Chest
Selected ReportsTracheobronchomalacia and Tracheal Hemorrhage in Patients With Duchenne Muscular Dystrophy Receiving Long-term Ventilation With Uncuffed Tracheostomies
Section snippets
Materials and Methods
Rancho Los Amigos National Rehabilitation Center (RLANRC) is a teaching facility of the University of Southern California that has provided care for patients with neuromuscular and chest wall disorders with respiratory complications. Many of these patients included those with DMD, most of whom lived in the community. A small group of patients with DMD lived for most of their lives at RLANRC because of the unavailability of community resources. Individuals who received respiratory assistance
Results
The adult chest medicine service at RLANRC observed approximately 75 respiratory impaired individuals with myopathies who were ≥ 18 years of age between 1957 and 1995. During this period, approximately 30 ventilator-dependent patients lived at the facility for variable lengths of time because of the unavailability of community resources. The autopsy records were reviewed for seven patients with DMD who died after having lived at the facility for many years. All patients had spinal deformities.
Discussion
The key findings in this clinicopathologic study are: (1) the development of acquired tracheobronchial malacia in some adult patients with DMD after many years of tracheostomy positive-pressure ventilation, (2) the development of TBM despite the use of uncuffed tracheostomies, and (3) the occurrence of fatal hemorrhage in some patients. The severity of the symptoms of TBM depends on the location, length, and severity of the abnormal airway segments.9 Some patients with severe disease respond
ACKNOWLEDGMENT
The authors thank Theresa Behdjet for the preparation of this manuscript.
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