Brazilian Journal of Pulmonology

ISSN (on-line): 1806-3756 | ISSN (printed): 1806-3713

SBPT

Publication continuous and bimonthly

SCImago Journal & Country Rank
Advanced Search

 

Current Issue: 2017 - Volume 43 - Number 2 (March/April)

CASE REPORT

Tracheobronchopathia osteochondroplastica

Traqueobroncopatia osteocondroplástica

 

Mara Graziele Maciel Silveira1; Maria Vera Cruz de Oliveira Castellano2; Clarice Emiko Fuzi2; Ester Nei Aparecida Martins Coletta2; Guilherme Nogueira Spinosa2

 

1. Serviço de Clínica Médica, Hospital do Servidor Público Estadual de São Paulo, São Paulo (SP) Brasil.
2. Serviço de Doenças do Aparelho Respiratório, Hospital do Servidor Público Estadual de São Paulo,
São Paulo (SP) Brasil.
Submitted: 21 September 2016.
Accepted: 18 November 2016.
Study carried out at the Serviço de Doenças do Aparelho Respiratório, Hospital do Servidor Público Estadual de São Paulo, São Paulo (SP) Brasil.

Correspondence to:
Mara Graziele Maciel Silveira. Rua Borges Lagoa, 512, apto. 81B, Vila Clementino, CEP 04038-000, São Paulo, SP, Brasil.
E-mail: mgraziem@gmail.com
Financial support: None.


 

Abstract

Tracheobronchopathia osteochondroplastica is a rare benign disease, of unknown cause, characterized by numerous sessile, cartilaginous, or bony submucosal nodules distributed throughout the anterolateral walls, projecting into the laryngotracheobronchial lumen. In general, tracheobronchopathia osteochondroplastica is diagnosed incidentally during bronchoscopy or autopsy and is not associated with a specific disease. We report the case of a male patient who was diagnosed with tracheobronchopathia osteochondroplastica via bronchoscopy and biopsy.

 

Resumo

A traqueobroncopatia osteocondroplástica é uma doença benigna rara, de causa desconhecida, caracterizada por numerosos nódulos submucosos sésseis, cartilaginosos e/ou ósseos, distribuídos pelas paredes anterolaterais da traqueia, projetando-se no lúmen laringotraqueobrônquico. Em geral, a traqueobroncopatia osteocondroplástica é descoberta acidentalmente durante broncoscopias ou em necropsias e não é associada a uma doença específica. Relatamos o caso de um paciente que foi diagnosticado com traqueobroncopatia osteocondroplástica por broncoscopia e biópsia.

 

 

Keywords: Dyspnea; Tracheal diseases; Bronchoscopy.

 

Palavras-chave: Dispneia; Doenças da traqueia; Broncoscopia.

 

 

INTRODUCTION

Tracheobronchopathia osteochondroplastica (TO) is a rare benign disease, of unknown cause, characterized by numerous sessile, cartilaginous, or bony submucosal nodules distributed throughout the anterolateral walls of the trachea, projecting into the laryngotracheobronchial lumen. TO presents as round or polypoid osteocartilaginous projections covering the corrugated portion of the tracheobronchial mucosal surface and the narrow, rigid area in the respiratory tree.(1-3) The nodular lesions are sessile and calcified and vary from 1 to 10 mm in diameter. They are characterized by slow, progressive growth, being focal or diffuse, covered by metaplastic or normal epithelium, and extend from the perichondrium to the tracheal lumen, along the ring path, with active hematopoietic inclusion in nodular neoformations. TO can cause stenosis of the laryngotracheobronchial lumen, but without involvement of the posterior wall, with the possibility of progression to the main bronchi.(4)

The first case of TO was described by Wilks, as early as in the 19th century, in a 38-year-old male patient with tu-berculosis. Since then, hundreds of cases have been reported worldwide.(5) However, many patients go undiagnosed because of a lack of knowledge about TO on the part of physicians. The etiology and pathogenesis of TO remain unknown. In general, it is diagnosed incidentally during bronchoscopy or autopsy and is not associated with a specif-ic disease. TO predominantly affects males in the fifth to seventh decade of life(1,2) and is usually asymptomatic.(6)

In the present study, we report a case of TO found incidentally on a male patient's CT scan performed to investigate dyspnea.

CASE REPORT

A 59-year-old male patient presented with a 1-month history of dyspnea on severe exertion, associated with a pro-gressive cough and throat clearing. The patient had hepatitis B and chronic atrial fibrillation. He reported being a nonsmoker. His physical examination yielded normal findings. A chest X-ray revealed no significant changes. Pulmo-nary function testing indicated mild obstructive lung disease with a response to bronchodilator use. Echocardiography showed an ejection fraction of 49% and mild, diffuse left ventricular hypocontractility. Chest CT images are shown in Figure 1. At bronchoscopy, multiple granular lesions were seen in the trachea and bronchi (Figure 2). Biopsy re-vealed nodular areas consisting of ossified cartilaginous tissue in the subepithelial regions, with the underlying res-piratory epithelium exhibiting a characteristic pattern and no atypia (Figure 3).







DISCUSSION

TO is a rare benign disease of unknown cause. It was described macroscopically by Rokitansk in 1855 and micro-scopically by Wilks in 1857. Some etiopathogenic theories have been postulated. In 1863, Virchow postulated that echondrosis and exostosis lead to calcium deposition in and ossification of the tracheal rings. In 1947, Dalgaard postulated that the elastic tissue undergoes metaplasia to form cartilage and calcium deposition. In 1910, Aschoff-Freiburg attributed TO to changes in the elastic tissue in the trachea, introducing the term tracheopathia osteoplastica; and, in 1964, Secrest et al. labeled the disease as tracheobronchopathia osteoplastica.(7)

TO is usually asymptomatic. The most common symptoms are dyspnea; chronic cough; expectoration, which is of-ten abundant; and occasionally hemoptysis. Pulmonary function testing generally does not show airflow limitation. Only a small fraction of patients have ventilation anomalies, most commonly bronchial obstruction. CT and bronchos-copy remain the gold standard for identifying TO.

The clinical incidence of TO is estimated to range from 2 to 7 cases per 1,000 population, and its onset occurs be-tween 25 and 85 years of age, most commonly in the fifth decade of life. There is no gender predominance. The interval between the first symptoms and diagnosis is approximately 4 years in 45% of cases; however, it may be longer than 25 years.(7) Incidental findings on bronchoscopy occur at a ratio of approximately 3:2,000-5,000 popula-tion. According to Secrest et al., it is estimated that only 51% of cases will be diagnosed during the patient's life-time.(7) No correlation has been found between TO and smoking; however, some studies indicate an association between TO and chronic tracheal inflammation, arguing that this is possibly a factor of disease progression.(8,9)

Histologically, the mucosal bed may appear normal, with alternating areas of inflammation and necrosis and prolif-erative abnormal cartilage or bone formation in the submucosa, and there may be squamous metaplasia of the co-lumnar epithelium, calcium deposits, fragments of adipocytes, and active hematopoietic bone marrow tissue. (9,10) There is bony tissue, which is often cartilaginous, adjacent to the cartilaginous rings. Microscopically, the benign bony-cartilaginous tissue grows and replaces the bronchial submucosa and compresses the mucous glands. Bone marrow may be present. The epithelium covering these nodules is usually intact, metaplastic, sometimes dysplastic, or ulcerated. In the case presented here, transbronchial biopsy revealed the presence of cartilaginous tissue, with foci of metaplastic ossification, in the submucosal region. Metaplastic squamous epithelium, which was intact and exhibited no atypia, covered the osteocartilaginous nodule.

The differential diagnosis of TO consists mainly of papillomatoses, sarcoidosis, chondrosarcoma, hamartomas, amyloidoses, tuberculoid calcifications, dermatomyositis, scleroderma, Wegener's granulomatosis, and paratracheal calcified lymph nodes.(11,12)

To date, there is no definitive therapy for eradicating TO. Treatment is nonspecific; antibiotics are used to treat res-piratory tract infections, and antitussives and inhaled corticosteroids are used to treat cough. Surgical treatment is indicated when the symptoms do not respond to clinical treatment; tracheal segmental resection, anterior laryngofis-sure, partial laryngectomy, and bronchoscopic removal of lesions can be performed. (8) Neodymium:yttrium-aluminum-garnet (Nd:YAG) laser photocoagulation, as well as placement of a silicone mold, can be a treatment op-tion.(13-16)

The prognosis of patients with TO is favorable. Many cases in the literature have reported little progression over the years. However, it has been demonstrated that some patients died from severe respiratory infections.(17)

REFERENCES

1. Corrin B, Nicholson AG. Diseases of the airways. In: Corrin B, Nicholson AG. Pathology of the lungs. 2nd ed. London: Churchill Livingstone; 2006. p. 87-130.
2. Miscellaneous diseases of uncertain etiology. In: Travis WD, Colby TV, Koss MN, Rosado-di-Christenson ML, Muller NL, King Jr TE. Non-neoplastic disorders of the lower respiratory tract: Atlas of Nontumor Pathology. 1st ed. Washington: Armed Forces Institute of Pathology (U.S.); American Registry of Pathology; 2002. p. 857-93.
3. Kanat F, Teke T, Ozer F. Tracheopathia osteoplastica associated with iron deficiency anemia. Indian J Chest Dis Allied Sci. 2005;47(1):47-51.
4. Härmä RA, Suukari S. Tracheopathia condro-osteoplastica: a clinical study of thirty cases. Acta Otolaryngol.1997;84(1-2):118-23.
5. Hussain K, Gilbert S. Tracheopathia osteochondroplastica. Clin Med Res. 2003;1(3):239-42. https://doi.org/10.3121/cmr.1.3.239
6. Faig-Leite FS, Defaveri J. Traqueobroncopatia osteocondroplástica em portador de tumor de Klatskin. J Bras Patol Med Lab. 2008;44(6):459-62. https://doi.org/10.1590/S1676-24442008000600010
7. Jabbardarjani HR, Radpey B, Kharabian S, Masjedi MR. Tracheobronchopathia osteochondroplastica: presentation of ten cases and review of the literature. Lung. 2008;186(5):293-7. https://doi.org/10.1007/s00408-008-9088-4
8. Willms H, Wiechmann V, Sack U, Gillissen A. Tracheobronchopathia osteochondroplastica: A rare cause of chronic cough with haemoptysis. Cough. 2008;4:4. https://doi.org/10.1186/1745-9974-4-4
9. Doshi H, Thankachen R, Philip MA, Kurien S, Shukla V, Korula RJ. Tracheobronchopathia osteochondroplastica presenting as an isolated nodule in the right upper lobe bronchus with upper lobe collapse. J Thorac Cardiovasc Surg. 2005;130(3):901-2. https://doi.org/10.1016/j.jtcvs.2005.01.020
10. Pinto JA, Silva LC, Perfeito DJ, Soares Jdos S. Osteochondroplastic tracheobronchopathy: report on 02 cases and bibliographic review. Braz J Otorhinolaryngol. 2010;76(6):789-93. https://doi.org/10.1590/S1808-86942010000600019
11. Chroneou A, Zias N, Gonzalez AV, Beamis JF Jr. Tracheobronchopathia Osteochondroplastica. An underrecognized entity? Monaldi Arch Chest Dis. 2008;69(2):65-9.
12. Marom EM, Goodman PC, McAdams HP. Diffuse abnormalities of the trachea and main bronchi. AJR Am J Roentgenol. 2001;176(3):713-7. https://doi.org/10.2214/ajr.176.3.1760713
13. Tadjeddein A, Khorgami Z, Akhlaghi H. Tracheobronchopathia osteoplastica: cause of difficult tracheal intubation. Ann Thorac Surg. 2006;81(4):1480-2. https://doi.org/10.1016/j.athoracsur.2005.04.013
14. Lazor R, Cordier JF. Tracheobronchopathia osteochondroplastica. Orphanet Encyclopedia [serial on the Internet]. 2002 Jun [updated 2004 Jun; cited 2008 October 13]. [Adobe Acrobat document, 4p.]. Available from: https://www.orpha.net/data/patho/GB/uk-TO.pdf
15. Dutau H, Musani AI. Treatment of severe tracheobronchopathia osteochondroplastica. J Bronchol. 2004;11(3):182-5. https://doi.org/10.1097/01.lab.0000131026.05007.34
16. Abu-Hijleh M, Lee D, Braman SS. Tracheobronchopathia osteochondroplastica: a rare large airway disorder. Lung. 2008;186(6):353-9. https://doi.org/10.1007/s00408-008-9113-7
17. Leske V, Lazor R, Coetmeur D, Crestani B, Chatté G, Cordier JF, et al. Tracheobron-chopathia osteochondroplastica: a study of 41 patients. Medicine (Baltimore). 2001;80(6):378-90. https://doi.org/10.1097/00005792-200111000-00004

 

 


The Brazilian Journal of Pulmonology is indexed in:

Latindex Lilacs SciELO PubMed ISI Scopus Copernicus pmc

Support

CNPq, Capes, Ministério da Educação, Ministério da Ciência e Tecnologia, Governo Federal, Brasil, País Rico é País sem Pobreza
Secretariat of the Brazilian Journal of Pulmonology
SCS Quadra 01, Bloco K, Salas 203/204 Ed. Denasa. CEP: 70.398-900 - Brasília - DF
Fone/fax: 0800 61 6218/ (55) (61) 3245 1030/ (55) (61) 3245 6218
E-mails: jbp@jbp.org.br
jpneumo@jornaldepneumologia.com.br

Copyright 2019 - Brazilian Thoracic Association

Logo GN1