All issues > Volume 9(6); 1966

Original Article

J Korean Pediatr Soc. 1966;9(6):385-398. Published online November 30, 1966.

Clinlco-Pathologlc Study on Biliary Cirrhosis followed by Congenital Extrahepatic Biliary Atresia In Infants

Dong Won Chun¹

¹Department of Pediatrics College of Medicine, Seoul National University

Abstract

In spite of the abundant literature on the neonatal prolonged obstructive jaundice, there remain many unresolved problems regarding the natural history and management on it. Differentiation. between congenital atresia of extrahepatic biliary tract and neonatal hepatitis, and criteria and relevant time for surgical intervention, and operative procedures are sources of considerable controversy. In obstructive cirrhosis, the common cause in infancy is a congenital malformation duct, whereas in the adult, it results from tumors, stones and inflammatory strictures. Clinico-pathologic study in four cases with biliary cirrhosis followed by congenital extrahepatic biliary atresia, which had been proved at surgical exploration and confirmed on liver sections on surgical biopsy, were undertaken in the pediatric ward of Han-Il Hospital and Seoul National Univ- ersity Hospital during the period from January 1965 to March 1966, and review of the literatures was also made. The results are as follows: 1. The time of onset of jaundice varied from 3 days to 1 month of age, and acholic stool, dark urine and some degree of hepatosplenomegaly were noted in all cases. 2. The clinical courses and laboratory findings were of no aids in differentiating the congenital extrahepatic biliary atresia and neonatal hepatitis, and in diagnosing biliary cirrhosis followed by congenital malformations of extrahepatic biliary system. 3. Surgical explorations with liver biopsy were performed in all cases, and malformations of the extrahepatic biliary tract were confirmed by the naked eye. 4. Histopathologic findings of surgical liver biopsy specimens showed early changes of biliary cirrhosis in two cases, and moderate to advanced cirrhotic changes in another two cases, and their age distributions were ranged from 2 to 5 1/2 months. 5.In this series, there were no surgically correctable cases.

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