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Anesthetic management of a parturient with Emery-Dreifuss Muscular Dystrophy
Abstract Number: FCH-345
Abstract Type: Case Report Case Series
Emery-Dreifuss muscular dystrophy (EDMD) is characterized by contractures of elbows and posterior cervical muscles limiting their range of motion, progressive muscle wasting and weakness. In addition, these patients are very susceptible to cardiac conduction defects and cardiomyopathy.1,2 Modes of inheritance can be either X-linked autosomal recessive or dominant. The prevalence of this muscular dystrophy is unknown, but it is thought to be less than 1-2/100,000.1,2
A 31-year-old female, gravida 1 para 0, at 40 weeks gestation, with a past medical history of Emery-Dreifuss muscular dystrophy (EDMD) and paroxysmal atrial fibrillation, was admitted in labor to our hospital. Her past surgical history revealed an Achilles tendon lengthening without any anesthetic complication. Her family history was significant for arrhythmia and cardiomyopathy in her father, brother and sister. All her aforementioned relatives had automatic implantable cardioverter-defibrillator (AICD) in place secondary to this. The patient’s echocardiogram performed during this pregnancy revealed normal left and right ventricular size and function, with an estimated ejection fraction of 64%. Her paroxysmal atrial fibrillation was asymptomatic and managed only with aspirin as rate control medication was not deemed necessary. Otherwise, this patient’s prenatal course had been relatively uncomplicated.
An early labor epidural at the L3-L4 level was performed in order to minimize catecholamine surge associated with labor pain. After confirming negative intrathecal or intravascular placement, 8 ml of bupivacaine 0.25% was administered over a 10-minute period and a bupivacaine 0.125%- 8ml/h infusion started. The patient tolerated procedure well. As per cardiology recommendation, the patient was on continuous telemetry throughout the peripartum period, and a transcutaneous pacemaker/defibrillator was immediately available given her risk of peripartum arrhythmias. Her intrapartum and postpartum course were uncomplicated, and she was discharged home 3 days after delivery.
There seems to be paucity of literature regarding the anesthetic management of patients with EDMD, despite the challenges this disease poses to anesthesiologists. AICD’s are frequently placed for patients with this dystrophy. Hence, the physiologic changes of pregnancy, the risk of cardiomyopathy and AICD precautions should be kept in mind when caring for patients with EDMD. Neuraxial analgesia/anesthesia, when possible, avoids the potential challenges of difficult intubation secondary to limited neck range of motion and an unknown, yet rare, susceptibility to malignant hyperthermia.1,2 A multidisciplinary approach is recommended when caring for a pregnant patient with EDMD.
1. Rev Esp Anestesiol Reanim 1996;43:288–90.
2. Anaesth Intensive Care 2010;38:744–7.