Laryngeal sarcoidosis: Presentation and management in the pediatric population

doi:10.1016/j.ijporl.2015.06.017

International Journal of Pediatric Otorhinolaryngology

Volume 79, Issue 9, September 2015, Pages 1382-1387

https://doi.org/10.1016/j.ijporl.2015.06.017 Get rights and content

Abstract

Background

Sarcoidosis is a disease characterized by systemic non-necrotizing granulomas of unknown etiology. Laryngeal sarcoidosis is extremely uncommon, especially among pediatric patients. The clinical presentation and management of this entity in the pediatric population are poorly understood.

Methods

A comprehensive search in PubMed was conducted to identify all cases in the published literature. We also present a case of isolated pediatric laryngeal sarcoidosis and outline the multidisciplinary approach to evaluation and management.

Results

A previously healthy 13-year-old female presented with a five-month history of mild dysphonia, dyspnea on exertion, and diffuse supraglottic edema. Biopsy showed non-necrotizing granulomas. Treatment with methotrexate led to marked improvement. The literature search identified seven previously published cases of pediatric laryngeal sarcoidosis, four in which disease was isolated to the larynx. All patients presented with a symptomatic and diffusely edematous supraglottis. Diagnoses were based on supraglottic biopsies showing non-necrotizing granulomas; all other possible etiopathologies were excluded. Three patients responded to corticosteroid therapy alone, one patient to tumor necrosis factor (TNF) inhibitor and methotrexate, and the remainder to a combination of corticosteroid therapy and surgical debulking.

Conclusions

Laryngeal sarcoidosis in the pediatric population is challenging to diagnose and manage. When epithelioid granulomas are encountered histologically, other causes of granulomatous inflammation must be ruled out before a diagnosis of sarcoidosis can be made. Corticosteroid therapy alone may be ineffective. Medical therapy with methotrexate alone or in combination with TNF inhibitors versus surgical debulking alone or as part of multimodality treatment should be considered. A multidisciplinary approach with involvement of an otolaryngologist, pathologist, and rheumatologist is suggested.

Introduction

Sarcoidosis is a multi-system disease that can affect most organ systems and is uncommon among pediatric patients [1]. The clinical presentation of this systemic granulomatous disease can vary greatly, and cases of laryngeal sarcoidosis are exceedingly rare. To our knowledge, only seven previous cases of have been reported in the literature. The purpose of this paper is to discuss the presentation, evaluation, and management of pediatric laryngeal sarcoidosis via a multidisciplinary team approach.

Section snippets

Materials and methods

A relevant case of a pediatric laryngeal sarcoidosis is presented. A literature search was conducted for peer-reviewed English-language publications using the online search database PubMed on January 26, 2015. Search terms included: laryngeal sarcoidosis, pediatric sarcoidosis, and pediatric laryngeal sarcoidosis. Reference lists were scanned for additional case reports.

Patient presentation

A previously healthy and physically active 13-year-old Caucasian female presented with a five-month history of mild dysphonia and dyspnea on physical exertion. She reported nighttime coughing and difficulty sleeping in the supine position. She denied dysphagia, constitutional symptoms including fever and weight loss, epistaxis, hemoptysis, trauma or vocal abuse, recent respiratory tract infection, exposure to fumes or gases, or illicit drug use. Review of systems was otherwise negative. The

Discussion

Sarcoidosis is a disease characterized by systemic non-necrotizing granulomas [1]. There are three criteria for diagnosis: (1) clinical and radiologic manifestations of disease, (2) presence of non-necrotizing granulomas, and (3) no evidence of alternative diseases [1]. Laryngeal sarcoidosis is a rare entity, especially in the pediatric population. The case herein is the eighth reported case in the literature [2], [3], [4], [5], [6], [7], [8].

The patient herein, at age 13, can be said to have

Conclusions

Pediatric laryngeal sarcoidosis presents a serious clinical challenge due to the difficulty in diagnosis and management of the disease. Airway evaluation and biopsy should be considered. A keen clinical eye, appropriate work-up, and multidisciplinary approach including consultation with an otolaryngologist, pathologist, and rheumatologist is paramount to the accurate diagnosis and management. Corticosteroid treatment, systemic or by supraglottic injection, may be unsuccessful at managing the

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