Article, Otolaryngology

Forestier disease: an unusual cause of upper airway obstruction

Case Report

Forestier disease: an unusual cause of upper airway obstruction

We report the case of a 75-year-old man who presented at the emergency department complaining of difficult breath- ing, especially when in supine position. He had cough, dysphonia, and dysphagia for fluids in the last few months. After ruling out other causes of dyspnea, a lateral cervical radiograph was performed that showed large osteophytes compressing the airway, which was confirmed by cervical computed tomography. Marked improvement of symptoms was observed after resection of the osteophytes compressing the airway. Diffuse idiopathic skeletal hyperostosis, or Forestier disease, is an ossifying disease of ligaments and tendons and affects mainly elderly men. Anterior long- itudinal ligament ossification is characteristic. Although it is asymptomatic in some occasions, the disease may produce dysphagia, dysphonia, and exceptionally breathing difficul- ties due to airway compromise. In these cases, early surgery is recommended to alleviate airway obstruction.

A 75-year-old man presented at our emergency depart- ment complaining of a 3-week history of progressive breathlessness clearly related with the supine position. In the last few days, he had to sleep in a sitting position. He was under treatment with Oral anticoagulation because of atrial fibrillation. In the last few months, he had persistent cough, dysphonia, and dysphagia especially for fluids.

After examination the patient did not present any symptoms or signs of acute cardiac failure or of acute respiratory tract infection. Chest radiograph was unremark- able except for large bone spurs in the dorsal spine.

A lateral cervical spine radiograph showed large osteo- phytes that displaced the trachea and the esophagus anteriorly observed prominently at the C3 and C4 levels (Fig. 1). Cervical computed tomography confirmed com- pression of the airway, and the spinal cord was intact (Fig. 2). Surgical intervention was performed by right ante- rolateral cervicotomy, carrying out resection and grind- ing of osteophytes at C3 and C4 levels, before a

programmed tracheotomy.

This article was presented at the 13th Congress of the Spanish Society of Neruosurgery (Valencia, Spain; May 28-30, 2008).

After the intervention, the patient showed marked symptomatic improvement from dyspnea and dysphagia.

Diffuse idiopathic skeletal hyperostosis, also known as Forestier disease, is a non-inflammatory ossifying entheso- pathy, which manifests principally as an ossification of the anterior longitudinal ligament and the formation of inter- vertebral osseous bridges. It mostly affects men [1], and the mean age of patients is 60 years [2]. The thoracic region is mostly affected followed by the lumbar and Cervical regions. Ossification does not always occur solely in vertebral column, but also in other parts of the musculoskeletal system such as peripatellar ligaments, plantar fascia, and the Achilles tendon [3].

This pathology was first described on 1950 by Forestier and Querol [4] though Resnick established radiologic criteria

Fig. 1 Lateral cervical radiograph showing large osteophytes displacing the trachea and the esophagus anteriorly at the C3 and C4 levels.

0735-6757/$ – see front matter (C) 2008

1072.e2 Case Report

Fig. 2 Cervical computed tomographic scan demonstrating the compression of the airway.

[5]: (1) calcification or ossification of the anterior long- itudinal ligament in at least 4 consecutive vertebral bodies,

(2) conservation of the height of the intervertebral disk in the affected areas, and (3) absence of apophyseal joint ankylosis and sacroiliac joint sclerosis [2].

This is a disease of unknown cause. Various causal factors have been investigated, of metabolic, environmental, and endocrine origin, but no conclusive results have been found. It is not a frequent disease though, found in 12% of autopsies [2,6], with a prevalence of 2.6% and 3.8% in women and men older than 40 years, respectively, and increases up to 26% and 28%, respectively, in those older

than 80 years [7,8].

It is generally asymptomatic, discovered by coincidence as a remarkable radiologic finding when soliciting a radiograph for another disease. The most frequent symptoms are dorsolumbar pain and stiffness [3]. Nearly 20% of patients complain of dysphagia especially for solids (con- trary to our patients’ complaints) that exacerbates on extension and improves with flexion of the neck. Besides extrinsic compression of the esophagus, various mechanisms have been suggested to explain the cause of dysphagia.

These include inflammatory edema of the esophagus and periesophageal fibrosis [1,2,9,10]. Moreover, choking, bronchial aspiration, dysphonia [10], and more exceptionally respiratory compromise due to compression of the upper respiratory airway [6,11,12] can occur. Nelson et al [13] have presented a case series in which 3 patients required tracheotomy for acute airway obstruction. McCaffrey [2] observed that the size of the bone spurs do not relate to the severity of the symptoms, although Strasser et al [14] showed by videofluoroscopy a correlation between the size of the osteophytes (N10 mm) and the possibility of aspiration. neurologic complications have been described such as quadriplegia due to ossification of the posterior longitudinal ligament [15].

If no symptoms are produced, and diagnosis is made as a coincidental finding, then an expectant attitude should be taken in regard to treatment. Symptomatic cases can be managed with nonsteroid anti-inflammatory drugs, muscular relaxants, and soft foods. Surgical treatment consists of extirpation of the osteophytes that cause compression and is reserved for severe cases, such as progressive dysphagia, that leads to important weight loss, airway obstruction, and disabling dysphonia, or those in which medical treatment fails [6,10,16].

Although there is a high incidence, especially in elderly patients, Forestier disease is usually asymptomatic. Respira- tory compromise is exceptional and may require emergency tracheotomy until bone decompression and the airway is permeable. Results of bone extirpation are generally good.

Carlos Beaumont Caminos MD Emergency Department Hospital of Navarra

Pamplona, Navarra 31008, Spain E-mail address: [email protected]

Idoya Zazpe Cenoz MD, PhD Department of neurosurgery Hospital of Navarra

Pamplona, Navarra 31008, Spain

Clint Jean Louis MD Tomas Belzunegui Otano MD, PhD Bernabe Fenandez Esain MD

Maria Teresa Fortun Perez de Ciriza MD, PhD

Emergency Department Hospital of Navarra

Pamplona, Navarra 31008, Spain

doi:10.1016/j.ajem.2008.03.048

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