Progressive Upper Airway Obstruction and Dysphagia in a Child With Supraglottic Edema

Abstract

A 9-year-old, vaccinated boy with glucose-6-phosphate dehydrogenase deficiency and β-thalassemia trait presented with 1 month of progressive dyspnea, snoring, dysphagia, and weight loss. Both parents had a history of latent tuberculosis infection treated after immigrating to the US from Syria shortly after the patient's birth. The patient's pediatrician diagnosed him with reactive airway disease and adenotonsillar hypertrophy. He was referred to the otolaryngology clinic, where flexible laryngoscopy revealed diffuse upper airway lymphoid hyperplasia and supraglottic edema. He was sent to the emergency department, where he was stridulous and afebrile with a normal white blood cell count and an elevated erythrocyte sedimentation rate. A computed tomography scan of the neck revealed diffuse edema and contrast enhancement of the nasopharynx, oropharynx, hypopharynx, and supraglottis. A chest radiograph was normal.The patient was taken urgently to the operating room for direct microlaryngoscopy (Figure 1A and B) and intubated. Results of biopsies and cultures of the tonsils, adenoids, and supraglottis revealed diffuse non-necrotizing granulomas (Figure 1C) and oropharyngeal flora with negative acid-fast bacilli and Grocott methenamine silver stains. He remained intubated, and treatment with systemic corticosteroids and antibiotics was initiated. During this time, he had a normal angiotensin-converting enzyme (ACE) level and normal titers for antinuclear antibody (ANA), cytoplasmic antineutrophilic cytoplasmic antibody, perinuclear antineutrophilic cytoplasmic antibody, antimyeloperoxidase antibody, antiproteinase 3 antibody,anti-double-strandedDNAantibody,Smithantibody,Sjogrenantibodies,andhistoplasma antibodies. He also had normal results on tuberculin purified protein derivative and interferon-γ release assay (QuantiFERON; Qiagen).

Uncommon presentation of isolated laryngeal sarcoidosis in a young man

“…Sarcoidosis is a chronic multisystemic granulomatous disease with unknown etiology that commonly affects the lungs, eyes, and lymph nodes [ 3 ]. Furthermore, the disease commonly presents in middle-age at the third to the fourth decade of life with a female predominance [ 4 , 5 ]. However, sarcoidosis affects the head and neck region in only 10%–15% of cases [ 4 ].…”

“…Furthermore, the disease commonly presents in middle-age at the third to the fourth decade of life with a female predominance [ 4 , 5 ]. However, sarcoidosis affects the head and neck region in only 10%–15% of cases [ 4 ]. Laryngeal involvement accounts for 0.5%–5% of sarcoid patients, while isolated laryngeal sarcoid is very uncommon [ 2 , 4 ].…”

“…However, sarcoidosis affects the head and neck region in only 10%–15% of cases [ 4 ]. Laryngeal involvement accounts for 0.5%–5% of sarcoid patients, while isolated laryngeal sarcoid is very uncommon [ 2 , 4 ]. Patients usually present with dysphagia, dyspnea, pharyngeal globus, dysphonia, and dry cough.…”

“…This occurs in 15% of the cases [ 6 , 7 ]. Flexible laryngoscopic examination usually shows pale and edematous mucosa mainly involving the epiglottis as the most common site with a characteristic turban-like appearance, which could be related to rich lymphatic supply to this tissue, followed by involvement of the arytenoids and aryepiglottic folds [ 4 ]. Involvement of the glottic and subglottic areas is less common, and vocal cord mobility could be affected in approximately 24% of cases either due to sarcoid infiltration of the larynx or direct neural involvement of the disease or by the compressive effect of lymphadenopathy [ 7 , 8 ].…”

“…However, due to limited data, treatment options for laryngeal sarcoidosis are still under investigation [ 4 ]. Systemic steroids are the first-line therapy, and prednisone (40–60 mg/day in adults) is the most favorable medication for a total period of 6–12 months [ 7 ].…”