The anesthetic management of a patient with Emery-Dreifuss muscular dystrophy for orthopedic surgery

General Anesthesia



To report a patient with Emery-Dreifuss muscular dystrophy (EDMD) coming for an orthopedic procedure, with potential problems of sudden cardiac death, difficult airway, and neuromuscular disorders who was managed successfully by permanent pacemaker insertion, total iv anesthesia (TIVA), laryngeal mask insertion (LMA) insertion and continuous epidural blockade.

Clinical features

A 22-yr-old man with known EDMD presented for triple arthrodesis of his right foot and fractional lengthening of his hamstrings bilaterally. Anesthesia was induced with a TIVA technique, and maintained throughout the operative period. A suspected difficult airway was managed by the use of a LMA, and analgesia for the peri-, and postoperative period provided by a continuous epidural infusion. The patient’s perioperative course was uneventful.


EDMD is a rare disorder. However, anesthesia is often required for orthopedic procedures. This case report illustrates the many potential difficulties that may be encountered. Regional anesthesia combined with light general anesthesia offers a method of avoiding many of these difficulties.


Muscular Dystrophy Laryngeal Mask Airway Difficult Airway Malignant Hyperthermia Flexion Contracture 
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La prise en charge anesthésique d’un patient de chirurgie orthopédique atteint de dystrophie musculaire de Emery-Dreifuss



Présenter le cas d’un patient, atteint de dystrophie musculaire de Emery-Dreifuss (DMED), qui devait subir une intervention orthopédique. Cette opération comportait des risques potentiels de mort cardiaque soudaine, d’intubation difficile et de troubles neuromusculaires dont la prise en charge a été réussie grâce à l’insertion d’un stimulateur cardiaque permanent, à l’anesthésie exclusivement iv (AEIV), à l’utilisation d’un masque laryngé (ML) et d’un blocage péridural continu.

Éléments cliniques

Un homme de 22 ans, dont la DMED était connue, devait subir une triple arthrodèse du pied droit et l’allongement fractionné bilatéral des ischio-jambiers. L’anesthésie a été induite selon une technique AEIV qui a été maintenue tout au long de l’opération. Lusage d’un ML, et d’une perfusion péridurale continue pour l’analgésie périopératoire et postopératoire, ont permis de contourner les difficultés d’intubation soupçonnées. Aucun incident périopératoire n’est survenu.


La DMED est une affection rare. La présente observation illustre les nombreuses difficultés anesthésiques possibles dans les circonstances. Lanesthésie régionale, combinée à une anesthésie générale légère, représente une méthode efficace de contourner ces difficultés.


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Copyright information

© Canadian Anesthesiologists 2002

Authors and Affiliations

  1. 1.Department of AnaesthesiaDerriford Hospital, Derriford HospitalPlymouthUK

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