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Anesthetic management for surgery in a nemaline myopathy patient with difficult airway: A CARE-compliant case report
Hoon Jung1, Hyunjee Kim, See Woo Lee
1Department of Anesthesiology and Pain Medicine, School of Medicine, Kyungpook National University, Daegu, Republic of Korea.
Rationale:
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.
Patient Concerns:
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.
Diagnoses:
The patient was diagnosed with NM (ACTA1 mutation) and coronoid hyperplasia.
Interventions:
Awake fiberoptic nasal intubation was performed following preparations for analgesia. General anesthesia was maintained using inhalational anesthetics and opioids without using neuromuscular blocking agents.
Outcomes:
General anesthesia remained well maintained during surgery, with no movement or spontaneous breathing of the patient and he recovered from anesthesia uneventfully without complications.
Lessons:
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.
Insights
Anesthesia management for nemaline myopathy (NM) requires careful planning due to respiratory risks. This case demonstrates successful general anesthesia induction and recovery in a high-risk NM patient, ensuring safety.
Area of Science:
- Anesthesiology
- Neuromuscular Disorders
Background:
- Nemaline myopathy (NM) presents with muscle weakness and potential respiratory/cardiac compromise, increasing anesthesia risks.
- Patients with NM may require surgical intervention, necessitating careful anesthetic planning.
Observation:
- A 35-year-old male with NM, ACTA1 mutation, and difficult airway underwent surgery for limited mouth opening.
- The patient had restrictive ventilatory pattern and pulmonary hypertension, posing significant anesthetic challenges.
Findings:
- Awake fiberoptic nasal intubation was successfully performed under analgesia.
- General anesthesia was maintained with inhalational agents and opioids, avoiding neuromuscular blockers.
- The patient experienced uneventful recovery without complications.
Implications:
- This case underscores the feasibility of safe anesthesia in high-risk NM patients.
- Careful preoperative assessment and tailored anesthetic techniques are crucial for managing NM patients.
- Highlights the importance of avoiding neuromuscular blocking agents in NM patients.
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