Abstract

During the period 1984-1991, 21 infants with biliary atresia were treated with Kasai's portoenterostomy. The median survival in infants operated on before the age of 60 days (55 (range 5-82) months) was significantly 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 median age of 54 (17-96) months; 6 with portoenterostomy and 4 after liver 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 laparotomy in infants with cholestatic jaundice. Scintigraphy and liver biopsy added no further decisive information. Because early diagnosis and surgical treatment is important, only the well documented presence of a normal gallbladder can warrant postponement of an operation.

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