An Unexpected Hepatic Hydrothorax After a Successful Kasai Portoenterostomy: A Case Report.

Hepatic hydrothorax (HH) represents a rare complication of portal hypertension among adult cirrhotic patients. Here, we describe a pediatric case of HH, observed in a biliary atresia infant. The child presented with recurrent right-sided pleural effusion, after a successful Kasai portoenterostomy with restoration of bile flow and without overt signs of hepatic failure.

Unusual Clinical Course for Untreated Malformative Biliary Atresia Infant: Is Portal Hypertension an Important Driver of Liver Fibrosis?

In biliary atresia, infants left untreated, and in those with unsuccessful porto-enterostomy, hepatic condition and function worsen rapidly towards cirrhosis, malnutrition, portal hypertension with ascites, and variceal haemorrhage; many die within the first 3 years of life unless they benefit from liver replacement. We describe a girl with biliary atresia splenic malformation syndrome, who had portal vein cavernoma and microsplenia; she did not undergo porto-enterostomy. She survived with her native liver over the age of 3 years.