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

Tülay Örki Lale Kurul Özcan Deniz Avan

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.

Anahtar Kelimeler

MELAS syndrome, toxic megacolon, anesthetic management

MELAS sendromlu toksik megakolonlu olguda anestezi yönetimi

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.

Anahtar Kelimeler

MELAS sendromu, toksik megakolon, anestezi yönetimi

Kaynakça

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