Comparison of volume-reductive therapies for massive polycystic liver disease in autosomal dominant polycystic kidney disease

Aim: Polycystic liver disease (PLD) in autosomal dominant polycystic kidney disease (ADPKD) patients can induce massive hepatomegaly-related symptoms. Volume-reductive therapies for symptomatic PLD include transcatheter arterial embolization (TAE), liver resection and liver transplantation; however, consensus has not been reached regarding treatment selection. We compared three volume-reductive therapies for a better understanding of PLD treatment strategies.

Methods: We retrospectively analyzed 28 ADPKD patients who underwent TAE, liver resection or liver transplantation for PLD at a single center, and compared their outcomes.

Results: Of 18 TAE patients, five required repeat TAE, and five required rescue liver transplantation or liver resection because of refractory symptoms or hepatic failure. The treatment failure rate for TAE was high (69.6%). Nine underwent liver resection, and the degree of volume reduction in the liver resection group was greater than that in the TAE group (52.4% vs 7.6%, P < 0.001). One liver resection patient required rescue liver transplantation because of hepatic failure. Seven patients underwent liver transplantations. All liver transplant patients had successfully controlled symptoms or hepatic failure, and had good graft function. Three patients in the TAE group died of infections or hepatic failure, whereas no mortality occurred after surgical therapy.

Conclusion: Liver resection is a good first-line therapy in patients that have severe symptoms, cyst involvement in several segments with some spared segments and preserved liver function. Liver transplantation is a preferred first-line therapy in patients with poor liver function or whole-liver involvement. Liver transplantation is also a good rescue therapy following TAE or liver resection.