Biliary Atresia Overview
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.
Henry Dumas is a Pediatrics specialist and an Intensive Care Medicine provider in Orlando, Florida. Dr. Dumas and is rated as an Advanced provider by MediFind in the treatment of Biliary Atresia. His top areas of expertise are Biliary Atresia, Microcephaly, Undescended Testicle, and Epilepsy in Children.
Legacy Healthcare Services Inc
Jeremy Benjaminson is a Family Medicine specialist and a General Practice provider in Tampa, Florida. Dr. Benjaminson and is rated as an Experienced provider by MediFind in the treatment of Biliary Atresia. His top areas of expertise are Familial Hypertension, Glucocorticoid-Remediable Aldosteronism, Hypertension, Type 2 Diabetes (T2D), and Ureteroscopy. Dr. Benjaminson is currently accepting new patients.
University Medical Service Association Inc
Amarilis Sanchez-Valle is a Medical Genetics specialist and a Pediatrics provider in Tampa, Florida. Dr. Sanchez-Valle and is rated as an Experienced provider by MediFind in the treatment of Biliary Atresia. Her top areas of expertise are Phenylketonuria (PKU), Early Infantile Epileptic Encephalopathy, Ornithine Transcarbamylase Deficiency, and Ornithine Translocase Deficiency. Dr. Sanchez-Valle is currently accepting new patients.
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: This is a multi-center retrospective chart review to compile a data repository of the management and outcomes of children with biliary atresia. Overall, investigators aim to evaluate which specific factors contribute to improved patient outcomes, to help guide potential improvements in patient care and resource utilization.
Summary: To investigate the role of preoperative serum FGF19 level in the prognosis of biliary atresia.
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.
Berlin SC. Diagnostic imaging of the neonate. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 38.
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.