Correct!
1. OMD has been associated with respiratory failure because of retained secretions and aspiration
The work up of this patient with recurrent respiratory failure without severe pulmonary disease suggested a neuromuscular component. The episodes of poor airway clearance, atelectasis, contributed to this suspicion. The patient was alert and able to follow commands, and without a lateralizing deficit, suggesting a more peripheral cause of neuro-muscular abnormality. Diaphragmatic weakness was excluded as a cause via ultrasound, myasthenia gravis and Guillain-Barre were found to be less likely due to the exam and the laboratory finding of a negative acetylcholine receptor antibody.
Oculopharyngeal muscular dystrophy (OPMD) is an increasingly recognized autosomal dominant, late onset neuromuscular disorder primarily characterized by ptosis, pharyngeal muscle weakness and proximal skeletal muscle weakness (4). It is most prevalent in the French-Canadian population from Quebec; however, in recent years there has been an unexpected large OPMD population of Hispanic offspring identified in New Mexico (5).
We report a rare case in which OPMD complicated mechanical ventilation management in a 54-year-old Hispanic gentleman admitted to the ICU for respiratory failure secondary to alcohol withdrawal seizures. To our knowledge, this is the first case report of OPMD in the Hispanic population resulting in tracheostomy placement. The first case report of OPMD complicating airway management was reported by Christopher et al. (4) in an 83-year-old male of French-Canadian descent after elective surgery.
Despite frequent and aggressive airway clearance therapy, OPMD resulted in re-intubation, tracheostomy placement, aspiration, extensive neurological workup and a prolonged hospitalization course in our patient. Early recognition and tracheostomy should be advocated in such patients to prevent these complications.
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