R. Emblem et al., PROGRESS IN THE TREATMENT OF BILIARY ATRESIA - A PLEA FOR SURGICAL INTERVENTION WITHIN THE 1ST 2 MONTHS OF LIFE IN INFANTS WITH PERSISTENT CHOLESTASIS, Acta paediatrica, 82(11), 1993, pp. 971-974
During the period 1984 1991, 21 infants with biliary atresia were trea
ted with Kasai's portoenterostomy. The median survival in infants oper
ated on before the age of 60 days(55(range 5-82)months) was significan
tly longer than the survival of children operated on after the age of
60 days(15(1.5-38) months). At present there are 10 survivors with a m
edian age of 54 (17-96) months; 6 with portoenterostomy and 4 after li
ver transplantation. Eight patients died of progressive liver failure
and 3 died of causes not related to biliary atresia. Apart from blood
tests, ultrasonography was the most important investigation before lap
arotomy in infants with cholestatic jaundice. Scintigraphy and liver b
iopsy added no further decisive information. Because early diagnosis a
nd surgical treatment is important. only the well documented presence
of a normal gallbladder can warrant postponement of an operation.