Oculopharyngeal muscular dystrophy complicating airway management.
Oculopharyngeal muscular dystrophy (OPMD) is an uncommon autosomal dominant disorder characterized by late onset and slow progression. Complications of OPMD include ptosis and progressive dysphagia leading to eventual malnutrition and aspiration. We report a rare case of OPMD complicating mechanical ventilator management following emergent surgery. OPMD and the resulting dysphagia contributed to multiple intubations, tracheostomy, aspiration pneumonia, and a prolonged hospital course. Awareness of the possibility of OPMD in intubated patients with a history of dysphagia is crucial for avoidance of complications.
Christopher
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K
Division of Critical Care Medicine, the Miriam Hospital, Brown University School of Medicine, Providence, RI 02906, USA. Kenneth_Christopher_MD@Brown.edu
Horkan
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C
Patterson
R B
RB
Yodice
P C
PC
D000368:Aged; D000369:Aged, 80 and over; D003937:Diagnosis, Differential; D005528:Foot / Q000098:blood supply*; D006801:Humans; D007511:Ischemia / Q000601:surgery*; D008297:Male; D009136:Muscular Dystrophies / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D011015:Pneumonia, Aspiration / Q000175:diagnosis / Q000209:etiology*; D011183:Postoperative Complications / Q000175:diagnosis / Q000209:etiology*; D012121:Respiration, Artificial; D015300:Ventilator Weaning / Q000009:adverse effects