Two patients with biliary atresia with different outcomes – case reports

Biliary atresia is part of the category of immune-mediated cholangiopathies, along with neonatal sclerosing cholan­gi­tis and primary sclerosing cholangitis, progressive and se­vere inflammatory diseases that cause injury to the bile ducts. Although rare, biliary atresia represents the lea­ding cause of neonatal and infant cholestasis. Early diag­nos­is is crucial, and timely intervention with a Kasai por­to­en­te­ro­sto­my (KPE) can significantly improve the prognosis. Due to the severe and irreversible evolution, biliary atresia is the most common indication for liver transplantation in pe­dia­trics. Delayed disease diagnosis, late patient referral to a hepatologist, and untimely surgery remain key problems in the management of biliary atresia. We report two cases of biliary atresia, both diagnosed in the first weeks of life, that were treated with KPE, having different outcomes. We chose to present these cases comparatively to emphasize the importance of early diagnosis in biliary atresia, as well as of proper care following the Kasai portoenterostomy, in order to offer patients the best possible prognosis. Although both cases benefited from prompt diagnosis and timely inter­ven­tion, living conditions and the quality of care play an equally important role.