Chest
Volume 123, Issue 4, April 2003, Pages 1307-1311
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Selected Reports
Tracheobronchomalacia and Tracheal Hemorrhage in Patients With Duchenne Muscular Dystrophy Receiving Long-term Ventilation With Uncuffed Tracheostomies

https://doi.org/10.1378/chest.123.4.1307Get rights and content

Study objectives:

Autopsy evaluation of tracheobronchomalacia (TBM) in patients with Duchenne muscular dystrophy (DMD) who were receiving long-term ventilation through uncuffed tracheostomies.

Design:

Necropsies were performed in seven patients with DMD who had received positive-pressure ventilation through uncuffed tracheostomies for a duration of 5 to 30 years.

Setting:

Rehabilitation facility affiliated with a university medical center.

Results:

The range of peak airway pressures sustained during ventilation by all the patients was 23 mm Hg to 36 mm Hg. Bronchoscopy (which was performed in four of the five patients) detected tracheomalacia in only one of the patients. Five of the seven patients demonstrated variable degrees of airway malacia. Two patients also had tracheal perforations, one of which resulted in a fatal hemorrhage from a tracheovascular fistula.

Conclusions:

Given enough time, patients receiving positive-pressure ventilation can develop airway thinning and dilation even without the use of an inflated tracheostomy cuff. There is also a potential for tracheal erosion into an adjacent artery that can lead to fatal hemorrhage. Such findings also have implications for individuals receiving noninvasive positive-pressure ventilation, who could develop TBM as a result of the continuous cycling pressures on the airway wall.

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.

References (13)

There are more references available in the full text version of this article.

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