In an extremely rare case of Budd Chiari Syndrome with Biliary Atresia, doctors at Indraprastha Apollo hospitals saved the life of a 5-month old baby girl Surampudi Sehitha from Kakinada, Andhra Pradesh. The baby developed jaundice when she was just one month old. Her condition worsened as her abdomen started distending due to progressive swelling. Dr Anupam Sibal, Group Medical Director and Senior Pediatric Gastroenterologist and Hepatologist, Apollo Hospitals Group said, “In Biliary Atresia, which is seen 1 in 12000 babies there is no connection between the liver and the intestine. The first step in treatment is to create a connection between the liver and the intestine. This was attempted at the local hospital initially to treat the baby’s condition but as Surampudi’s liver was already severely shrunken (due to cirrhosis), the procedure was abandoned. She was referred to ApolloHospital for a liver transplant.” “She weighed 5.5 kg when she first came to our hospital, including abdominal fluid of about 1 litre. So effectively, her weight was 4.5 kg. CT Angiography showed that she was suffering from occluded hepatic venous channels which drain the liver. The diagnosis of Budd Chiari Syndrome was made. It is a very rare disease which afflicts 1 in 2 million children. Budd Chiari Syndrome had resulted in rapid worsening of the baby’s condition which necessitated an urgent liver transplant. While Biliary Atresia is most common condition for which liver transplantation in children are performed, Budd Chiari syndrome is extremely rare. Of the 320 liver transplants that we have performed in children, more than 140 have been for children with Biliary Atresia. The combination of Budd Chiari syndrome with Biliary Atresia in a baby is the rarest of rare case” Dr Sibal added.