Biliary Atresia

Biliary atresia is the most common cause of obstructive jaundice that requires surgical correction in infants &. The obstruction is of unknown etiology, and it is the result of a necro-inflammatory and progressive obliterative process that destroys the entire extrahepatic ductal system, leading to hepatic cirrhosis and death within 2 years, if the disease in not treated. Its incidence is 1 in 10,000 to 15,000 live births. Several hypotheses on the ethiology of the disease had been postulated, proposing fetal insult to hepatobiliary system, of infectious, vascular or immunologic origin, as the originating event leading to biliary atresia &. The hepatic histological picture of biliary atresia is characterized by portal tract widening, biliary duct edema, fibrosis, and bile duct proliferation $. The adopted classification of biliary atresia is the one proposed by the Japanese Society of Pediatric Surgeons &. 
Diagnosis of biliary atresia is usually obtained with the first 2-3 weeks after birth, on the basis of persistent jaundice, with acholic stools and dark urine. A diagnostic work-up needs to be performed to exclude other causes of neonatal jaundice. Once the diagnosis is confirmed, the patient must be submitted to surgery. Untreated patients with biliary atresia have an average life expectancy of 12 months. Liver failure due to biliary cirrhosis and gastrointestinal hemorrhages are the most common causes that lead to death.
The surgical treatment for biliary atresia is the Kasai portoenterostomy  & or a variant of this surgical procedure, proposed to avoid ascending cholangitis and other Kasai-associated complications &. The success of the surgical treatment depends on the residual patency of the tiny bile ducts which have replaced the right and left hepatic duct and that are found in the fibrous cone at porta hepatis &. The number of these tiny bile ducts  progressively diminishes  with time, and they completely disappear after three to four months of age. At this time biliary cirrhosis is usually established. For this reason portoenterostomy should be not attempted, except in patients with biliary atresia type I or II, after the 4th month. If performed by a skilled surgeon before 8 weeks of birth, up to 90 % of patients will have a postoperative bile drainage. With later surgery less than 35% will have good prospects.
Despite an early success with the Kasai operation, a substantial proportion of  patients develop growth failure $and progressive liver disease. Several prognostic factors have been identified.
When the portoenterostomy fails, patient should undergo liver transplantation, which represents the curative treatment of biliary atresia and allows good results in terms of patient survival rates.
Liver transplantation has been advocated as the first operation for patients with biliary atresia &. However no advantages in performing liver transplantation in these very small patients has been demonstrated &.