摘要: Objective To evaluate the efficacy of liver transplantation for severe autosomal dominant polycystic liver disease (APLD).Methods A patient with severe APLD (only two liver segments left in the periphery of the right lobe)was treated by percutaneous aspiration and ethanol sclerotheraphy but in vain.She was then treated by partial hepatectomy and fenestration, but developed severe postoperative complications.Orthotopic liver transplantation (OLT) had to be performed 8 months after the first operation.Results A 26-month follow-up showed that the patient recovered well.Conclusions OLT is an effective method to cure severe APLD patient,who treated by partial hepatectomy and fenestration but in vain.