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Communications to the Editor |

Tracheobronchomalacia and Noninvasive Ventilation Revisited FREE TO VIEW

Ahmet Baydur, MD, FCCP; Gary Kanel, MD; Michael Koss, MD
Author and Funding Information

Keck School of Medicine, University of Southern California, Los Angeles, CA

Correspondence to: Ahmet Baydur, MD, FCCP, 2025 Zonal Ave, GNH 11900, Los Angeles, CA 90033; e-mail: baydur@hsc.usc.edu



Chest. 2004;126(4):1390-1392. doi:10.1378/chest.126.4.1390-a
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To the Editor:

We appreciate the comments by Dr. Bach (November 2003)1regarding our report on autopsy-demonstrated cases of tracheobronchomalacia in seven patients with Duchenne muscular dystrophy (DMD) receiving positive-pressure ventilation through uncuffed tracheostomies.2 Two of these patients also experienced tracheal perforations, one of whom died from a massive tracheoarterial hemorrhage. We fully agree with Dr. Bach that tracheostomies directly led to the erosion through the airway in two of the five patients. The purpose of our report was not to dissuade clinicians from initiating noninvasive positive pressure ventilation (NIPPV) as the first choice of ventilatory support in such patients, but to report a previously unreported finding in patients with DMD receiving long-term ventilation with uncuffed tracheostomies, namely tracheobronchomalacia. At the same time, there are a number of points we would like to emphasize that Dr. Bach has omitted in his correspondence.

As noted in the table, at least four of the five patients who underwent autopsy exhibited grossly visible generalized tracheobronchomalacia, far distal to the point of direct contact between the tube opening and trachea. In addition, careful histologic re-examination of the lung sections by two pathologists (G.K. and M.K.) confirmed dilatation of small bronchi in at least one of the patients when compared to equivalent airways in otherwise healthy control lung sections. Furthermore, intramural cartilage was noted to be markedly atrophic (Fig 1 ) or to have completely disappeared in airways extending two to three generations distal to the trachea in three of the patients. This finding must be accounted for by factors other than flow turbulence, which usually occurs in the trachea3and, as pointed out by Dr. Bach, is augmented by the presence of an abrupt change in cross-sectional area at the junction of the tracheostomy and trachea.4 It is for this reason that we think that any positive-pressure ventilation, whether delivered by tracheostomy (even uncuffed) or oral/nasal interface, could also lead to distal bronchomalacia, a finding that can only be verified by direct examination of lung tissue. Without surgical tissue or autopsy evidence to the contrary, Dr. Bach cannot argue that none of his patients who received NIPPV had tracheobronchomalacia.

While it is now universally accepted that NIPPV is the preferred means of long-term respiratory support in patients with neuromuscular disease, one must remember that this modality has been increasingly available only since the mid-1980s. A review of our article shows that our patients with DMD began receiving tracheostomy-assisted positive pressure ventilation as far back as 1958, long before NIPPV was reintroduced as an alternative to tracheostomy-assisted ventilation. Most of Dr. Bach’s descriptions of patients successfully initiating or weaning on to noninvasive ventilation occurred after 1985.5 Furthermore, as Dr. Bach himself has reported, trials of mouthpiece intermittent positive pressure ventilation to switch patients with DMD off tracheostomy-assisted ventilation are sometimes unsuccessful because of the inability to grab the mouthpiece.5 Under these circumstances, a life span of up to 30 years on ventilation for patients with DMD, even by tracheostomy, is a remarkable achievement, and attests to the superb care these patients received while residing in a chronic-care/rehabilitation facility. Respiratory care, incidentally, included all the cough-assist modalities Dr. Bach advocates,5with the exception of the in-exsufflation device, which was reintroduced in the early 1990s. We have separately described our experience with patients with DMD (as well as other musculoskeletal disorders) managed with long-term NIPPV and other respiratory aids elsewhere.6Even Dr. Bach has reported that some patients with neuromuscular disease and unmeasurable vital capacities eventually require or prefer tracheostomies despite the use of cough-assist measures,78 as others have reported.9

In connection with these different outcomes, two of our patients died as a direct result of cardiomyopathy (confirmed at autopsy), and two others had autopsy evidence of cardiomyopathy at the time of their deaths. Thus, not all patients died of respiratory complications, as Dr. Bach implies.

It is now clearly established that oral/nasal interfaces have their own inherent problems,5,9including abdominal distention,10 which sometimes preclude patient acceptance. Ultimately, only a prospective, randomized controlled trial (RCT) comparing NIPPV to tracheostomy-assisted ventilation in patients with neuromuscular disease would help resolve the question of which modality results not only in prolongation of life, but also in preservation of quality of life and in the overall reduction of complications and hospitalizations. Because of potential logistical and ethical issues, however, it may not be possible to conduct such an RCT. Given the current evidence available, there is no doubt that NIPPV should be the modality initially attempted in patients with neuromuscular disorders, but the clinician has to rely on care decisions mutually arrived at with the patient and family, often depending on available resources and patient and family acceptance.7,11

Figure 1.Microscopic section of lung of patient 2 showing dilated bronchus and atrophic cartilage (center of photomicrograph).2 Focal areas of inflammation surround the airway (hematoxylin-eosin, original × 100).

Grahic Jump LocationImage not available.

Figure Jump LinkFigure 1. Microscopic section of lung of patient 2 showing dilated bronchus and atrophic cartilage (center of photomicrograph).2 Focal areas of inflammation surround the airway (hematoxylin-eosin, original × 100).Grahic Jump Location
Bach, JR (2003) Tracheobronchomalacia and noninvasive ventilation [letter]?.Chest124,2038. [CrossRef]
 
Baydur, A, Kanel, G Tracheobronchomalacia and tracheal hemorrhage in patients with Duchenne muscular dystrophy receiving long-term ventilation with uncuffed tracheostomies.Chest2003;123,1307-1311. [CrossRef]
 
Dubois, AR Resistance to breathing. Fenn, WO Hahn, H eds.Respiration: I. Handbook of physiology1964,451-462 American Physiological Society. Washington, DC:
 
Chang, HK, Mortola, JP Fluid dynamic factors in tracheal pressure measurement.J Appl Physiol: Respir Environ Exerc Physiol1981;51,218-225
 
Bach, JR. Noninvasive mechanical ventilation. 2002; Hanley and Belfus. Philadelphia, PA:.
 
Baydur, A, Layne, E, Aral, H, et al Long term non-invasive ventilation in the community for patients with musculoskeletal disorders: 46 year experience and review.Thorax2000;55,4-11. [CrossRef]
 
Bach, JR A comparison of long-term ventilatory support alternatives from the perspective of the patient and care giver.Chest1993;104,1702-1706. [CrossRef]
 
Bach, JR, O’Brien, J, Krotenberg, R, et al Management of end stage respiratory failure in Duchenne muscular dystrophy.Muscle Nerve1987;10,177-182. [CrossRef]
 
Meyer, TJ, Hill, NS Noninvasive positive pressure ventilation to treat respiratory failure.Ann Intern Med1974;120,760-770
 
Leger, P, Bedicam, JM, Cornette, A, et al Nasal intermittent positive pressure ventilation: long-term follow-up in patients with severe chronic respiratory insufficiency.Chest1994;105,100-105. [CrossRef]
 
Gay, PC, Patel, AM, Viggiano, RW, et al Nocturnal nasalventilation for treatment of patients with hypercapnic respiratory failure.Mayo Clin Proc1991;66,695-703. [CrossRef]
 

Figures

Figure Jump LinkFigure 1. Microscopic section of lung of patient 2 showing dilated bronchus and atrophic cartilage (center of photomicrograph).2 Focal areas of inflammation surround the airway (hematoxylin-eosin, original × 100).Grahic Jump Location

Tables

References

Bach, JR (2003) Tracheobronchomalacia and noninvasive ventilation [letter]?.Chest124,2038. [CrossRef]
 
Baydur, A, Kanel, G Tracheobronchomalacia and tracheal hemorrhage in patients with Duchenne muscular dystrophy receiving long-term ventilation with uncuffed tracheostomies.Chest2003;123,1307-1311. [CrossRef]
 
Dubois, AR Resistance to breathing. Fenn, WO Hahn, H eds.Respiration: I. Handbook of physiology1964,451-462 American Physiological Society. Washington, DC:
 
Chang, HK, Mortola, JP Fluid dynamic factors in tracheal pressure measurement.J Appl Physiol: Respir Environ Exerc Physiol1981;51,218-225
 
Bach, JR. Noninvasive mechanical ventilation. 2002; Hanley and Belfus. Philadelphia, PA:.
 
Baydur, A, Layne, E, Aral, H, et al Long term non-invasive ventilation in the community for patients with musculoskeletal disorders: 46 year experience and review.Thorax2000;55,4-11. [CrossRef]
 
Bach, JR A comparison of long-term ventilatory support alternatives from the perspective of the patient and care giver.Chest1993;104,1702-1706. [CrossRef]
 
Bach, JR, O’Brien, J, Krotenberg, R, et al Management of end stage respiratory failure in Duchenne muscular dystrophy.Muscle Nerve1987;10,177-182. [CrossRef]
 
Meyer, TJ, Hill, NS Noninvasive positive pressure ventilation to treat respiratory failure.Ann Intern Med1974;120,760-770
 
Leger, P, Bedicam, JM, Cornette, A, et al Nasal intermittent positive pressure ventilation: long-term follow-up in patients with severe chronic respiratory insufficiency.Chest1994;105,100-105. [CrossRef]
 
Gay, PC, Patel, AM, Viggiano, RW, et al Nocturnal nasalventilation for treatment of patients with hypercapnic respiratory failure.Mayo Clin Proc1991;66,695-703. [CrossRef]
 
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