(Chest. 2001;120:1018-1020.)
© 2001
American College of Chest Physicians
A 38-Year-Old Man With Tracheomegaly, Tracheal Diverticulosis, and Bronchiectasis*
Luiz Claudio Lazzarini-de-Oliveira, MD;
Carlos Alberto Costa de Barros Franco, MD;
Cristiane Linhares Gomes de Salles, MD and
Amarino C. de Oliveira, Jr, MD
*
From the Pulmonary Department (Drs. Lazzarini-de-Oliveira, Barros Franco, and Salles), Clementino Fraga Filho University Hospital, Federal University of Rio de Janeiro; and Radiology Department (Dr. Oliveira), Pro-Cardíaco Hospital, Rio de Janeiro, Brazil.
Correspondence to: Luiz Claudio Lazzarini-de-Oliveira, MD, Hospital Universitário Clementino Fraga Filho, Av. Brigadeiro Trompowski s/n°, Ilha do Fundão, Rio de Janeiro, Brazil CEP 21941–590; e-mail: lazzarini{at}openlink.com.br
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Introduction
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A
38-year-old man was referred to our University Hospital for
evaluation of chronic cough with sputum production. During the last 15
years, he had experienced an increased expectoration of mucoid sputum
that became purulent during infectious exacerbations, sometimes with
bloody sputum. He denied fever, wheezes, chest pain, dyspnea, or weight
loss. No other respiratory illness was present. His parents and
siblings were normal. He works as a security officer. He has never
smoked and denies regular alcohol intake.
Physical examination revealed a thin but well-nourished patient.
Vital signs were normal. Clinical respiratory examination disclosed
decreased breath sounds over both lungs and inspiratory crackles at the
lower third of both lung fields. Finger clubbing was present. The
results of blood analysis were within normal limits. A chest radiograph
and helical CT scan (Fig 1
, 2
) were performed, showing tracheomegaly with transversal diameters of
the trachea and right-main bronchus of 36 mm and 26 mm,
respectively. Many diverticular outpouchings were present from the
trachea to the main bronchi. There was also cystic bronchiectasis
involving both lower lobes (Fig 3
).
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Figure 1.. Chest CT scan in coronal plane. Note the
presence of tracheomegaly and bronchomegaly. Some of the tracheal
diverticula are identified surrounding the trachea and main bronchus.
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A bronchoscopy was performed and disclosed several openings in
the posterior and lateral wall of trachea and main bronchus; some of
the openings could be easily penetrated by the tip of bronchoscope. In
these openings, we could identify a collection of great amounts of
secretion. The rest of the examination was normal. Sputum and tracheal
aspirate results were negative for mycobacteria.
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What is the diagnosis?
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Diagnosis: Mounier-Kuhn syndrome
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Mounier-Kuhn syndrome is a rare disorder characterized by
marked dilatation of the trachea and main bronchi, sometimes with
tracheal diverticulosis, bronchiectasis, and recurrent lower
respiratory tract infection.1
2
The clinical and
endoscopic features of tracheobronchomegaly were described by
Mounier-Kuhn in 1932.3
The etiology is uncertain. Autopsy
studies suggest a congenital defect or atrophy of the elastic and
smooth muscle tissue of the trachea and main bronchi.4
5
Because of this weakened trachea, some patients also develop mucosal
herniations between the tracheal rings, leading to tracheal
diverticulosis and retention of secretions in them. The airways distal
to the fourth-order and fifth-order division are usually normal in
diameter. A familial form has been described with a possible recessive
inheritance6
and acquired forms as a complication of
pulmonary fibrosis in adults7
and of mechanical
ventilation in preterm neonates.8
Secondary
tracheobronchomegaly was also described in association with
Ehlers-Danlos syndrome, Marfan syndrome, Kenny-Caffey syndrome,
Brachmann-de Lange syndrome, connective tissue diseases,
ataxia-telangiectasia, Bruton-type agammaglobulinemia, ankylosing
spondylitis, cutis laxa, and light chain deposition
disease.9
10
11
Nevertheless, the majority of cases
appear to be sporadic. The disease predominantly occurs in men in their
third and fourth decades of life.1
The symptoms of tracheobronchomegaly are nonspecific, with sputum
production secondary to bronchiectasis and lower respiratory tract
infection. The grossly enlarged but weakened airways and inefficient
cough mechanisms block mucociliary clearance leading to mucus retention
with resultant recurrent pneumonia, bronchiectasis, and fibrosis.
Excessive sputum production with occasional hemoptysis occurs and
patients may develop dyspnea and respiratory failure as the lungs
become progressively damaged. In addition, spontaneous pneumothorax,
hemoptysis, pneumonia, and finger clubbing may develop.10
On a plain chest radiograph, the increased caliber of the central
airways may be visible. This is usually best seen in the lateral
projection. For an adult, any diameter of the trachea, right main
bronchus, and left main bronchus that exceeds 3.0 cm, 2.4 cm, and 2.3
cm, respectively, on a standard chest radiograph or bronchogram is
diagnostic of tracheobronchomegaly because these are the upper limits
of the means plus three standard deviations.12
For chest
CT, these values are 3.0, 2.0, and 1.8, respectively.9
Tracheal diverticulosis is seen in approximately one third of patients
and most commonly originate from the right posterolateral
wall.11
The experience with MRI is still limited to one
case report in the literature.13
Pulmonary function tests
may reveal an obstructive pattern and increased residual volume.
Treatment is limited to physiotherapy to assist in clearing secretions
and appropriate antibiotics during infectious
exacerbations.10
14
There is no role for surgery because
of the diffuse nature of the disease. There is one case report of
tracheal stenosis requiring surgical correction secondary to tracheal
intubation for 15 days with a high-volume, low-pressure cuff. It is
recommended that Mounier-Kuhn syndrome patients who require mechanical
intubation should use an uncuffed tube.15
Tracheal
stenting has been shown to be useful in advanced cases.16
Received for publication February 16, 2000.
Accepted for publication September 28, 2000.
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References
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Bateson, EM, Woo-Ming, M (1973) Tracheobronchomegaly. Clin Radiol 24,354-358[CrossRef][Medline]
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Gay, S, Dee, P (1984) Tracheobronchomegaly. Br J Radiol 57,640-644[ISI][Medline]
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Mounier-Kuhn, P (1932) Dilatation de la trachée: constatations, radiographiques et bronchoscopies. Lyon Med 150,106-109
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Katz, I, Levine, M, Hermam, P (1962) Tracheobronchomegaly (Mounier-Kuhn Syndrome): CT diagnosis. AJR Am J Roentgenol 88,1084-1094
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Spencer, H (1985) Congenital abnormalities of the lung: congenital tracheobronchomegaly Spencer, H eds. Pathology of the lung 4th ed. ,129-130 Pergamon Press Oxford, UK.
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Johnson, RF, Green, RA (1965) Tracheobronchomegaly: report of five cases and demonstration of familial occurrence. Am Rev Respir Dis 91,35-50[ISI][Medline]
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Woodring, JH, Barrett, PA, Rehm, SR, et al (1989) Acquired tracheomegaly in adults as a complication of diffuse pulmonary fibrosis. Am J Roentgenol 152,743-747[Abstract/Free Full Text]
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Bhutani, VK, Ritchie, WG, Schaffer, TH (1986) Acquired tracheomegaly in very preterm neonates. Am J Dis Child 140,449-452[Abstract]
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Blake, MA, Chaoui, AS, Barish, MA (1999) Thoracic case of the day: Mounier-Kuhn syndrome (tracheobronchomegaly). Am J Roentgenol 173,822,824-825
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Schoor, JV, Joos, G, Pauwels, R (1991) Tracheobronchomegaly: the Mounier-Kuhn syndrome; report of two cases and review of the literature. Eur Respir J 4,1303-1306[Abstract]
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Sane, AC, Effmann, EL, Brown, SD (1992) Tracheobronchiomegaly: the Mounier-Kuhn syndrome in a patient with the Kenny-Caffey syndrome. Chest 102,618-619[Abstract/Free Full Text]
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Katz, I, Levine, M, Hermam, P (1962) Tracheobronchomegaly: the Mounier-Kuhn Syndrome. Am J Roentgenol Radiother Nucl Med 88,1084-1094
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Rindsberg, S, Friedman, AC, Fiel, SB, et al (1987) MRI in tracheobronchomegaly. J Can Assoc Radiol 38,126-128
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Guest, JL, Anderson, JN (1977) Tracheobronchomegaly (Mounier-Kuhn Syndrome). JAMA 238,1754-1755[CrossRef][ISI][Medline]
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Messahel, FM (1989) Tracheal dilatation followed by stenosis in Mounier-Kuhn Syndrome. Anesthesia 44,227-229[ISI][Medline]
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Pilavaki, M (1995) Tracheobronchomegaly (Mounier-Kuhn Syndrome) roentgen findings and tracheal stent instrumentation. Pneumologie 49,556-558[Medline]
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Introduction
What is the diagnosis?
