- Faculty of Medicine, Universidade Federal do Paraná, Brazil
- Department of Neurosurgery of Hospital Universitário Cajuru, Curitiba, Paraná, Neurosurgeon, Fellow of Interventional Neuroradiology, Neurosurgery Resident, Curitiba, Paraná, Brazil
- Department of Neurosurgery of Hospital Universitário Cajuru, Curitiba, Paraná, Brazil
- Radiologist in Clínica X Leme, Curitiba, Paraná, Brazil
- Department of Neurosurgery of Hospital Universitário Cajuru, Curitiba, Paraná, Neurosurgeon and Interventional Neuroradiologist, Curitiba, Paraná Brazil
- Department of Neurosurgery of Hospital Universitário Cajuru, Curitiba, Paraná, Interventional Neuroradiologist in Hospital Universitário Cajuru, Curitiba, Paraná, Brazil
Fábio A. Nascimento
Department of Neurosurgery of Hospital Universitário Cajuru, Curitiba, Paraná, Interventional Neuroradiologist in Hospital Universitário Cajuru, Curitiba, Paraná, Brazil
DOI:10.4103/2152-7806.130675Copyright: © 2014 Nascimento FA This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Fábio A. Nascimento, Maranha Gatto LA, Lages RO, Neto HM, Demartini Z, Koppe GL. Diffuse idiopathic skeletal hyperostosis: A review. Surg Neurol Int 16-Apr-2014;5:
How to cite this URL: Fábio A. Nascimento, Maranha Gatto LA, Lages RO, Neto HM, Demartini Z, Koppe GL. Diffuse idiopathic skeletal hyperostosis: A review. Surg Neurol Int 16-Apr-2014;5:. Available from: http://sni.wpengine.com/surgicalint_articles/diffuse-idiopathic-skeletal-hyperostosis-a-review/
Background:Diffuse idiopathic skeletal hyperostosis (DISH) is a systemic noninflammatory disease characterized by ossification of the entheses.
Methods:This paper reviews the etiopathogenesis, epidemiology, clinical features, differential diagnosis, and treatment of DISH, based on current available literature.
Results:Exact prevalence and incidence of DISH remains undetermined. Many external and genetic factors have been reported as being contributors to the pathogenesis of DISH. Current theories focus on the pathologic calcification of the anterior longitudinal ligament of the spine as the main physiopathological mechanism of disease. Clinical features are variable from monoarticular sinovitis to airway obstruction, and can be associated to systemic conditions. Comorbidities include obesity, hypertension, diabetes mellitus, hyperinsulinemia, dyslipidemia, and hyperuricemia according to a number of reports.
Conclusions:DISH is a disease which involves the calcification of the anterior longitudinal ligament of the spine and can be associated with numerous clinical presentations and comorbidities.
Keywords: Anterior Longitudinal Ligament, diffuse idiopathic skeletal hyperostosis, Forestier's disease
Forestier's disease was first described by Jacques Forestier and his student Jaume Rotes-Querol in 1950 under the name “senile ankylosing vertebral hyperostosis”.[
While the cause of DISH remains unclear, mechanical factors (such as the location of the aorta contributing to the development of bony bridging on specific sites), genetic factors (HLA genes), environmental exposures (fluoride, vitamin A/retinol), drugs (isotretinoin, etretinate, acitretin and other vitamin A derivatives), and metabolic conditions have been hypothesized to be relevant [
Although many external and genetic factors have been reported as being contributors of the pathogenesis of DISH, most of the current theories focus on the pathologic calcification of the anterior longitudinal ligament of the spine. The majority of these theories postulate that this process is due to the abnormal growth and function of the osteoblasts in the osteoligamentary binding.[
In terms of epidemiology of DISH, it varies in numerous reports – the absence of a consensus about the exact definition of the disease certainly contributes to not being able to determine its exact epidemiology. However, there are a few well-designed studies that try to estimate its prevalence. Holton et al. assessed 298 men aged older than 65 years from the general population. It was found that the prevalence of this disease (using Resnick's definition) in this age group was 42%.[
Various signs and symptoms have been described in patients suffering from DISH, such as polyarticular pain, neck/thoracic/lumbar/extremity pain, acute monoarticular sinovitis, limited range of spinal motion, dysphagia, increased susceptibility to unstable spinal fractures, and different degrees of airway obstruction [
The most commonly used classification criteria were defined by Resnick and Niwayama and required following anterolateral ossifications of at least four contiguous thoracic vertebral segments, preservation of the intervertebral disk spaces, and absence of apophyseal joint degeneration or sacroiliac inflammatory changes [
Despite the fact that the presence of constitutional and metabolic abnormalities is not mandatory for making a formal diagnosis of DISH, it is known that systemic conditions are associated with DISH in varying degrees. These comorbidities include obesity, hypertension, diabetes mellitus, hyperinsulinemia, dyslipidemia, and hyperuricemia, according to a number of reports.[
The most common conditions that may also present with bony excrescences, similar to those related to DISH, are Spondylosis Deformans and Ankylosing Spondylitis. The former disease is by far the most common of the disorders to be considered in the differential diagnosis of DISH. Spondylosis Deformans, however, does not affect the anterior longitudinal ligament in the thoracic spine, and that is how one can differentiate these two conditions. The latter disease shares some features seen with DISH, such as a preponderance in males and an association with ligamentous ossification and syndesmophytes. One may distinct these two conditions by noting that in Ankylosing Spondylitis, the bony bridges are slender, vertical, and involve the outer margin of the annulus fibrosus and do not involve the anterior longitudinal ligament. In addition, erosions and bony ankylosis of the sacroiliac and apophyseal joints are not seen in DISH.
Therapy for DISH is based on symptomatic and empiric treatment. There have been no well-designed studies evaluating the effectiveness of any therapy in this disease. In general, physical therapy, analgesics, sedation, antiinflammatory drugs, and muscle relaxants, associated with appropriate diet, have all been successful in managing the majority of patients with DISH.[
Even though few articles until now have focused on indications for surgery, it is generally accepted that surgery is indicated for patients with severe symptoms (such as airway obstruction and/or dysphagia) in whom conservative approach has failed.
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