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Cricoarytenoiditis as a cause of acute airway obstruction in children
CASE REPORT cricoarytenoiditis, pediatric
Cricoarytenoiditis as a Cause of A c u t e Airway Obstruction in Children A 4-1~2-year-old girl with a one-year history of juvenile rheumatoid arthritis was admitted with severe respiratory distress requiring endotracheal intubation. Arthritis of the cricoarytenoid joint was diagnosed and she responded well to intubation and high-dose steroids. Although well described in adults, this is only the third case report of a child afflicted with arthritis of the cricoarytenoid joint. [Goldhagen JL: Cricoarytenoiditis as a cause of acute airway obstruction in children. Ann Emerg Med May 1988;17:532-
533.] INTRODUCTION Arthritis of the cricoarytenoid joint as a cause of airway obstruction rarely has been described in children. This report describes acute airway obstruction in a 4V2-year-old girl with cricoarytenoiditis as a complication of juvenile rheumatoid arthritis.
CASE REPORT The patient was hospitalized at 42 months of age due to a limp and left leg pain and was diagnosed as having juvenile rheumatoid arthritis. At 46 months of age she experienced chest pain and sternal swelling along the costochondral junction without pulmonary symptoms. Physical examination revealed only a previously unnoted pectus carinatum. A chest radiograph was normal, and sedimentation rates remained elevated despite therapeutic salicylate levels. At 48 months of age the patient experienced symptoms of sore throat and cough. Recurrent symptoms of "croup" with the gradual onset of respiratory distress during the ensuing m o n t h necessitated hospitalization. She remained afebrile during the three-day hospitalization but awakened at night with air hunger, apprehension, and increased inspiratory stridor. There was improvement with racemic epinephrine nebulizations, but stridor persisted at hospital discharge. The discharge diagnosis was laryngotracheobronchiris. C o n t i n u i n g stridor unresponsive to nebulizations necessitated rehospitNization nine days later. IV Decadron ® and antibiotics were administered. Laryngoscopy revealed an occlusive "cherry" mass at the level of the cricoarytenoid joint. Intubation and inspection of the hypopharynx, epiglottis, vocal cords, and subglottic area revealed normal tissue. Repeat laryngoscopy three days later revealed no masses and extubation proceeded without difficulty. Diagnoses were laryngotracheobronchitis, a resolved subglottic mucous cyst, and juvenile rheumatoid arthritis. Rehospitalization was again required four days later for stridor, cough, and respiratory distress. Examination was unremarkable except for the pectus carinatum, a violaceous hue and tenderness to palpation at the costochrondal joints, and inspiratory stridor with soft tissue retractions. The stridor was unresponsive to nebulization therapy. Plain radiographs and fluoroscopy revealed distention of the hypopharynx and a segment of symmetrical narrowing characteristic of croup or congenital subglottic stenosis. Laryngoscopy without muscle relaxation showed no spontaneous m o v e ment of the vocal cords and no purulent secretions. The supraglottic area was erythematous and edematous and had a funnel-shaped appearance. There were no mass lesions and the epiglottis was normal. Bronchosc0py was normal. The patient was intubated and IV Decadron ® initiated. 17:5 May 1988
Annals of Emergency Medicine
Jeffrey L Goldhagen, MD, MPH Cleveland, Ohio From the Department of Emergency Medicine, Minneapolis Children's Medical Center, Minneapolis, Minnesota. Received for publication January 4, 1988. Accepted for publication February 8, 1988. Address for reprints: Jeffrey L Goldhagen, MD, MPH, Department of General Academic Pediatrics, Rainbow Babies and Children's Hospital, Case Western Reserve University, 2074 Abbington Road, Cleveland, Ohio 44106.
532/147
CRICOARYTENOIDITIS Golclhagen
Laryngoscopy and bronchoscopy after 24 hours showed complete resolution of the inflammation and edema, as well as normal vocal cord mobility. Extubation proceeded without complications. The sternal inflammation and tenderness resolved and a diagnosis was made of cricoarytenoiditis and costochondritis secondary to her juvenile r h e u m a t o i d arthritis. Oral steroid therapy was initiated. The patient subsequently required tracheostomy after multiple hospitalizations when stridor and respiratory compromise reemerged following discontinuation of steroid therapy. DISCUSSION First noted in 1894 as arthritis deformans of the larynx, 1 arthritis of the cricoarytenoid joint was not well described in adults u n t i l the 1950s. Clinical symptoms and post-mortem pathology of the cricoarytenoid joint since have been documented in many adults with rheumatoid arthritis. In contrast, only two reports of this disease appear in the pediatric literature.2, 3 It is unclear whether the difficulty of defining s y m p t o m s and examining the larynx in children with juvenile rheumatoid arthritis has prevented documentation of this entity, or whether symptomatic cricoarytenoiditis is an insidious process requiring many years to develop. Alternatively, mild cases may be misdiagnosed as laryngotracheobronchitis or asthma. The cricoarytenoid joint is true diarthrodial joint. In the acute stage of cricoarytenoiditis, erythematous swelling over the arytenoids with normal or slightly edematous vocal cords and variable cord motility is found. The chronic stage reveals mucosal changes, bowing of the vocal cords with inspiration, and fixation of the
148/533
arytenoid.4, s This p a t i e n t showed findings of acute cricoarytenoiditis with reversible fixation of the arytenoids. Symptoms in adults include foreign body sensation, hoarseness, dyspnea, radiation of pain to the ears, nocturnal stridor, dysphagia, odynophagia, and pain with speech. Stridor in adults appears to be most marked when the patient is relaxed or asleep, due to the ability to m o d i f y the o b s t r u c t i o n while awake and alert. 6-s This child's stridor and dyspnea also were most prominent at night and were confused with laryngotracheobronchitis. The initial presentation and recurrence of the syml~toms in this patient may have been due to an associated upper respiratory viral infection, which can worsen stridor. Parents of children with juvenile rheumatoid arthritis should watch for signs of respiratory distress during otherwise routine viral illnesses, and especially at night. Diagnosis of cricoarytenoiditis is established by direct laryngoscopy in the ED. Other potential indications of stridor (ie, laryngotracheobronchitis, epiglottitis, foreign body aspiration) should be evaluated with neck radiographs prior to laryngoscopy. Rigid fixation as opposed to passive mobility of the cricoarytenoid joint differentiates this entity from paralysis of the vocal cords. Diagnosis of acute cricoarytenoid arthritis in pediatrics mandates hospitalization and observation for progressive respiratory compromise, especially during sleep. Racemic epinephrine nebutizations are sometimes helpful, but steroids are necessary for sustained remission of symptoms. Treatment regimens for children have not been well defined. An initial response to steroids has been noted,
Annals of Emergency Medicine
but tracheostomy has been required in b o t h c h i l d r e n and a d u l t s despite chronic steroid therapy. 2 Unilateral arytenoidectomy has been performed for chronic symptoms. 9
SUMMARY Arthritis of the cricoarytenoid joint must be considered in the differential diagnosis of stridor and acute airway obstruction in children. Initial treatm e n t should include racemic epinephrine nebulization and steroids, but long-term therapy may necessitate t r a e h e o s t o m y or cricoarytenoidectomy. The author thanks Murale Srinivasan, MD, for referral of this patient and Gary Garvis, MD, for surgical support.
REFERENCES
1. CasselberryW: Arthritis deformans larynx.
Trans A m Laryng Assoc 1894;3:18-23.
2. Jacobs J, Hui R: Cricoarytenoidarthritis and airway obstruction in juvenile rheumatoid arthritis. Pediatrics 1977~59:291-293. 3. SehlesingerBE, ForsythCC, White RH, et al: Observations on the clinical course and treatment of one hundred cases of Still's Disease. Arch Dis Child 1961;36:65-76. 4. Lofgren R, Montgomery W: Incidence of laryngeal involvement in rheumatoid arthritis. N Engl J Med 1962;267:193-195. 5. MontgomeryW: Pathologyof cricoarytenoid arthritis. N Engl l Med 1959;260:66-69.. 6. Grossman A, Martin J, Root H: Rheumatoid arthritis of the cricoarytenoid joint. Laryngoscope 1961;71:530-544. 7. Bienenstock H, Ehrlich GE, YreybergRH: Rheumatoid arthritis of the cricoarytenoid joint: A clinicopathologic study. A r t h r i t i s Rheum 1963;6:48-62. 8. BridgerMWM, Jahn AF, van Nostrand AWP: Laryngeal rheumatoid arthritis: Laryngoscope 1980;90:296-303. 9. Polisar I, Burbank B, Levitt L, et al: Bilateral midline fixation of cricoarytenoidjoints as a serious medical emergency. ] A M A 1960; 172:901-906.
17:5 May 1988
Cricoarytenoiditis as a Cause of A c u t e Airway Obstruction in Children A 4-1~2-year-old girl with a one-year history of juvenile rheumatoid arthritis was admitted with severe respiratory distress requiring endotracheal intubation. Arthritis of the cricoarytenoid joint was diagnosed and she responded well to intubation and high-dose steroids. Although well described in adults, this is only the third case report of a child afflicted with arthritis of the cricoarytenoid joint. [Goldhagen JL: Cricoarytenoiditis as a cause of acute airway obstruction in children. Ann Emerg Med May 1988;17:532-
533.] INTRODUCTION Arthritis of the cricoarytenoid joint as a cause of airway obstruction rarely has been described in children. This report describes acute airway obstruction in a 4V2-year-old girl with cricoarytenoiditis as a complication of juvenile rheumatoid arthritis.
CASE REPORT The patient was hospitalized at 42 months of age due to a limp and left leg pain and was diagnosed as having juvenile rheumatoid arthritis. At 46 months of age she experienced chest pain and sternal swelling along the costochondral junction without pulmonary symptoms. Physical examination revealed only a previously unnoted pectus carinatum. A chest radiograph was normal, and sedimentation rates remained elevated despite therapeutic salicylate levels. At 48 months of age the patient experienced symptoms of sore throat and cough. Recurrent symptoms of "croup" with the gradual onset of respiratory distress during the ensuing m o n t h necessitated hospitalization. She remained afebrile during the three-day hospitalization but awakened at night with air hunger, apprehension, and increased inspiratory stridor. There was improvement with racemic epinephrine nebulizations, but stridor persisted at hospital discharge. The discharge diagnosis was laryngotracheobronchiris. C o n t i n u i n g stridor unresponsive to nebulizations necessitated rehospitNization nine days later. IV Decadron ® and antibiotics were administered. Laryngoscopy revealed an occlusive "cherry" mass at the level of the cricoarytenoid joint. Intubation and inspection of the hypopharynx, epiglottis, vocal cords, and subglottic area revealed normal tissue. Repeat laryngoscopy three days later revealed no masses and extubation proceeded without difficulty. Diagnoses were laryngotracheobronchitis, a resolved subglottic mucous cyst, and juvenile rheumatoid arthritis. Rehospitalization was again required four days later for stridor, cough, and respiratory distress. Examination was unremarkable except for the pectus carinatum, a violaceous hue and tenderness to palpation at the costochrondal joints, and inspiratory stridor with soft tissue retractions. The stridor was unresponsive to nebulization therapy. Plain radiographs and fluoroscopy revealed distention of the hypopharynx and a segment of symmetrical narrowing characteristic of croup or congenital subglottic stenosis. Laryngoscopy without muscle relaxation showed no spontaneous m o v e ment of the vocal cords and no purulent secretions. The supraglottic area was erythematous and edematous and had a funnel-shaped appearance. There were no mass lesions and the epiglottis was normal. Bronchosc0py was normal. The patient was intubated and IV Decadron ® initiated. 17:5 May 1988
Annals of Emergency Medicine
Jeffrey L Goldhagen, MD, MPH Cleveland, Ohio From the Department of Emergency Medicine, Minneapolis Children's Medical Center, Minneapolis, Minnesota. Received for publication January 4, 1988. Accepted for publication February 8, 1988. Address for reprints: Jeffrey L Goldhagen, MD, MPH, Department of General Academic Pediatrics, Rainbow Babies and Children's Hospital, Case Western Reserve University, 2074 Abbington Road, Cleveland, Ohio 44106.
532/147
CRICOARYTENOIDITIS Golclhagen
Laryngoscopy and bronchoscopy after 24 hours showed complete resolution of the inflammation and edema, as well as normal vocal cord mobility. Extubation proceeded without complications. The sternal inflammation and tenderness resolved and a diagnosis was made of cricoarytenoiditis and costochondritis secondary to her juvenile r h e u m a t o i d arthritis. Oral steroid therapy was initiated. The patient subsequently required tracheostomy after multiple hospitalizations when stridor and respiratory compromise reemerged following discontinuation of steroid therapy. DISCUSSION First noted in 1894 as arthritis deformans of the larynx, 1 arthritis of the cricoarytenoid joint was not well described in adults u n t i l the 1950s. Clinical symptoms and post-mortem pathology of the cricoarytenoid joint since have been documented in many adults with rheumatoid arthritis. In contrast, only two reports of this disease appear in the pediatric literature.2, 3 It is unclear whether the difficulty of defining s y m p t o m s and examining the larynx in children with juvenile rheumatoid arthritis has prevented documentation of this entity, or whether symptomatic cricoarytenoiditis is an insidious process requiring many years to develop. Alternatively, mild cases may be misdiagnosed as laryngotracheobronchitis or asthma. The cricoarytenoid joint is true diarthrodial joint. In the acute stage of cricoarytenoiditis, erythematous swelling over the arytenoids with normal or slightly edematous vocal cords and variable cord motility is found. The chronic stage reveals mucosal changes, bowing of the vocal cords with inspiration, and fixation of the
148/533
arytenoid.4, s This p a t i e n t showed findings of acute cricoarytenoiditis with reversible fixation of the arytenoids. Symptoms in adults include foreign body sensation, hoarseness, dyspnea, radiation of pain to the ears, nocturnal stridor, dysphagia, odynophagia, and pain with speech. Stridor in adults appears to be most marked when the patient is relaxed or asleep, due to the ability to m o d i f y the o b s t r u c t i o n while awake and alert. 6-s This child's stridor and dyspnea also were most prominent at night and were confused with laryngotracheobronchitis. The initial presentation and recurrence of the syml~toms in this patient may have been due to an associated upper respiratory viral infection, which can worsen stridor. Parents of children with juvenile rheumatoid arthritis should watch for signs of respiratory distress during otherwise routine viral illnesses, and especially at night. Diagnosis of cricoarytenoiditis is established by direct laryngoscopy in the ED. Other potential indications of stridor (ie, laryngotracheobronchitis, epiglottitis, foreign body aspiration) should be evaluated with neck radiographs prior to laryngoscopy. Rigid fixation as opposed to passive mobility of the cricoarytenoid joint differentiates this entity from paralysis of the vocal cords. Diagnosis of acute cricoarytenoid arthritis in pediatrics mandates hospitalization and observation for progressive respiratory compromise, especially during sleep. Racemic epinephrine nebutizations are sometimes helpful, but steroids are necessary for sustained remission of symptoms. Treatment regimens for children have not been well defined. An initial response to steroids has been noted,
Annals of Emergency Medicine
but tracheostomy has been required in b o t h c h i l d r e n and a d u l t s despite chronic steroid therapy. 2 Unilateral arytenoidectomy has been performed for chronic symptoms. 9
SUMMARY Arthritis of the cricoarytenoid joint must be considered in the differential diagnosis of stridor and acute airway obstruction in children. Initial treatm e n t should include racemic epinephrine nebulization and steroids, but long-term therapy may necessitate t r a e h e o s t o m y or cricoarytenoidectomy. The author thanks Murale Srinivasan, MD, for referral of this patient and Gary Garvis, MD, for surgical support.
REFERENCES
1. CasselberryW: Arthritis deformans larynx.
Trans A m Laryng Assoc 1894;3:18-23.
2. Jacobs J, Hui R: Cricoarytenoidarthritis and airway obstruction in juvenile rheumatoid arthritis. Pediatrics 1977~59:291-293. 3. SehlesingerBE, ForsythCC, White RH, et al: Observations on the clinical course and treatment of one hundred cases of Still's Disease. Arch Dis Child 1961;36:65-76. 4. Lofgren R, Montgomery W: Incidence of laryngeal involvement in rheumatoid arthritis. N Engl J Med 1962;267:193-195. 5. MontgomeryW: Pathologyof cricoarytenoid arthritis. N Engl l Med 1959;260:66-69.. 6. Grossman A, Martin J, Root H: Rheumatoid arthritis of the cricoarytenoid joint. Laryngoscope 1961;71:530-544. 7. Bienenstock H, Ehrlich GE, YreybergRH: Rheumatoid arthritis of the cricoarytenoid joint: A clinicopathologic study. A r t h r i t i s Rheum 1963;6:48-62. 8. BridgerMWM, Jahn AF, van Nostrand AWP: Laryngeal rheumatoid arthritis: Laryngoscope 1980;90:296-303. 9. Polisar I, Burbank B, Levitt L, et al: Bilateral midline fixation of cricoarytenoidjoints as a serious medical emergency. ] A M A 1960; 172:901-906.
17:5 May 1988