Lancet 1989 Feb 25;1(8635):421-3

Late referral for biliary atresia--missed opportunities for effective surgery

Mieli-Vergani G, Howard ER, Portman B, Mowat AP
Department of Child Health, King's College School of Medicine, Denmark Hill,
London 

Abstract
To assess whether clinicopathological features other than the age at operation
influence prognosis after surgery for extrahepatic biliary atresia (EHBA) and to
determine whether the age at referral has fallen since a previous survey, 50
consecutive cases with EHBA referred between February, 1985, and December, 1987,
were reviewed. Liver or spleen size, liver function tests, or histological
appearance of liver biopsy specimen before surgery were not predictive of
outcome. The jaundice cleared up in 12 of 14 children operated on by age 8
weeks, but in only 13 of 36 operated on later. In 41 referral was delayed. All
25 children in whom surgery was successful are alive and well, while 13 of 25
with unsuccessful surgery have died, at a median age of 1 year. To improve the
prognosis of infants with EHBA parents and health staff need a better awareness
of the early clinical features of EHBA and of the necessity for prompt referral.
Liver disease should be suspected in any infant jaundiced after 14 days of age