Primary Budd-Chiari syndrome: its significance for the choice of surgical management of a patent right inferior hepatic vein.
Budd-Chiari syndrome is still a major problem and the overall prognosis for the patients is dismal and disappointing. The case history of a patient with not only outflow obstruction of the hepatic vein but also complete obstruction of the retrohepatic inferior vena cava is presented. She had a patent right inferior hepatic vein which partially decompressed the liver via a rich network of venous collaterals. Management included an inferior vena cava to inferior vena cava shunt using a Goretex graft. The patient fared well and the postoperative course was satisfactory. The case history of this patient illustrates the importance of precise pre-operative investigations for the choice of the type of surgical management. Each patient has to be considered individually on the basis of his or her mechanical peculiarities.