Anesthetic management for surgery in a nemaline myopathy patient with difficult airway: A CARE-compliant case report.

Abstract

Nemaline myopathy (NM) is a congenital disease characterized by nonprogressive or slowly progressing muscle weakness and may increase the risk of anesthesia in case of respiratory muscle or cardiac involvement. Care should be taken to prevent respiratory failure after surgery. A 35-year-old man with NM, who had difficult airway, restrictive ventilatory pattern, and pulmonary hypertension, required general anesthesia for surgery because of limited mouth opening. The patient was diagnosed with NM (ACTA1 mutation) and coronoid hyperplasia. Awake fiberoptic nasal intubation was performed following preparations for analgesia. General anesthesia was maintained using inhalational anesthetics and opioids without using neuromuscular blocking agents. General anesthesia remained well maintained during surgery, with no movement or spontaneous breathing of the patient and he recovered from anesthesia uneventfully without complications. This report highlights the safe performance of anesthesia induction and recovery in a case where anesthesia management is necessary for surgery in a patient of NM at a high risk of anesthesia-related complications.