Abstract
The syndromic form of biliary atresia accounts for 10-25% and is associated with a poor prognosis due to associated anomalies. We report a case of extrahepatic biliary atresia and polysplenia syndrome with jaundice since 19th day of life and who had undergone surgical correction of malrotation in the neonatal period. Inspite of successful Kasai’s portoenterostomy at 52nd day of life, the child succumbed to post-operative sepsis.
Highlights
Extra-hepatic Biliary atresia is a destructive inflammatory obliterative cholangiopathy of neonates and infants that affects varying lengths of the intrahepatic and extra-hepatic biliary ducts. [1] It is usually an isolated malformation, but in 10-25% cases it is associated with other anomalies
The most common association is the Polysplenia syndrome, known as the biliary atresia splenic malformation which is reported in about 10% of cases
We report here a case of neonate operated for intestinal malrotation on the third day of life who presented with cholestasis on the 19th day of life
Summary
Extra-hepatic Biliary atresia is a destructive inflammatory obliterative cholangiopathy of neonates and infants that affects varying lengths of the intrahepatic and extra-hepatic biliary ducts. [1] It is usually an isolated malformation, but in 10-25% cases it is associated with other anomalies. [1] It is usually an isolated malformation, but in 10-25% cases it is associated with other anomalies. The most common association is the Polysplenia syndrome, known as the biliary atresia splenic malformation which is reported in about 10% of cases.
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