Abdulkadir YEKTAŞ, Sıtkı Nadir ŞİNİKOĞLU, Melehat EROL, Mevlüt ÇÖMLEKÇİ, Kerem ERKALP

Kearns-Sayre sendromlu bir hastada anestezi yöntemi

Kearns-Sayre sendromu; Kronik-progresif, eksternal oftalmopleji ve pigmenter retina dejenerasyonu ile karakterize, 20 yaşından önce bulgu veren, mitokondriyal bir hastalıktır. Kearns-Sayre sendromlu hastalarda; potansiyel aspirasyon riski, zayıf faringeal kas kontrolü ve oral sekresyonlara bağlı hava yolu komplikasyonları nedeniyle, genel anestezi uygulaması dikkatli bir şekilde yürütülmelidir. Kalp iletim defektleri de hastalığın sık görülen ve önlenebilen ölümcül komplikasyonlarındandır. Bu sunuda; Kearns-Sayre sendromlu bir hastaya olan anestezik yaklaşımımızı sunmayı amaçladık.

Anahtar Kelimeler:

Anestezi, genel, Kadın, Genç erişkin, Kearns-Sayre sendromu

Anesthetic management in a patient with Kearns-Sayre syndrome

Kearns-Sayre syndrome is a mitochondrial dissease characterized by a triad of features including oncet in persons younger than 20 years, chronic progressive external ophtalmoplegia and pigmentary degeneration of retina. In patient with Kearns-Sayre syndrome, General anesthetic management should be carefully implemented in consideration of the potantial risk of aspiration and air-way complications due to poor pharyngeal muscle control and oral secretions. Cardiac conduction defects are significant and preventable cause of mortality. Here we aimed to report our anesthetic management of a patient with Kearns- Sayre syndrome.

Keywords:

Anesthesia, General, Female, Young Adult, Kearns-Sayre Syndrome,

PDF

___

1. Carod-Artal FJ, Lopez Gallardo E, Solano A, Dahmani Y, Herrero MD, Montoya J. Mitochondrial DNA deletion in Kearns Sayre syndrome. Neurologia 2006; 21: 357-364.
2. Moraes CT, Dimauro S, Zeviani M, et al. Mitocondrial DNA deletions in progressive external ophthalmoplegia and Kearns Sayre syndrome. N Engl J Med 1989; 320: 1293-1299.
3. Kearns TP, Sayre GP. Retinitis pigmentosa external ophthalmoplegia and complete heart block. AMA Arch Ophtalmol 1958; 60: 280-289.
4. Sundaran C, Kanikannan MA, Jagarlapudi MM, Bhompally VR, Surath M. Diagnosis of mitochondrial disease. Clinical and histological study of sixty patients with ragged red fibers. Neurol India 2004; 52: 353-358.
5. Klockgether-Radke A, Henze T, Braun U, Ketler D. General anesthesia in two patients with mitochondrial myopathy. Anesthesist 1993; 42: 111-114.
6. Lauwers MH, Van Lersberghe C, Camu F. Inhalation anesthesia and the Kearns Sayre syndrome. Anaesthesia 1994; 49: 876-878.
7. Hara K, Sata T, Shigematsu A. Anesthetic management for cardioverter-defibrillator implantation in a patient with Kearns Sayer syndrome. J Clin Anesth 2004; 16: 539-541.
8. Sabate S, Ferrandiz M, Paniagua P, Vilamor JM, Vilanova F, Villar-Landeira JM. Aneshesia in Kearns Sayre syndrome(mitochondrial myophaty). Rev Esp Anesthesiol Reanim 1996; 43: 255-257.
9. Mayer WJ, Remy M, Rudolph G. Kearns-Sayre syndrome: A mitochondrial disease. Ophtalmologe 2010; 18.
10. Celik U, Celik T, Avcı A, et al. Metabolic acidosis in a patient with type 1 diabetes mellitus complicated by methanol and amitriptiline intoxication. Eur J Emerg Med 2009; 16: 45-48.
11. Axel F, Berthold B, Peter HT. Propofol infusion syndrome in anesthesia and intensive care medicine. 2006; 19: 404-410.
12. Vasile B, Rasulo F, Candiani A, Latronico N. The pathophysiology of propofol infusion syndrome: a simple name for a complex syndrome. İntensive care med 2003; 29: 1417-1425.
13. Parke TJ, Stevens JE, Rice AS, et al. Metabolic acidosis and fatal myocardial failure after propofol infusion in children. BMJ 1992; 305: 613-616.
14. Arzu G, Selin T, Deniz K, Memet Ö. Malign hipertermi öyküsü olan çocuk olguda genel anestezi yöntemi. Türk Anestezi Reanimasyon Dergisi 2008; 36: 250-252.
15. Stevens JB, Wheatley L. Tracheal intubation in ambulatory surgery patients: using remifentanil and propofol without muscle relaxants. Anesth and Analg 1998; 86: 45-49.
16. Erhan E, Ugur G, Alper I, Gunusen I, Ozyar B. Tracheal intubation without muscle relaxants: remifentanil or alfentanil in combination with propofol. Europen J Anaesthesiology 2003; 20:37-43.
17. Editorial II. Tracheal intubation without neuromuscular blocking agents: is there any point. British J Anaesthesia 2010; 104: 535-537.
18. Migita T, Mukaida K, Kawamoto M, et al. Propofol induced changes in myoplasmic calcium concentrations in cultured human skeletal muscles from RYR 1 mutation carriers. Anaesth Intensive Care 2007; 35: 894-898.
19. Brandom BW. Malignant hyperthermia.In: Motoyoma EK, Davis PJ, ed.Smith’s Anesthesia for infants and children. 7thed. Philadelphia: Mosby 2006; 521-570.