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Anestheshetic management in a patient with MELAS syndrome receiving the diagnosis of toxic megacolon

Yıl 2018, Cilt: 10 Sayı: 2, 58 - 61, 01.08.2018

Öz

MELAS syndrome is a progressive, neurodegenerative genetic disorder characterized by point mutations in the mitochondrial DNA, mitochondrial myopathy, lactic acidosis, encephalopathy, and stroke-like episodes. The syndrome has high morbidity and mortality, affecting all the organs, especially the brain, cardiovascular, gastrointestinal and neuromuscular systems. Due to metabolic acidosis, tendency to hypothermia and to malign hyperthermia, increased resistance to muscle relaxants as well as prolonged effects, the method for anesthetic management, monitorization, medication and fluid regimes are of crucial significance for anesthesiologists in these patients. In this article, we present anesthetic management performed on a patient with MELAS syndrome who had received the diagnosis of toxic megacolon and underwent emergency hemicolectomy.

Kaynakça

  • 1. Sproule DM, Kaufmann P. Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes. basic concepts, clinical phenotype, and therapeutic management of MELAS syndrome. Ann NY Acad Sci.2008;1142:133–158.
  • 2. F Koç, Y Sarıca, D Yerdelen. Mitokondrial Hastalıklar: Klinik Özellikleri-Hastaya Yaklaşım- Arşiv Kaynak Tarama Dergisi, 2003;12: 32; 36-43.
  • 3 Fricker RM, Raffelsberger T, Rauch-Shorny S, Finsterer J, Müller-Reible C, Gilly H, et al. Positive malignant hyperthermia susceptibility in vitro test in a patient with mitochondrial myopathy and myoadenylate deaminase deficiency. Anesthesiology. 2002;97:1635–1637.
  • 4. Cholley F, Edery P, Ricquier D, Peudenier S, Slama A, Tardieu M. Mitochondrial respiratory chain deficiency revealed by hypothermia. Neuropediatrics. 2001;32:104–106.
  • 5. Gurrieri C, Kivela JE, Bojanic K, Gavrilova RH, Flick RP, Sprung J, Weingarten TN. Anesthetic condsiderations in mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes syndrome:a case series.Can J Anesth. 2011; 58:751-763.
  • 6. Parikh S,Saneto R, Falk MJ,et al. A modern approach to the treatment of mitochondrial disease. Curr Treat Options Neurol.E 2009;11:414-30.
  • 7. Finsterer J, Stratil U, Bittner R, Sporn P. Increased sensitivity to rocuronium in mitochondrial myopathy. Can J Anaesth. 1998;45:781–784.
  • 8. Wisely NA, Cook PR. General anesthesia in a man with mitochondrial myopathy undergoing eye surgery. Eur J Anaesthesiol. 2001;18:333–335.
  • 9. Aouad MT, Gerges FJ, Bararka AS. Resistance to cisatracurium in a patient with MELAS syndrome. Paediatr Anaesth. 2005;15:1124–1127.
  • 10. Jin Suk Park, Chong Wha Baek, Hyun Kang, Su Man Cha, Jung Won Park, Yong Hun Jung, and YoungCheol Woo, Total intravenous anesthesia with propofol and remifentanil in a patient with MELAS syndrome -A case report-Korean J Anesthesiol. 2010;58(4): 409– 412.
  • 11. Haas A, Wappler F.Characteristics of anesthesia in patients with MELAS syndrome: Case report of anesthesia in video-assisted thoracoscopy.Anaesthesist. 2015;64(10):747-53.
  • 12. Flick RP, Gleich SJ,Herr MM, Wedel DJ, The risk of malignant hyperthermia in children undergoing muscle biopsy for suspected neuromuscular disorder. Pediatr Anesth. 2007; 17:22-27.
  • 13. Vasile B, Rasulo F, Candiani A, Latronico N. The pathophysiology of propofol infusion syndrome: a simple name for a complex syndrome. Intensive Care Med. 2003;29:1417–1425.

MELAS sendromlu toksik megakolonlu olguda anestezi yönetimi

Yıl 2018, Cilt: 10 Sayı: 2, 58 - 61, 01.08.2018

Öz

MELAS sendromu; mitokondrial düzeyde nokta mutasyon sonucu gelişen, mitokondrial miyopati, laktik asidoz, ensefalopati ve inme benzeri epizotlarla karakterize progresif nörodejeneratif genetik bir hastalıktır. Yüksek morbidite ve mortaliteye sahip olan bu sendrom; özellikle beyin, kardiyovasküler gastrointestinal ve nöromuskuler sistem olmak üzere tüm organları etkiler. Metabolik asidoz, hipotermi, malign hipertermiye yatkınlık, kas gevşeticilere karşı artmış direnç ve uzamış etkiden dolayı bu hastalarda uygulanacak anestezi yöntemi, monitörizasyon, ilaç ve sıvı rejimi anestezistler açısından önem arz eder. Bu yazıda toksik megakolon tanısı ile acil hemikolektomi geçiren MELAS sendromlu bir olguda uyguladığımız anestezi yönetimini sunulmuştur.

Kaynakça

  • 1. Sproule DM, Kaufmann P. Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes. basic concepts, clinical phenotype, and therapeutic management of MELAS syndrome. Ann NY Acad Sci.2008;1142:133–158.
  • 2. F Koç, Y Sarıca, D Yerdelen. Mitokondrial Hastalıklar: Klinik Özellikleri-Hastaya Yaklaşım- Arşiv Kaynak Tarama Dergisi, 2003;12: 32; 36-43.
  • 3 Fricker RM, Raffelsberger T, Rauch-Shorny S, Finsterer J, Müller-Reible C, Gilly H, et al. Positive malignant hyperthermia susceptibility in vitro test in a patient with mitochondrial myopathy and myoadenylate deaminase deficiency. Anesthesiology. 2002;97:1635–1637.
  • 4. Cholley F, Edery P, Ricquier D, Peudenier S, Slama A, Tardieu M. Mitochondrial respiratory chain deficiency revealed by hypothermia. Neuropediatrics. 2001;32:104–106.
  • 5. Gurrieri C, Kivela JE, Bojanic K, Gavrilova RH, Flick RP, Sprung J, Weingarten TN. Anesthetic condsiderations in mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes syndrome:a case series.Can J Anesth. 2011; 58:751-763.
  • 6. Parikh S,Saneto R, Falk MJ,et al. A modern approach to the treatment of mitochondrial disease. Curr Treat Options Neurol.E 2009;11:414-30.
  • 7. Finsterer J, Stratil U, Bittner R, Sporn P. Increased sensitivity to rocuronium in mitochondrial myopathy. Can J Anaesth. 1998;45:781–784.
  • 8. Wisely NA, Cook PR. General anesthesia in a man with mitochondrial myopathy undergoing eye surgery. Eur J Anaesthesiol. 2001;18:333–335.
  • 9. Aouad MT, Gerges FJ, Bararka AS. Resistance to cisatracurium in a patient with MELAS syndrome. Paediatr Anaesth. 2005;15:1124–1127.
  • 10. Jin Suk Park, Chong Wha Baek, Hyun Kang, Su Man Cha, Jung Won Park, Yong Hun Jung, and YoungCheol Woo, Total intravenous anesthesia with propofol and remifentanil in a patient with MELAS syndrome -A case report-Korean J Anesthesiol. 2010;58(4): 409– 412.
  • 11. Haas A, Wappler F.Characteristics of anesthesia in patients with MELAS syndrome: Case report of anesthesia in video-assisted thoracoscopy.Anaesthesist. 2015;64(10):747-53.
  • 12. Flick RP, Gleich SJ,Herr MM, Wedel DJ, The risk of malignant hyperthermia in children undergoing muscle biopsy for suspected neuromuscular disorder. Pediatr Anesth. 2007; 17:22-27.
  • 13. Vasile B, Rasulo F, Candiani A, Latronico N. The pathophysiology of propofol infusion syndrome: a simple name for a complex syndrome. Intensive Care Med. 2003;29:1417–1425.
Toplam 13 adet kaynakça vardır.

Ayrıntılar

Birincil Dil Türkçe
Bölüm Case Report
Yazarlar

Tülay Örki Bu kişi benim

Lale Kurul Özcan Bu kişi benim

Deniz Avan Bu kişi benim

Yayımlanma Tarihi 1 Ağustos 2018
Yayımlandığı Sayı Yıl 2018 Cilt: 10 Sayı: 2

Kaynak Göster

Vancouver Örki T, Özcan LK, Avan D. MELAS sendromlu toksik megakolonlu olguda anestezi yönetimi. Maltepe tıp derg. 2018;10(2):58-61.