HEPATIC VENOUS OUTFLOW OBSTRUCTION IN AUTOSOMAL-DOMINANT POLYCYSTIC KIDNEY-DISEASE

To discuss the clinical presentation, diagnosis, and treatment of hepa tic venous outflow obstruction as a complication of polycystic liver d isease, four cases diagnosed and treated at our institution have been reviewed and the information from six previously published case report s has been summarized. Eight of the 10 patients were women. All presen ted with severe ascites. Nine had polycystic kidneys. Three had modera te-to-advanced renal insufficiency, four were on hemodialysis, and one had a renal allograft. Possible predisposing factors were identified in seven patients; the most common was recent abdominal surgery, which , in three cases, was a bilateral nephrectomy. All patients had extrin sic compression of the hepatic veins and the inferior vena cava by hep atic cysts, and four had proven superimposed thrombosis of the inferio r vena cava and/or hepatic veins. In the patients seen in this institu tion, magnetic resonance imaging was helpful in determining the level of obstruction in the inferior vena cava and the patency of the hepati c and portal veins. The outcome was worse in the patients with thrombo sis; one recovered after a portocaval shunt, and the remaining three p atients died. On the other hand, five of the six patients without thro mbosis recovered after alcohol sclerosis of a large dominant cyst (one patient) or after hepatic resection and cyst fenestration (four patie nts). Hepatic venous outflow obstruction probably has been underrecogn ized as a cause of portal hypertension, ascites, and liver dysfunction in polycystic liver disease. The diagnosis can be reliably establishe d with current imaging techniques, especially magnetic resonance imagi ng. The treatment is aimed at decompressing the congested liver by rel ieving the obstruction of the intrahepatic inferior vena cava and, in the case of thrombosis of the hepatic veins, by a portosystemic shunt.