Biliary Atresia: advances in diagnosis, treatment, and multidisciplinary management

Abstract

Biliary atresia is a rare and severe congenital condition primarily affecting infants, characterized by the obstruction and destruction of the bile ducts, leading to cholestasis and progressive liver damage. This condition is the leading cause of liver transplantation in young children and can result in severe complications such as biliary cirrhosis if not treated early. The etiology of biliary atresia is complex and multifactorial, involving genetic and environmental factors, although a complete understanding of its mechanisms is still under development. Diagnosis is challenging and relies on a combination of clinical signs, laboratory tests, and imaging, with confirmation typically achieved through cholangiography or exploratory laparotomy. The standard treatment is the Kasai procedure, which aims to create an alternative pathway for bile flow by connecting the liver to the small intestine. The effectiveness of this surgery can be influenced by the patient’s age and the severity of atresia at the time of the operation. Recent studies show that early detection and performing the surgery at a younger age are associated with better long-term outcomes and a reduced need for liver transplantation. In addition to surgery, managing biliary atresia requires continuous and multidisciplinary support, including hepatologists, pediatric surgeons, and nutritionists, to optimize patient outcomes and quality of life.