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Table of Contents
CASE REPORT
Year : 2016  |  Volume : 6  |  Issue : 1  |  Page : 14-17

Arytenoid sclerosis in diffuse idiopathic skeletal hyperostosis presenting with acute stridor in elderly: Chance or association?


1 Department of Otorhinolaryngology - Head and Neck Surgery, Jawaharlal Nehru Medical College, DMIMSU, Wardha, Maharashtra, India
2 Department of Medicine, Jawaharlal Nehru Medical College, DMIMSU, Wardha, Maharashtra, India
3 Department of Radiodiagnosis, Jawaharlal Nehru Medical College, DMIMSU, Wardha, Maharashtra, India

Date of Web Publication5-Apr-2017

Correspondence Address:
Shraddha Jain
Department of Otorhinolaryngology - Head and Neck Surgery, Jawaharlal Nehru Medical College, DMIMSU, Sawangi, Wardha - 442 004, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2230-9748.203888

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   Abstract 

Forestier's disease or diffuse idiopathic skeletal hyperostosis (DISH) is a skeletal pathology characterized by paravertebral osteophyte formation and calcification and ossification of the anterior longitudinal vertebral ligament. It is being increasingly recognized as a cause of dysphagia and rarely leads to obstructive airway symptoms such as snoring, dyspnea on exertion, and laryngeal stridor. We report an unusual case presenting with acute respiratory distress due to obstructing laryngeal edema and bilateral arytenoid sclerosis, with the fixation of left cricoarytenoid joint in association with DISH.

Keywords: Arytenoid sclerosis, diffuse idiopathic skeletal hyperostosis, Forestier's disease, stridor, vocal cord immobilization


How to cite this article:
Jain S, Singh P, Agrawa G, Kumar S, Banode P. Arytenoid sclerosis in diffuse idiopathic skeletal hyperostosis presenting with acute stridor in elderly: Chance or association?. J Laryngol Voice 2016;6:14-7

How to cite this URL:
Jain S, Singh P, Agrawa G, Kumar S, Banode P. Arytenoid sclerosis in diffuse idiopathic skeletal hyperostosis presenting with acute stridor in elderly: Chance or association?. J Laryngol Voice [serial online] 2016 [cited 2017 Aug 20];6:14-7. Available from: http://www.laryngologyandvoice.org/text.asp?2016/6/1/14/203888


   Introduction Top


Diffuse idiopathic skeletal hyperostosis (DISH) or Forestier's disease, also known as ankylosing hyperostosis, is characterized by noninflammatory pathological ossification and calcification of the anterolateral spinal ligaments, mostly in the middle and lower thoracic regions in association with hypertrophic anterior cervical osteophytes.[1],[2] Resnick and Niwayama coined the term DISH, in 1970, for this systemic disease, which is characterized by exuberant proliferation of bone at various osseous sites of ligaments and tendinous attachment throughout the body.[3],[4] About 17%–28% of patients with DISH present with dysphagia due to anterior osteophytes of the cervical spine from the fourth to seventh vertebra.[1],[3] However, laryngotracheal symptoms such as dyspnea and noisy breathing in the form of snoring or stridor are rare and usually occur due to a large osteophyte at C3 to C4 level.[1],[4] Very few cases of acute respiratory distress due to DISH have been reported.[5],[6],[7] An unusual case of acute respiratory distress due to unilateral cricoarytenoid joint fixation with bilateral arytenoid sclerosis associated with DISH is being reported.


   Case Report Top


A 69-year-old male admitted to the Intensive Care Unit for diarrhea, with a 1-month history of cerebrovascular accident secondary to acute infarct in the paramedullary region and bilateral gangliocapsular region developed sudden respiratory distress with biphasic stridor. He also developed change in voice which was initially missed due to aphasia. The patient was tracheostomized for a duration of 1 month before the present episode, and tracheostomy closure had been done 15 days back. Initial impression was that of tracheal or subglottic stenosis as the cause of stridor. The patient was a nonsmoker, nonalcoholic, hypertensive, and nondiabetic.

Laryngoscopic evaluation was not possible in the emergency department due to pooling of secretions and increase in distress on examination. Considering supraglottis as a possibility due to pooling of secretions, injectable antibiotics and steroids along with nebulization with steroids were started. Arterial blood gas analysis showed pH of 7.4, PO2 of 99 mmHg, and PCO2 of 21 mmHg. The patient responded immediately. Stridor decreased with antibiotics and steroids and tracheostomy was not required. His erythrocyte sedimentation rate, serum uric acid, kidney function tests, liver function tests, and serum electrolytes were normal. Rheumatoid factor and ELISA for HIV were negative. High-resolution computed tomographic (CT) scan of the larynx revealed bilateral arytenoid sclerosis with calcification of anterior longitudinal ligament at the D2, D3, D4, and D5 levels, suggestive of early features of DISH. Prominent anterior osteophytes were seen at C4–C5 level, causing compression of the posterior aspect of larynx with edema of the laryngeal inlet and hypopharynx [Figure 1] and [Figure 2]. Rigid videolaryngoscopy was performed after stridor decreased. It revealed an immobile left vocal cord in a median position with restricted right vocal cord movements and mucosal edema of both arytenoids, more on the left side. Glottic chink was reduced on maximum inspiration. Under local anesthesia, a blunt curved laryngeal biopsy forceps was passed through the mouth and left arytenoid palpated and found to be fixed. Right arytenoid was found to be mobile. As patient's relatives were not willing for any surgical intervention, he was discharged in a stable condition after conservative management.
Figure 1: Computed tomographic scan of the cervical spine showing large anterior osteophytes at C4–C5 (red small arrow) leading to edematous hypopharynx and laryngeal inlet with calcification of anterior longitudinal ligament at the D2, D3, D4, and D5 level ( yellow arrow)

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Figure 2: Computed tomographic of larynx showing bilateral arytenoid sclerosis (Right = Orange arrowhead, Left = Orange arrow) with left cricoarytenoid joint ankylosis

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   Discussion and Review Top


Stridor is a rare manifestation of DISH and can be life-threatening. Initially, DISH was mainly implicated as a rare cause of chronic respiratory distress.[8] Recently, cases of DISH as a cause of sudden respiratory distress are being reported in the elderly. The following reasons have been implicated. First is the impaired function of the vocal folds or vocal cord paralysis caused by an osteophyte at the cricoid level.[9] Long-standing cases may develop cricoarytenoid joint ankyloses with unilateral or bilateral adduction-fixation of the vocal cords.[8],[9] Postcricoid ulceration at pressure point between the posterior aspect of cricoid cartilage and a protruding osteophyte can lead to chondritis of the cricoid cartilage and arytenoid, with subsequent ankylosis of the cricoarytenoid joint as occurred in our case. Stridor can rarely be a result of direct airway obstruction by the osteophytic mass.[5] The other contributing factor for airway compromise in DISH associated with excessively enlarged cervical osteophytes could be the edema of the laryngeal inlet and consequent severe dyspnea.[7] Compression of the venous structures in region of laryngeal inlet due to hyperostosis may thus lead to edema, fibrosis, and immobilization of the arytenoids.

In our patient, bilateral arytenoid sclerosis with sclerosis of cricoid cartilage and fixation of left cricoarytenoid joint were diagnosed on CT scan and laryngeal examination. Anterior spinal ligament calcification was limited to the thoracic region in our patient. In this disease, the thoracic region is most commonly involved (96%), followed by lumbar (90%) and cervical regions (78%) in that order.[10]

Although the figure quoted is different in different studies, it is well established that DISH is more frequent in men, and the incidence increases with age, mainly affecting patients over the age of 40 years.[4] Holton et al. found the prevalence of DISH, in the age group of more than 65 years in general population, to be as high as 42%.[11] Hence, in geriatric population, DISH should be considered in the causes of sudden stridor as in our case and CT scan should be undertaken in every case. In elderly, large projecting anterior cervical osteophytes can also be associated with senile degenerative skeletal disease and present in a similar manner. However, most of the patients with cervical osteophytes are asymptomatic; still, osteophytic compression leading to acute stridor should be included in the list of conditions causing acute respiratory distress in elderly. As DISH is more common in diabetics, the adduction-fixation could be misdiagnosed as vocal cord palsy of diabetic neuropathy. We therefore suggest that palpation of arytenoid by a probe for mobility testing, along with a CT scan, should be mandatory in all elderly diabetic patients with isolated impaired vocal cord mobility, to rule out underlying DISH and not to simply dismiss it as peripheral neuropathy.

Ours is probably the first reported case of bilateral arytenoid sclerosis in association with DISH. Bilateral or unilateral arytenoid sclerosis is more common in females and may occur as a normal variant, more on the left side.[12] In males, laryngeal cancer has been implicated as a cause of sclerosis, with only limited information regarding other causes of arytenoid cartilage sclerosis.[12],[13] Sclerosis is defined as increase in medullary density. In DISH, new bone formation is believed to be the result of abnormal osteoblast cell growth/activity under the influence of metabolic factors. The vertebral blood supply has been implicated as a predisposing factor that contributes to the occurrence and/or localization of DISH.[14] We propose that arytenoid sclerosis could be a result of the same underlying pathology as contributing to abnormal calcification and ossification in DISH. Benjamin and Roche reported association between vocal cord granuloma and focal or diffuse sclerosis of the arytenoid cartilage in all the 21 of their male patients being evident on CT scans.[15] Sclerosis, according to them, could be of reactive nature, secondary to hyperemia and other inflammatory changes in the perichondrium. This could form basis of another possible explanation of arytenoid sclerosis in our patient. In elderly, more studies are needed to implicate DISH as a direct cause of arytenoid sclerosis.

Obesity, type 2 diabetes mellitus, hypervitaminosis A, high body mass index, and hyperuricemia are considered as risk factors for DISH in that order. Our patient did not have any of these risk factors.

Conservative treatment has been indicated for the initial management of patients with DISH due to the potential risks of surgery in the elderly. Surgery should be reserved only for severe and resistant cases.[2],[4],[10]


   Conclusion Top


  • DISH should be considered in the differential diagnosis of all cases of acute respiratory distress and unilateral or bilateral vocal cord immobility in elderly
  • Further studies are needed to establish any direct association between arytenoid sclerosis and DISH
  • We propose CT scan and laryngeal probing in all cases of respiratory distress in elderly to rule out DISH as a cause.


Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Wang J, Chichra A, Koenig S. An unusual cause of acute hypercapneic respiratory failure. Clin Med Insights Circ Respir Pulm Med 2011;5:81-5.  Back to cited text no. 1
    
2.
Ohki M. Dysphagia due to diffuse idiopathic skeletal hyperostosis. Case Rep Otolaryngol 2012;2012:123825.  Back to cited text no. 2
    
3.
Resnick D, Niwayama G. Radiographic and pathologic features of spinal involvement in diffuse idiopathic skeletal hyperostosis (DISH). Radiology 1976;119:559-68.  Back to cited text no. 3
    
4.
Nascimento FA, Gatto LA, Lages RO, Neto HM, Demartini Z, Koppe GL. Diffuse idiopathic skeletal hyperostosis: A review. Surg Neurol Int 2014;5 Suppl 3:S122-5.  Back to cited text no. 4
    
5.
Papakostas K, Thakar A, Nandapalan V, O'Sullivan G. An unusual case of stridor due to osteophytes of the cervical spine: (Forestier's disease). J Laryngol Otol 1999;113:65-7.  Back to cited text no. 5
    
6.
Dagher WI, Nasr VG, Patel AK, Flis DW, Wein RO. An unusual and rare cause of acute airway obstruction in the elderly: Forestier's disease. J Emerg Med 2014;46:617-9.  Back to cited text no. 6
    
7.
Marks B, Schober E, Swoboda H. Diffuse idiopathic skeletal hyperostosis causing obstructing laryngeal edema. Eur Arch Otorhinolaryngol 1998;255:256-8.  Back to cited text no. 7
    
8.
Verstraete WL, De Cauwer HG, Verhulst D, Jacobs F. Vocal cord immobilisation in diffuse idiopathic skeletal hyperostosis (DISH). Acta Otorhinolaryngol Belg 1998;52:79-84.  Back to cited text no. 8
    
9.
Anand V, Vikram Vel VR, Purushothaman PK, Rajesh Kumar MS. Crico arytenoid joint fixation in diffuse idiopathic skeletal hyperostosis (DISH): A case report. Indian J Otolaryngol Head Neck Surg 2011;63 Suppl 1:55-7.  Back to cited text no. 9
    
10.
Najib J, Goutagny S, Peyre M, Faillot T, Kalamarides M. Forestier's disease presenting with dysphagia and disphonia. Pan Afr Med J 2014;17:168.  Back to cited text no. 10
    
11.
Holton KF, Denard PJ, Yoo JU, Kado DM, Barrett-Connor E, Marshall LM; Osteoporotic Fractures in Men (MrOS) Study Group. Diffuse idiopathic skeletal hyperostosis and its relation to back pain among older men: The MrOS study. Semin Arthritis Rheum 2011;41:131-8.  Back to cited text no. 11
    
12.
Schmalfuss IM, Mancuso AA, Tart RP. Arytenoid cartilage sclerosis: Normal variations and clinical significance. AJNR Am J Neuroradiol 1998;19:719-22.  Back to cited text no. 12
    
13.
Zan E, Yousem DM, Aygun N. Asymmetric mineralization of the arytenoid cartilages in patients without laryngeal cancer. AJNR Am J Neuroradiol 2011;32:1113-8.  Back to cited text no. 13
    
14.
El Miedany YM, Wassif G, El Baddini M. Diffuse idiopathic skeletal hyperostosis (DISH): Is it of vascular aetiology? Clin Exp Rheumatol 2000;18:193-200.  Back to cited text no. 14
    
15.
Benjamin B, Roche J. Vocal granuloma, including sclerosis of the arytenoid cartilage: Radiographic findings. Ann Otol Rhinol Laryngol 1993;102:756-60.  Back to cited text no. 15
    


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