Primary Localized Laryngeal Amyloidosis: an Exceptional Clinical Entity

Case Report

Austin J Otolaryngol. 2015;2(6): 1049.

Primary Localized Laryngeal Amyloidosis: an Exceptional Clinical Entity

Sinha R¹, Singh RK¹* and Kumar R²

¹Department of Otorhinolaryngology, Indira Gandhi Institute of Medical Sciences University, India

²Department of Medicine, Katihar Medical College, India

*Corresponding author: Rakesh Kumar Singh,Department of Otorhinolaryngology, Indira Gandhi Institute of Medical Sciences, Sheikhpura, Patna, Zip code: 800014, India

Received: May 10, 2015; Accepted: July 22, 2015; Published: July 25, 2015

Abstract

Laryngeal amyloidosis is a rare condition encountered by otolaryngologists, though it is the most common site for amyloidosis in head and neck region. It often presents with dysphonia as it involves the vocal cords. This again makes its diagnosis and management challenging. Precise surgical excision is mandatory for functional preservation of voice along with close follow-up for quick detection of any recurrence. We present a case of laryngeal amyloidosis managed with microlaryngeal surgery resulting in complete resolution of symptoms and no recurrence.

Keywords: Amyloidosis; Larynx; Localized

Introduction

Amyloidosis is a rare benign disease of head and neck region. It is a disorder that results from the extracellular deposition of an abnormal fibrinous protein called amyloid in various organs of the body. It can be of two types, systemic and localized. Although the localized form of amyloidosis is extremely rare in larynx, it is the commonest site of involvement in head and neck region [1]. The condition involves the true vocal cords and laryngeal ventricle, which is primary in nature; nevertheless in some incidence, it may be a part of generalized systemic form [2]. The presenting complaints are hoarseness, fullness in throat and sometimes cough. Diagnosis is based on histopathological evidence of amyloid confirmed by Congo red staining under polarized light or by electron microscopy. Surgery is the mainstay of treatment directed at improving the airway and restoration of quality of voice. We present one such case managed with microlaryngeal surgery resulting in complete resolution of symptoms and no recurrence.

Case Presentation

A 45 years old female presented with progressive hoarseness of voice for past 6 months. There was no associated breathing difficulty, cough or sense of fullness in throat. There were no signs and symptoms of any systemic disease. She was a non-smoker with no other associated debilitating disease. Endolaryngeal examination revealed granular polypoidal mass in right ventricle. Both the vocal cords were mobile with no airway compromise. The remaining head and neck examination and systemic examination was unremarkable. Her routine blood investigations were within normal limits. The pulmonary, cardiac, abdominal and urinary evaluations were normal. Microlaryngeal surgery with excision biopsy under general anesthesia was planned. Microlaryngoscopy showed multiple smooth polypoidal tissue in entire right ventricle and extending through anterior commissure to partly involve the left ventricle also (Figure 1). The mass was completely excised with the help of microlaryngeal forceps and scissors without causing any damage to the vocal cords or vestibular folds. Histopathological examination by haematoxylin and eosin revealed polypoidal lesion lined by squamous to respiratory lining with an acellular amorphous eosinophilic infiltrate in stroma and mild chronic inflammatory infiltrate. Congo red stain was positive along with apple green birefringence with polarized light (Figure 2). The immediate post-operative period was uneventful and patient showed gradual improvement in voice quality. On one year follow up visit, the patient had reasonable speech with no hoarseness. The vocal cords were mobile with no edema or scarring. No residual or recurrent disease was seen.

Citation: Sinha R, Singh RK and Kumar R. Primary Localized Laryngeal Amyloidosis: an Exceptional Clinical Entity. Austin J Otolaryngol. 2015;2(6): 1049. ISSN :2473-0645