ABSTRACT
Biliary atresia is a neonatal cholestatic disease caused by complete organic obstruction of the extrahepatic biliary system, probably of congenital origin. The intrahepatic biliary system is simultaneously involved with pericholangeal fibrosis. The etiology of this disease is unknown. The pathologic changes of the biliary systems are sequelae of inflammatory changes which might have occurred during fetal life. It does not appear to be a developmental anomaly. The overall incidence is one out of 10 000 live births. There is no racial significance in incidence. Female infants are more commonly affected than males (f:m ratio 1.27:1). Patients initially present with acholic stools and jaundice persisting beyond the age of 2 weeks.1,2
In 1928, Ladd reported successful operations in selected patients with biliary atresia in whom a large duct containing bile was present at the porta hepatis proximal to the atretic portion.3 In this situation, a lumen-to-lumen anastomosis could be performed between the bile duct and bowel. The lesion observed in these patients was regarded as the ‘correctable form’ of biliary atresia, allowing the surgeon to perform reconstruction of the biliary tract. However, such a large bilecontaining duct is not available in more than 80% of patients.1,2 In the majority of patients, the extrahepatic biliary system has been totally involved in fibrotic changes with absence of a bile duct with a visible lumen. Such a lesion was previously called the ‘incorrectable form’, because a surgical reunion of the bile duct to the bowel was impossible. Since Ladd’s report, the classification dividing the lesions into two forms – correctable and incorrectable – has been used.
