Perioperative Considerations in a Patient With Oculopharyngeal Muscular Dystrophy: A Case Report

Oculopharyngeal muscular dystrophy (OPMD) is a rare, late-onset neuromuscular disorder that is classically characterized by dysphagia, ptosis, and proximal limb weakness. Although OPMD is typically mild in progression, it presents unique challenges in the perioperative setting due to pharyngeal muscle weakness, increased risk of aspiration, and sensitivity to neuromuscular blocking agents. Here, we discuss the anesthetic management of a 75-year-old male with OPMD undergoing an elective reverse total shoulder arthroplasty. Though preoperative pulmonary function testing showed preserved respiratory mechanics, the patient had previously reported dysphagia, voice changes, and fatigue. Due to the increased risk for perioperative complications, an anesthetic plan was developed with the primary goals of reducing aspiration risk, minimizing neuromuscular blockade, and facilitating a smooth emergence. For induction of anesthesia, total intravenous agents were used. Inhaled anesthetics were avoided to decrease the risk of residual respiratory depression. Endotracheal intubation was performed under video laryngoscopy after administering a reduced dose of rocuronium. Intraoperatively, train-of-four stimulation was utilized for close monitoring of neuromuscular blockade. Following partial recovery of consciousness after case completion, rocuronium was reversed with sugammadex. The patient was then extubated and monitored in the post-anesthesia care unit. No respiratory sequelae were noted. This case highlights the need for individualized anesthetic planning in patients with OPMD, especially in the context of airway protection and neuromuscular management. Avoiding succinylcholine, limiting non-depolarizing agents, and utilizing neuromuscular monitoring may help mitigate the risk of prolonged paralysis and respiratory compromise. As more aging patients with neuromuscular disorders present for surgery, it is essential to promote increased awareness of the specific anesthetic implications of OPMD. This case contributes to the limited body of literature guiding safe perioperative care in this patient population.