Biliary Atresia is a rare disorder (approximately 1 in 15,000 infants) where the bile ducts (tubes that carry bile fluid from the liver to the digestive system) become blocked shortly after birth or shortly thereafter. The exact cause of this blockage is unknown and remains a focal point of extensive research. The obstruction leads to increased pressure on the liver and the development of scars that can eventually progress to liver inflammation and acute liver failure if not appropriately addressed. Infants with biliary atresia often appear healthy at birth. They may develop jaundice and become irritable. One significant sign is pale or clay-colored stools (since the green-brown color of stool comes from bile), and dark urine might also occur.
The diagnosis is made based on characteristic features and is supported by a combination of tests, such as blood tests, ultrasound, liver biopsy, and a specialized endoscopic examination. If the test results indicate biliary atresia, a surgical procedure is performed to confirm the diagnosis and undertake appropriate correction.
Initial treatment involves surgical intervention, as there is no medication that can treat biliary atresia effectively. The surgical procedure is called the Kasai procedure, in which the bile ducts are reconnected to the intestine to allow proper drainage of bile fluid into the digestive system. The Kasai procedure succeeds in approximately 2/3 of infants with biliary atresia. Liver damage will likely persist, yet despite this, we often observe a gradual progression of improvement in most cases, leading to liver inflammation.
The situation is dynamic and necessitates close medical supervision, as the outcomes can vary depending on the specific response of each child's body. Early intervention and specialized care are essential in managing biliary atresia effectively and giving affected children the best possible chance for healthy development.