[Idiopathic portal hypertension, multiple focal nodular hepatic hyperplasia, the CREST syndrome and protein S deficiency].

A 54 year old woman with idiopathic portal hypertension, CREST syndrome, protein "S" deficiency and multiple focal nodular hyperplasia is reported. The patient presented several episodes of upper digestive bleeding due to portal hypertension gastropathy and to esophageal varices. Treatment with propanolol and isosorbide 5-mononitrate failed to control recurrent bleeding and a portacaval shunt was performed. At operation, ten hepatic nodes were found, being diagnosed as focal nodular hyperplasia in the pathologic study. Congestive gastropathy, esophageal varices and splenomegaly disappeared after portacaval shunt and bleeding did not recur after a follow-up of twelve months. This results support the role of portal decompressive surgery in the bleeding due to portal hypertension gastropathy.