Primary laryngeal amyloidosis is a rare benign disease of unknown aetiology. It can present with dysphonia or stridor. A woman presenting with airway compromise, who required a tracheostomy, is reported. (Postgrad Med J 2000;76:364-365) Keywords: laryngeal disease; amyloidosis Case report A 47 year old woman presented with a seven year history of dysphonia which had deteriorated recently. Fibre optic laryngoscopy showed polyps on the right false vocal cord with a suggestion of subglottic stenosis (fig 1). The true vocal cords were mobile and appeared normal.While awaiting further investigation, she presented with airway compromise requiring urgent tracheostomy. Direct laryngoscopy revealed subglottic oedema. The polypoid lesion was biopsied and showed features suggestive of amyloidosis and this was confirmed after staining with Congo red. A flexible bronchoscopy through the tracheostomy tube showed isolated nodules within the trachea (fig 2). Full blood count and erythrocyte sedimentation rates were normal.At follow up a year later she was noted to have a change in voice. Fibre optic laryngoscopy showed new lesions on the right and left true vocal cords. These were resected with a carbon dioxide laser. The result was a subjective improvement in the quality of her voice and airway. She was decannulated two months later. She remains under review and requires no further intervention.
DiscussionAmyloidosis is a benign, slowly progressive condition that is characterised by extracellular fibular proteins. Diagnosis is confirmed by histopathological specimens that stain with Congo red.1 Amyloidosis can be classified as either primary, developing spontaneously, or secondary to some other longstanding inflammatory disease such as rheumatoid arthritis. The primary form can be further subdivided into a localised form, where deposits are confined to a single organ or location, or generalised, where deposits are found to some extent in all tissues.The most common presenting symptoms of primary laryngeal amyloidosis are dysphonia and stridor. Rarely, airway compromise occurs and an alternative airway may be necessary. The presence of tender bones, lymphadenopathy, or splenomegaly should alert the clinician to the possibility of generalised amyloidosis. Learning points x Amyloidosis is a rare benign disease of unknown aetiology. x Laryngeal amyloidosis can present with hoarseness or stridor which may require tracheostomy. x Histological diagnosis from a biopsy specimen can be confirmed with characteristic staining with Congo red. x Treatment is by surgical resection using the carbon dioxide laser. Repeated resections may be necessary. Localisation of lesions in the larynx is to the ventricle, false vocal cords, true vocal cords, aryepiglottic folds, and subglottis in that order of frequency.2 4 Histology of the biopsied specimen using routine haematoxylin and eosin stain shows amyloid as eosinophilic extracellular infiltrate. Further staining with Congo red reveals characteristic apple green birefringence with a ...
We describe a man who, after a presumed encephalitic illness, was "locked-in" for 27 years. His CT and autopsy findings showed atrophy of the brainstem and a cystic lesion at the base of the pons. He survived longer than most other patients in a similar state.
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