P014: ANESTHETIC CONSIDERATIONS AND MANAGEMENT OF EMERY DREIFUSS MUSCULAR DYSTROPHY
Emily Chung, DO; Kayla Yoshida, DO; Matthew Maggio, MD; Kalina Nedeff, MD; Kendall Regional Medical Center
Introduction: A 55-year-old female with past medical history of autosomal dominant Emery Dreifuss Muscular Dystrophy and first-degree atrioventricular block presented for robotic total abdominal hysterectomy and bilateral salpingo-oophorectomy. Her only medication is midodrine for chronic hypotension.
Methods: After extensive discussion with the patient and the surgeon regarding risks and benefits, it was determined that converting the procedure from robotic to open total abdominal hysterectomy and bilateral salpingo-oophorectomy would be safer for the patient due to history of Emery Dreifuss Muscular dystrophy. In the operating room, ASA standard monitors were placed and patient sat on the surgical bed. A combined spinal-epidural was placed using bupivacaine 0.75% with dextrose in the L3-L4 intervertebral space. Patient was then positioned in supine position and dermatomal tests were performed to check for adequate surgical block. Patient was then positioned into lithotomy position. Following the surgical procedure, a transversus abdominis plane block was performed using ropivacaine 0.25%.
Results: The surgery occurred without complications. She was transported to the post-anesthesia care unit. She was admitted to the hospital overnight for pain management and observation for possible complications. She was discharged from the hospital the next day.
Discussion/Conclusion: In patients with any type of muscular dystrophy, there are many factors to consider to provide safe anesthesia. The disease process affects multiple organ systems, including respiratory, cardiac, and central nervous system. Patients can be more predisposed to aspiration due to weakness of pharyngeal muscles and delayed gastric emptying. Additionally, due to respiratory muscle weakness, patients are at risk for post-operative atelectasis, pneumonia, and respiratory failure requiring prolonged ventilation. Due to the nature of robotic cases, generally, patients would require use of neuromuscular blockade. However, in patients with muscular dystrophy, the use of any neuromuscular blockade is a relative contraindication as patients tend to be more sensitive and have prolonged muscle weakness. Due to the multiple factors, it was decided to perform a neuraxial technique to provide the appropriate level analgesia for the procedure as well as minimizing the risk of exacerbating her condition.