The anaesthetic management of a patient with Emery-Dreifuss muscular dystrophy

Purpose

This case report presents a patient with Emery-Dreifuss Muscular Dystrophy and describes the anaesthetic considerations.

Clinical features

The features of Emery-Dreifuss Muscular Dystrophy are contractures, humeroperoneal muscle weakness and cardiomyopathy. The anaesthetic considerations for this syndrome are difficult tracheal intubation, difficult spinal anaesthetic, heart block, gastric reflux, rhabdomyolysis, and unproved malignant hyperthermia susceptibility.

Conclusion

The major anaesthetic problem for the patient with Emery-Dreifuss Muscular Dystrophy could be a life-threatening cardiomyopathy.