A Case of Extensive Nasopharyngeal and Tracheobronchial Amyloidosis

Abstract

INTRODUCTION: Upper respiratory tract amyloidosis with obstructive symptoms is an exceedingly rare presentation. Deposition of amyloid in the submucosa leads to progressive airway narrowing, often requiring endoscopic debridement1. We report a case with near complete narrowing of the pharynx and trachea by polyploidy submucosal amyloid lesions that were treated successfully with Nd:YAG-laser CASE PRESENTATION: A 58 year-old Caucasian male presented with a 10 day history of upper respiratory symptoms including congestion, rhinorrhea, shortness of breath and hoarseness. Flexible laryngoscopy revealed hard polypoid lesions on the posterior and right lateral walls of the nasopharynx as well as on the posterior surface of the soft palate. These lesions extended down into the trachea, mandating bronchoscopy for further evaluation. This showed diffuse endobronchial thickening and irregular nodules extending into the main stem bronchi. Of note, both the cartilaginous and membranous portions were markedly thickened with mass-like lesions. Debridement of the lesions was performed to decrease airway obstruction and establish diagnosis. A 4 x 3 cm nodule was shaved off the soft palate and was sent for pathology along with subglottic tissue. Pathology revealed acellular eosinophilic, waxy material depositions characterized by positive staining for Congo red and amyloid A protein, consistent with localized amyloidosis. As a result of the resection the patient's rhinorrhea, congestion and hoarseness improved. However, progressive dyspnea on exertion persisted and was attributed to subglottic disease; a staged debridement was planned for this. Microlaryngoscopy was performed to excise laryngeal nodules using carbon dioxide laser. This facilitated intubation with 7.5 endotracheal tube, followed by bronchoscopy. Near total occlusion of lower trachea and mainstem bronchi was again visualized. The soft tissue nodules protruding into lumen were cauterized with Nd:YAG laser. Secondary bronchi and smaller airways were relatively free of disease. The postoperative course was unremarkable and the patient was discharged home after overnight observation in the hospital. DISCUSSION: Localized amyloidosis of the tracheobronchial tree with laryngeal involvement is extremely rare2. We report a case of extreme tracheobronchial amyloidosis with laryngeal and nasopharyngeal involvement. Symptomology at presentation in patients with localized amyloidosis varies with location of disease load1. Our patient presented with voice changes, congestion and rhinorrhea which resolved after excision of the soft palate lesion. He later presented with progressive shortness of breath secondary to airway narrowing. Excision is often required to improve symptoms and prevent post-obstructive pneumonia and prevent sudden death from airway obstruction or hemorrhage. Use of Nd:YAG laser has been reported to have excellent results not only in achieving adequate excision and symptomatic relief but also in reducing bleeding complications3. CONCLUSIONS: Tracheobroncial amyloidosis is a rare diagnosis and patients present with a wide variety of symptoms including airway obstruction. Bronchoscopy findings usually raise the suspicion but biopsy is needed to confirm diagnosis. Excision with Nd:YAG laser is effective and safe in providing symptomatic relief.