Infants with biliary atresia who are not treated, or who have failed porto-enterostomy, have a rapid deterioration of hepatic condition and function, leading to cirrhosis, malnutrition, portal hypertension with ascites, and variceal haemorrhage; many die within the first three years of life unless they receive liver replacement. The study reported a girl with biliary atresia splenic malformation syndrome who had portal vein cavernoma and microsplenia but did not have a porto-enterostomy. She lived for three years with her natural liver.

Despite the lack of ascites or significant hepatic failure, she remained in excellent health, but four other comparable patients handled during the same time period all had clinical deterioration and ascites, necessitating liver transplant. There is no such account in the literature that we are aware of. Possible pathogenetic processes are explored, as well as the involvement of portal hypertension as an essential component.