(Chest. 2005;128:3671-3673.)
© 2005
American College of Chest Physicians
Hemoptysis With Multiple Tracheal Nodules*
Jeff S. W. Wong, MBChB, MRCS;
Calvin S. H. Ng, MBBS(Hons), MRCS and
Anthony P. C. Yim, MD, FRCS
* From the Division of Cardiothoracic Surgery, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, N.T., Hong Kong.
Correspondence to: Anthony P. C. Yim, MD, FRCS, Professor of Surgery and Chief of Cardiothoracic Surgery, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, NT, Hong Kong; e-mail: yimap{at}cuhk.edu.hk
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Introduction
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A 59-year-old man presented with on-and-off mild hemoptysis for 2 months. Physical examination, chest radiograph, and sputum examinations including culture, acid-fast bacilli tests, and cytology were unremarkable. Flexible bronchoscopy was performed that showed multiple protruding nodules inside the tracheal lumen. Subsequent CT of the neck revealed multiple irregular calcified nodular protrusions into the tracheal lumen (Fig 1
). Sagittal CT reconstruction showed multiple nodules of varying sizes over the anterior tracheal wall, involving the whole length of the trachea (Fig 2
).
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What is the diagnosis?
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Diagnosis: tracheopathia osteoplastica
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Flexible bronchoscopy showed multiple tracheal nodules without involvement of the distal endobronchial tree (Fig 3
). These tracheal nodules were located in the anterior and lateral tracheal walls with sparing of the posterior tracheal membrane. The consistency of the tracheal nodule was hard on bronchoscopic biopsy.
Finally, tracheal nodule biopsy reported normal respiratory and squamous mucosa with fragments of lamellar bone in the submucosa. The clinical and radiologic evidence suggested the diagnosis of tracheopathia osteoplastica that was confirmed by histology. The patient had no shortness of breath, and the hemoptysis resolved spontaneously without treatment. The patient remained asymptomatic during subsequent follow-up.
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Discussion
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Tracheopathia osteoplastica (TPO) is a rare disorder characterized by multiple submucosal cartilaginous and osseous nodules in the respiratory tract that can involve the entire trachea and mainstem bronchi.1 Typically, the posterior tracheal membrane is spared, and the overlying mucosa is normal. The pathogenesis of TPO is unclear, although it can be familial and sometimes associated with chronic inflammation or trauma.2 Most patients remain asymptomatic, although occasionally they may present with hemoptysis, chronic coughing, and dyspnea.2
Diagnosis of TPO is made by the characteristic radiologic, bronchoscopic, and histologic appearances. CT may show multiple, broad-based calcified protrusions inside the major airway. The calcification typically occurs in the submucosa separate from the cartilaginous tracheal rings.3 The bronchoscopic image is usually diagnostic, displaying multiple papilla-like protrusions from the anterior and lateral walls of the trachea, making the typical cobblestone appearance. The posterior tracheal membrane is characteristically spared from the lesions. In more advance cases, these protrusions may coalesce into plaque-like lesions causing airway deformity and obstruction.2 Histologically, metaplastic cartilage and bone are found in the submucosa. The overlying mucosa is intact with normal or metaplastic epithelium.1 Occasionally, the bony lamellae may protrude into the mucosa, giving the appearance of multiple whitish tracheal nodules on bronchoscopy. As these nodules are hard in consistency, biopsy can be difficult to perform and often results in inadequate tissue sampling.
The clinical differential diagnosis of TPO includes tracheobronchomegaly, tracheomalacia, and papillomatosis. Tracheobronchomegaly and tracheomalacia cause softening and dilation of the trachea, while TPO causes rigidity and narrowing of the trachea. In contrast to TPO, papillomatosis manifests as multiple soft, noncalcified endotracheal nodules. CT is helpful in making the diagnosis.
Management of TPO ranges from conservative treatment, bronchoscopic intervention, to operative correction. Asymptomatic patients should be treated conservatively with regular follow-up, as the natural history of TPO is relatively benign with slow progression.4 Surgical intervention is indicated to treat symptoms of airway stenosis. For short segmental tracheal stenosis, bronchoscopic curettage and resection of tracheal nodules have been reported as an effective treatment in relieving airway obstruction.24 However, bronchoscopic dilatation and laser therapy are not effective in this condition.24 For long segmental tracheal stenosis, different surgical approaches have been reported. Kutlu et al5 successfully treated a case of TPO with modified sliding tracheoplasty, with the patient remaining asymptomatic at 15 months following surgery. Recently, Grillo and Wright6 reported four cases of TPO treated with linear tracheoplasty. Despite one patient being unavailable for follow-up, the other three patients showed significant improvements in symptoms and peak expiratory flow rate after surgery. Nevertheless, the experience in treating this rare condition is still limited in the literature.
In summary, tracheopathia osteoplastica is a rare disease. Despite the alarming bronchoscopic features, TPO is a relatively benign condition. Patients are usually asymptomatic, and diagnosis is based on characteristic bronchoscopic, radiologic, and histologic appearances. Most cases can be managed conservatively with close monitoring. Surgical intervention is indicated to treat symptoms of airway obstruction.
Received for publication June 8, 2005.
Accepted for publication June 27, 2005.
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References
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- Penner, CR, Thompson, LD (2003) Tracheopathia osteoplastica. Ear Nose Throat J 82,427[Medline]
- Prakash, UB, McCullough, AE, Edell, ES, et al Tracheopathia osteoplastica: familial occurrence. Mayo Clin Proc 1989;64,1091-1096[Medline]
- Hirsch, M, Tovi, F, Goldstein, J, et al Diagnosis of tracheopathia osteoplastica by computed tomography. Ann Otol Rhinol Laryngol 1985;94,217-219[Medline]
- Tibesar, RJ, Edell, ES Tracheopathia osteoplastica: effective long-term management. Otolaryngol Head Neck Surg 2003;129,303-304[Medline]
- Kutlu, CA, Yeginsu, A, Ozalp, T, et al Modified slide tracheoplasty for the management of tracheopathia osteochondroplastica. Eur J Cardiothorac Surg 2002;21,140-142[Abstract/Free Full Text]
- Grillo, HC, Wright, CD Airway obstruction owing to tracheopathia osteoplastica: treatment by linear tracheoplasty. Ann Thorac Surg 2005;79,1676-1681[Abstract/Free Full Text]
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Introduction
What is the diagnosis?
