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Bilateral cricoarytenoid joint ankylosis in scleroderma
Bilateral cricoarytenoid joint ankylosis in scleroderma DANIEL D. VINER, MD, ALAIN SABRI, MD, and HARVEY M. TUCKER, MD, Cleveland, Ohio
S
cleroderma is an uncommon disease of uncertain cause that is characterized by progressive fibrosis of multiple organ systems. Because of the pervasive nature of the disease, presentations can be varied. Mortality and morbidity are significant by virtue of the relentless progression of the systemic sclerosis. We present a case of acute respiratory distress as a result of a bilateral vocal fold fixation in a patient with advanced scleroderma. CASE REPORT A 40-year-old white man with known scleroderma presented to the internal medicine clinic complaining of subacute progressive dysphagia and acute progressive dyspnea. He had modified his diet at home to avoid thin liquids and noted that his voice “sounded older” for the few days before admission. He denied subjective aspiration, fevers, and chest pain. His scleroderma had been diagnosed 31 years previously, and, subsequently, he had developed several classic complications of the disease. All of his digits had autoamputated, and severe vascular compromise had necessitated bilateral abovethe-knee amputations. He had chronic pain and recalcitrant gastroesophageal reflux as well as esophageal strictures that required dilation every few months. The last dilation had been performed only 10 days before this presentation. On physical examination, he demonstrated the stigmata of scleroderma and was in mild respiratory distress with tachypnea and mild inspiratory stridor. He was admitted directly to the medical intensive care unit and received racemic epinephrine and oxygen with adequate response. By morning, his dyspnea and stridor had worsened, and the otolaryngology department was consulted. The patient was in mild respiratory distress with biphasic stridor and a weak, breathy voice. Fiberoptic laryngoscopy revealed bilateral immobile vocal folds nearly in the median position along with mild postcricoid edema and erythema. His airway was compromised, and an emergent tracheotomy and direct laryngoscopy were performed. At laryngoscopy, the arytenoid car-
From the Department of Otolaryngology–Head and Neck Surgery, Case Western Reserve University. Reprint requests: Harvey M. Tucker, MD, Department of Otolaryngology–Head and Neck Surgery, University Hospitals of Cleveland, 11100 Euclid Ave, Cleveland, OH 44106. Otolaryngol Head Neck Surg 2001;124:696-7. Copyright © 2001 by the American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc. 0194-5998/2001/$35.00 + 0 23/4/115910 doi:10.1067/mhn.2001.115910 696
Fig 1. Photomicrograph of laryngeal biopsy demonstrating mucosal and submucosal infiltration with chronic inflammatory cells.
tilages were firmly fixed in essentially full adduction. The mucosa over the right arytenoid was biopsied and revealed a nonspecific chronic inflammation (Fig 1). The patient breathed without difficulty through the tracheotomy. DISCUSSION
The cricoarytenoid joint is a true synovial joint and, therefore, is susceptible to local and systemic diseases of the synovium. Rheumatoid arthritis is most commonly associated with cricoarytenoid joint involvement; 25% to 35% of rheumatoid arthritis patients have symptomatic cricoarytenoid arthritis, although postmortem histological examination of rheumatoid larynges finds evidence of cricoarytenoid arthritis in more than 50% of specimens.1 When symptomatic, patients may complain of pain on phonation, globus sensation, hoarseness, dysphagia, odynophagia, otalgia, or dysp-
Otolaryngology– Head and Neck Surgery Volume 124 Number 6
nea.2 Physical examination during acute episodes may reveal inspiratory or biphasic stridor and, occasionally, respiratory distress.2 Laryngoscopy demonstrates restricted vocal fold mobility as well as erythematous, edematous arytenoid cartilages.2 In a chronically inflamed larynx, cricoarytenoid arthritis can progress to ankylosis, and the overlying mucosa may appear thickened and coarse.2 Although most commonly associated with rheumatoid arthritis, cricoarytenoid arthritis has been described in gout,3 Crohn’s disease,3 systemic lupus erythematosis,3 ankylosing spondylitis,3 infections (eg, mumps3 and Streptococcus4), Teitze’s syndrome,3 and Reiter’s syndrome.3 The management of cricoarytenoid arthritis centers around securing a safe airway and, secondarily, relieving the arthritis. When respiratory distress is present, emergent tracheotomy may be necessary.2,5 If the airway is not in jeopardy, the arthritis can be treated with antiinflammatory medications such as oral steroids.5 Nonemergent surgical options include arytenoidectomy or arytenoidopexy.2 Good results have also been described with periarticular steroid injection in selected cases.5 Arthritis is not a common feature of scleroderma; when present in acral joints, scleroderma-associated arthritis resembles rheumatoid arthritis. The synovium demonstrates an infiltrate of lymphocytes and plasma cells, and there is a thick layer of fibrin deposited in the joint space that can eventually lead to fibrous ankylosis.6 If such fibrous ankylosis were to occur in the cricoarytenoid joint, the airway could be compromised, necessitating tracheotomy.
VINER et al
697
Presumably, in this case, the cricoarytenoid joints had been chronically inflamed and were undergoing gradual ankylosis. Most likely, one of the joints was fixed earlier in time, perhaps in the median position. For a few days before this presentation, the other joint may have been nearly fixed in a more lateral position, allowing for an adequate airway while yielding to the “older” vocal character that was described. Then, with a strong adducting force such as a cough, more lateral fold shifted into a median position and was unable to lateralize, improving the voice but also establishing a critical airway stenosis at the glottis, which mandated emergent tracheotomy. SUMMARY
We report an unusual case of upper airway obstruction due to bilateral cricoarytenoid joint fixation in a patient with scleroderma. To our knowledge, there are no previous reports of cricoarytenoid joint ankylosis due to scleroderma in the English-language literature. REFERENCES 1. Bridger MWM, Jahn AF, van Nostrand AWP. Laryngeal rheumatoid arthritis. Laryngoscope 1980;90:296-303. 2. Montgomery WW. Cricoarytenoid arthritis. Laryngoscope 1963; 73:801-36. 3. Miller FR, Wanamaker JR, Hicks DM, et al. Cricoarytenoid arthritis and ankylosing spondylitis. Arch Otolaryngol 1994;120:214-6. 4. Berger AJ, Calcaterra VE. Septic cricoarytenoid arthritis. Otolaryngol Head Neck Surg 1983;91:211-3. 5. Simpson GT, Javaheri A, Janfaza P. Acute cricoarytenoid arthritis: local periarticular steroid injection. Ann Otol Rhinol Laryngol 1980;89:558-562. 6. Blocka KLN, Bassett LW, Furst DE, et al. The arthropathy of advanced progressive systemic sclerosis. Arthritis Rheum 1981; 24:874-84.
S
cleroderma is an uncommon disease of uncertain cause that is characterized by progressive fibrosis of multiple organ systems. Because of the pervasive nature of the disease, presentations can be varied. Mortality and morbidity are significant by virtue of the relentless progression of the systemic sclerosis. We present a case of acute respiratory distress as a result of a bilateral vocal fold fixation in a patient with advanced scleroderma. CASE REPORT A 40-year-old white man with known scleroderma presented to the internal medicine clinic complaining of subacute progressive dysphagia and acute progressive dyspnea. He had modified his diet at home to avoid thin liquids and noted that his voice “sounded older” for the few days before admission. He denied subjective aspiration, fevers, and chest pain. His scleroderma had been diagnosed 31 years previously, and, subsequently, he had developed several classic complications of the disease. All of his digits had autoamputated, and severe vascular compromise had necessitated bilateral abovethe-knee amputations. He had chronic pain and recalcitrant gastroesophageal reflux as well as esophageal strictures that required dilation every few months. The last dilation had been performed only 10 days before this presentation. On physical examination, he demonstrated the stigmata of scleroderma and was in mild respiratory distress with tachypnea and mild inspiratory stridor. He was admitted directly to the medical intensive care unit and received racemic epinephrine and oxygen with adequate response. By morning, his dyspnea and stridor had worsened, and the otolaryngology department was consulted. The patient was in mild respiratory distress with biphasic stridor and a weak, breathy voice. Fiberoptic laryngoscopy revealed bilateral immobile vocal folds nearly in the median position along with mild postcricoid edema and erythema. His airway was compromised, and an emergent tracheotomy and direct laryngoscopy were performed. At laryngoscopy, the arytenoid car-
From the Department of Otolaryngology–Head and Neck Surgery, Case Western Reserve University. Reprint requests: Harvey M. Tucker, MD, Department of Otolaryngology–Head and Neck Surgery, University Hospitals of Cleveland, 11100 Euclid Ave, Cleveland, OH 44106. Otolaryngol Head Neck Surg 2001;124:696-7. Copyright © 2001 by the American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc. 0194-5998/2001/$35.00 + 0 23/4/115910 doi:10.1067/mhn.2001.115910 696
Fig 1. Photomicrograph of laryngeal biopsy demonstrating mucosal and submucosal infiltration with chronic inflammatory cells.
tilages were firmly fixed in essentially full adduction. The mucosa over the right arytenoid was biopsied and revealed a nonspecific chronic inflammation (Fig 1). The patient breathed without difficulty through the tracheotomy. DISCUSSION
The cricoarytenoid joint is a true synovial joint and, therefore, is susceptible to local and systemic diseases of the synovium. Rheumatoid arthritis is most commonly associated with cricoarytenoid joint involvement; 25% to 35% of rheumatoid arthritis patients have symptomatic cricoarytenoid arthritis, although postmortem histological examination of rheumatoid larynges finds evidence of cricoarytenoid arthritis in more than 50% of specimens.1 When symptomatic, patients may complain of pain on phonation, globus sensation, hoarseness, dysphagia, odynophagia, otalgia, or dysp-
Otolaryngology– Head and Neck Surgery Volume 124 Number 6
nea.2 Physical examination during acute episodes may reveal inspiratory or biphasic stridor and, occasionally, respiratory distress.2 Laryngoscopy demonstrates restricted vocal fold mobility as well as erythematous, edematous arytenoid cartilages.2 In a chronically inflamed larynx, cricoarytenoid arthritis can progress to ankylosis, and the overlying mucosa may appear thickened and coarse.2 Although most commonly associated with rheumatoid arthritis, cricoarytenoid arthritis has been described in gout,3 Crohn’s disease,3 systemic lupus erythematosis,3 ankylosing spondylitis,3 infections (eg, mumps3 and Streptococcus4), Teitze’s syndrome,3 and Reiter’s syndrome.3 The management of cricoarytenoid arthritis centers around securing a safe airway and, secondarily, relieving the arthritis. When respiratory distress is present, emergent tracheotomy may be necessary.2,5 If the airway is not in jeopardy, the arthritis can be treated with antiinflammatory medications such as oral steroids.5 Nonemergent surgical options include arytenoidectomy or arytenoidopexy.2 Good results have also been described with periarticular steroid injection in selected cases.5 Arthritis is not a common feature of scleroderma; when present in acral joints, scleroderma-associated arthritis resembles rheumatoid arthritis. The synovium demonstrates an infiltrate of lymphocytes and plasma cells, and there is a thick layer of fibrin deposited in the joint space that can eventually lead to fibrous ankylosis.6 If such fibrous ankylosis were to occur in the cricoarytenoid joint, the airway could be compromised, necessitating tracheotomy.
VINER et al
697
Presumably, in this case, the cricoarytenoid joints had been chronically inflamed and were undergoing gradual ankylosis. Most likely, one of the joints was fixed earlier in time, perhaps in the median position. For a few days before this presentation, the other joint may have been nearly fixed in a more lateral position, allowing for an adequate airway while yielding to the “older” vocal character that was described. Then, with a strong adducting force such as a cough, more lateral fold shifted into a median position and was unable to lateralize, improving the voice but also establishing a critical airway stenosis at the glottis, which mandated emergent tracheotomy. SUMMARY
We report an unusual case of upper airway obstruction due to bilateral cricoarytenoid joint fixation in a patient with scleroderma. To our knowledge, there are no previous reports of cricoarytenoid joint ankylosis due to scleroderma in the English-language literature. REFERENCES 1. Bridger MWM, Jahn AF, van Nostrand AWP. Laryngeal rheumatoid arthritis. Laryngoscope 1980;90:296-303. 2. Montgomery WW. Cricoarytenoid arthritis. Laryngoscope 1963; 73:801-36. 3. Miller FR, Wanamaker JR, Hicks DM, et al. Cricoarytenoid arthritis and ankylosing spondylitis. Arch Otolaryngol 1994;120:214-6. 4. Berger AJ, Calcaterra VE. Septic cricoarytenoid arthritis. Otolaryngol Head Neck Surg 1983;91:211-3. 5. Simpson GT, Javaheri A, Janfaza P. Acute cricoarytenoid arthritis: local periarticular steroid injection. Ann Otol Rhinol Laryngol 1980;89:558-562. 6. Blocka KLN, Bassett LW, Furst DE, et al. The arthropathy of advanced progressive systemic sclerosis. Arthritis Rheum 1981; 24:874-84.