Edit concept Question Editor Create issue ticket

Diffuse Idiopathic Skeletal Hyperostosis

Forestier Rotes Querol Syndrome

Diffuse idiopathic skeletal hyperostosis is a disorder in which additional bone growth and ossification of ligaments and tendons occur, most commonly in the thoracal spine. It primarily develops in older adults, and it is asymptomatic in the majority of individuals. Symptoms, when present, include pain and stiffness of the affected area, and radiological findings confirm large calcifications. Treatment is indicated in symptomatic patients, and in those with respiratory or gastrointestinal compromise.


Presentation

Despite the fact that many patients with DISH are asymptomatic, numerous symptoms have been reported, and they depend on the localization of skeletal changes. In the case involvement of the thoracal spine, which is the most common localization, back and neck pain, limited range of motion, stiffness, and increased frequency of spinal fractures have all been documented [10], while dysphagia is the principal symptom in cervical spine involvement. Since other parts of the skeletal system may be affected, polyarticular pain, tendinitis, and in severe cases, spinal compression and numbness in lower extremities may be observed [11]. The differential diagnosis of DISH is broad and includes numerous systemic diseases, which is why a thorough diagnostic workup is necessary to exclude other diseases, including ankylosing spondylitis, different spondyloarthropathies, and spondylosis deformans.

Pathologist
  • Treatment provided by a speech and language pathologist focused on the dysphagia and consisted of compensatory management for 2 weeks.[ncbi.nlm.nih.gov]
Pseudotumor
  • A rare case of retro-odontoid pseudotumor combined with diffuse idiopathic skeletal hyperostosis is presented. To discuss the pathomechanism of retro-odontoid pseudotumor in diffuse idiopathic skeletal hyperostosis.[ncbi.nlm.nih.gov]
Localized Pain
  • Other symptoms include localized pain and development of fractures.[symptoma.com]
Excessive Daytime Sleepiness
  • Polysomnography was used for patients with excessive daytime sleepiness for detection of obstructive sleep apnea (OSA).[ncbi.nlm.nih.gov]
Dysostosis
  • Medienarchiv Zur Navigation springen Zur Suche springen Morbus Forestier Krankheit Medium hochladen Wikipedia Ist ein(e) Krankheit, developmental defect during embryogenesis Unterklasse von Kalzinose, spinal disease, developmental anomaly of metabolic origin, dysostosis[commons.wikimedia.org]
Back Pain
  • We report on a 69-year-old male who had severe back pain due to spontaneous symptomatic pseudoarthrosis at the T11-T12 intervertebral space with diffuse idiopathic skeletal hyperostosis.[ncbi.nlm.nih.gov]
  • Kopec JA, Esdaile JM, Abrahamowicz M et al (1995) The Quebec Back Pain Disability Scale. Spine 20:341–352 PubMed CrossRef Google Scholar 10.[doi.org]
Hip Pain
  • Also, he had mild bilateral hip pain [numeric rating scale (NRS): 2]. Hip joint range of motion was 60.3% of normal.[ncbi.nlm.nih.gov]
Spine Stiffness
  • However, spine stiffness and decreased mobility are referred to as possible symptoms. The etiology of DISH is still unknown.[radiopaedia.org]
Spine Pain
  • Chiropractic management of a patient with lumbar spine pain due to synovial cyst: a case report. J Chiropr Med. 2012;11(1):7-15. Kruse RA, Gudavalli S, Cambron J. Chiropractic treatment of a pregnant patient with lumbar radiculopathy.[dcorthoacademy.org]
  • ., Semin Arthritis Rheum, 38 (2009), pp. 361–365 (Level of evidence: 2B) Giles LGF, Singer KP, Vol.3: Clinical anatomy and management of cervical spine pain.[physio-pedia.com]
Urinary Retention
  • We further consider some conditions associated with metabolic syndrome as possible causes of Tycho Brahe's final symptoms (urinary retention, renal failure and coma), including diabetes, alcoholic ketoacidosis and benign prostatic hypertrophy.[ncbi.nlm.nih.gov]

Workup

The diagnosis of DISH is performed according to specific diagnostic criteria supported by various imaging studies. Resnick and Niwayama proposed criteria that include the following [12]:

  • Calcification and ossification of ventrolateral aspects of at least four contiguous vertebrae, with or without excrescences at the vertebral disc-body junctions.
  • Preservation of disc height and integrity in areas where DISH developed, and absence of radiological findings that may indicate degenerative disease.
  • The absence of apophyseal joint, bony ankylosis, and absence of erosion and sclerosis of the sacroiliac joint.

For patients to be evaluated using this criterion, imaging studies are imperative. Usually, plain radiography can show abnormal bone formation and multiple large calcifications along the vertebral column, particularly if the changes occur in the thoracal spine, and almost all patients have involvement of the thoracic vertebrae. Extraspinal calcifications may be observed in numerous joints, including the calcaneus, olecranon, metacarpal and phalangeal joints of the hand, in which capsular hyperostosis is observed in more than 10% of patients [13].

Despite the fact that plain radiography is effective in recognizing the changes in DISH, CT and MRI studies are more effective in determining the magnitude of the lesions. More importantly, they are helpful in assessing complications and associated findings, such as compression of the spinal cord, as well as tracheal and esophageal obstruction. These findings can be of great value in determining whether surgical therapy should be instated in these patients.

Schmorl's Nodes
  • Parameters evaluated were disc space height, disc protrusion, subchondral cysts/sclerosis, Schmorl nodes, vacuum phenomenon, and posterior elements including costovertebral and facet joints.[ncbi.nlm.nih.gov]
Increased Bone Density
  • Although most cases of hypercalcemia are found to have decreased bone mineralization, inactivation of calcium sensing receptor may induce a promitogenic response to hypercalcemia resulting in increased bone density.[ncbi.nlm.nih.gov]

Treatment

Treatment of DISH is targeted at alleviating symptoms that occur as a result of the disease, and treatment is often unnecessary in asymptomatic patients. Depending on which part of the skeletal system is involved and the severity of complaints, treatment involves rehabilitation, lifestyle changes, pain alleviation with non-steroidal anti-inflammatory drugs (NSAIDs), and in severe cases, surgery.

For patients with little or no complaints, pain can be managed with NSAIDs, including paracetamol and ibuprofen (unless these patients present with contraindications, such as peptic ulcer disease), while regular exercise and proper dietary habits have been established to be important in these patients.

For patients with dysphagia, and mechanical obstruction of respiratory and gastrointestinal passages, initial tracheostomy and subsequent osteophysectomy is indicated, and shows good results [14].

Prognosis

Because DISH uncommonly produces symptoms, the long-term prognosis is generally good. In rare cases, however, cervical spine involvement may lead to significant complications, which necessitate surgical treatment. For these reasons, a prompt workup is necessary for patients who are symptomatic, but with proper therapy, the outcomes are very good. The disease itself does not pose a significant risk to the patient and shows a slow progression. In fact, several studies established a less frequent presence of back pain and similar symptoms in patients with DISH compared to control subjects.

Etiology

The cause and pathogenesis of DISH remain unknown. However, several factors, including mechanical, genetic and environmental have been determined to play a role in the etiology of this disorder [4]. Aortic pulsations and its pressure on the adjacent skeletal structures, HLA mutations, exposure to fluoride, use of isotretinoin and other derivatives of vitamin A, and degenerative changes have all been hypothesized to contribute to this disease, but no solid conclusions have been made yet. Metabolic diseases, such as diabetes mellitus and acromegaly, and other diseases in which cartilage development is impaired, have also been investigated as potential factors in the development of DISH.

Epidemiology

Certain studies estimated that prevalence rates for DISH are approximately 19% for men and 4% for women older than 50 years, with small variations [5] [6]. Prevalence rates increase as the age increases, and certain studies have determined that DISH is present in more than 30% of males older than 65 years, and more than 50% of males older than 80 years [7]. These studies have also identified that blood pressure and increased body mass index (BMI) positively correlated with the development of this disorder. DISH very rarely occurs in adults under 40 years of age, while ethnic predilection has not been documented yet.

Sex distribution
Age distribution

Pathophysiology

The hallmark of DISH is ossification of entheses - bony attachments of ligaments, tendons, and joint capsules, and development of osteophytes. As a result of these changes, numerous pathological features are observed, including focal and diffuse calcifications, periosteal bone formation on the surface of vertebral bodies, paraspinal connective tissue fibrosis, and many other [8]. When these changes occur in the cervical spine, development of large osteophytes may lead to direct compression of the esophagus, resulting in dysphagia, while tracheal displacement and airway compromise may also occur [9]. Spinal cord compression open link and increased risk of fractures are also established in these patients because of the changes that occur in patients with DISH.

It is important to distinguish DISH from other skeletal diseases of similar pathogenesis, such as ankylosing spondylitis since this disorder produces non-inflammatory changes in the skeletal system.

Prevention

The means of direct prevention of DISH are currently not established. However, since this disease is associated with obesity, hypertension, and metabolic diseases such as diabetes mellitus, preventive measures may include proper dietary habits and regular physical exercise.

Summary

Diffuse idiopathic skeletal hyperostosis (DISH) is a generalized skeletal disease which is characterized by ossification of bony entheses, including tendons, ligaments, and joint capsules, with resultant development of osteophytes [1]. Since its initial description in the 1950s [2], numerous advances have been made in terms of epidemiology, diagnosis, and treatment, but the cause of DISH remains unknown. It is established that this disease is more present in men than women and that it is most commonly diagnosed in males older than 50 years. The thoracal spine and the anterior longitudinal ligament is described as the most common location of this disease. Cervical spine, the pelvis, calcaneum and ulnar olecranon are also sites where this disorder is reported [3]. Although the majority of patients are asymptomatic, patients may have significant complaints, depending on the site of ossification. Thoracal spine involvement is usually accompanied with back pain and stiffness, and if the cervical spine is affected, hyperostosis may lead to dysphagia and breathing difficulties, as a result of mechanical obstruction of the trachea and the esophagus. Other symptoms include localized pain and development of fractures. The diagnosis is made by imaging studies, which reveal large calcifications and computed tomography (CT scan) and magnetic resonance imaging (MRI) are superior to plain X-rays in diagnosing DISH and other associated findings. Diagnostic criteria include ossification of at least 4 contiguous vertebrae, preservation of the intervertebral disc space and its height, and absence of apophyseal joints, ankylosis, erosion or other sacroiliac inflammatory changes. Treatment is often delayed, as many patients do not report symptoms. Physical therapy is an important part of the long-term management of patients who report pain and reduced motion, and lifestyle, as well as dietary habit changes, are also indicated. Appropriate therapeutic and surgical procedures are necessary for patients with the compromise of the airway and the esophagus, including tracheostomy and osteophysectomy, to reestablish safe passage of air and food content.

Patient Information

Diffuse idiopathic skeletal hyperostosis (DISH) is a disease in which the sites where tendons and ligaments attach to the bone tend to transform into bone tissue and result in the extensive bone growth and calcifications. It is still unknown why this disease occurs, and it is thought that mechanical, genetic, and environmental factors all play a role in the pathogenesis of this disease. It is established that the risk factor for DISH is male gender and increased age, because the rates of DISH rise as the age increases, and certain studies indicate that more than 30% of males over 65 years have this disease. This disease is very rarely seen before 40 years of age. Additionally, it was established that blood pressure and obesity correlate with this disease, which provides evidence that dietary and lifestyle habits are important in preventing this disorder. DISH can occur at any site in the body. The most common location where DISH develops is the spine, but other sites are reported as well, including the hips, elbows, the patella, and the bones of the hands and feet. The majority of individuals with DISH are asymptomatic, but when symptoms are present, they include pain, back stiffness, and limited range of motion. When DISH develops in the vertebrae of the neck, direct compression of the airways and gastrointestinal tract may occur, which can lead to symptoms of painful and difficult swallowing, voice changes, and breathing difficulties. The diagnosis of DISH is made by performing a plain X-ray, which can clearly show the presence of calcifications, and virtually all patients have these calcifications in the spine. Computed tomography and magnetic resonance imaging are used in assessing potential complications, including compression of the airways, and can detect other associated findings, such as spinal cord compression, which occurs in rare cases. Treatment involves symptomatic therapy, including pain relief, rehabilitation, and if necessary, surgical treatment to correct the changes that occurred as a result of this disease. The prognosis is generally good, as the disease does not pose a threat to the patient, but in the case of the airway compromise, prompt surgical therapy is vital in preventing the occurrence of other complications. Because this disease is correlated with increased blood pressure and obesity, proper dietary habits and regular physical exercise have been implicated as measures of prevention against DISH.

References

Article

  1. Miyazawa N, Akiyama I. Ossification of the ligamentum flavum of the cervical spine. J Neurosurg Sci. 2007;51(3):139-144.
  2. Forestier J, Rotes-Querol J. Senile Ankylosing Hyperostosis of the Spine . Annals of the Rheumatic Diseases. 1950;9(4):321-330.
  3. Cammisa M, De Serio A, Guglielmi G. Diffuse idiopathic skeletal hyperostosis. Eur J Radiol. 1998y;27(1):S7-11.
  4. Nascimento FA, Gatto LAM, Lages RO, Neto HM, Demartini Z, Koppe GL. Diffuse idiopathic skeletal hyperostosis: A review. Surgical Neurology International. 2014;5(3):S122-S125.
  5. Mader R. Diffuse Idiopathic Skeletal Hyperostosis: Time for a Change. J Rheumatol. 2008;35;377-379.
  6. Westerveld LA, van Ufford HM, Verlaan JJ, et al. The prevalence of diffuse idiopathic skeletal hyperostosis in an outpatient population in the Netherlands. J Rheumatol. 2008;35(8):1635-8.
  7. Holton KF, Denard PJ, Yoo JU, et al. Diffuse idiopathic skeletal hyperostosis (DISH) and its relation to back pain among older men: The MrOS Study. Seminars in arthritis and rheumatism. 2011;41(2):131-138.
  8. Taljanovic MS, Hunter TB, Wisneski RJ et-al. Imaging characteristics of diffuse idiopathic skeletal hyperostosis with an emphasis on acute spinal fractures: review. AJR Am J Roentgenol. 2009;193(3):S10-19.
  9. Fox TP, Desai MK, Cavenagh T, Mew E. Diffuse idiopathic skeletal hyperostosis: a rare cause of dysphagia and dysphonia. BMJ Case Reports. 2013;bcr2013008978.
  10. Olivieri I, D'Angelo S, Cutro MS, et al. Diffuse idiopathic skeletal hyperostosis may give the typical postural abnormalities of advanced ankylosing spondylitis. Rheumatology (Oxford). 2007;46(11):1709-1711.
  11. Rothschild BM. Diffuse idiopathic skeletal hyperostosis (DISH): misconceptions and reality. Clin Rheumatol. 1985;4:207-12.
  12. Resnick D, Niwayama G. Diagnosis of bone and joint disorders. 2nd ed. Philadelphia: WB Saunders;1988.
  13. Scutellari PN, Orzincolo C, Princivalle M, Franceschini F. Diffuse idiopathic skeletal hyperostosis. Review of diagnostic criteria and analysis of 915 cases. [Article in Italian] Radiol Med. 1992;83(6):729-36.
  14. Burduk PK, Wierzchowska M, Grzelalak L, et al. Diffuse idiopathic skeletal hyperostosis inducted stridor and dysphagia. Otolaryngol Pol. 2008;62(2):138-40.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2019-07-11 19:57