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(Chest. 2001;120:2101-2103.)
© 2001 American College of Chest Physicians

Oculopharyngeal Muscular Dystrophy Complicating Airway Management*

Kenneth Christopher, MD; Clare Horkan, MB; Robert B. Patterson, MD and Paul C. Yodice, MD, FCCP

* From the Division of Critical Care Medicine (Drs. Christopher and Yodice) and Department of Vascular Surgery (Dr. Patterson), the Miriam Hospital, Brown University School of Medicine, Providence, RI; and Department of Neurology (Dr. Horkan), Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA.

Correspondence to: Kenneth Christopher, MD, Division of Critical Care Medicine, The Miriam Hospital Brown University, 164 Summit Ave, Providence, RI 02906; e-mail: Kenneth_Christopher_MD{at}Brown.edu

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.

Key Words: dysphagia • mechanical ventilation • oculopharyngeal muscular dystrophy




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D. L. Dedrick and L. K. Brown
Obstructive Sleep Apnea Syndrome Complicating Oculopharyngeal Muscular Dystrophy
Chest, January 1, 2004; 125(1): 334 - 336.
[Abstract] [Full Text] [PDF]


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