Living related liver transplantation for polycystic liver disease.
Polycystic liver disease (PCLD) is a rare inherited disorder, often associated with polycystic disease of the kidney. Although liver failure is unusual, some patients suffer from hepatic enlargement associated with severe complications such as abdominal distention, cachexia and dyspnea. Until recently, many surgical attempts had been made to reduce hepatic size, however, results have been unsatisfactory [3, 9, 10]. Today, liver transplantation is recommended as a therapeutic option, and excellent outcome has been demonstrated [1, 2, 4, 5, 6, 8, 11]. In this paper, we present the first case study of total hepatectomy and partial liver transplantation for PCLD, from a living, related donor. The patient is a 38-year-old man with PCLD who underwent living related liver transplantation (LRLT). He is alive and well 21 months after the operation, with complete resolution of the symptoms. He has returned to his previous job, with a marked improvement in his quality of life. Our experience demonstrates that LRLT can be an option for treatment of PCLD.
Koyama
Ichiro
I
Present address: Department of Surgery, White 546, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA. ichiro.koyama@tbrc.mgh.harvard.edu
Fuchinoue
Shohei
S
Urashima
Yoshinori
Y
Kato
Yojiro
Y
Tsuji
Kazuhiko
K
Kawase
Tomonori
T
Murakami
Toru
T
Tojimbara
Tamotsu
T
Nakajima
Ichiro
I
Teraoka
Satoshi
S
D000328:Adult; D003560:Cysts / Q000000981:diagnostic imaging / Q000601:surgery*; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000000981:diagnostic imaging / Q000601:surgery*; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome