Hepatology 1999 Sep;30(3):606-11

Prognosis of biliary atresia in the era of liver transplantation: Frenc
national study from 1986 to 1996

Chardot C, Carton M, Spire-Bendelac N, Le Pommelet C, Golmard JL, Auvert B
Departement de pediatrie, Hopital de Bicetre, Le Kremlin Bicetre, France

Abstract
Since the sequential treatment of Kasai operation with or without liver
transplantation became available, the overall prognosis of biliary atresia
remains unclear. This study examined the prognostic factors from diagnosis. All
patients with biliary atresia living in France and born in the years 1986 to
1996 were reviewed. Actuarial survival rates were calculated for survival with
native liver, survival after liver transplantation, and overall survival.
Potential prognostic factors were analyzed using the logrank test and the Cox
model. A total of 472 patients were identified. Ten-year overall survival was
68%. Independent prognostic factors for overall survival were (S = 10-year
rates) performance of Kasai operation (performed: S = 69%; not performed: S =
50%), age at Kasai operation (</=45 days: S = 80%; >45 days: S = 66%),
anatomical pattern of extrahepatic bile ducts, polysplenia syndrome, experience
of the center (</=2 new biliary atresia [BA] patients/year [24 centers]: S =
54%; 3 to 5 [2 centers]: S = 60%; >/=20 [1 center]: S = 78%). Survival with
native liver depended on the same independent prognostic factors. In conclusion
(1) Kasai operation remains the first line treatment of BA, and (2) early
performance of Kasai operation and treatment in an experienced center reduces
the need for liver transplantation in infancy and childhood and provides
children with the best chance of survival