Prior to 1999, infants in the United Kingdom who were born with suspected biliary atresia were investigated in regional centers. If the diagnosis was confirmed a Kasai operation was done at the regional center.
Currently, infants with suspected biliary atresia in England and Wales are referred to 1 of 3 designated centers where the Kasai operation and liver transplantation (if necessary) can be done.
In this study, doctors assessed clearance of jaundice (bilirubin <20 µmol/L) as an early outcome in all cases of biliary atresia.
The team estimated survival using the Kaplan-Meier method with endpoints of liver transplantation or death.
|Overall 4-year estimated actuarial survival was 89%.|
The team found that 148 infants with biliary atresia were treated between 1999 and 2002.
A primary portoenterostomy was done in 96% of infants and a primary liver transplant in 3%. One child died before any intervention.
The doctors established that early clearance of jaundice after portoenterostomy was achieved in 57% of 142 infants.
Liver transplantation was done in 37% of those undergoing portoenterostomy.
Overall, 9% of infants died.
The team found that of the 135 children who survived 62% still have their native liver.
The team calculated the overall 4-year estimated actuarial survival to be 89%, while the 4-year estimated actuarial survival with native liver was 51%.
Dr Mark Davenport and colleagues concluded, "Our early results suggest that surgical outcome can be improved by centralization of care to supra-regional centers".