Article, Otolaryngology

Diffuse idiopathic skeletal hyperostosis as an acute airway presentation requiring urgent tracheostomy

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American Journal of Emergency Medicine

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Diffuse idiopathic skeletal hyperostosis as an acute airway presentation requiring urgent tracheostomy?

Abstract

Diffuse idiopathic skeletal hyperostosis is an idiopathic disorder characterized by intervertebral bridges of ossification along the anterior and lateral aspects of the spine. This case report describes an 87-year-old man presenting with an acute onset of stridor. Computed tomography revealed a large osteophyte originating from the cervical spine requiring tracheostomy due to a critical narrowing of the laryngeal inlet. Diffuse idiopathic skeletal hyperostosis is a common cause of dysphagia but rarely results in critical airway obstruction. This condition should be considered in anyone presenting with dysphagia without an obvious cause. As demonstrated, it can be a very rare cause of airway obstruction causing significant clinical challenges.

Diffuse idiopathic skeletal hyperostosis (DISH), also referred to as Forestier or Forestier-Rotes-Querol disease, is an idiopathic disorder, affecting middle aged and Elderly people. It has been found to occur in up to 10% of patients older than the age of 65 years and is characterized by intervertebral bridges of ossification along the anterior and lateral aspects of the spine. Most reported cases have documented significant involvement of the cervical spine; but alternative extra spinal calcifica- tion sites include the ligamentous attachments of the pelvis, calcaneus, tarsal bones, patella, and olecranon [1].

Stridor secondary to osteophyte compression of the upper airway is a very rare but recognized complication of DISH [2]. However, cases requiring emergency tracheostomy at first presentation have not been documented previously. Therefore, we present a previously undiag- nosed case of DISH, presenting with acute airway obstruction requiring emergent tracheostomy.

An 87-year-old man was referred from a local district general hospi- tal to a tertiary referral otolaryngology center. He was an inpatient under the physicians for investigation of severe dysphagia (10 kg weight loss over 8 weeks) and dehydration but became increasingly breathless and stridulous over the preceding few hours, prompting urgent referral. His medical history included ischemic heart disease and atrial fibrillation, requiring warfarinization. He had previously been under investigation by the gastroenterologists with an 18-month history of dysphagia and weight loss, having recently undergone an esophageal gastroduodenoscopy reported to be normal.

Examination of the patient on arrival revealed an inspiratory and expiratory stridor with tachypnea and increasing fatigue. Flexible laryn- goscopy revealed a protruding posterior pharyngeal wall abutting the epiglottis, causing a critical narrowing of the laryngeal inlet. Mucosa of the posterior pharyngeal wall and supraglottis appeared normal, with no other abnormality detected.

? Acknowledgments: None.

After examination and identification of a posterior pharyngeal mass, an urgent computed tomography scan of the head, neck, and chest was performed. This demonstrated exaggeration of the cervical lordosis, together with anterior osteophytosis resulting in compression of the oesophagus and trachea (Figure). It was concluded that the patient’s air- way had been compromised from a combination of cervical lordosis, an- terior cervical bridging osteophytes, and a thickened oesophagus.

Because of the findings of respiratory distress and worsening clinical parameters, a tracheostomy under local anaesthetic was planned. This was further complicated by the patient’s kyphotic cervical spine resulting in the use of a surgical cricothyroidotomy to initially secure the airway. After this, the patient was anaesthetized and intubated with considerable difficulty. A surgical tracheostomy was then per- formed through the first tracheal ring, due to the kyphosis and severe cervical osteophytes. With a definitive airway in place, further examina- tion was carried out using direct laryngoscopy. This revealed bilateral vocal cord palsy with massive bony posterior pharynx swelling at the level of the epiglottis and upper oesophagus. No mucosal abnormality was detected, and esophagoscopy was normal to a length of 28 cm.

After surgical tracheostomy, the patient’s airway remained stable allowing transfer to the ward. In discussion with the patient and family, it was decided not to proceed with surgical excision of the osteophytic mass. Over the following 2 months, the patient remained stable from an airway perspective but subsequently developed a lower respiratory tract infection and progression of preexisting congestive cardiac failure. Despite further hospital admission and medical optimization, he died 48 days after his initial presentation due to decompensating congestive cardiac failure.

Diffuse idiopathic skeletal hyperostosis is a condition characterized by the ossification of the enthesis (enthesopathy). For DISH to be diag- nosed at least 2, according to Forestier and Rotes-Querol [3], or 3, according to Resnick et al [1], contiguous intervertebral bridges must be fused. This is in the absence of severe disk alterations, in contrast to degenerative spinal disease or ankylosis of the sacroiliac or facet joints as seen in spondylarthritis.

Diffuse idiopathic skeletal hyperostosis can cause a variety of symp- toms (pain, outlet obstruction, respiratory compromise, and Chronic cough), but dysphagia is the most common reported in 6% to 28% of pa- tients as a result of cervical osteophytes [4]. Despite radiographic evi- dence of osteophytes, one must not overlook the possibility of a malignant etiology given the age of these patients. Stridor, however, is a rare presentation of DISH but can occur when osteophyte formation occurs at the level of C2 to C3 region. In addition to the extrinsic effect of the osteophytes, there can also be an intrinsic mass-like effect. Fur- ther methods of diagnosis may include plain lateral and anteroposterior x-rays, video fluoroscopy, contrast swallow, computed tomography, magnetic resonance imaging, and endoscopy.

0735-6757/(C) 2014

image of Figure

Figure. Sagittal computerized tomography scan revealing significant anterior cervical osteophyte (black arrow) causing critical narrowing of the laryngeal inlet (white arrow).

The management of DISH depends on the degree of symptoms. Ini- tial management of dysphagia includes general dietary modifications, postural changes when swallowing, Muscle relaxants, antireflux medi- cations, antiinflammatories, and steroids [5]. Patients should undergo videofluoroscopic swallow study or functional endoscopic examination of swallowing to exclude aspiration, especially when vocal fold function is decreased or absent. Tracheostomy and feeding tube placement should be reserved for patients who are poor surgical candidates or those with a short Life expectancy, whereas neuroSurgical consultation and decompression of osteophytes should be offered to surgically fit pa- tients with refractory symptomatic cervical osteophytic disease [5]. Sur- gical approaches include anterolateral, posterior lateral, or transoral routes. Reported complications include paralysis, recurrent laryngeal nerve damage, stroke, and cervical instability [2,5].

This case is the first of its kind to describe stridor secondary to DISH requiring an emergent tracheostomy at first presentation. Because of

the nature of this condition, it should be considered as a differential diag- nosis in patients presenting with dysphagia of unknown origin and may occur rarely in those presenting with acute airway compromise.

Jonathan H. Bird, MRCS

Facility of Medicine, University of Southampton

Southampton, United Kingdom

Timothy C. Biggs, MRCS Department of ENT Surgery, Southampton University Hospital NHS Foundation Trust, Southampton, United Kingdom

Petros D. Karkos, MD, PhD, MPhil

Department of ENT Surgery, Ahepa University Hospital

Thessaloniki, Greece Corresponding author. Department of Otolaryngology Ahepa Univeristy Hospital, Thessaloniki, 54636, Greece

Fax: +30 2314 022 009

E-mail address: [email protected]

Costa Repanos, FRCS(ORL-HNS) Department of ENT Surgery, Portsmouth Hospitals NHS Foundation Trust, Portsmouth, United Kingdom

http://dx.doi.org/10.1016/j.ajem.2014.10.050

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