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.

The Budd-Chiari Syndrome: A Case Report and Review of Current Management Options

“…The course of the disease, and the out-come in a given patient, is difficult to predict [17] and the natural history is still uncertain [18]. The fact remains that the prognosis is dismal if the disease is left untreated.…”

“…However, shunts like these do not last longer because of their length. When the IVC is patent, then a portocaval or preferably mesocaval shunt would be more appropriate [17][18][19][20][21][22][23][24]. The latter is preferred partly because of the ease of construction, and partly because of the lower incidence of peroperative and postoperative complications.…”

“…Experience with side-to-side portocaval shunts has shown this to be a reliable and effective procedure also, and survival of up to 5 years has been reported [17,21].…”

Tubular Handling of Sodium and Phosphate in Cirrhosis with Salt Retention

“…Moreover, a similar pattern of tubular handling of salt as seen in man with advanced liver disease was demonstrated in various experimental models of edema-forming states. Thus, the blunted natriuresis seen in chronic caval dogs [19], in animals with AV fistula [20], in patients with Budd-Chiari syndrome [21], or following selective hepatic venous outflow block [22] is a consequence of an exaggerated reabsorption of sodium in the distal nephron.…”

“…Such a possibility seems remote, however, in view of our pre vious work suggesting the primacy of the distal nephron for sodium reabsorption [5,21], at least in early stages of liver disease.…”