two types:

  • Biliary atresia without birth defects

According to a recent study, 84 percent of
infants with biliary atresia have no other major birth defects. This type is
also called biliary atresia perinatal or isolated biliary atresia.

  • Biliary atresia with birth defects

Some infants suffering from biliary atresia have major birth defects such as problems with their heart, spleen, or intestines. Doctors may call this condition fetal or embryonic biliary atresia.

Symptoms

Infants with biliary atresia may grow as normal at the beginning. However, they may have jaundice by the second or third week of life. They may also begin to lose weight and become irritable.

Other possible symptoms may involve:

  • Dark urine
  • Enlarged spleen
  • Floating stools
  • Foul-smelling stools
  • Pale or clay-colored stools
  • Slow or no weight gain
  • Slow growth

Causes

The exact cause of biliary atresia is still
unknown. The following factors that may contribute to biliary atresia are being
studied:

  • Infections with certain viruses
  • Coming into contact with harmful chemicals
  • Problems with the immune system
  • A problem that affects liver and bile duct development in the womb
  • Certain genes or changes in genes

Diagnosis

Doctors will perform a physical exam to
feel for an enlarged liver. In addition to this, doctors may also order other
tests such as:

  • Abdominal x-ray
  • Abdominal ultrasound
  • Blood tests: to check bilirubin levels
  • Hepatobiliary iminodiacetic acid (HIDA) scan
  • Liver biopsy
  • X-ray of the bile ducts

Treatment

The most common treatment for biliary
atresia is an operation called the Kasai procedure which is done if the blocked
bile ducts are outside the baby’s liver. This procedure will replace the blocked
bile ducts with part of the intestine, letting bile drain from the liver
through this new “duct” and into the intestine. Within three months after the birth,
the success rate of the Kasai procedure is 80%. If it fails, a liver transplant
within 1 to 2 years is usually needed.

If the blocked bile ducts are inside the liver, the doctor may prescribe medications that help get rid of bile as well as vitamin A, D, and E supplements. A liver transplant may be done when necessary.

Keyword: biliary atresia.

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