Rare Manifestation of Idiopathic Tracheobronchopathia Osteochondroplastica: Misdiagnosed and Untreated Entity?

Abdul Rana,Haitem Mezughi,Shuja A Malik,Kanaan Mansoor,Amro Al-Astal

doi:10.7759/cureus.9407

Abstract

Tracheobronchopathia osteochondroplastica (TBPO) is a rare benign disease of unknown cause, in which multiple cartilaginous or bony submucosal nodules project into the trachea and proximal bronchi. It usually occurs in men in their fifth decade and can cause airway obstruction, bleeding and chronic cough; patients are more prone to post-obstructive pneumonia and chronic lung infection in some instances. We report a case of a 69-year-old female who presented with shortness of breath and lower extremity swelling over the past couple of weeks. Echocardiography (ECHO) was consistent with heart failure with preserved ejection fraction, and she was treated with diuretics accordingly. Imaging revealed persistent pleural effusions bilaterally, more pronounced on the right side. During the course of her hospitalization, the patient coded once and had to be resuscitated. She had bronchoscopy done and pathology was consistent with TBPO. In this condition, there are numerous osseous or cartilaginous submucosal nodules in the trachea and the main bronchus and nodules are formed due to the deposition of calcium phosphate that results in the proliferation of osseous and cartilaginous structures resulting in the obstruction of large airways. Treatment for the most part is supportive and resolves around bronchodilators for symptomatic relief.

Highlights

Tracheobronchopathia osteochondroplastica (TBPO) is an idiopathic, rare, benign pulmonary condition with an incidence of 0.01 to 4.2 per 100,000 people [1]
Tracheobronchopathia osteochondroplastica (TBPO) is a rare benign disease of unknown cause, in which multiple cartilaginous or bony submucosal nodules project into the trachea and proximal bronchi. It usually occurs in men in their fifth decade and can cause airway obstruction, bleeding and chronic cough; patients are more prone to post-obstructive pneumonia and chronic lung infection in some instances
There are numerous osseous or cartilaginous submucosal nodules in the trachea and the main bronchus and nodules are formed due to the deposition of calcium phosphate that results in the proliferation of osseous and cartilaginous structures resulting in the obstruction of large airways

Summary

Introduction

Tracheobronchopathia osteochondroplastica (TBPO) is an idiopathic, rare, benign pulmonary condition with an incidence of 0.01 to 4.2 per 100,000 people [1] It was first described in 1857 and observed in an autopsy of a patient with pulmonary tuberculosis. This condition is characterized by numerous osseous or cartilaginous submucosal nodules in the trachea and main bronchus It usually occurs in men in their fifth decade and cause airway obstruction, bleeding and chronic cough, and patients are more prone to postobstructive pneumonia and chronic lung infection in some instances. We present a case of 69-year-old female with past medical history significant for hypertension, hyperlipidemia and diabetes who came to the hospital with shortness of breath and lower extremity swelling that had progressively worsened over the last two weeks These symptoms were accompanied with weight gain, orthopnea and chest pressure. The family pursued comfort care measures for her and the patient passed away the following day

Discussion

Conclusions

Disclosures