Ahmed Mussanna, Armani Hamidreza, Salahi Navid, Hammill Patrick
Department of Radiology, SUNY Downstate Health Sciences University/Kings County Hospital, 451 Clarkson Ave, 3rd Fl, Rm B-3304, Brooklyn, NY 11203, USA.
St. George's University School of Medicine, True Blue, Grenada.
Radiol Case Rep. 2022 Aug 27;17(11):4096-4099. doi: 10.1016/j.radcr.2022.07.068. eCollection 2022 Nov.
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.
原发性淀粉样变性是一种罕见疾病,每百万人口中有6至10例,其中局灶性受累占这些病例的9%至15%[1,2]。孤立性气管支气管淀粉样变性极为罕见,一旦出现,可导致声门、气管和支气管局灶性或弥漫性增厚,导致声音嘶哑、呼吸急促和发音困难。计算机断层扫描(CT)通常显示气管和支气管环形增厚,伴有或不伴有钙化以及受累节段相关的气道狭窄。磁共振成像(MRI)在T1上显示中等至低信号,在T2上显示低信号以及可变的不均匀强化。多种情况可导致气道增厚,包括但不限于炎症、感染和肿瘤性病因。组织学相关的活检可提供明确诊断。活检组织在刚果红染色下显示特征性的苹果绿双折射。淀粉样变性无法治愈,预后因气道受累程度而异。目前的治疗旨在缓解症状,包括支气管镜清创、激光治疗和辅助放射治疗的球囊扩张。在此,我们报告一例罕见的47岁男性病例,患有孤立性喉气管淀粉样变性,伴有明显的气道狭窄和声襞受累。