Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1016/j.bjane.2021.07.037
Brazilian Journal of Anesthesiology
Case Report

Several challenges associated with the anesthetic management of Emery-Dreifuss muscular dystrophy patients: case report

Ana Isabel Leite, Sofia da Silva Ramos, Raquel Inácio, Isabel Rute Vilhena, Joana Carvalhas

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Abstract

Emery-Dreifuss Muscular Dystrophy is a very rare type of muscular dystrophy, associated with contractures, atrophy and muscle weakness, besides cardiomyopathy with severe arrhythmias. Published studies focusing on this disorder are scarce. We describe the anesthetic management of a male patient with Emery-Dreifuss Muscular Dystrophy, to be submitted to umbilical and inguinal hernioplasty and hydrocele repair under epidural anesthesia. The anesthesia approach enabled us to circumvent the patient’s susceptibility to malignant hyperthermia and his potentially difficult airway, in addition to maintaining hemodynamic stability. The day after surgery the patient resumed walking, and two days later he was discharged from the hospital.

Keywords

Emery-Dreifuss muscular dystrophy;  Epidural anesthesia;  Malignant hyperthermia;  Airway management; Case report
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Braz J Anesthesiol

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