New observations in the clinical spectrum of the Budd-Chiari Syndrome

Abstract

Some observations on the natural history, surgical management and metabolic phenomena in B.C.S. are presented. In one patient side to side portocaval shunt brought dramatic improvement with asymptomatic followup of 5 years. The patient died of causes unrelated to B.C.S. At postmortem the shunt was patent and normal liver architecture was preserved. The second patient presented with a fulminant course culminating in acute hepatorenal syndrome. There was marked hypofibrinogenemia and extreme elevation of SGOT. Emergency portocaval shunt was followed by marked improvement in liver status. The patient died of respiratory complications on the 8th postoperative day. The third patient is one year on conservative treatment only and well. In this patient excessive renal tubular reabsorption of sodium at a distal site was documented. This may explain the generalised edema formation in patients with B.C.S. even without inferior vena cava obstruction. This is apparently the first reported instance of renal tubular function studied in B.C.S.