ArticleRadiology case reports2022
A rare case of isolated laryngotracheal amyloidosis with airway narrowing and vocal fold involvement.
Article in Radiology case reports, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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4 authors at 2 institutions in 2 countries.
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Abstract
Primary amyloidosis is a rare condition with 6-10 cases in a million, with focal involvement representing 9%-15% of those cases [1,2]. Isolated tracheobronchial amyloidosis is extremely rare and when present, can result in focal or diffuse thickening of the glottis, trachea and bronchi, leading to hoarseness, shortness of breath, and dysphonia. Computed tomography (CT) usually shows circumferential thickening of trachea and bronchi with or without calcifications and associated airway narrowing of affected segments. MRI demonstrates intermediate to low signal on T1, low signal on T2 and variable heterogeneous enhancement. Multiple conditions can result in thickening of the airway including but not limited to inflammatory, infectious, and neoplastic etiologies. Biopsy with histologic correlation provides a definitive diagnosis. Biopsied tissue demonstrates characteristic apple-green birefringence with Congo red stain. There is no cure for amyloidosis and the prognosis is quite variable depending on the extent of airway involvement. Current treatments are aimed at alleviating symptoms and include bronchoscopic debridement, laser therapy, and balloon dilation with adjuvant radiation therapy. Here, we present a rare case of a 47-year-old male with isolated laryngotracheal amyloidosis with marked airway narrowing and vocal fold involvement.
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