Combined Liver and Kidney Transplant in a Patient with Budd-Chiari Syndrome Secondary to Autosomal Dominant Polycystic Kidney Disease Associated with Polycystic Liver Disease: Report of a Case with a 9-Year Follow-Up

Abstract

Polycystic liver disease (PLD) is a hereditary disease inherited by autosomal dominant trait that occurs as a frequent extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). We report a case of a 59-year-old woman diagnosed with ADPKD associated with PLD. End-stage chronic renal failure with a secondary Budd-Chiari syndrome developed during the patient's clinical course. She underwent combined liver and kidney transplantation, with a successful response over a 9-year follow-up period.

Functional Budd-Chiari Syndrome Associated With Severe Polycystic Liver Disease

“…8 Polycystic liver disease is the most frequent extrarenal manifestation in this disorder, affecting up to 94% of the patients. [1][2][3][4] Patients with PLD are most often asymptomatic, usually not requiring treatment directed to the liver phenotype. In some cases, however, cyst expansion may lead to compression of neighboring structures, giving rise to clinical manifestations.…”

“…Alterations in transaminases are not common. 1,3 Ascites is an uncommon finding in patients with ADPKD and ADPLD with significant cystic liver involvement. 4 When displaying ascites, these patients must be investigated for cardiogenic, renal, and infectious causes, as well as for concomitant parenchymal hepatic diseases progressing to cirrhosis, congenital hepatic fibrosis, and conditions associated with obstruction of hepatic venous flow.…”

“…Although most cases are asymptomatic, the anatomic changes in liver parenchyma resulting from the progressive nature of cyst growth can occasionally lead to massive hepatomegaly and compressive effects. [1][2][3][4] Obstruction of hepatic venous flow secondary to vascular compression by liver cysts is rare in PLD. [3][4][5][6] Given the anatomic relationship among the local structures, however, the compression of the portal vein, inferior vena cava, and hepatic veins may potentially lead to functional Budd-Chiari syndrome (BCS).…”

“…[1][2][3][4] Obstruction of hepatic venous flow secondary to vascular compression by liver cysts is rare in PLD. [3][4][5][6] Given the anatomic relationship among the local structures, however, the compression of the portal vein, inferior vena cava, and hepatic veins may potentially lead to functional Budd-Chiari syndrome (BCS). 3,5,7 We describe a case of a woman with severe PLD and subsequent development of functional BCS.…”

“…[3][4][5][6] Given the anatomic relationship among the local structures, however, the compression of the portal vein, inferior vena cava, and hepatic veins may potentially lead to functional Budd-Chiari syndrome (BCS). 3,5,7 We describe a case of a woman with severe PLD and subsequent development of functional BCS. Kidney-liver transplant resulted in successful treatment of the patient.…”