The Anesthetic Management in a Patient with Friedreich Ataxia

Friedreich ataksisi (FA) nadir gorulen herediter otozomal resesif bir hastaliktir. Friedreich ataksisi skolyoz gelismesi- ne neden olur ve eger ciddi boyutlarda skolyoz varsa nefes almayi da guclestirebilir. Skolyoz cerrahisi uygulanan, 6 yil once FA teshisi konan ve anestezi riski ASA III olarak tespit edilen, 14 yasindaki erkek hastayi sunmayi amacladik. Propofol ve remifentanil infuzyonu ile total intravenoz anestezi (TIVA) uygulanarak anestezi saglandi. Hastaya arter ve santral venoz basinc monitorizasyonu uygulama- sinin yaninda BIS ve noromuskuler monitorizasyon da yapildi. Operasyon suresince hasta hemodinamik olarak stabil seyretti. Biz bu olgu sunumunda, skolyoz cerrahisi icin anestezi uyguladigimiz Friedreich ataksili bir hastada anestezi deneyimimizi literatur bilgileri isiginda tartismayi amacladik. Friedreich ataxia is a rare inherited autosomal recessive disorder. Friedreich's ataxia develops scoliosis which, if severe, may impair breathing. A 14-year-old patient with Friedreich's ataxia, which was diagnosed 6 years ago, and determined the anesthetic risk ASA III is presented for scoliosis surgery. Anesthesia was induced with total intra- venous anesthesia (TIVA), remifentanil and propofol. With arterial pressure monitoring and central venous pressure monitoring, BIS monitoring and neuromuscular monitoring were used, too. The patient was hemodynamically stable during the operation. In this case, we discuss the anesthetic experience of a patient with Friedreich's ataxia underwent scoliosis surgery and literature review.