Topic Collection: Biliary Atresia

Biliary atresia (BA) is a potentially lethal disease of the newborn with an incidence varying from 1 in 10,000 to 1 in 20,000 depending on country or region. The aetiology is very much arguable with possible mechanisms including an early developmental defect, a ciliopathy, a perinatal viral infection, in utero environmental toxic damage and possibly prenatal ischaemia in the bile ducts – though the presentation is stereotypical with conjugated jaundice from birth, pale stool and dark urine amid a backdrop of worsening liver fibrosis. There is variation in the clinical phenotype with defined syndromic variants [e.g. biliary atresia splenic malformation (BASM) syndrome, cat-eye syndrome]; cystic biliary atresia and CMV IgM+ve BA. Isolated BA can be defined by the lack of any of these associated features, and remains the commonest variant world-wide (85-90%). Progression from a purely cholestatic condition to one involving hepatic inflammation; infiltration of immune-active mononuclear cells; Kupffer cell and macrophage activation and ultimately EMT and initiation of hepatic fibrosis explains much of its morbidity. Early diagnosis remains essential to try and abbreviate this process and typically includes exclusion of other neonatal cholestatic conditions and efforts to establish a more specific pre-surgical diagnosis typically involving ultrasonography, percutaneous liver biopsy, and newer entities such MMP-7 estimation and possibly shear-wave elastography. Initial treatment is invariably surgical with an attempt at restoration of bile flow and salvage of the native liver (Kasai portoenterostomy) (~95%) or primary liver transplantation (~5%). The balance in individual countries is currently contentious depending on resources and outcomes. The outcome following Kasai portoenterostomy is unpredictable and the role of adjuvant therapy controversial, but clearance of jaundice should be expected in >50% of infants and a 5 year native liver survival of >50% attainable. Suggested topics include: - The syndromic variants of biliary atresia - The genetic background - Organoids in biliary atresia- The microbiome in infants with biliary atresia – a possible contributing factor - Pathophysiology of the native liver in biliary atresia- The Texas experience of screening for biliary atresia - Current attitudes to diagnosis of biliary atresia - Laparoscopic approach to the Kasai operation - Lessons from the Japanese Biliary Atresia Registry - The role of adjuvant therapies in biliary atresia: current and on the horizon - Evolution of the Pathophysiology of the liver post-Kasai operation Submissions Open | Submission Deadline: 16 June 2025