Atrezia de căi biliare – management clinic şi chirurgical (prezentare de caz)

Biliary atresia is characterized by the obliteration or dis­con­ti­nuity of the extrahepatic or intrahepatic biliary sys­tem, resulting in the obstruction of bile flow, the di­sor­der representing the most common surgically trea­table cause of cholestasis encountered during the new­born pe­riod. Recent studies show that biliary atresia is the most common hepatobiliary malformation with ma­ni­fes­­ta­tions in the neonatal period, characterized by a progressive inflammatory lesion, which suggests a role of infectious and/or toxic agents causing bile duct obli­te­ration. No single etiologic factor has been identified, but infectious agents seem to be the most plausible can­di­dates, particularly in the isolated (neonatal) form of atre­sia. Prior to the development of liver transplantation as a therapeutic option for children with end-stage liver disease, the long-term survival rate for infants with bilia­ry atresia following portoenterostomy was 47-60% at 5 years and 25-35% at 10 years. Extrahepatic biliary atre­sia is more common in females than in males. Biliary atresia is a disorder unique to the neonatal period. Once biliary atresia is suspected, surgical intervention is the only option available for a definitive diagnosis (intraoperative cholangiogram) and therapy (Kasai portoenterostomy).