Clinical Challenges

Clinical Challenges - June 2016
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What's Your Diagnosis?

He did not have any personal or family history of cancer. He also denied alcohol and tobacco use. Within the past 2 months, the patient had been experiencing a globus sensation in his throat unassociated with swallowing. Even though his appetite was intact, the patient still had difficulty swallowing both solid and liquid food without odynophagia.

Upon arrival, his vital signs were stable. Physical examination was unremarkable. No cervical lymphadenopathy or masses were appreciated in his neck. Hemoglobin, platelets, and white blood cell count were within normal reference ranges. An esophagogram was initially performed, illustrating mildly thickened folds of distal esophagus with no obstruction or ulcer (Figure A).

Upper endoscopy was subsequently performed, revealing normal esophageal mucosa without any strictures. An ENT specialist was consulted because of the concern of oropharyngeal dysphagia. Laryngoscope and video swallowing evaluations were performed; the results were normal. Ultimately, a neck radiograph and computed tomography (CT) of the neck were performed (Figures B and C).


 

The diagnosis

The radiograph (Figure B) and CT scan (Figure C) of the neck revealed severe calcification of soft tissue along the anterior portion of cervical spine starting from C2 to T1. The calcification is pressing on the esophagus and also displaces the trachea forward.

These findings are consistent with diffuse idiopathic skeletal hyperostosis (DISH). Initially, the patient was treated with partial liquid diet and nutritional supplement. Subsequently, the patient underwent orthopedic consultation for surgical consideration.

DISH is a noninflammatory ossification and calcification of ligaments and tendons anterior to the spine. Even though this condition was first described by Forestier et al. in 1950, 1 the etiology and pathogenesis of DISH remain unknown. Many risk factors have been postulated to be associated with DISH, such as mechanical factors, diet, and drugs. DISH is more prevalent among the elderly, especially, males; however, it is a rare cause of dysphagia. 2 The clinical manifestation varies depending on the location of calcification. There have been case reports of DISH causing dysphagia, difficult airway management, and spinal cord root compression. 3

For esophageal involvement, the severity of symptoms is correlated with degree of compression. Initially, DISH may cause only an isolated globus sensation without any significant dysphagia. However, as the compression progresses, it can cause significant progressive dysphagia that mimics the presentation of esophageal cancer. Thus, the effort to rule out esophageal cancer or other structural diseases is needed before the diagnosis of DISH can be established, such as an upper endoscopy or CT of the neck.

Because DISH can resemble benign degenerative osteophytes on a plain neck radiograph, it is sometimes overlooked by physicians. DISH is an uncommon condition; it should be considered in the differential diagnosis of dysphagia, especially in the elderly population.References

1. Forestier, J., Rotes-Querol, J. Senile ankylosing hyperostosis of the spine. Ann Rheum Dis. 1950;9:321-30.

2. Schlapbach, P., Beyeler, C., Gerber, N.J., et al. Diffuse idiopathic skeletal hyperostosis (DISH) of the spine: a cause of back pain? (A controlled study). Br J Rheumatol. 1989;28:299-303.

3. Verlaan, J.J., Boswijk, P.F., de Ru, J.A., et al. Diffuse idiopathic skeletal hyperostosis of the cervical spine: an underestimated cause of dysphagia and airway obstruction. Spine J. 2011;11:1058-67.

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