Anesthetic Management for Mandibular Fracture in a Patient with Spinocerebellar Ataxia Type 1

Abstract

  Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant disorder that is clinically characterized by progressive ataxia, dysarthria, and the eventual deterioration of bulbar functions. A 43-year-old man (height, 171 cm ; weight, 54 kg) with SCA1 was scheduled to undergo an open reduction and internal fixation of a mandibular fracture under general anesthesia. The clinical manifestations of this patient included gait disturbance, dysarthria, dysphagia, oculomotor deficits and anxiety disorder. A preoperative fiberoptic laryngeal examination revealed that his vocal cord was not paralyzed. A video laryngoscope and bronchial fiberscope were prepared before the induction of anesthesia in case of a difficult tracheal intubation and to evaluate the vocal cord movements after emergence from anesthesia. After the placement of a radial arterial line and electrodes for neuromuscular monitoring, general anesthesia was induced with propofol and remifentanil. The initial dose of rocuronium was titrated using neuromuscular monitoring, and nasotracheal intubation was performed with a McGRATH^TM MAC video laryngoscope. General anesthesia was maintained with desflurane and remifentanil. Etilefrine and phenylephrine were continuously administered to avoid hypotension, which could have been induced by autonomic neuropathy. The surgery was completed uneventfully. Acetaminophen was used for postoperative pain relief instead of opioids. After extubation, the patient had no symptoms of vocal cord paralysis and was transferred to the ICU. When anesthetizing patients with SCA1, perioperative evaluation of the airway and preparation for anticipated difficult airway management are crucial.