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 18F-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.
Similar content being viewed by others
References
Sprolue DM, Kaufmann P. Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes: basic concepts, clinical phenotype, and therapeutic management of MELAS syndrome. Ann N Y Acad Sci 2008;1142:133–158.
Thambisetty M, Newman NJ. Diagnosis and management of MELAS. Expert Rev Mol Diagn 2004;4:631–644.
Scaglia, Northrop JL. The mitochondrial myopathy encephalopathy, lactic acidosis with stroke-like episodes (MELAS) syndrome: a review of treatment options. CNS Drugs 2006;20:443–464.
Mita S, Tokunaga M, Kumamoto T, Uchino M, Nonaka I, Ando M. Mitochondrial DNA mutation and muscle pathology in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. Muscle Nerve 1995;3:S113–S118.
Majamaa K, Moilanen JS, Uimonen S, Remes AM, Salmela PI, Kärppä M. Epidemiology of A3243G, the mutation for mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes: prevalence of the mutation in an adult population. Am J Hum Genet. 1998;63:447–454.
Manwaring N, Jones MM, Wang JJ, Rochtchina E, Howard C, Mitchell P, et al. Population prevalence of the MELAS A3243G mutation. Mitochondrion 2007;7:230–233.
Sarnat HB, Marín-García J. Pathology of mitochondrial encephalopathies. Can J Neurol Sci 2005;32:152–166.
Marko P, Gabrielli A, Caruso LJ. Case conference of the University of Florida “Too much lactate or too little liver?” J Clin Anesth 2004;16:389–395.
Vilela H, Garcìa-Fernández J, Parodi E, Reinoso-Barbero F, Durán P, Gilsanz F. Anesthetic management of a patient with MERRF syndrome. Paediatr Anesth 2005;15:77–79.
Sasano N, Fujita Y, So M, Sobue K, Sasano H, Katsuya H. Anesthetic management of a patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) during laparotomy. J Anesth 2007;21:72–75.
Chioléro R, Tappy L, Gillet M, Revelly JP, Roth H, Cayeux C, et al. Effect of major hepatectomy on glucose and lactate metabolism. Ann Surg 1999;229:505–513.
Furukawa H, Ikuma H, Asakura-Yokoe K, Uesaka K. Preoperative staging of biliary carcinoma using 18F-fluorodeoxyglucose PET: prospective comparison with PET+CT, MDCT and histopathology. Eur Radiol 2008;18:2841–2847.
Higashi T, Tamaki N, Honda T, Torizuka T, Kimura T, Inokuma T, et al. Expression of glucose transporters in human pancreatic tumors compared with increased FDG accumulation in PET study. J Nucl Med 1997;38:1337–1344.
Higashi T, Tamaki N, Torizuka T, Nakamoto Y, Sakahara H, Kimura T, et al. FDG uptake, GLUT-1 glucose transporter and cellularity in human pancreatic tumors. J Nucl Med 1998;39:1727–1735.
Läuffer JM, Baer HU, Maurer CA, Stoupis C, Zimmerman A, Büchler MW, et al. Biliary cystadenocarcinoma of the liver: the need for complete resection. Eur J Cancer 1998;34:1845–51.
Pojchamarnwiputh S, Na Chiangmai W, Chotirosniramit A, Lertprasertsuke N. Computed tomography of biliary cystadenoma and biliary cystadenocarcinoma. Singapore Med J 2008;49:392–396.
Del Poggio P, Buonocore M. Cystic tumors of the liver: a practical approach. World J Gastroenterol 2008;14:3616–3620.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Ohno, A., Mori, A., Doi, R. et al. Successful left hemihepatectomy and perioperative management of a patient with biliary cystadenocarcinoma, complicated with MELAS syndrome: Report of a case. Surg Today 40, 878–882 (2010). https://doi.org/10.1007/s00595-009-4145-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00595-009-4145-z