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

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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.

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B. Sarcoidosis

Sarcoidosis is a chronic granulomatous disease of unclear etiology. Its prevalence is about 10 to 35 cases per 100 000 people, with a significant African American and female predominance.1 The disease tends to affect those in the third or fourth decade of life, most often manifesting as cough or dyspnea.2 Sarcoidosis affects the head and neck region in 10% to 15% of patients and usually presents as cervical lymphadenopathy.3

The reported rate of laryngeal sarcoidosis ranges from 0.5% to 5% of all patients with sarcoidosis, and isolated laryngeal sarcoidosis is rarer still.2,4 Symptoms may include hoarseness, dyspnea, snoring, dysphagia, globus, and throat pain.2 Laryngoscopy shows pale edema of the epiglottis most commonly, followed by the arytenoids and aryepiglottic folds. The epiglottis may have a characteristic turbanlike appearance, as seen in this patient (Figure 1A).5 The glottis and subglottis are less frequently involved, likely owing to less lymphoid tissue in these areas.2,5 While sarcoidosis is a diagnosis of exclusion, serum ANA and ACE levels may be elevated, although the latter has low sensitivity (60%).2 Because ACE is primarily secreted by pulmonary endothelial cells and alveolar macrophages, levels may be normal in patients without pulmonary involvement.6 Histopathology will reveal noncaseating granulomas, as it did in this patient.

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Article Information

Corresponding Author: Scott A. Hong, MD, Department of Otolaryngology–Head and Neck Surgery, St Louis University Hospital, 1008 S Spring Ave, 3rd Floor, St Louis, MO 63104 (scott.hong@health.slu.edu).

Published Online: September 3, 2020. doi:10.1001/jamaoto.2020.2577

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s parent for granting permission to publish this information.

References
1.
Leedman  SR , Hendriks  T , Leahy  TW , Hall  D .  Supraglottic laryngeal sarcoidosis masquerading as supraglottitis.   BMJ Case Rep. 2020;13(1):e232369. doi:10.1136/bcr-2019-232369PubMedGoogle Scholar
2.
Dean  CM , Sataloff  RT , Hawkshaw  MJ , Pribikin  E .  Laryngeal sarcoidosis.   J Voice. 2002;16(2):283-288. doi:10.1016/S0892-1997(02)00099-1PubMedGoogle ScholarCrossref
3.
Badhey  AK , Kadakia  S , Carrau  RL , Iacob  C , Khorsandi  A .  Sarcoidosis of the head and neck.   Head Neck Pathol. 2015;9(2):260-268. doi:10.1007/s12105-014-0568-yPubMedGoogle ScholarCrossref
4.
Ahmadi  A , Dehghani Firouzabadi  F , Dehghani Firouzabadi  M , Roomiani  M .  Isolated laryngeal sarcoidosis.   Respirol Case Rep. 2019;8(1):e00502.PubMedGoogle Scholar
5.
van den Broek  EMJM , Heijnen  BJ , Verbist  BM , Sjögren  EV .  Laryngeal sarcoidosis: a case report presenting transglottic involvement.   J Voice. 2013;27(5):647-649. doi:10.1016/j.jvoice.2013.03.010PubMedGoogle ScholarCrossref
6.
Mandel  L , Wolinsky  B , Chalom  EC .  Treatment of refractory sarcoidal parotid gland swelling in a previously reported unresponsive case.   J Am Dent Assoc. 2005;136(9):1282-1285. doi:10.14219/jada.archive.2005.0345PubMedGoogle ScholarCrossref
7.
Strychowsky  JE , Vargas  SO , Cohen  E , Vielman  R , Son  MB , Rahbar  R .  Laryngeal sarcoidosis: presentation and management in the pediatric population.   Int J Pediatr Otorhinolaryngol. 2015;79(9):1382-1387. doi:10.1016/j.ijporl.2015.06.017PubMedGoogle ScholarCrossref
8.
Butler  CR , Nouraei  SA , Mace  AD , Khalil  S , Sandhu  SK , Sandhu  GS .  Endoscopic airway management of laryngeal sarcoidosis.   Arch Otolaryngol Head Neck Surg. 2010;136(3):251-255. doi:10.1001/archoto.2010.16PubMedGoogle ScholarCrossref
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