Case Report
3Tracheobronchopathia Osteochondroplastica in a Patient with Leukoplakia
Dhillon KV and Akst ML*
Department of Otolaryngology-Division of Laryngology, Johns Hopkins University, USA
*Corresponding author: Lee M Akst, Department of Otolaryngology-Division of Laryngology, Johns Hopkins University, 601 N Caroline Street 6th floor, Baltimore, MD 2123, USA
Published: 11 Jul, 2016
Cite this article as: Dhillon KV, Akst ML. Tracheobronchopathia Osteochondroplastica in a Patient with Leukoplakia. Clin Surg. 2016; 1: 1071.
Abstract
Objectives: We present a case of Tracheo Bronchopathia Osteo Chondroplastica (TBOC) found incidentally in a patient being managed for recurrent vocal fold leukoplakia and dysplasia.
Tracheobronchopathia osteochondroplastica is a rare disease of the airway
Methods: Single case report
Results: We review the clinical manifestations, diagnosis, pathophysiology and treatment for our patient with this rare disease.
Conclusion: Tracheobronchopathia Osteochondroplastica is a rare benign disease of the airway
which usually presents incidentally with no evidence of symptom. It is important to consider this
disease on the differential of patients with classic airway findings.
Introduction
Tracheo Bronchopathia Osteo Chondroplastica (TBOC) is a rare benign disease of the
tracheobronchial tree that is characterized by multiple submucosal osseous and cartilaginous
nodules along the entire length of the trachea. Histopathology has demonstrated these lesions as
calcium phosphate deposits that form islands underneath the mucosa of the tracheal lumen. The
overall incidence of this disease varies as reported in the literature—on average 1:125 to 1:6000
cases during bronchoscopy [1]. The presentation is often asymptomatic or may instead include nonspecific
respiratory complaints, with cough and dyspnea the most common. The etiology of TBOC
is unclear, but it is considered secondary to chronic airway inflammation. The diagnosis is made on
bronchoscopic findings and at times, CT imaging of the chest, with little role in histopathology to
confirm findings other than to exclude other pathologies. Treatment is symptomatic, ranging from
cough suppression to excision and dilation depending on severity of the airway compromise [2,3].
In this report, we present a case of tracheobronchopathia osteochondroplastica found
incidentally in a patient being managed for recurrent vocal fold leukoplakia and dysplasia. We
review the clinical manifestations, diagnosis, pathophysiology and treatment for our patient with
this rare disease.
Case Presentation
A 77 year-old male presented with dysphonia and history of prior outside surgery for
leukoplakia, with outside pathologic diagnosis of dysplasia. At time of presentation to our clinic,
transoral stroboscopy revealed scar of the right vocal fold consistent with his prior surgery and
persistent/recurrent leukoplakia. The patient had no complaints of dyspnea, cough or inspiratory
stridor. With persistent disease and desire to re-establish pathologic diagnosis, the patient was taken
to the operating room for suspension microlaryngoscopy and microflap excision of diseased vocal
fold epithelium. During microlaryngoscopy, views of the anterior subglottis demonstrated a small
nodular submucosal lesion along the anterior tracheal wall just beneath the cricoid cartilage. This
anterior tracheal lesion was biopsied during the procedure. While the vocal fold leukoplakia was
found on histopathology to be dysplasia without evidence of invasion, the final pathology of the
proximal tracheal lesion was chronic inflammation.
Serial follow-up for the patient’s vocal fold dysplasia was recommended, and approximately three
years after surgery, recurrence of the vocal fold leukoplakia was noted. The patient elected in-office
KTP laser treatment of his lesion, and with benefit of laryngeal anesthesia, tracheobronchoscopy
was done with a flexible scope at the time of the procedure; of note, all prior exams in the office
had been done with a rigid scope for magnified, brilliantly illuminated visualization of his vocal
folds, and this was the first time a flexible scope had been used for
this patient in this practice. Findings of tracheobronchoscopy (Figure
1) were characteristic for TBOC. The patient never endorsed any
airway symptoms, cough, hemoptysis, pneumonia etc, and therefore
no further treatment was indicated. To the present day, the patient
continues to be monitored for his vocal cord leukoplakia with no
evidence of disease and no evidence of any airway complaints.
Figure 1
Discussion
The existing knowledge about the rare entity of TBOC is largely
from case reports and case series, with the first report of TBOC
based on autopsy findings. The condition was described by Wilks
in 1857 as ossific deposits in the anterior portion of the trachea [4].
In 1896, Von Schroetter became the first to use a laryngeal mirror
to document these lesions on a living patient [5]. Shortly thereafter,
Killian described these lesions using bronchoscopy in 1899 [2]. In
1910, Ascoff was credited for coining the name tracheobronchopathia
osteochondroplastica [4].
The largest case study described is by Leske et al with 41 patients
[6]. Most other case series are smaller, between one and three patients,
all with variable presentations.
Overall the disease entity is rare, but its relevance is important in
the laryngology field with respect to presentation and evaluation on
bronchoscopy.
The best approximation of incidence of TBOC is cited as 11 per
10,000 cases [7]. There is no difference in distribution between males
and females, and family history does not appear to play role. The
average age of diagnosis is between the fourth and seventh decade
[2]. It is a benign disease, with most cases incidentally found on
bronchoscopy or autopsy. Patients are normally asymptomatic on
diagnosis, and it is thought that those who become symptomatic
may have acquired a superimposed infection or progressive airway
stenosis secondary to the lesions. In turn, they present with nonspecific
complaints, the most common ones cited being cough and
dyspnea with exertion [7].
The etiology of TBOC is unknown. Theories have been
formulated regarding the pathophysiology of the disease. These have
included chondrosis and exostosis of the tracheal cartilaginous rings
or metaplasia of the submucosal stroma [7]. Associations with other
diseases such as atrophic rhinitis and neoplastic processes have been
reported, but there is no science to consistently demonstrate this
relationship [2,7].
The histopathology of these lesions demonstrates calcium and
phosphate deposits within small islands under the tracheal mucosa.
More recently, factors such as bone morphogenetic protein 2 and
transforming growth factor beta 1 have been implicated in potentially
playing a role in formation of new bone and cartilage [5]. These calcium
and bone deposits are in direct contact with the natural C-shaped
anatomical structure of the tracheal rings - and therefore TBOC
deposits are limited to the anterior and lateral walls of the trachea.
The posterior wall of the trachea is membranous and is therefore
spared [7]. This is an important trait in narrowing the differential
diagnosis of tracheal lesions, and posterior wall involvement of the
trachea increases suspicion for other disease pathologies including
amyloidosis, sarcoidosis, polychondritis and malignancies such
as lung cancer [7,8]. The role of biopsy is to confirm diagnosis and
evaluate for associated conditions. Bronchoscopy is considered the
gold standard for the diagnosis of TBOC.
Visualization of the bony or cartilaginous nodules on the tracheal
walls is diagnostic of TBOC, more commonly found in the distal 2/3
of the trachea [8]. Some case studies have discussed involvement of
the proximal trachea including subglottis and larynx [8], in which
laryngoscopy may be sufficient to establish a diagnosis.
Though laryngoscopy should be pathognomonic for the
condition, its rarity means that many bronchoscopists are unfamiliar
with its appearance, and biopsy is often performed [9].
The role of imaging in diagnosis is uncertain. Jabbardarjani
et al. [3] demonstrated the low yield of chest X-ray in detection of
tracheal lesions. Computed Tomography (CT) of the chest plays a role
in demonstrating the sessile submucosal nodules along the anterior
and lateral tracheal cartilaginous rings, when present. Such findings
however may not be universally present [3]. Other ancillary tests such
as pulmonary function tests and laboratory tests such as CRP and
ESR are non-specific [1].
There is no specific treatment for TBOC with most treatment
reserved for patients presenting with severe airway obstruction
presenting with debilitating symptoms. A recent case series
demonstrated all symptomatic patients (n=10) underwent removal
of nodules with the use of Nd:YAG laser [3]. The other options
include surgical resection, mechanical debulking with the use of rigid
bronchoscope, stent placement, and medial management including
inhaled steroids, antibiotics and expectorants [1-3,8]. Medical
therapies are considered palliative or for symptomatic treatment
only. There is a relatively good prognosis for these patients, with slow
progression of the disease. Leske et al. [6] reported that over 55% of
the patients do not show any disease progressive following diagnosis.
Treatment modalities should be implemented on a case-by-case basis
pending the severity of the disease.
Our case presentation is an incidental finding of TBOC on
bronchoscopy in a patient that was otherwise asymptomatic from an
airway standpoint. Our goals at this time are continued surveillance
of these lesions, as there is evidence for progression of disease as
documented in other case reports. Given the rarity of this disease,
it is important to consider those who have been undiagnosed.
Tracheobronchopathia osteochondroplastica is a disease that should
remain on the differential when there are diffuse lesions along
the tracheal wall. Treatment is done on a case-by-case basis, but
continued surveillance of findings and symptoms is the mainstay in
management for the majority of these patients.
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