P054: ANESTHETIC CONSIDERATIONS FOR A PATIENT WITH DUCHENNE MUSCULAR DYSTROPHY ON PLV-100 HOME VENTILATOR
Anirudh K Gorti, MD; Kamil M Sochacki, DO; Andrew J Iskander, MD; Akshay Goyal, MD; Rutgers-Robert Wood Johnson Hospital
Introduction: Duchenne Muscular dystrophy (DMD) is a hereditary muscular dystrophy affecting approximately 1 in 3,500 to 1 in 5,000 newborn males globally each year (1). DMD is an X-linked recessive disorder of the dystrophin gene—the largest known gene in the human cells—predisposing it to spontaneous mutation. The role of the dystrophin protein is to anchor cytoskeleton of cardiac and skeletal muscle cells to the extracellular matrix, linking actin filaments to extracellular laminins. Disruption of dystrophin has systemic, progressive life-threatening consequences. Arrhythmias, hypoventilation, cardiomyopathies, dysphagia all induce morbidity and mortality in these patients (2), and in turn have multiple anesthetic implications. Lifespan is estimated to be 30 years of age and mortality stems from cardiorespiratory failure.
Here we present a case of a 28-year-old male with DMD who is dependent on a PLV-100 home ventilator. He was admitted to the hospital for failure to thrive in the setting of starvation ketosis secondary to dysphagia and aspiration. This patient required an endoscopic percutaneous gastrostomy tube placement. Here we review the anesthetic considerations for this population and the potential complications associated with late-stage DMD which include multi-systemic deterioration characterized by dysphagia, aspiration, arrhythmias, hypercapnia, hypoventilation, and failure to thrive (3). Moreover, in a patient chronically supported by PLV-100 ventilator—which is no longer manufactured—endoscopic intervention further complicates airway management. Airway obstruction resulting in hypercapnic respiratory failure is a feared complication in the endoscopy suite and this risk is elevated in neuromuscular disorders and ventilated patients. An understanding of analog ventilators such as the PLV-100 is paramount to reducing complications associated with limited knowledge of dated technology.
Methods: Our anesthetic plan involved awake endoscopy with local topicalized benzocaine. The patient maintained spontaneous ventilation with nasal CPAP via PLV-100 ventilator to minimize risk of respiratory failure and preoperative electrolyte derangements were corrected to minimize risk of fatal arrhythmia. All relevant data was gathered from electronic medical record, and those participating in the case. Patient identifying information was omitted. For literature review, relevant articles were identified in PubMed using keywords “Duchenne Muscular Dystrophy”, “home ventilator”, “anesthesia”, “complications”
Results: The patient underwent the procedure with no complications and returned to his room in the hospital. He was trialed on tube feeds, and was able to tolerate them appropriately. His failure to thrive improved and was ultimately discharged home shortly after.
Discussion/Conclusion: Patients who suffer from Duchenne Muscular Dystrophy are limited by lack of therapeutic advances, with supportive therapy as mainstay of treatment. Percutaneous gastrostomy tubes aid in minimizing risk of aspiration, malnutrition and circumvent the severe dysphagia that accompanies advanced disease due to muscle degeneration. Patients with DMD require a carefully tailored anesthetic plan and close monitoring during any invasive procedure as general anesthesia and sedation increase risk of aspiration and exacerbation of pulmonary disease leading to cardiorespiratory arrest (4). A diagnosis of DMD will also alter choice of neuromuscular blockade, decision for mechanical ventilation, pre-operative cardiac screening, and gastrointestinal prophylaxis (5).