Şemsi Mustafa AKSOY, Ayşe LAFÇI, Ercan BAL, Muhammed KÖSE

Friedreich Ataksisi Olan Hastaların Skolyoz Cerrahisinde Anestezi Yönetimi: Dört Olgu Sunumu

Friedreich ataksisi (FRDA), kardiyak, endokrin ve pulmoner komorbiditelerin yanısıra skolyozun da eşlik edebileceği otozomal resesif geçişli nörodejeneratif bir hastalıktır. Bu raporda Friedreich ataksili dört hastada, skolyoz cerrahisinde total intravenöz anestezi (TIVA) yöntemi ile anestezi deneyimlerimizi sunmayı amaçladık. Hastalar, santral venöz basınç (CVP), bispektral indeks (BIS), invaziv kan basıncı ve near infrared spectroscopy (NIRS) ile izlendi. Friedreich ataksisi olan hastalarda malign hipertermi ve zor hava yolu riski yüksektir. Bu nedenle zor hava yolu için gerekli ekipman hazırlığı ve malign hipertermi riski için dantrolen sağladık. Bir olguda anestezi indüksiyonunda kas gevşemesi sağlayabilmek için kas gevşetici kullandık, diğer üç olguda ise kas gevşetici kullanmadık. Friedreich ataksisi olan hastalarda kalpte hipertrofik kardiyomiyopati sıktır. Bu nedenle Friedreich ataksili hastalarda hemodinamik stabilite için detaylı preoperatif değerlendirme, intraoperatif yakın kanama takibi, kan gazı analizi, hemodinamik izleme ve postoperatif multimodal analjezi önemlidir. Tıbbi literatür rehberliğinde, skolyoz cerrahisi geçiren Friedreich ataksili dört hastadaki anestezi yönetimi deneyimimizi sunmayı amaçladık.

Anahtar Kelimeler:

Anestezi, Skolyoz, Friedreich Ataksisi

Anesthesia Management in Scoliosis Surgery of Patients with Friedreich’s Ataxia: A Report of Four Cases

Friedreich's ataxia (FRDA) is an autosomal recessive neurodegenerative disease, which may be accompanied by scoliosis, cardiac, endocrine and pulmonary comorbidities. We present our anesthesia experiences using total intravenous anesthesia (TIVA) method in scoliosis surgery of four patients with Friedreich ataxia. Patients were monitored with central venous pressure (CVP), bispectral index (BIS),invasive blood pressure and near infrared spectroscopy (NIRS). The risk of malignant hyperthermia and difficult airway are increased in patients with Friedreich's ataxia. We were prepared for difficult airway and provided dantrolene preoperatively. Neuromuscular blocker was used for anesthesia induction in one case, we did not use it in the other cases. Hypertrophic cardiomyopathy may accompany patients with Friedreich's ataxia. Therefore, detailed preoperative examination, intraoperative close bleeding, blood gas analysis, hemodynamic monitoring and postoperative multimodal analgesia for hemodynamic stability in patients with Friedreich's ataxia were performed. We aimed to discuss our anesthesia management with four patients with Friedreich's ataxia who underwent scoliosis surgery.

Keywords:

Anesthesia, Scoliosis, Friedreich's Ataxia,

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