Case Report

Laryngeal sarcoidosis causing acute upper airway obstruction

Abstract

Sarcoidosis is a chronic granulomatous disease affecting people worldwide. Rarely, it can affect the larynx, causing edema and thickening of the supraglottic structures resulting in upper airway obstruction. A 34-year-old African-Amer- ican man presented to the ED with stridor, dysphonia, and chronic cough. He had been treated for chronic sinus congestion with inhalers but his symptoms progressed. In the ED, he was treated with nebulized racemic epinephrine and intravenous steroids while computed tomography scan and laboratory exams were obtained. Computed tomography scan and fiberoptic laryngoscopy performed in the ED showed a severely thickened right aryepiglottic fold with central epiglottic swelling which had caused narrowing of the supraglottic airway. The patient reported symptomatic relief in the ED with medical management. In consult with an ear- nose-throat specialist, the decision was made to continue medical therapy and postpone emergent intubation and/or cricothyrotomy because sarcoid has a high rate of sponta- neous remission. However, on day 3, the patient had progressive dyspnea and required emergent tracheotomy in the operating room.

Laryngeal sarcoidosis should be considered in any patient with upper airway obstruction and a history of sarcoidosis or hilar adenopathy on chest radiograph. Fiberoptic laryngo- scopy and neck computed tomography scan may help assess laryngeal structures. Pale and diffusely edematous supra- glottic structures are pathognomonic for sarcoidosis. Intuba- tion of such a patient must be considered a difficult airway and the emergency physician should be prepared to perform a Surgical airway if oral intubation attempts fail. Patients who are observed without prophylactic intubation should be monitored in the intensive care unit with surgical airway equipment readily available.

Sarcoidosis is a chronic granulomatous disease affecting people worldwide, with African Americans having the highest prevalence. The disease can be acute, with a high

rate of spontaneous remission, or chronic, with organ- specific manifestations and a more sinister course [1].

Rarely laryngeal sarcoidosis causes edema and thickening of the supraglottic structures resulting in upper airway obstruction (UAO) [2,3]. Management of this disease presents a difficult challenge for emergency physicians (EPs). A 34-year-old African American man presented to the ED with progressive shortness of breath and voice changes. He was treated by his physician for chronic sinus congestion and cough with inhalers for weeks but these were no longer effective. Past medical history was significant for sarcoidosis, diagnosed 18 months previously. There were no additional medical

problems, family history, or smoking/alcohol/drug use.

Initial examination showed temperature of 36.8?C, heart rate of 93 beats per minute, blood pressure of 136/87 mm Hg, respiratory rate of 16, room air oxygen saturation of 99%. The patient was resting comfortably in a chair, talking in complete sentences, with no respiratory distress despite inspiratory stridor and hoarseness. Breath sounds were slightly dimin- ished. The patient had chronic cervical lymphadenopathy. The oropharynx was normal as was the remainder of the physical exam.

Initial treatment for UAO was nebulized racemic epinephrine and intravenous methylprednisolone. Although his stridor persisted, the patient symptomatically improved. Neck and chest computed tomography scan, ordered to further evaluate possible obstructive processes, showed cervical and hilar adenopathy and a severely thickened right aryepiglottic fold with epiglottic swelling narrowing the supraglottic airway (Figs. 1 and 2). Differential diagnosis included epiglottitis or laryngeal sarcoidosis. Ceftriaxone was administered empirically.

The patient remained stable in the ED for 4 hours. The EPs performed bedside fiberoptic laryngoscopy. Supraglot- tic swelling was consistent with CT findings. Oral intubation was predicted to be difficult. Given the slow progression of symptoms and symptomatic improvement in the ED, emergent intubation and/or cricothyrotomy was not performed. The on-call otolaryngologist was consulted and agreed with medical management in the intensive care unit. The surgical team was alerted and a surgical airway tray was ordered to bedside as a precaution. The patient’s condition remained unchanged until day 3 when he developed agitation and increased work of

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114.e2 Case Report

breathing. Anesthesiology attempted oral intubation and ultimately required an ear-nose-throat specialist to perform a tracheotomy emergently in the operating room. The patient did well and on day 7 was discharged on oral steroids with close follow-up.

Sarcoidosis is a disease that involves the formation of noncaseating granulomas. A genetic predisposition combined with an environment trigger is thought to be responsible for the disease process [3]. Most commonly the Pulmonary system, the skin, the eyes, and the lymphatics are affected. Differential diagnosis includes tuberculosis, histoplasmosis, blastomycosis, syphilis, actinomycosis, neoplasm, Wegen- er’s granulomatosis, amyloidosis, and berylliosis [4]. angiotensin-converting enzyme inhibitor levels and ery- throcyte sedimentation rate can both be elevated during acute exacerbation and can be useful to monitor a response to therapy [5].

Sarcoidosis affects the upper respiratory tract in approxi- mately 6% of cases. Laryngeal involvement is seen less frequently in 0.9% to 5% of cases [6]. Rich lymphatics in the supraglottic region favor involvement of the epiglottis, aretenoids, and false vocal cords. The true vocal cords are rarely affected [5].

Common presenting symptoms of laryngeal sarcoidosis include dysphagia, dysphonia, and cough [4,7]. Patients may describe a lump in throat sensation, chronic nasal congestion, or dyspnea [2]. Gradual onset of symptoms and lack of sore throat support sarcoid or neoplasm over infection [8]. Fiberoptic images frequently reveal an enlarged, pale, and edematous epiglottis, often with turban-like thickening, which is pathognomonic for sarcoidosis [9].

Fig. 1 Computed tomography scan showing swelling of the epiglottis and narrowing of the supraglottic airway.

Fig. 2 Computed tomography scan showing severe swelling of the right aryepiglottic fold and narrowing of the supraglottic airway.

The mainstay of treatment is Systemic corticosteroids [5]. Steroid nonresponders may benefit from investigational therapies: clofazamine, Cytotoxic agents (methotrexate, azathioprine), intralesion steroid injection, radiation therapy, CO₂ laser, or surgical excision [10,11]. Racemic epinephrine may promote vasoconstriction and reduce edema. Early consultation with an ear-nose-throat specialist is warranted in all patients owing to the potential need for surgical manage- ment of the airway or intralesional steroid injections [12].

Intubation of a patient with laryngeal sarcoid must be considered a difficult airway. The EP should be prepared for cricothyrotomy if intubation fails [8]. Alternatively, an awake intubation with sedation but no paralytic agent is an option [13]. If time warrants, the attempt at intubation can be made in the operating room [14]. In one study, successful intubation was achieved in only one of four patients with airway compromise; the remaining three required tracheot- omy [8]. Patients with symptoms of stridor who are being observed without prophylactic intubation should be admitted to an intensive care unit with surgical airway equipment readily available.

This case illustrates the life-threatening nature of laryngeal sarcoidosis and the difficult decisions it presents to the EP. Laryngeal sarcoidosis should be considered in a patient with UAO and a history of sarcoidosis or hilar adenopathy on chest radiograph. Fiberoptic laryngoscopy and neck CT may help assess laryngeal structures. ED treatment includes Systemic corticosteroids, nebulized racemic epinephrine, and otolar- yngology consultation. The EP must monitor the patient’s airway status closely while prepared to intervene with intubation or cricothyrotomy if acute obstruction occurs.

Case Report 114.e3

Carrie Davis MD Daniel V. Girzadas Jr MD Department of Emergency Medicine Advocate Christ Medical Center Oak Lawn, IL 60453, USA

E-mail addresses: [email protected];

[email protected]

doi:10.1016/j.ajem.2007.06.030

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