Cricoarytenoid arthritis in children NEAL D. FUTRAN, MD, DMD, DAVID SHERRIS, MD, and JOHN D. NORANTE, MD, Rochester, New York
c r i c o a r y t e n o i d arthritis (CA) is clinically present in 17% to 54% of adult patients with rheumatoid arthritis. l4 Autopsy evidence of cricoarytenoid arthritis has been found in 45% to 86% of adult patients with three well-documented rheumatoid a r t h r i t i ~ . Only ~ cases of cricoarytenoid arthritis in children have been One other review mentions three cases in a series of 100 patients with Still’s disease, but provides no documentation.’ We present a 4%-year-old girl with upper airway obstruction resulting from cricoarytenoid arthritis. In the context of this case, we review all other reported cases of cricoarytenoid arthritis in children with juvenile rheumatoid arthritis, and comment o n diagnosis and treatment of this disorder in children. Clearly, the most important factor in considering this unusual entity is the need for prompt, early diagnosis. Early diagnosis, along with aggressive medical therapy, may circumvent the need for tracheotomy in these patients. CASE REPORT
H.D., a 4%-year-old girl, was first diagnosed with juvenile rheumatoid arthritis (JRA) in 1986, at 18 months of age. At that time, she had a I-month history of fever, truncal rash, generalized lymphadenopathy, and bilateral wrist and knee arthritides. Antinuclear antibodies and rheumatoid factor were negative, and erythrocyte sedimentation rate (ESR) was 90. She responded well to aspirin and tolectin (a nonsteroidal anti-inflammatory drug [NSAID]) and these drugs were tapered off until the patient went into remission in May 1987. The patient remained well until July 1988, when she manifested daily fevers, truncal rash, and polyarthritis. Additionally, she reported dyspnea, and was found to have a large heart shadow and bilateral pleural effusions on chest x-ray film. Echocardiography confirmed the diagnosis of pericardial effusion, and this entity was treated effectively with pericardiocentesis. She again responded well to NSAID (naprosyn) therapy, and all symptoms subsided. From the Division of Otolaryngology-Head and Neck Surgery, Strong Memorial Hospital, University of Rochester. Received for publication Nov. 24, 1989; revision received June 11, 1990; accepted June 12, 1990. Reprint requests: John D. Norante, MD, Division of OtolaryngologyHead and Neck Surgery, Strong Memorial Hospital, 601 Elmwood Ave., Box 629, Rochester, NY 14642. 23/4/23109
On September 29, 1988, the patient was brought to the emergency department in acute upper airway distress, with stridor, substernal retractions, nasal flaring, air hunger, and anxiety. The parents stated that over the 2 to 3 weeks before this presentation, noisy breathing developed in the patient at night, and would resolve each morning when the patient awoke. The duration of noisy breathing increased each subsequent day, until the day of presentation to the emergency department, when the patient had continuous noisy breathing and shortness of breath. Auscultatory examination in the emergency department revealed no wheezing, good air exchange, and inspiratory stridor. The patient was afebrile and also reported bilateral wrist and knee pain. Lateral and anterior neck films revealed a normal epiglottis shadow, subglottic narrowing, and ballooning of the hypopharynx (Figs. 1 and 2). Laboratory findings at the time included ESR = 84, white blood cell count = 17,100, arterial blood gas on 2 L/min oxygen via nasal cannula of pH 7.48, pC0, = 33, PO, = 121, and 98% saturation. The patient was given racemic epinephrine without relief, and was then transferred to the operating room for airway management. In the operating room, general anesthesia was induced. Initial direct laryngoscopy before intubation revealed a small glottic chink resulting from bilateral vocal cord immobility. The patient was orally intubated without difficulty. Tracheotomy was immediately performed because of the inadequacy of the glottic airway, and a no. 2 pediatric Shiley tracheotomy tube was placed without difficulty or complication. We believed that the presence of an indwelling endotracheal tube could only exacerbate the acute inflammatory process and possibly lead to a chronic inflammatory process. It was thought that tracheotomy would help to avoid this sequela and could be maintained indefinitely, awaiting the return of vocal cord mobility. With the endotracheal tube out of the airway, the larynx was visualized with an anterior commissure laryngoscope. With the patient paralyzed, there was a slit-like airway present. The right arytenoid cartilage appeared erythematous, and the left appeared normal. Each arytenoid cartilage was palpated with a laryngeal spatula separately, and both were found to be completely fixed. The paralysis was reversed and the patient was allowed to awaken. Both vocal cords were fixed in the paramedian position and did not abduct with inspiratory effort. The slit-like airway remained unchanged, and there was no evidence of laryngospasm. Subglottic narrowing was not apparent at the time of endoscopic examination. The diagnosis of cricoarytenoid arthritis (CA) was supported. The patient had a stable postoperative course and was started on NSAID (naprosyn), intravenous corticosteroids,
366 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 5, 2016
Volume I W Number 3 March 1991
Case Reports
i Fig. 1. Portable anterior-posterior chest radiograph shows significont subglottic norrowing
Fig. 2. Portable cross-table lateral neck radiogroph shows bollooning of the hypopharynx. N o epiglottic edema or thickening.
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367
OtolaryngologyHead and Neck Surgery
368 CaseReports
Table 1. Comparison of clinical findings* JRA classifications and subjects' ages in years Systemic (4.5 Yr)
Systemic findings
Neck radiograph
Laryngoscopy findings
Polyarthritis Pericarditis Pleuritis Truncal rash Fever Subglottic narrowing, ballooning of hypopharynx Vocal cords adducted and immobile, fixed arytenoid cartilages, erythema, and edema, R > L
ESR Airway control
84 Tracheotomy
Medical therapy
Naprosyn, gold, intravenous and oral corticosteroids Decannulated in 2.5 months, normal voice, oral NSAID, gold and steroid therapy Present study
Outcome
Source
Pauciarticuiar
Pauciarticular (4.5 v )
(2.3vr)
Systemic (3.25 yr)
Sternal swelling along costochondral junction
Left knee effusion with flexion/contracture
Polyarthritis Pericarditis Truncal rash Fever
Subglottic narrowing, distention of the hypopharynx Vocal cords adducted and immobile, supraglottic edema and erythema with funnelshaped appearance Not reported Overnight intubation, subsequent tracheotomy after several recurrences Intravenous and oral corticosteroids Oral antibiotic
Narrowing of the glottic region
Not reported
Vocal cords adducted and immobile, fixed arytenoid cartilages with edema and erythema, R > L
Left vocal cord adducted and immobile, fixed left arytenoid cartilage
Not reported 02 tent
Tracheotomy
118
Aspirin, oral corticosteroids
Aspirin, intravenous and oral corticosteroids
Maintained tracheotomy Unknown medications
Symptom free, aspirin and oral steroid therapy
Decannulated in 10 days, hoarse voice Aspirin therapy
Goldhagen (1988)
Malleson et al. (1986)
Jacobs and Hui (1977)
_
_
_
_
~
*All subjects in comparison are female JRA, Juvenile rheumatoid arthritis,ESR.
erythrocyte sedimentation rate, NSAID, nonsteroidal anti-inflammatory drug
and gold therapy. She was discharged on postoperative day 5 , with a tracheotomy tube in place. The other systemic symptoms of JRA resolved with continued outpatient NSAID, oral steroid, and gold therapy. On October 31, 1988, the patient underwent elective direct laryngoscopy with the anterior commissure laryngoscope under sedation, but with spontaneous respirations. No movement of the left vocal cord was seen. The right vocal cord had moderate lateral excursion. Both arytenoid cartilages appeared mildly erythematous, and the tracheotomy was left in place because of the inadequacy of the airway. The patient continued on NSAID, oral steroid, and gold therapy, with no further exacerbation of disease. In December 1988, flexible fiberoptic laryngoscopy was performed while the girl was under mild sedation. The ary-
tenoids appeared normal, and both vocal cords and arytenoid cartilages had full, symmetric range of motion. The patient was decannulated, the tracheotomy site healed well, and the patient currently speaks well. She has had no further clinical evidence of CA and is now on tapering doses of medications. As of December 1990, this patient's arthritis remains wellcontrolled with low-dose oral NSAID therapy.
DISCUSSION
The cricoarytenoid joint is a multiaxial, diathrodial joint that allows motion between the arytenoid cartilages and the cricoid cartilage during phonation and respiration. The structure and mechanics of the joint are well presented by other authors.I0." Arthritis of the
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Volume 104 Number 3 March 1991
CaseReports 369
subglottic narrowing and distention of the hypopharcricoarytenoid joint was first described by Mac Kenzie ynx. The other patient demonstrated only glottic narin 1880.12 Since then, this clinical entity has been derowing. The measurements of this narrowing was not scribed in adult patients numerous times. 1 3 ~ 1 4The sympdocumented in any of the other cases. Our patient’s toms of CA in adults are fullness or tension in the throat subglottic airway measured 3 mm radiographically in aggravated by swallowing, hoarseness, odynophagia, the anteroposterior projection. Normal measurements pain with speaking and coughing, pain radiating to the of the subglottic airway in a patient this age should be ears, tenderness of the thyroid cartilage, dyspnea, and 8 mm in the anteroposterior dimen~ion.~’ inspiratory stridor.” The stridor is worse during sleep and sedation because of the decreased voluntary abDirect laryngoscopy (DL) results corrolated well in duction of the vocal cords in these relaxed ~ t a t e s . ~ , ” . ’ ~all four cases. DL results were characterized by deDefinitive diagnosis of CA is by laryngoscopy. Lacreased vocal cord mobility, erythema and swelling of ryngoscopic findings in adults in the acute phase of CA one or both arytenoid cartilages, fixation of one or both include erythematous, swollen arytenoids, normal or arytenoid cartilages in adduction, and a slit-like airway slightly edematous vocal cords, variable vocal cord moin cases of bilateral arytenoid cartilage fixation. The bility, and immobility and tenderness of the arytenoid subglottic narrowing noted on radiographs was not vicartilages on palpation with a laryngeal ~patula.~.” Lasualized during endoscopy in our case. This may have ryngoscopic findings in the chronic stage of disease in represented subglotticedema that resolved with medical adults include thickening of the mucosa overlying the therapy before surgical intervention. Subglotticnarrowarytenoids, narrow vocal cord chink, and midline, iming was not addressed in the other case reports, and the mobile arytenoid cartilages by the spatula t e ~ t . ~ . ’ ~ etiology of that radiographic finding remains unclear. Though this entity is well described in adults, there The narrowness of the airway in all of the cases dictates are only three well-documented cases in children .&’ concern over the ability to intubate these patients. The One other report mentions three patients in a group of use of an endotracheal tube one size smaller than ap100 patients with Still’s disease who exhibited inspipropriate for the age and size of the patient allows for ratory ~ t r i d o rYet, . ~ this last-mentioned report only doceasier intubation and provides an adequate temporary uments that one of the patients demonstrated “paralyzed airway. cords” on direct laryngoscopy, and required emergent All of the patients responded well to either steroids tracheotomy. Because of the poor documentation in this alone, or in combination with an NSAID or aspirin. latter report, only the three first-mentioned reports of There was mild, if any, decreased stridor reported with CA in children will be discussed. Table 1 presents perracemic epinephrine-nebulizer therapy. The excellent tinent information from the four (including ours) wellresponse to medical therapy seen in children with CA documented cases of CA in children. is not shared by adults who acquire this disease. Some adults with mild, acute airway compromise Interestingly, three of the four patients were female. have been reported to respond to anti-inflammatory Two of the patients had systemic JRA, while the other medications; antibiotics for exacerbating infections; hutwo had pauciarticular (three or less joints involved, midified, cool oxygen tents; and bedre~t.~’” Many minimal systemic symptoms) JRA. Of note here is that times, however, treatment failures occur, and progresthe three poorly documented cases all had systemic JRA sive airway obstructure ensues. Fatalities have been (Still’s disease).’ Each patient experienced inspiratory reported as a result of airway obstruction before adestridor that progressively worsened over a period of quate surgical intervention.I 3 . I 6 weeks, until medical intervention was sought. Three of the patients reportedly experienced worse stridor at In contrast to pediatric CA, adult-onset CA is charnight, while sleeping. When stridor became constant, acterized by chronic, insiduous fixation of the arytenoid acute upper airway distress occurred and was characcartilages. Acute exacerbations of this process are reterized by air hunger, nasal flaring, use of accessory sponsible for emergent airway compromise, and fremuscles of respiration, and apprehension. Three of the quently necessitate surgical intervention. The progrespatients required airway management consisting of eisive nature of the adult form of CA has lead many ther tracheotomy (early, two; late, one) or intubation authorities to recommend surgery in the form of tra(one). One patient was observed closely in an oxygen cheotomy, arytenoidectomy, or arytenoidopexy at the tent and responded to therapy quickly enough to avoid first sign of airway compromise, as gauged by diminformal airway management. In our case, fixation of the ished vocal chink.l 3 3 l 6 Though the reports of pediatric arytenoid cartilages in adduction dictated aggressive CA are limited, the reversibility of joint fixation with therapy. Lateral and anterior neck film results were medical therapy has been the rule with every case, and reported in three cases. Two cases were associated with we do not advocate routine, aggressive surgical therapy.
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Otolaryngology-
370 Case Reports
Head and Neck Surgery
Rather, if this entity can be diagnosed early, aggressive medical therapy consisting of corticosteroids alone or in combination with an NSAID or aspirin seems to be the treatment of choice. Tracheotomy may very well be avoided in many cases. As evidenced by the cases reported, CA manifests essentially the same signs, symptoms, and direct laryngoscopic findings in the pediatric patient as in the adult patient. Yet, CA is rarely reported in the pediatric patient, whereas 17% to 54% of adult patients with rheumatoid arthritis have clinical evidence of CA. Several factors may account for the disparity between adults and children. First, examination of the larynx in children is much more difficult than in adults. Mild-to-moderate cases of CA may be misdiagnosed because of the larynx not being directly examined. Because of the more common causes of pediatric stridor, CA may be misdiagnosed as viral croup, bacterial laryngotracheitis, retained foreign body, allergic edema, asthma, or epiglottitis. Additionally, nocturnal stridor may be overlooked by parents, or wrongly interpreted as snoring. Possibly, CA is an insidious process that normally takes years to develop, and these cases represent the rare exceptions from the rule.’ Also, it may be that CA is more common in patients with systemic JRA, as five cases have been reported (6,9 and our patient). Only two cases of CA have been reported in patients with pau~iarticular’~~ and no cases of CA have been reported in patients with polyarticular JRA. Thus, though rare, CA must be considered in the differential diagnosis of stridor, and more generally in the differential diagnosis of acute and chronic upper airway obstruction in children with JRA. Early diagnosis of the entity, along with immediate corticosteroid therapy-alone or in combination with NSAIDs-may prevent the need for emergent airway control and tracheotomy in affected patients.
REFERENCES 1. Junk AG, Pedersen U. Rheumatoid arthritis of the cricoarytenoid
2.
3.
4. 5.
6. 7.
8. 9.
10.
11. 12.
13. 14.
15. 16.
and cnco-thyroid joints: a radiologic and clinical study. Clin Radio1 1984;35:233-6. Lawry GV, Finerman ML, Hanafee WN, et al. Laryngeal involvement in rheumatoid arthritis: a clinical, laryngoscopic, and computerized tomographic study. Arthritis Rheum 1984;27:87382. Bienenstock H, Ehrlich GE, Freyberg RH.Rheumatoid arthritis of the cricoarytenoid joint: a clinicopathologic study. Arthritis Rheum 1963;6:48-63. Lofgren RH, Montgomery WW. Incidence of laryngeal involvement in rheumatoid atluitis. N Engl J Med 1962;267:193-5. Grossman A, Martin JR, Root HS. Rheumatoid arthritis of the cricoarytenoid joint. Laryngoscope 1961;71:530-44. Jacobs JC, Hui RM. Cricoarytenoid arthritis and airway obstruction in juvenile rheumatoid arthritis. Pediatrics 1977;59:292-4. Malleson P, Riding K, Petty R. Stridor due to cricoarytenoid arthritis in pauciarticular onset juvenile rheumatoid arthritis. J Rheum 1986;13:952-3. Goldhagen JL. Cricoarytenitis as a cause of acute airway obstruction in children. AM Emerg Med 1988;17:532-3. Schlesinger BE, Forsyth CC, White RHR, et al. Observations on the clinical course and treatment of one hundred cases of Still’s disease. Arch Dis Child 1961;36:65-76. Von Leden H, Moore P. The mechanics of the cricoarytenoid joint. Arch Otolaryngol 1961;73:541-55. Sellars I, Sellars S . Cncoarytenoid joint structure and function. J Laryngol Otol 1983;97:1027-34. Mac Kenzie M. Manual of disease of throat and nose, including pharynx, larynx, trachea, esophagus, nose, and nasopharynx. New York William Wood, 1880:347. Montgomery WW. Cricoarytenoid arthritis. Laryngoscope 1963; 73:801-36. Bridger MWM, Jahn AF, van Nostrand AWP. Laryngeal rheumatoid arthritis. Laryngoscope 1980;90:296-303. Bluestone CP, Stool SE. Pediatric otolaryngology. Philadelphia: WB Saunders, 1983;1109:1191-3. Leicht MS, Harrington CM, Davis DE. Cricoarytenoid arthritis: a cause of laryngeal obstruction. Ann Emerg Med 1987;16: 885-8.
Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 5, 2016
c r i c o a r y t e n o i d arthritis (CA) is clinically present in 17% to 54% of adult patients with rheumatoid arthritis. l4 Autopsy evidence of cricoarytenoid arthritis has been found in 45% to 86% of adult patients with three well-documented rheumatoid a r t h r i t i ~ . Only ~ cases of cricoarytenoid arthritis in children have been One other review mentions three cases in a series of 100 patients with Still’s disease, but provides no documentation.’ We present a 4%-year-old girl with upper airway obstruction resulting from cricoarytenoid arthritis. In the context of this case, we review all other reported cases of cricoarytenoid arthritis in children with juvenile rheumatoid arthritis, and comment o n diagnosis and treatment of this disorder in children. Clearly, the most important factor in considering this unusual entity is the need for prompt, early diagnosis. Early diagnosis, along with aggressive medical therapy, may circumvent the need for tracheotomy in these patients. CASE REPORT
H.D., a 4%-year-old girl, was first diagnosed with juvenile rheumatoid arthritis (JRA) in 1986, at 18 months of age. At that time, she had a I-month history of fever, truncal rash, generalized lymphadenopathy, and bilateral wrist and knee arthritides. Antinuclear antibodies and rheumatoid factor were negative, and erythrocyte sedimentation rate (ESR) was 90. She responded well to aspirin and tolectin (a nonsteroidal anti-inflammatory drug [NSAID]) and these drugs were tapered off until the patient went into remission in May 1987. The patient remained well until July 1988, when she manifested daily fevers, truncal rash, and polyarthritis. Additionally, she reported dyspnea, and was found to have a large heart shadow and bilateral pleural effusions on chest x-ray film. Echocardiography confirmed the diagnosis of pericardial effusion, and this entity was treated effectively with pericardiocentesis. She again responded well to NSAID (naprosyn) therapy, and all symptoms subsided. From the Division of Otolaryngology-Head and Neck Surgery, Strong Memorial Hospital, University of Rochester. Received for publication Nov. 24, 1989; revision received June 11, 1990; accepted June 12, 1990. Reprint requests: John D. Norante, MD, Division of OtolaryngologyHead and Neck Surgery, Strong Memorial Hospital, 601 Elmwood Ave., Box 629, Rochester, NY 14642. 23/4/23109
On September 29, 1988, the patient was brought to the emergency department in acute upper airway distress, with stridor, substernal retractions, nasal flaring, air hunger, and anxiety. The parents stated that over the 2 to 3 weeks before this presentation, noisy breathing developed in the patient at night, and would resolve each morning when the patient awoke. The duration of noisy breathing increased each subsequent day, until the day of presentation to the emergency department, when the patient had continuous noisy breathing and shortness of breath. Auscultatory examination in the emergency department revealed no wheezing, good air exchange, and inspiratory stridor. The patient was afebrile and also reported bilateral wrist and knee pain. Lateral and anterior neck films revealed a normal epiglottis shadow, subglottic narrowing, and ballooning of the hypopharynx (Figs. 1 and 2). Laboratory findings at the time included ESR = 84, white blood cell count = 17,100, arterial blood gas on 2 L/min oxygen via nasal cannula of pH 7.48, pC0, = 33, PO, = 121, and 98% saturation. The patient was given racemic epinephrine without relief, and was then transferred to the operating room for airway management. In the operating room, general anesthesia was induced. Initial direct laryngoscopy before intubation revealed a small glottic chink resulting from bilateral vocal cord immobility. The patient was orally intubated without difficulty. Tracheotomy was immediately performed because of the inadequacy of the glottic airway, and a no. 2 pediatric Shiley tracheotomy tube was placed without difficulty or complication. We believed that the presence of an indwelling endotracheal tube could only exacerbate the acute inflammatory process and possibly lead to a chronic inflammatory process. It was thought that tracheotomy would help to avoid this sequela and could be maintained indefinitely, awaiting the return of vocal cord mobility. With the endotracheal tube out of the airway, the larynx was visualized with an anterior commissure laryngoscope. With the patient paralyzed, there was a slit-like airway present. The right arytenoid cartilage appeared erythematous, and the left appeared normal. Each arytenoid cartilage was palpated with a laryngeal spatula separately, and both were found to be completely fixed. The paralysis was reversed and the patient was allowed to awaken. Both vocal cords were fixed in the paramedian position and did not abduct with inspiratory effort. The slit-like airway remained unchanged, and there was no evidence of laryngospasm. Subglottic narrowing was not apparent at the time of endoscopic examination. The diagnosis of cricoarytenoid arthritis (CA) was supported. The patient had a stable postoperative course and was started on NSAID (naprosyn), intravenous corticosteroids,
366 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 5, 2016
Volume I W Number 3 March 1991
Case Reports
i Fig. 1. Portable anterior-posterior chest radiograph shows significont subglottic norrowing
Fig. 2. Portable cross-table lateral neck radiogroph shows bollooning of the hypopharynx. N o epiglottic edema or thickening.
Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 5, 2016
367
OtolaryngologyHead and Neck Surgery
368 CaseReports
Table 1. Comparison of clinical findings* JRA classifications and subjects' ages in years Systemic (4.5 Yr)
Systemic findings
Neck radiograph
Laryngoscopy findings
Polyarthritis Pericarditis Pleuritis Truncal rash Fever Subglottic narrowing, ballooning of hypopharynx Vocal cords adducted and immobile, fixed arytenoid cartilages, erythema, and edema, R > L
ESR Airway control
84 Tracheotomy
Medical therapy
Naprosyn, gold, intravenous and oral corticosteroids Decannulated in 2.5 months, normal voice, oral NSAID, gold and steroid therapy Present study
Outcome
Source
Pauciarticuiar
Pauciarticular (4.5 v )
(2.3vr)
Systemic (3.25 yr)
Sternal swelling along costochondral junction
Left knee effusion with flexion/contracture
Polyarthritis Pericarditis Truncal rash Fever
Subglottic narrowing, distention of the hypopharynx Vocal cords adducted and immobile, supraglottic edema and erythema with funnelshaped appearance Not reported Overnight intubation, subsequent tracheotomy after several recurrences Intravenous and oral corticosteroids Oral antibiotic
Narrowing of the glottic region
Not reported
Vocal cords adducted and immobile, fixed arytenoid cartilages with edema and erythema, R > L
Left vocal cord adducted and immobile, fixed left arytenoid cartilage
Not reported 02 tent
Tracheotomy
118
Aspirin, oral corticosteroids
Aspirin, intravenous and oral corticosteroids
Maintained tracheotomy Unknown medications
Symptom free, aspirin and oral steroid therapy
Decannulated in 10 days, hoarse voice Aspirin therapy
Goldhagen (1988)
Malleson et al. (1986)
Jacobs and Hui (1977)
_
_
_
_
~
*All subjects in comparison are female JRA, Juvenile rheumatoid arthritis,ESR.
erythrocyte sedimentation rate, NSAID, nonsteroidal anti-inflammatory drug
and gold therapy. She was discharged on postoperative day 5 , with a tracheotomy tube in place. The other systemic symptoms of JRA resolved with continued outpatient NSAID, oral steroid, and gold therapy. On October 31, 1988, the patient underwent elective direct laryngoscopy with the anterior commissure laryngoscope under sedation, but with spontaneous respirations. No movement of the left vocal cord was seen. The right vocal cord had moderate lateral excursion. Both arytenoid cartilages appeared mildly erythematous, and the tracheotomy was left in place because of the inadequacy of the airway. The patient continued on NSAID, oral steroid, and gold therapy, with no further exacerbation of disease. In December 1988, flexible fiberoptic laryngoscopy was performed while the girl was under mild sedation. The ary-
tenoids appeared normal, and both vocal cords and arytenoid cartilages had full, symmetric range of motion. The patient was decannulated, the tracheotomy site healed well, and the patient currently speaks well. She has had no further clinical evidence of CA and is now on tapering doses of medications. As of December 1990, this patient's arthritis remains wellcontrolled with low-dose oral NSAID therapy.
DISCUSSION
The cricoarytenoid joint is a multiaxial, diathrodial joint that allows motion between the arytenoid cartilages and the cricoid cartilage during phonation and respiration. The structure and mechanics of the joint are well presented by other authors.I0." Arthritis of the
Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 5, 2016
Volume 104 Number 3 March 1991
CaseReports 369
subglottic narrowing and distention of the hypopharcricoarytenoid joint was first described by Mac Kenzie ynx. The other patient demonstrated only glottic narin 1880.12 Since then, this clinical entity has been derowing. The measurements of this narrowing was not scribed in adult patients numerous times. 1 3 ~ 1 4The sympdocumented in any of the other cases. Our patient’s toms of CA in adults are fullness or tension in the throat subglottic airway measured 3 mm radiographically in aggravated by swallowing, hoarseness, odynophagia, the anteroposterior projection. Normal measurements pain with speaking and coughing, pain radiating to the of the subglottic airway in a patient this age should be ears, tenderness of the thyroid cartilage, dyspnea, and 8 mm in the anteroposterior dimen~ion.~’ inspiratory stridor.” The stridor is worse during sleep and sedation because of the decreased voluntary abDirect laryngoscopy (DL) results corrolated well in duction of the vocal cords in these relaxed ~ t a t e s . ~ , ” . ’ ~all four cases. DL results were characterized by deDefinitive diagnosis of CA is by laryngoscopy. Lacreased vocal cord mobility, erythema and swelling of ryngoscopic findings in adults in the acute phase of CA one or both arytenoid cartilages, fixation of one or both include erythematous, swollen arytenoids, normal or arytenoid cartilages in adduction, and a slit-like airway slightly edematous vocal cords, variable vocal cord moin cases of bilateral arytenoid cartilage fixation. The bility, and immobility and tenderness of the arytenoid subglottic narrowing noted on radiographs was not vicartilages on palpation with a laryngeal ~patula.~.” Lasualized during endoscopy in our case. This may have ryngoscopic findings in the chronic stage of disease in represented subglotticedema that resolved with medical adults include thickening of the mucosa overlying the therapy before surgical intervention. Subglotticnarrowarytenoids, narrow vocal cord chink, and midline, iming was not addressed in the other case reports, and the mobile arytenoid cartilages by the spatula t e ~ t . ~ . ’ ~ etiology of that radiographic finding remains unclear. Though this entity is well described in adults, there The narrowness of the airway in all of the cases dictates are only three well-documented cases in children .&’ concern over the ability to intubate these patients. The One other report mentions three patients in a group of use of an endotracheal tube one size smaller than ap100 patients with Still’s disease who exhibited inspipropriate for the age and size of the patient allows for ratory ~ t r i d o rYet, . ~ this last-mentioned report only doceasier intubation and provides an adequate temporary uments that one of the patients demonstrated “paralyzed airway. cords” on direct laryngoscopy, and required emergent All of the patients responded well to either steroids tracheotomy. Because of the poor documentation in this alone, or in combination with an NSAID or aspirin. latter report, only the three first-mentioned reports of There was mild, if any, decreased stridor reported with CA in children will be discussed. Table 1 presents perracemic epinephrine-nebulizer therapy. The excellent tinent information from the four (including ours) wellresponse to medical therapy seen in children with CA documented cases of CA in children. is not shared by adults who acquire this disease. Some adults with mild, acute airway compromise Interestingly, three of the four patients were female. have been reported to respond to anti-inflammatory Two of the patients had systemic JRA, while the other medications; antibiotics for exacerbating infections; hutwo had pauciarticular (three or less joints involved, midified, cool oxygen tents; and bedre~t.~’” Many minimal systemic symptoms) JRA. Of note here is that times, however, treatment failures occur, and progresthe three poorly documented cases all had systemic JRA sive airway obstructure ensues. Fatalities have been (Still’s disease).’ Each patient experienced inspiratory reported as a result of airway obstruction before adestridor that progressively worsened over a period of quate surgical intervention.I 3 . I 6 weeks, until medical intervention was sought. Three of the patients reportedly experienced worse stridor at In contrast to pediatric CA, adult-onset CA is charnight, while sleeping. When stridor became constant, acterized by chronic, insiduous fixation of the arytenoid acute upper airway distress occurred and was characcartilages. Acute exacerbations of this process are reterized by air hunger, nasal flaring, use of accessory sponsible for emergent airway compromise, and fremuscles of respiration, and apprehension. Three of the quently necessitate surgical intervention. The progrespatients required airway management consisting of eisive nature of the adult form of CA has lead many ther tracheotomy (early, two; late, one) or intubation authorities to recommend surgery in the form of tra(one). One patient was observed closely in an oxygen cheotomy, arytenoidectomy, or arytenoidopexy at the tent and responded to therapy quickly enough to avoid first sign of airway compromise, as gauged by diminformal airway management. In our case, fixation of the ished vocal chink.l 3 3 l 6 Though the reports of pediatric arytenoid cartilages in adduction dictated aggressive CA are limited, the reversibility of joint fixation with therapy. Lateral and anterior neck film results were medical therapy has been the rule with every case, and reported in three cases. Two cases were associated with we do not advocate routine, aggressive surgical therapy.
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Otolaryngology-
370 Case Reports
Head and Neck Surgery
Rather, if this entity can be diagnosed early, aggressive medical therapy consisting of corticosteroids alone or in combination with an NSAID or aspirin seems to be the treatment of choice. Tracheotomy may very well be avoided in many cases. As evidenced by the cases reported, CA manifests essentially the same signs, symptoms, and direct laryngoscopic findings in the pediatric patient as in the adult patient. Yet, CA is rarely reported in the pediatric patient, whereas 17% to 54% of adult patients with rheumatoid arthritis have clinical evidence of CA. Several factors may account for the disparity between adults and children. First, examination of the larynx in children is much more difficult than in adults. Mild-to-moderate cases of CA may be misdiagnosed because of the larynx not being directly examined. Because of the more common causes of pediatric stridor, CA may be misdiagnosed as viral croup, bacterial laryngotracheitis, retained foreign body, allergic edema, asthma, or epiglottitis. Additionally, nocturnal stridor may be overlooked by parents, or wrongly interpreted as snoring. Possibly, CA is an insidious process that normally takes years to develop, and these cases represent the rare exceptions from the rule.’ Also, it may be that CA is more common in patients with systemic JRA, as five cases have been reported (6,9 and our patient). Only two cases of CA have been reported in patients with pau~iarticular’~~ and no cases of CA have been reported in patients with polyarticular JRA. Thus, though rare, CA must be considered in the differential diagnosis of stridor, and more generally in the differential diagnosis of acute and chronic upper airway obstruction in children with JRA. Early diagnosis of the entity, along with immediate corticosteroid therapy-alone or in combination with NSAIDs-may prevent the need for emergent airway control and tracheotomy in affected patients.
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