P374 Laryngeal sarcoidosis responsive to treatment with sirolimus: a first case report

• Published in: Archives of disease in childhood Vol. 104; no. Suppl 3; p. A306
• Main Authors: Kelleher, Karen, Russell, John, Killeen, Orla, MacDermott, Emma Jane, Leahy, Timothy Ronan
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group LTD 01.06.2019
• Subjects: Airway management; Biopsy; Childrens health; Corticosteroids; Dysphagia; Endoscopy; Epiglottis; Histology; Hyperplasia; Methotrexate; Ostomy; Pediatrics; Rapamycin; Respiratory tract; Sarcoidosis; Tracheostomy
• ISSN: 0003-9888; 1468-2044
• DOI: 10.1136/archdischild-2019-epa.720

We report the case of a 15 year old girl with a four month history of gradual onset dysphonia and dysphagia. Endoscopy revealed a significantly enlarged epiglottis and arytenoids causing supraglottic airway obstruction (pinpoint airway). She progressed to emergency tracheostomy and a biopsy of the epiglottis was taken. Histology revealed lymphoid hyperplasia with focal non-necrotizing granulomata. A screen for infection was negative, as was a serum ACE. The patient was commenced on corticosteroids and methotrexate, but after three months, there was no improvement. Therafter, she was switched to sirolimus, which led to resolution of her epiglottic swelling, allowing the tracheostomy to be reversed and corticosteroid therapy to be discontinued.ConclusionThis is the first case, to our knowledge, of sirolimus being successfully used in treatment of laryngeal sarcoidosis. Sirolimus could be considered a treatment option in these instances, particularly in the presence of lymphoid aggregates on histology.