Clinical manifestations of diffuse idiopathic skeletal hyperostosis (DISH)

Abstract

Diffuse idiopathic skeletal hyperostosis (DISH, Forestier disease) is in most cases a harmless, asymptomatic disease characterized by a massive, noninflammatory ossification with intensive formation of osteophytes affecting ligaments, tendons, and fasciae, especially of the spinal column. If the disease becomes symptomatic at all, the leading complaints will usually be dysphagia in the sense of a lump in the throat and difficulty in swallowing. However, in rare cases the osteophytes may influence the laryngeal function ranging from hoarseness and immobility of the vocal cord to life-threatening inspiratory stridor. Such laryngeal manifestations are almost unknown. We report on three patients, two female and one male, suffering not only from chronic dysphagia but also from increasing inspiratory stridor and difficult breathing. Especially one case illustrates how difficult it can be to establish the etiological relationship between a cancer-like ulcer in the postcricoid region and a bilateral inactivity of the vocal cord on the one hand and DISH on the other hand. Only the resistance of the ulceration to any therapy as well as rare case reports in the literature confirmed our suspicion that the ulcer and disturbance of laryngeal function were caused by chronic pressure exerted by the huge vertebral osteophytes. In all three patients surgical resection of the osteophytes resulted in complete relief of complaints. Uni- or bilateral immobility of the arytenoids, possibly associated with chronic inflammatory hyperplasia of the tissue of the arytenoids and the postcricoid region, may be a symptom of an unusual manifestation of DISH.