Tracheobronchomegaly associated with laryngo-tracheal amyloidosis: First case report

• Published in: Auris, nasus, larynx Vol. 43; no. 4; pp. 472 - 475
• Main Authors: Adachi, Kazuo, Umezaki, Toshiro, Komune, Shizuo
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ireland Ltd 01.08.2016
• Subjects: Amyloidosis; Amyloidosis - complications; Amyloidosis - pathology; Dyspnea - etiology; Female; Humans; Laryngeal Diseases - complications; Laryngeal Diseases - pathology; Laryngostenosis - diagnostic imaging; Laryngostenosis - etiology; Laryngostenosis - surgery; Lasers, Gas - therapeutic use; Middle Aged; Otolaryngology; Recurrence; Subglottic stenosis; Tomography, X-Ray Computed; Tracheal Diseases - complications; Tracheal Diseases - pathology; Tracheobronchomegaly; Tracheobronchomegaly - diagnostic imaging; Tracheobronchomegaly - etiology; Tracheostomy; Subglottic stenosis; Amyloidosis; Tracheobronchomegaly
• ISSN: 0385-8146; 1879-1476
• DOI: 10.1016/j.anl.2015.12.008

Abstract Tracheobronchomegaly (TBM) is a rare enlargement of the tracheal cartilage, also known as Mounier-Kuhn syndrome (MKS). Here, we describe an unusual case of acquired TBM in an adult, caused by amyloid regeneration and associated tracheal weakening, rather than by MKS. CT scan and fiberscopic examination of a 55-year-old woman suffering from severe dyspnea revealed TBM and subglottic stenosis, which was caused by deposition of amyloid tissue. We performed a tracheostomy and vaporized the subglottic stenosis with a CO2 laser, after which we installed a silicone T-tube. After the first operation, re-stenosis occurred, and the procedure was repeated; stenosis was subsequently cured and the dyspnea disappeared, after which the tracheostomy could be closed. This is the first report of adult TBM associated with amyloid deposition in the subglottis and trachea. This diagnosis is very difficult, as amyloid deposition in the trachea can have various clinical presentations.