Upper Airway Obstruction Requiring Emergent Tracheostomy Secondary to Laryngeal Sarcoidosis: A Case Report. 2017

Changwan Ryu, and Erica L Herzog, and Hongyi Pan, and Robert Homer, and Mridu Gulati
Department of Internal Medicine, Section of Pulmonary, Critical Care, and Sleep Medicine, Yale University School of Medicine, New Haven, CT, USA.

BACKGROUND Laryngeal sarcoidosis is a rare extrapulmonary manifestation of sarcoidosis, accounting for 0.33-2.1% of cases. A life-threatening complication of laryngeal sarcoidosis is upper airway obstruction. In this report we describe our experience in the acute and chronic care of a patient who required an emergent tracheostomy, with the aim to provide further insight into this difficult to manage disease. CASE REPORT A 37-year-old African American female with a 10-year history of stage 1 sarcoidosis presented with severe dyspnea. Laryngeal sarcoidosis was diagnosed three years previously, and she remained stable on low-dose prednisone until six months prior to admission, at which time she self-discontinued her prednisone for the homeopathic treatment Nopalea cactus juice. Her physical examination was concerning for impending respiratory failure as she presented with inspiratory stridor and hoarseness. Laryngoscopy showed a retroflexed epiglottis obstructing the glottis with edematous arytenoids and aryepiglottic folds. Otolaryngology performed an emergent tracheostomy to secure her airway and obtained epiglottic biopsies, which were consistent with sarcoidosis. She was eventually discharged home on prednisone 60 mg daily. Following months of corticosteroids, a laryngoscopy showed the epiglottis continuing to obstruct the glottis. The addition of methotrexate to a tapered dosage of prednisone 10 mg daily was unsuccessful, and she remains on prednisone 20 mg daily for disease control. CONCLUSIONS Laryngeal sarcoidosis, a rare extrapulmonary manifestation of sarcoidosis, uncommonly presents as the life-threatening complication of complete upper airway obstruction. As such, laryngeal sarcoidosis is associated with significant morbidity and mortality, requiring a high index of suspicion for timely diagnosis and treatment.

UI MeSH Term Description Entries
D007818 Laryngeal Diseases Pathological processes involving any part of the LARYNX which coordinates many functions such as voice production, breathing, swallowing, and coughing. Laryngeal Perichondritis,Larynx Diseases,Disease, Laryngeal,Disease, Larynx,Diseases, Laryngeal,Diseases, Larynx,Laryngeal Disease,Laryngeal Perichondritides,Larynx Disease,Perichondritides, Laryngeal,Perichondritis, Laryngeal
D007828 Laryngoscopy Examination, therapy or surgery of the interior of the larynx performed with a specially designed endoscope. Laryngoscopic Surgical Procedures,Surgical Procedures, Laryngoscopic,Laryngoscopic Surgery,Surgery, Laryngoscopic,Laryngoscopic Surgeries,Laryngoscopic Surgical Procedure,Laryngoscopies,Procedure, Laryngoscopic Surgical,Procedures, Laryngoscopic Surgical,Surgeries, Laryngoscopic,Surgical Procedure, Laryngoscopic
D011241 Prednisone A synthetic anti-inflammatory glucocorticoid derived from CORTISONE. It is biologically inert and converted to PREDNISOLONE in the liver. Dehydrocortisone,delta-Cortisone,Apo-Prednisone,Cortan,Cortancyl,Cutason,Dacortin,Decortin,Decortisyl,Deltasone,Encorton,Encortone,Enkortolon,Kortancyl,Liquid Pred,Meticorten,Orasone,Panafcort,Panasol,Predni Tablinen,Prednidib,Predniment,Prednison Acsis,Prednison Galen,Prednison Hexal,Pronisone,Rectodelt,Sone,Sterapred,Ultracorten,Winpred,Acsis, Prednison
D004630 Emergencies Situations or conditions requiring immediate intervention to avoid serious adverse results. Emergency
D005260 Female Females
D005938 Glucocorticoids A group of CORTICOSTEROIDS that affect carbohydrate metabolism (GLUCONEOGENESIS, liver glycogen deposition, elevation of BLOOD SUGAR), inhibit ADRENOCORTICOTROPIC HORMONE secretion, and possess pronounced anti-inflammatory activity. They also play a role in fat and protein metabolism, maintenance of arterial blood pressure, alteration of the connective tissue response to injury, reduction in the number of circulating lymphocytes, and functioning of the central nervous system. Glucocorticoid,Glucocorticoid Effect,Glucorticoid Effects,Effect, Glucocorticoid,Effects, Glucorticoid
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000402 Airway Obstruction Any hindrance to the passage of air into and out of the lungs. Choking,Airway Obstructions,Obstruction, Airway,Obstructions, Airway
D012507 Sarcoidosis An idiopathic systemic inflammatory granulomatous disorder comprised of epithelioid and multinucleated giant cells with little necrosis. It usually invades the lungs with fibrosis and may also involve lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands. Besnier-Boeck Disease,Boeck's Sarcoid,Besnier-Boeck-Schaumann Syndrome,Boeck Disease,Boeck's Disease,Schaumann Disease,Schaumann Syndrome,Schaumann's Syndrome,Besnier Boeck Disease,Besnier Boeck Schaumann Syndrome,Boeck Sarcoid,Boecks Disease,Boecks Sarcoid,Disease, Schaumann,Sarcoid, Boeck's,Sarcoidoses,Schaumann's Syndromes,Syndrome, Besnier-Boeck-Schaumann,Syndrome, Schaumann,Syndrome, Schaumann's

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