Learn About Biliary Atresia
Biliary atresia is a blockage in the tubes (ducts) that carry a liquid called bile from the liver to the gallbladder.
Jaundice newborns - biliary atresia; Newborn jaundice - biliary atresia; Extrahepatic ductopenia; Progressive obliterative cholangiopathy
Biliary atresia occurs when the bile ducts inside or outside the liver are abnormally narrow, blocked, or absent. The bile ducts carry a digestive fluid from liver to small bowel to break down fats and filter out waste from the body.
The cause of the disease is not clear. It may be due to:
- Viral infection after birth
- Exposure to toxic substances
- Multiple genetic factors
- Perinatal injury
- Some medicines such as carbamazepine
It more commonly affects people of East Asian and African-American descent.
The bile ducts help remove waste from the liver and carry salts that help the small intestine break down (digest) fat.
In babies with biliary atresia, bile flow from the liver to the gallbladder is blocked. This can lead to liver damage and cirrhosis of the liver, which can be deadly.
Symptoms usually start to occur between 2 to 8 weeks. Jaundice (a yellow color to the skin and mucus membranes) develops slowly 2 to 3 weeks after birth. The infant may gain weight normally for the first month. After that point, the baby will lose weight and become irritable, and will have worsening jaundice.
Other symptoms may include:
- Dark urine
- Swollen belly
- Foul-smelling and floating stools
- Pale or clay-colored stools
- Slow growth
An operation called the Kasai procedure is done to connect the liver to the small intestine. The abnormal ducts are bypassed. The surgery is more successful if done before the baby is 8 weeks old.
Liver transplant may still be needed before 20 years of age in most of the cases.
Ronald Sokol is a Pediatric Gastroenterologist and a Gastroenterologist in Aurora, Colorado. Dr. Sokol is rated as an Elite provider by MediFind in the treatment of Biliary Atresia. His top areas of expertise are Cholestasis, Biliary Atresia, Alagille Syndrome, Bile Duct Obstruction, and Liver Transplant. Dr. Sokol is currently accepting new patients.
Mureo Kasahara is a Transplant Surgeon in Setagaya, Japan. Kasahara is rated as an Elite expert by MediFind in the treatment of Biliary Atresia. Their top areas of expertise are Biliary Atresia, Urea Cycle Disorders (UCD), Liver Failure, Liver Transplant, and Hepatectomy.
Henkjan Verkade practices in Groningen, Netherlands. Mr. Verkade is rated as an Elite expert by MediFind in the treatment of Biliary Atresia. His top areas of expertise are Progressive Familial Intrahepatic Cholestasis Type 1, Cholestasis, Alagille Syndrome, Liver Transplant, and Hepato-Pancreato-Biliary Surgery.
Early surgery will improve the survival of more than one third of babies with this condition. The long-term benefit of a liver transplant is not yet known, but it is expected to improve survival.
Complications may include:
- Infection
- Irreversible cirrhosis
- Liver failure
- Surgical complications, including failure of the Kasai procedure
Contact your provider if your child appears jaundiced, or if other symptoms of biliary atresia develop.
Summary: Little is known about the factors that cause biliary atresia nor the factors that influence disease progression. The purpose of this study is to collect the pertinent clinical information, genetic material and body fluid samples to enable investigators to address the following aims: To identify the gene or genes implicated in the etiology of BA; To characterize the natural history of the older, no...
Summary: Biliary atresia, idiopathic neonatal hepatitis, and specific genetic cholestatic conditions are the most common causes of jaundice and hyperbilirubinemia that continue beyond the newborn period. The long term goal of the Childhood Liver Disease Research Network (ChiLDReN) is to establish a database of clinical information and plasma, serum, and tissue samples from cholestatic children to facilitat...
Published Date: February 17, 2024
Published By: Charles I. Schwartz, MD, FAAP, Clinical Assistant Professor of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, General Pediatrician at PennCare for Kids, Phoenixville, PA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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Cazares J, Ure B, Yamataka A. Biliary atresia. In: Holcomb GW, Murphy JP, St. Peter SD, eds. Holcomb and Ashcraft's Pediatric Surgery. 7th ed. Philadelphia, PA: Elsevier; 2020:chap 43.
Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC. Cholestasis. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 383.
O'Hara SM. The pediatric liver and spleen. In: Rumack CM, Levine D, eds. Diagnostic Ultrasound. 6th ed. Philadelphia, PA: Elsevier; 2024:chap 52.