Hepatic vein stenting for recurrent ascites in polycystic liver and kidney disease

Abstract

Case A 74-year-old female with autosomal-dominant polycystic kidney disease (ADPKD) had been on maintenance haemodialysis for end-stage kidney disease for 18 months, via an upper-arm arteriovenous fistula. The polycystic kidney disease was diagnosed in her 50s, and she was also known to have mild cystic disease of the liver on prior ultrasound scanning. She was otherwise well, and had no history of any other liver disease. Other past medical history was unremarkable. She was a non-smoker and did not consume alcohol. She was taking erythropoietin, calcium carbonate, folic acid, omeprazole and rosuvastatin. After 6 months of haemodialysis, she began to complain of abdominal distension, and an ultrasound examination was performed which revealed multiple, large, hypodense lesions with the appearance of simple cysts. There was marked ascites, and she was admitted to hospital for further diagnostic procedures. A paracentesis was performed, yielding ascitic fluid with biochemical characteristics of a transudate (serum to ascitic fluid albumin gradient of 7 g/L). Cytology and microbiologic culture were negative, and a total of 5.5 L of fluid was drained. Serum biochemistry at this time showed an albumin concentration of 35 g/L, and normal liver function tests. Her condition slowly deteriorated over several weeks with weight loss, anorexia and recurrent abdominal discomfort related to re-accumulation of the ascites. A computed tomography (CT) scan revealed no evidence of malignancy, but extensive ascites was again present. A diagnostic laparoscopy was also normal. Serology for hepatitis B and C was negative, and autoimmune liver disease was excluded. She proceeded to percutaneous liver biopsy to exclude cirrhosis. The biopsy showed a degree of architectural distortion, with fibrosis encircling some portal tracts, but with no cellular infiltrate and no hepatocyte necrosis. The criteria for a histopathological diagnosis of cirrhosis were therefore not met, and it was thought that the pathological changes were solely due to polycystic disease. Her body weight continued to decline, but the serum