Trachéopathie ossifiante

Abstract

Objectives

Report a case of tracheopathia osteoplastica and describe from a literature analysis the main clinical, radiological, and therapeutic features of this rare disease based on literature review.

Material and methods

A 74-year-old patient suffering from a nasal polyposis that had been repeatedly operated was admitted for surgery of the frontal sinus. The day of surgery, the difficult intubation required a laryngotracheoscopy, which found a dystrophic trachea, with a sprinkling of whitish nodules, and hard swelling. The pathologic exams of a large biopsy specimen using a rigid bronchoscopy provided the diagnosis of tracheopathia osteoplastic. A complementary workup to search for the etiology was undertaken. The risk and the difficulty of the intubation led to suspending the surgery.

Results

The CT scan as well as the histological, bacteriological, and immunological tests showed nothing specific. The etiopathogenic hypothesis was the association of chronic disease of the upper airways such as the ozena or the chronic inflammation of the respiratory tract, an endocrine factor, dermatomyositis, exposure to toxic substances such as silica, or tracheobronchial amyloidosis. The bronchial microbiology test is positive in more than 50% of patients, and often Klebsiella ozaenae or Pseudomonas aeruginosa is found.

Conclusion

Tracheopathia osteoplastica is a rare tumor, with unknown etiology and physiopathology. The discovery is most often incidental. Progession is slow and it does not compromise the vital prognosis. The treatment is symptomatic. Few surgical tracheal operations are described in the literature.