Şennur UZUN, Seher ALTINEL, İ. Aydın ERDEN, Ülkü AYPAR

Nöral seroid lipofusinozisli (batten sendromu) hastada anestezi

Nöronal seroid lipofusinozis (NCL), nöronlarda ve diğer hücre tiplerinde otoşoresan materyallerin lizozomal birikimi ile karakterize otozomal resesif, çocuklarda gözlenen, nörodejeneratif nadir bir hastalıktır. Genellikle bu tanılı hastalarda, anestezi sırasında hipotermi ve bradikardi sıklıkla görülür. Malign hipertermi ve zor entübasyon sendromun anestezisine spesifik özellikler değildir. Kas gevşetici kullanımında, miyotonik olgularda suksinilkolin kullanımından kaçınılmalıdır. Kas gevşekliği için non depolarizan ajanlar kullanılabilir. Bu olgu sunumunda, NCL sendromlu bir çocuğa trakeotomi açılması sırasındaki anestezi uygulaması sunulmuştur.

Anesthesia management in a child with neuronal ceroid lipofuscinoses (batten syndrome)

Neuronal Ceroid Lipofuscinoses (NCL) is a group of rare autosomal recessive neurodegenarative disorder seen in children, which is characterized by lisosomsal accumulation of autofluorescent materials. During anesthesia, hypothermia and bradycardia are often seen in patients with this diagnosis. Generally, in the patients who are diagnosed as NCL, hypothermia and bradycardia are frequently observed during anesthesia. Malign hyperthermia and difficult intubation are not specific for anesthesia of this syndrome. Succinylcholine should be avoided in the myotonic cases. For muscle relaxation, non depolarizing agents could be used. In this case, the administration of anesthesia in a child with Neuronal Ceroid Lipofuscinoses during tracheotomy was reported.

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