Biliary atresia: clinical aspects.

Biliary atresia (BA) remains an enigmatic disease with a degree of etiologic heterogeneity. A number of variants can be defined clinically, and these include the syndromic group (typically BA splenic malformation), cystic BA, and cytomegalovirus (CMV) IgM +ve associated BA. The remainder, and still the largest group, may be termed isolated BA(.) There is a wide variation in incidence across the globe from 1 in 5000 in Taiwan to 1 in 20,000 live births in Northern Europe, although the reasons for such a disparity remain obscure. Management remains primarily surgical with an attempt to restore bile flow by resection of extrahepatic biliary remnants and a reconstruction portoenterostomy (the Kasai procedure), reserving liver transplantation for those where this fails or complications of chronic liver disease supervene. Clearance of jaundice to normal values has been achieved in 40%-55% of cases in large series from around the world, with an expectation of 5-year native liver survival of similar proportions.