Successful living donor liver transplantation for polycystic liver in a patient with auto somal-dominant polycystic kidney disease

Orthotopic liver transplantation has been recommended for patients with dis abling polycystic liver disease (PCLD). Because of the shortage of cadaveri c donors, living donor liver transplantation (LDLT) has been developed as a n alternative. We describe the case of a woman with PCLD as an extrarenal m anifestation of autosomal-dominant polycystic kidney disease (ADPKD) who wa s successfully palliated by LDLT. The patient was a 48-year-old woman with abdominal distention. Computed tomography showed a massively enlarged liver containing innumerable cysts, as well as bilateral kidney cysts. Hepatic a nd renal functions were well preserved. Genetic analysis of the family did not exclude linkage to the PKD1 locus. Two and a half years after the first examination, the patient reported severely disabling symptoms caused by th e PCLD. Living donor liver transplantation was performed using a right-lobe graft. The recipient and donor were both well 8 months after the transplan tation. The excised liver weighed 7.4 kg, and the histopathology revealed m ultiple cysts and von Meyenburg complexes in the portal areas.