title: Successful left hemihepatectomy and perioperative management of a patient with biliary cystadenocarcinoma, complicated with MELAS syndrome: report of a case.
abstract: Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Stroke-like syndrome (MELAS) is a rare, fetal disease caused by a mutation in mitochondrial DNA that leads to impaired oxidative metabolism in skeletal muscle, the central nervous system, and liver function. This report presents the case of a 50-year-old woman with biliary cystadenocarcinoma complicated by MELAS who underwent a successful left hemihepatectomy. In this case, the diagnostic key for the malignant tumor was an (18)F-fluorodeoxyglucose positron emission tomography study, which was useful even in a patient with MELAS, which causes abnormal glucose metabolism. The perioperative management of such patients includes special precautions to prevent lactic acidosis and deterioration of the reserved liver function after a hepatectomy, since the mitochondrial function in MELAS patients is abnormal. The patient in this report has remained free of liver dysfunctions and cancer recurrence for 2 years following the hepatectomy. This is the first report of a successful major hepatectomy for a patient with MELAS.
authors.affiliation: Division of Hepato-Biliary-Pancreatic Surgery and Transplantation, Department of Surgery, Graduate School of Medicine, Kyoto University, 54 Shogoin, Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan.
authors.forename: Ayami
authors.initials: A
authors.lastname: Ohno
authors.forename: Akira
authors.initials: A
authors.lastname: Mori
authors.forename: Ryuichiro
authors.initials: R
authors.lastname: Doi
authors.forename: Yoshikuni
authors.initials: Y
authors.lastname: Yonenaga
authors.forename: Noboru
authors.initials: N
authors.lastname: Asano
authors.forename: Shinji
authors.initials: S
authors.lastname: Uemoto
mesh_terms: D001661:Biliary Tract Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D003536:Cystadenocarcinoma / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D005260:Female; D006498:Hepatectomy; D006801:Humans; D017241:MELAS Syndrome / Q000150:complications*; D008875:Middle Aged; D019990:Perioperative Care; D011877:Radionuclide Imaging