ENT Updates

Case Report

Otolaryngological findings in mucopolysaccharidosis

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https://doi.org/10.2399/jmu.2014003008
Yurttaş, V., Bozdemir, K., Tarlak, B., & Yazgan, A. (2014). Otolaryngological findings in mucopolysaccharidosis. ENT Updates, 4(3). https://doi.org/10.2399/jmu.2014003008
Volume 4 Issue 3 (2014)
Copyright (c) 2014 Veysel Yurttaş, Kazım Bozdemir, Behçet Tarlak, Aylin Yazgan
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This work is licensed under a Creative Commons Attribution 4.0 International License.

Authors

  • Veysel Yurttaş
    Department of Otorhinolaryngology, Abant İzzet Baysal University Medical Faculty, Bolu, Turkey
  • Kazım Bozdemir Department of Otorhinolaryngology, Ankara Atatürk Training and Research Hospital, Ankara, Turkey
  • Behçet Tarlak Department of Otorhinolaryngology, Ankara Atatürk Training and Research Hospital, Ankara, Turkey
  • Aylin Yazgan Department of Pathology, Ankara Atatürk Training and Research Hospital, Ankara, Turkey

A 49-year-old woman admitted to our clinic due to a progressive hoarse voice for 4 years, foreign body sensation in the back of her throat, otalgia, hoarseness and mouth breathing. Nasolaryngoscopy demonstrated a right nasopharyngeal mass extending to oropharynx. Indirect laryngoscopy revealed a granulomatous lesion originating from right band ventricle and extending on the right vocal fold. The granulomatous lesion did not alter correct laryngeal mobility. There was no history of difficulty in swallowing. A contrast enhanced computed tomography showed a smooth mass involving nasopharynx and glottis. A direct laryngoscopy was planned for excisional biopsy of the lesion. Histopathological examination revealed nasopharyngeal and laryngeal amyloidosis. Further evaluations were negative for systemic amyloidosis. The aim of the report is to present an extremely rare case of isolated primary nasopharyngeal and laryngeal amyloidosis and discuss by using current literature knowledge.

Keywords:

Mucopolysaccharidoses (MPSs) lysosomal storage diseases short neck mouth opening restriction airway problems