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Laryngeal amyloidosis as a cause of death

Laryngeal amyloidosis (LA) is a rare idiopathic condition manifesting with extracellular depositions of amyloid fibrils, which may cause airway symptoms varying from dysphonia to dyspnea. It constitutes a localized form of immunoglobulin light chain amyloidosis (AL amyloidosis), and usually not associated with systemic disease [1]. Thus, a symptomatic treatment approach with repeated endoscopic laser-assisted resections of obstructive amyloid deposits [2] and long-term follow up is recommended even in progressive LA [3]. Further, LA is described as an innocuous and rarely lifethreatening condition with good prognosis, and treatmentrelated mortality has extremely rarely been reported [4]. Also, Wierzbicka et al. [3] when presenting their experience based on 16 LA cases, explicitly mention that none of their patients suffered from dyspnea. In our series of nine patients with LA (mean age 51.3 years; range 34–80, six females), we experienced one disease/ treatment related death. All patients with nonfatal outcome were treated with a conservative approach either with open or endoscopic surgery or observation depending on disease extension. During follow up, varying from 18 to 192 months, they had either stable/persistent disease or local recurrences. Our latest patient was a 58-year-old woman with uneventful medical history who fell ill with cough and acute progressive dyspnea, with inspiratory and expiratory stridor. She was first emergency intubated and then tracheotomized, and scheduled for ENT consultation. However, she died two days later of an anoxic brain injury after an airway obstruction due to tracheostomy complication and recannulation failure. At autopsy, a subglottic obstructive submucosal mass with histopathologically proven amyloidosis was seen. Our case demonstrates that LA may present with an obstructive lesion with progressive growth warranting emergency airway management and consideration of surgical intervention. Here, a tracheotomy cannula-related complication obviously was the immediate cause of death. However, the degree of LA-induced airway obstruction was severe enough to cause her progressive, and finally critical airway condition. Therefore, localized LA – and not solely systemic AL amyloidosis interfering with vital organs – may also ultimately lead to death. Physicians managing these patients should be aware of this rare cause of laryngeal stridor and dyspnea and of the potential mortality. Also, various treatment options and their adequate timing should be considered according to the degree of compromised airway and extension of disease. Endoscopic and imaging studies are needed to stage disease extension prior to management decision. Surgical endoscopic debulking seems to be an effective and functional approach in most cases in terms of achieving adequate laryngeal lumen. Also, radiation has been proposed as an alternative [5]. More importantly, if LA is likely to cause total airway obstruction, tracheotomy and its proper maintenance are warranted.