Tracheobronchopathia osteochondroplastica: kömür işleme işi ve astma ile ilişkili bir olgu

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Introduction
Tracheobronchopathia osteochondroplastica (TO)
is a rare disease of unknown etiology, which was first
described by Wilks in 1857. TO is seen with a frequency of 0.4 percent at bronchoscopy (1-6). The disease is characterized by cartilaginous or bony nodular
projections into the tracheobronchial lumen, with
sparing of the posterior membranous portion of the
tracheobronchial tree. The nodules may be either focal or diffuse. There is a 3:1 male predilection, and
the disease typically manifests in patients in their
mid-50s. However, recently a 9-year-old girl with TO
has been reported (7). The diagnosis is usually established years after the initiation of the process because
of the chronic and asymptomatic nature of the condition. Most patients are asymptomatic, but presentation may include persistent cough, hoarseness, dyspnea at exertion, recurrent infection, wheezing and
hemoptysis. The last symptom occurs when opposing nodules rub against each other, causing erosion
of the mucosa and subsequent bleeding (1). Although
most patients have a favorable prognosis, respiratory
insufficiency may complicate (3,8). Diagnosis may be
difficult with a standard chest radiography because
of poor imaging technique and an inadequate search.
Computed tomography (CT) improves both the detection and characterization of central airway disease.
Bronchoscopy, however, remains the primary procedure for the diagnostic work-up of this disease entity.
There is currently no specific treatment to remove the
abnormal tissue growth or prevent the development
of new nodules. Linear tracheoplasty may be required
in patients with symptomatic airway obstruction (9).
Case Report
A 41-year-old male patient admitted with the complaint of chronic cough. He had never smoked. His
complaint had lasted for 5 years, beginning after a
10-year period of working at a coal processing facil-
* Department of Thoracic Diseases, Gülhane Military Medical Faculty
Haydarpaşa Teaching Hospital
** Department of Thoracic Surgery, Gülhane Military Medical Faculty
Haydarpaşa Teaching Hospital
*** Department of Pathology, Gülhane Military Medical Faculty Haydarpaşa
Teaching Hospital
This case was presented as a poster presentation at the 15th World Bronchology Congress
Reprint request: Dr. Erdoğan Kunter, Department of Thoracic Diseases,
Gülhane Military Medical Faculty Haydarpaşa Teaching Hospital, Acıbadem
Cad. Üsküdar-34660, Istanbul, Turkey
E-mail: erkunter@hotmail.com
Date submitted: April 17, 2008 • Date accepted: August 10, 2008Volume 51 • Issue 3 Tracheobronchopathia osteochondroplastica • 193
ity with remissions and exacerbations but becoming more persistent over time. He had changed his
work with the hope that he would feel better. He had
been treated with the diagnoses of acute bronchitis
and/or asthma for several occasions and numerous
antitussive prescriptions were given as well. Current
and previous posteroanterior chest radiographs were
not suggesting any abnormality. Minimal and scattered rhonchi were heard at chest examination while
other organ systems were normal at physical examination. His medical history was unremarkable except
that his complaint of coughing worsened when exposed to cigarette smoke, perfumes and some other
odors. His routine biochemical and hematologic
tests were within normal limits and he was not on
any medication at presentation. Pulmonary function tests including carbon monoxide diffusion test
were within normal limits but the early reversibility
test with inhaled salbutamol was positive. He had a
normal peripheral eosinophil count and slightly elevated serum total IgE level (140 IU/mL) but negative
prick test with common antigens including common
aeroallergens. The patient was put on inhaled long
acting beta-2 agonist plus corticosteroid (formoterol
and budesonide) along with n-acetyl cysteine as a
mucolytic agent for 2 weeks. Inappropriate and insufficient clinical response, i.e. persistent coughing
despite improved auscultation findings, to this treatment leaded us to investigate further, and a chest CT
was performed, which demonstrated irregular calcification and projections from the tracheal wall, sparing the posterior wall, into the lumen and bilateral
minimal cylindrical bronchiectatic changes (Figure
1). Although this appearance was strongly suggesting TO, bronchoscopy was performed to confirm the
diagnosis and investigate any coexisting condition.
With bronchoscopy multiple nodules with varying
dimensions (3-5 mm) were distributed over the anterolateral walls of the trachea. The lower two-third of
the trachea and the proximal portions of the primary
bronchi seemed to be the most affected sites. The
number, dimension and frequency of the nodules
were decreasing while going down through the main
bronchi (Figure 2). These lesions were difficult to
make a biopsy. Biopsy samples from the lesions and
adjacent tissues and bronchial lavage samples were
examined histopathologically and microbiologically.
Disseminated submucosal ossifications were the only
abnormality found with bronchoscopic procedures.
Taking together the CT, bronchoscopy and biopsy
findings, the diagnosis of TO was established.
Figure 2. Bronchoscopic image of the lower trachea
Figure 1. Computed tomographic image of the affected trachea
Discussion
Although TO has a pathognomonic CT image,
sometimes the radiological appearance may mimic
other entities such as tracheobronchial amyloidosis,
papillomatosis, rhinoscleroma, relapsing polychondritis, etc. Radiologically distinguishing tracheobronchial amyloidosis and papillomatosis from TO might
be even more difficult. The classic radiographic appearance of tracheobronchial amyloidosis is nodular
and irregular narrowing of the tracheal lumen. Lobar
or segmental collapse may be seen with endobronchial obstruction due to amyloid deposition. In certain cases of diffuse involvement, there is a significant component of calcification and ossification of
the lesions. In such circumstances, differentiation is
made on the basis of posterior membrane sparing in 194 • September 2009 • Gulhane Med J Kunter et al.
TO. On the other hand, papillomatosis results from
infection of the upper respiratory tract by the human
papillomavirus and may show malignant degeneration. Multifocal infection or aspiration of infected
tissue from laryngeal papillomas distally may lead
to infection of the trachea, the bronchi or even the
alveoli. Radiographic findings include nodular narrowing of the airway that may be either focal or diffuse. TO has also been reported as a focal disease but
in papillomatosis white and polypoid appearance at
bronchoscopy and lack of ossification may suffice to
diagnose (1,10). However, malignancies, including
lung cancer, have been reported to be associated with
TO and needs to be excluded (5,11,12).
The cause of the condition is unknown but several
theories, including exposure to inhaled noxious substances have been postulated. One of the patients of
Ashley has had pneumoconiosis and there are other
published cases of TO, in whom silicosis has been
accused for the development of the disease (11,13-
15). Our patient was exposed to coal dust but radiologically and clinically there was no evidence of
pneumoconiosis.
In TO, spirometry frequently shows a mild obstructive pattern, but in spite of marked radiographic changes, patients are only rarely symptomatic
since severe airway obstruction is unusual (6,16,17).
However, to our knowledge there is only one previous case report of TO that the patient meets the criteria of bronchial asthma as a coexisting disease (16).
TO is a rare condition but considering that most
of the cases stay asymptomatic for a long period, the
true prevalence of the disease might be higher than
the reported numbers. Clinicians should include this
disease in the list of differential diagnoses when confronted with symptoms like persistent and often productive cough, hemoptysis, dyspnea and wheeze. We
think that thorax CT findings are very helpful to establish a diagnosis, but bronchoscopic evaluation will
enable the clinician to prove it and look for the coexisting situations like malignancies. We hope that our
case of TO associated with bronchial asthma and coal
dust exposure contributes to future investigations,
since in diseases of unknown etiology, describing the
associated conditions may help to define the etiology
and develop prevention and treatment strategies.