Biliary Atresia: Causes, Symptoms & Treatment

Instructor: Lisa Cauthen

Lisa is a Registered Nurse with a 14 years of experience and a Masters Degree in Nursing Education. She has certifications in CPN, ACLS, PALS, and NRP.

Biliary atresia is a rare and serious condition affecting newborn infants. This lesson will review its causes, symptoms, diagnosis, and treatments while following a case study.

Meet Baby Jamie

Baby Jamie is at her 1-month-old baby check up. She was born full term, with no complications during pregnancy or delivery and has been growing well. While in the exam room, Jamie's mom comments on how yellow the fluorescent lights make her skin look. The doctor asks, ''How is she acting?''

Upon reflection, Jamie's mom replies, ''Well, now that you mention it, she's been a little sleepier these last few days and not eating well. Also her poop is tan colored. Do you think I should change her formula?''

Concerned that it may be something other than the lighting and formula, Jamie's doctor does a careful examination, focusing on her abdomen. He'll do a few blood tests, but fears Jamie may have biliary atresia.

Biliary Atresia

Bile is an enzyme that helps digest fats and carry waste products. Normally bile drains from the liver into the intestine through networks and ducts. Biliary atresia is a rare condition characterized by the absence or obstruction of bile ducts. It occurs in only one in every 18,000 infants.

This condition causes a buildup of bile in the liver, causing irreversible liver damage. These blockages can be intrahepatic (in the liver), extrahepatic (outside the liver) or both.

Intrahepatic bile ducts or the common bile duct can be affected in biliary atresia
bile ducts

Causes

Biliary atresia can be a congenital (present at birth) or an acquired condition (develops in the perinatal period (the first few weeks after birth). While the cause is unknown, it is possible that the congenital form is related to a chromosomal abnormality and suspected that the acquired form is related to a viral infection or autoimmune response.

Symptoms

Infants with biliary atresia usually appear healthy when they are born. The symptoms of biliary atresia develop in the first two to eight weeks after birth, just as in baby Jamie's case.

Jaundice, or a yellow tint to the skin and eyes, is the primary symptom of biliary atresia. A high level of bilirubin in the blood causes jaundice. Elevated bilirubin levels can also cause lethargy and poor eating.

Other symptoms include dark urine, and acholic, or clay-colored, stools (from the large amounts of bilirubin being excreted in the urine and stool).

A person with jaundiced skin and eyes
Janundice

Diagnosis

Prompt detection and diagnosis of biliary atresia is essential. No single test can definitively diagnose it, so a series of tests is needed. These may included blood tests, radiology tests, and biopsy.

  • The blood tests include bilirubin levels and liver enzymes.
  • Radiology exams can include x-rays and ultrasound to detect enlargement of the liver and presence (or absence) of a gallbladder.
  • A liver biopsy, which looks for cellular changes in the liver, can be used for a definitive diagnosis.

Without appropriate diagnosis and treatment, biliary atresia will lead to liver failure and death within two years. Jamie's lab, radiology, and biopsy tests have all pointed to extrahepatic biliary atresia.

Treatment

Surgery

For infants with extrahepatic biliary atresia, initial treatment involves surgery, called a Kasai hepatoportoenterostomy, to re-establish bile flow from the liver into the intestine. During a Kasai procedure, the pediatric surgeon removes the infant's damaged bile ducts and brings up a loop of intestine to replace them.

When successful, this surgery allows infants to grow and have good health for several years. Unfortunately, even when successful, this surgery is not a cure for biliary atresia. Fibrosis and destruction of the intrahepatic bile ducts can continue, resulting in long-term damage.

Transplant

Liver transplant is the treatment for those who have unsuccessful surgery, those with intrahepatic blockages, and eventually many of those who had a successful Kasai surgery. A transplant will only be recommended after all other treatments have been exhausted. Eighty-five percent of all children with biliary atresia require a liver transplant before age 20.

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