Hypoplasia of the right hepatic lobe and portal hypertension A forty years follow-up

In 1960, hepatosplenomegaly was discovered in five- year old girl. Diagnosis of juvenile cirrhosis was put for- ward by an eminent french consultant. To confirm the diagnosis, laparoscopy and splenoportography were done in 1962 ; normal bulging liver without edge, non- visualised gallbladder and enlarged spleen were noticed. The splenoportography showed a portal vein rising to the upper right diaphragm and irrigating the liver down below. Splenic pressure was 17.6 mm Hg and hepatic veins gradient was 4 mm Hg. Oral cholecystography revealed a supra-hepatic gallbladder. The only biological abnormality was a low platelet count.