HOARSENESS AS PRESENTING SYMPTOM OF PEDIATRIC LARYNGEAL AMYLOIDOSIS M. Naraghi* and M. Kazemi Department of Otolaryngology, Head and Neck Surgery, School of Medicine, Otolaryngology Research Center, Tehran University of Medical Science, Tehran, Iran Abstract- Amyloidosis of the upper aerodigestive tract is rare in the pediatric age group. We present two cases of pediatric laryngeal amyloidosis. Both of them had hoarseness as the presenting symptom. Diagnostic evaluation included flexible nasopharyngoscopy, rigid laryngoscopy, biopsy, computed tomography scan and laboratory tests. The results of Congo red staining of the specimen were characteristic of amyloid. The most common area of reported laryngeal involvement have been the vestibule, followed by the false cords, the aryepiglottic folds and subglottic region. In our cases, the masses were limited to supraglottic larynx. Management depends on the severity of the symptoms regarding the individual patient. Acta Medica Iranica, 44(4): 277-280; 2006 © 2006 Tehran University of Medical Sciences. All rights reserved. Keywords: Amyloidosis; Hoarseness; Larynx; Pediatric; Supraglottic INTRODUCTION Amyloidosis is deposition of amyloid which is an extracellular fibrillar protein with unique ultrastructural, X-ray diffraction and biochemical properties in one or more sites in body (1-6). Virchow in 1851 was the first to use the term amyloid because of its starch like reaction when treated with iodine and sulfuric acid (1). Laryngeal amyloid was first recognized in 1875. Deposits of amyloid in the larynx are rare, accounting for between 0.2 and 1.2% of benign tumors of the larynx (4). However the larynx is the most common site for isolated amyloid deposits to occur in the head and neck (3). Amyloidosis has male to female ratio ranging from 1/1 to 3/1 in different studies (7, 8). It typically presents in middle life. Here we report 2 cases of pediatric laryngeal amyloidosis. Received: 15 Feb. 2004, Revised: 22 Oct. 2005, Accepted: 2 Nov. 2005 * Corresponding Author: M. Naraghi, Department of Otolaryngology, Head and Neck Surgery, School of Medicine, Otolaryngology Research Center, Tehran University of Medical Science, Tehran, Iran Tel: +98 21 66703037 Fax: +98 21 66760245 E-mail: naraghim@sina.tums.ac.ir CASE REPORTS The patients included two children, one male and one female. Both patients experienced progressive hoarseness and duration was from 4 to 36 months. Patients had negative systemic work up which included physical examinations, laboratory tests, radiological studies and biopsies. In both of them, amyloid deposition occurred in the false cord submucosally. Case 1 A ten-year-old girl presented with four-month history of hoarseness. She had also dyspnea in sleep. There were no other symptoms like dysphagia, odynophagia, cough or having sputum. Indirect laryngoscopy revealed a large mass located submucosally in the supraglottic area. The mass was limited to the right false vocal cord and aryepiglottic fold sparing true vocal cords. Other otorhinolaryngologic examinations were normal. Routine laboratory, rheumatoid and liver function tests were normal. High-resolution computed tomography (CT) was used to evaluate extension of the laryngeal involvement (Fig 1).