CASE 15583 Published on 30.03.2018

Forestier syndrome - A rare cause of dysphagia

Section

Head & neck imaging

Case Type

Clinical Cases

Authors

D. Campos Correia, Eduardo Miranda, Rui Cabral, Fátima Cruz

Centro Hospitalar de Lisboa Ocidental
Rua da Junqueira
126 1349-019 Lisboa, Portugal
Email:davidcorreia7@gmail.com

Patient

53 years, female

Categories

Area of Interest Head and neck ; Imaging Technique Conventional radiography, Fluoroscopy

Procedure Diagnostic procedure ; Special Focus Calcifications / Calculi ;

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Clinical History

A 53-year-old otherwise healthy woman presented with a long-standing history of progressive dysphagia, currently to solid foods.
Fiberoptic endoscopic evaluation of swallowing and upper gastrointestinal endoscopy were unremarkable.

Imaging Findings

A videofluoroscopic barium swallow was performed, revealing a filling defect with luminal diameter reduction of the oesophagus due to very prominent osteophytes at C2-C4 vertebrae, which compressed the hypopharynx posteriorly (Fig. 1a and b). The patient mentioned greater difficulty in swallowing a thicker viscosity, even though videofluoroscopic findings (Fig. 1b) were identical to those with liquid viscosity (Fig. 1a).
A cervical spine radiograph was obtained, confirming prominent horizontal osteophytes at the C2-C4 vertebrae and ossification of the anterior longitudinal ligament, with normal intervertebral disk heights, findings in keeping with Forestier syndrome or diffuse idiopathic skeletal hyperostosis (DISH).

Discussion

Diffuse idiopathic skeletal hyperostosis (DISH) - also referred to as Forestier disease - is a common pathology characterised by exuberant spinal and/or peripheral enthesopathy. Resnick et al. established three diagnostic criteria: a) flowing ossifications of the anterior longitudinal ligament of at least four contiguous vertebral bodies, b) absence of apophyseal bone ankyloses or sacroiliac joint erosions and c) normal invertertebral disk height. [1] The paraspinal ligaments, namely the anterior longitudinal ligament, are most frequently affected. [2]
DISH most frequently affects the elderly and has a male predominance. [3, 4]
DISH is an uncommon cause of dysphagia [5], which arises either due to direct mechanical compression of the pharynx/oesophagus [1], impairment of normal epiglotic tilting [6] or osteophytic inflammatory reactions. [7]
Initial management usually involves conservative treatment. [1] Surgery (osteophyte resection) is warranted when symptoms are severe, with the appearance of aphagia, neurological compressive signs or airway obstruction. [5]
Our patient was only treated conservatively since her symptoms were only mild.

Differential Diagnosis List

Forestier syndrome (diffuse idiopathic skeletal hyperostosis).

Degenerative spine disease

Retinoid arthropathy

Ankylosing spondylitis

Final Diagnosis

Forestier syndrome (diffuse idiopathic skeletal hyperostosis).

References

[1] Resnick D, Niwayama G (1976) Radiographic and pathologic features of spinal involvement in diffuse idiopathic skeletal hyperostosis (DISH). Radiology 119(3):559–68 (PMID: 935390)

[2] Najib J, Goutagny S, Peyre M, Faillot T, Kalamarides M (2014) Forestier’s disease presenting with dysphagia and disphonia. Pan Afr Med J 17:168 (PMID: 25120881)

[3] Forestier J, Rotes Querol J (1950) Senile ankylosing hyperostosis of the spine. Ann Rheumat Dis 9:321–330 (PMID: 14800245)

[4] Mader R, Sarzi-Puttini P, Atzeni F, et al (2009) Extraspinal manifestations of diffuse idiopathic skeletal hyperostosis. Rheumatology 48(12):1478–81 (PMID: 19783587)

[5] Masafumi Ohki (2012) Dysphagia due to Diffuse Idiopathic Skeletal Hyperostosis. Case Reports in Otolaryngology Article ID 123825, 3 pages (PMID: 22953098)

[6] Lambert JR, Tepperman PS, Jimenez J, et al (1981) Cervical spine disease and dysphagia: Four new cases and a review of the literature. Am J Gastroenterol Jul;76(1):35–40 (PMID: 7304539)

[7] Akhtar S, O'flynn PE, Kelly A, et al (2000) The management of dysphagia in skeletal hyperostosis. J Laryngol Otol 114(2):154–157 (PMID: 10748839)

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