Genel Tip Dergisi, vol.32, no.6, pp.789-792, 2022 (Scopus, TRDizin)
Friedreich’s ataxia (FRDA) is an autosomal recessive neurodegenerative disease, which may be accompanied by scoliosis, cardiac, endocrine and pulmonary comorbidities. In this paper the anesthesia experiences are presented using thetotal intravenous anesthesia (TIVA) method in scoliosis surgery of four patients with Friedreich ataxia. The patients were monitored with central venous pressure (CVP), bispectral index (BIS), invasive blood pressure, and near infrared spectroscopy (NIRS). The risks of difficult airway and malignant hyperthermia increased in patients with Friedreich’s ataxia. Specialized equipment for difficult airway management was prepared and dantrolene was administered preoperatively. Neuromuscular blocker was used for anesthesia induction in one case, but was not required in the other 3 patients. Hypertrophic cardiomyopathy may accompany patients with Friedreich’s ataxia. Therefore, detailed preoperative examination, intraoperative close monitoring of bleeding, blood gas analysis, hemodynamic monitoring and postoperative multimodal analgesia for hemodynamic stability were applied for patients with Friedreich’s ataxia. The aim of this paper was to present our anesthesia management experience in four patients with Friedreich’s ataxia who underwent scoliosis surgery, guided by the medical literature.