Laryngeal sarcoidosis: presentation and management in the pediatric population

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.

Keywords: Granulomatous disease; Pediatric laryngeal sarcoidosis; Pediatric sarcoidosis; Supraglottic edema.