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Duchenne muscular dystrophy and malignant hyperthermia--two case reports
Canadian Anaesthetists' Society Journal
|July 1, 1986
Summary
Duchenne muscular dystrophy (DMD) patients are at risk for malignant hyperthermia during anesthesia. Succinylcholine can trigger severe reactions in undiagnosed DMD, while known DMD patients may develop hyperthermia post-operatively.
Area of Science:
- Anesthesiology
- Pediatric Medicine
- Neuromuscular Disorders
Background:
- Duchenne muscular dystrophy (DMD) is a genetic disorder affecting muscle strength.
- Anesthetic management in DMD patients requires careful consideration due to potential complications.
- Malignant hyperthermia (MH) is a rare, life-threatening reaction to certain anesthetic agents.
Observation:
- A two-year-old with undiagnosed DMD experienced a severe reaction, including cardiac arrest, following succinylcholine administration during anesthesia.
- The patient presented with hyperkalemia, acidosis, and hepatosplenomegaly, with muscle biopsy consistent with MH.
- A three-year-old with known DMD developed postoperative hyperthermia and metabolic acidosis, responsive to dantrolene treatment.
Findings:
- Succinylcholine can precipitate malignant hyperthermia in patients with undiagnosed Duchenne muscular dystrophy.
- Known DMD patients may develop hyperthermia and acidosis post-anesthesia, which can be managed with dantrolene.
- Early recognition and appropriate anesthetic protocols are crucial for managing DMD patients.
Implications:
- This study highlights the critical need for pre-anesthetic screening for DMD, especially in young children.
- Anesthesiologists must be aware of the MH risk in DMD patients and employ specific anesthetic strategies.
- Prompt diagnosis and treatment of MH or related hyperthermic responses are vital for patient survival and recovery.