Diffuse Idiopathic Skeletal Hyperostosis (DISH)
Dr. KS Dhillon
Introduction
Diffuse idiopathic skeletal hyperostosis (DISH) is also known as Forestier disease. It describes a phenomenon that is characterized by a tendency toward ossification of ligaments. It usually affects the spine [1,2]. Ossification of the longitudinal ligaments, especially the anterior ligaments, of the spine produces a tortuous paravertebral mass anterior to and distinct from the vertebral bodies (fig 1) [3]. When the thoracic anterior longitudinal ligament is ossified, the areas of ossification usually meet without fusion. Motion is actually possible, unlike lumbar vertebral bridging, which is associated with loss of lumbar motion. The zygapophyseal and sacroiliac joints are not involved in DISH. The intervening intervertebral disk space is preserved.
DISH is a phenomenon that is primarily age-related, rather than a disease. It is usually asymptomatic and is discovered incidentally on imaging studies taken for some other reason. Clinical manifestations, such as stiffness and back pain [4], may result from neuropathy or from physical impingement by bony overgrowth.
There is no cure for DISH. Treatment is symptomatic and empirical. Surgery may be indicated to provide relief of severe symptoms, such as dysphagia or airway obstruction.
Fig 1
Background
Diffuse idiopathic skeletal hyperostosis (DISH) was first described by Forestier and Rotes-Querol in 1948. They reported nine patients, ranging in age from 50 to 73 years, who suffered from spinal rigidity and had exuberant osteophytes on radiologic studies. The authors termed the condition as senile vertebral ankylosing hyperostosis [5].
DISH is well represented in the paleontologic and zoologic records. It is found in 1-3% of monkeys and baboons, as well as in gorillas, bears, horses, camels, bison, canids, musk oxen, felids, and whales [6,7,8,9,10, 11,12]. DISH was also present in dinosaurs [13,14,15]. It occurs in 15-25% of older mammals.
Pathophysiology
Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by a tendency toward ossification of tendons, ligaments, and joint capsule insertions [16]. DISH is usually a completely asymptomatic phenomenon, with no alterations detectable on history taking or through physical examination.
Etiology
The causes of DISH are unknown. DISH results from calcification of entheses. Higher rates of DISH are seen with age over 50 years, in men compared with women, and in the White population compared with Black [4]. Additional risk factors include [17] :
Hypertension
Metabolic syndrome
Obesity
Type 2 diabetes mellitus
Hyperuricemia
A study by Lantsman et al [18] found that abdominal computed tomography showed significantly more visceral adipose tissue, as well as a significantly higher ratio of visceral to subcutaneous adipose tissue, in 43 patients with DISH as compared to 42 controls. The authors also noted that visceral adipose tissue is by itself associated with bone proliferation. They suggested that it is potentially a pathogenic pathway for enthesopathic excessive bone production in DISH.
Epidemiology
In the United States DISH is present in approximately 19% of men and 4% of women older than 50 years [19].
Mori et al [20] carried out a study of 3013 Japanese patients (1261 females and 1752 males) with a mean age of 65 years. The computed tomography based prevalence of thoracic DISH was 8.7%. The posterior longitudinal ligament of the cervical spine is ossified in 2% of Japanese people and in 0.16% of white people [21]. The anterior longitudinal ligament is calcified in 24% of patients who have posterior longitudinal ligament ossification [22].
In the Netherlands, DISH was reported in 17% of individuals [23]. A South African study of patients 40 years of age and older found that the prevalence of DISH in African blacks was 3.8% in men and 4.2% in women. The prevalence increased with increasing age, from 1% in the 40-49 year age group to 13.6% in those over 70 years of age [24].
DISH is present in approximately 19% of men over the age of 50 years but is found in only 4% of women in the same age group. It is uncommon in patients less than 50 years of age and is extremely rare in patients who are younger than 40 years.
A Finnish study [25] revealed the age frequency in Finnish men to be as follows:
40-49 years - 0.3%
50-59 years - 2.7%
60-69 years - 8.4%
70 years or older - 11.2%
The same study showed the age frequency in Finnish women to be as follows:
40-49 years - 0.2%
50-59 years - 1.7%
60-69 years - 4.3%
70 years or older - 6.9%
A study of middle-aged and elderly people in a rural town in Japan identified DISH in 17.5% of the 413 participants. The prevalence of DISH tends to increase with age, from 3.1% in individuals in their 50s to 14.0% in those in their 60s, 24.3% in those in their 70s, and 29.0% in those in their 80s. Besides aging, other independent factors associated with DISH were hypertension, male sex, and elevated body mass index [27].
Prognosis
Patients with DISH have an excellent prognosis. The condition is rarely life-threatening and it causes limited morbidity.
DISH does not appear to be a disease but it appears to be a phenomenon. A double-blind controlled evaluation in which controls and patients with DISH were drawn from the same population revealed no associated pathology. The incidence of arthritis, bursitis, and tendinitis is the same in patients with DISH and in controls. The character of back pain and duration is the same in patients with DISH and in controls. A history of back injury was found to be twice as frequent in control subjects as compared to patients with DISH. Back flexibility was also similar in the two groups. Patients with DISH who had decreased lumbar spinal motion had a lower incidence of back pain, implying that DISH may have a protective effect [19,27]. One study has shown that DISH may be protective against back pain [28].
Another study showed that people with DISH were more likely to experience physical functional impairment. This included a 1.72-fold increased odds of self-reported difficulty in bending and worse grip strength [29].
A study by Yamada et al [30] involving 1063 patients treated surgically for lumbar spinal stenosis found that reoperations were more frequent in patients with DISH that extended to the lumbar segment. Twenty-two percent of such patients underwent reoperation as compared with 7.3% of patients without lumbar DISH. The authors were of the opinion that the unfavorable outcomes in patients with lumbar DISH may be due to the decreased number of mobile lumbar segments.
Excessive ligamentous calcification can impinge on other structures such as the esophagus. There are reports of stridor and dysphagia in patients with DISH [31,32]. When compression of the larynx occurs patients can develop vocal cord paresis and airway obstruction [33].
Ossification of the posterior longitudinal ligament can impinge on the spinal cord on rare occasions.
Reduced vertebral column flexibility that occurs with DISH predisposes to fractures of the vertebra. Sixty percent of the fractures occur in the cervical spine, 34.5% in the thoracic spine, and 5.5% in the lumbar spine. These fractures result from minor trauma, preoperative and postoperative positioning, or intraoperative maneuvers [3,34,35]. Besides fully ankylosed spines, partially ankylosed spines also are at risk, with fractures occurring adjacent to the fused regions [36]. The risk of fracture increases with the number of ankylosed vertebrae [37]. Obesity is an additional risk factor for fracture [38].
Clinical Presentation
DISH is usually an asymptomatic phenomenon. It is usually discovered incidentally on imaging studies taken for some other reason.
Clinical manifestations, which may result from neuropathy or from physical impingement by bony overgrowth include [1] :
Acute monoarticular synovitis
Limited range of spinal motion
Polyarticular pain
Neck/thoracic/lumbar/extremity pain
Dysphagia [39]
Increased susceptibility to unstable spinal fractures
Airway obstruction of varying degrees
If vertebral fusion is quite extensive physical examination will show reduction in the range of spinal motion [40]. Since uniform vertebral involvement is extremely rare, usually there are no relatable findings.
Diagnostic Considerations
Other problems to consider in the differential diagnosis are:
Hypervitaminosis A
Retinoids
Pachydermoperiostosis
Spondylosis deformans
Fluorosis
Osteomalacia
Acromegaly
Hypophosphatemia
Hypoparathyroidism
Ossified posterior longitudinal ligament (OPLL)
Differential Diagnoses
Ankylosing Spondylitis
Ankylosing Spondylitis and Undifferentiated Spondyloarthropathy
Laboratory Studies
No laboratory tests are needed. Studies have found that there is no difference between patients with vertebral hyperostosis and control patients for prevalence of diabetes mellitus, and plasma glucose [41,42].
Imaging Studies
DISH involves the thoracic vertebrae in 100% of cases, the lumbar vertebrae in 68-90%, and the cervical vertebrae in 65-78% of the cases. Ligamentous ossification affects both sides of the lumbar vertebral column. Generally, it tends to be unilateral in the human spine.
In the thoracic spine, DISH prominence is seen on the right lateral aspect. This is apparently related to aortic pulsations. The left-sided overgrowth is much reduced, also probably because of the influence of aortic pulsations. There is left-sided prominence in individuals with situs inversus.
The earliest sign of DISH appears to be new bone formation adjacent to the midportion of the vertebral body. Radiologically there is a dense line paralleling the longitudinal axis of the spine but separated by a clearly definable space.
The most commonly used diagnostic criteria for DISH are those set by Resnick and Niwayama [43,44]. The criteria include:
Calcification and ossification along the ventrolateral aspects of at least four contiguous vertebral bodies. There may or may not be localized pointed excrescences at intervening vertebral body–disk junctions.
Relative preservation of intervertebral disc height in the involved areas, with the absence of extensive radiographic changes of degenerative disc disease, including vacuum phenomena and vertebral body marginal sclerosis.
Absence of apophyseal joint bony ankylosis and sacroiliac joint sclerosis, erosion, or intra-articular bony fusion
Julkunen and colleagues [45] also suggested similar criteria as those from Resnick and Niwayama but also included the presence of bridges connecting two vertebral bodies in at least two sites on the thoracic spine. are as follows:
The ligamentous ossification phenomenon is not limited to the spine. Exuberant ossification at sites of tendon, ligamentous, or joint capsule insertion is suggestive of the diagnosis. Such ossification can occur at any site of ligament and perhaps tendon insertion. A study by Slonimsky et al [46] found pelvic enthesopathy on CT to be significantly more prevalent in patients with DISH compared with matched control patients.
Entheseal reaction at the iliac crest and ischial tuberosities is referred to as pelvic whiskering. It is usually quite exuberant. Such whiskering has been noted in two-thirds of iliac crests studied and in 53% of ischial tuberosities. Entheseal reaction has been noted in 42% of lesser and 36% of greater trochanters of the femur. Enthesial spurs at the site of insertion of the quadriceps into the patella were present in 29% of patients studied. Osseous bridging of the tibia and fibula was noted in 10% of patients. Distal metacarpal and phalangeal capsular hyperostosis was present in 13% of patients with DISH [19].
DISH is associated with greater kyphosis in older men and women. DISH, however, is not significantly associated with a change in kyphosis over 4-5 years. In women followed over 15 years, those with DISH had less progression of kyphosis than those without DISH [47].
In patients with DISH who have experienced low-impact trauma,
whole spine computed tomography (CT) is mandatory. The rigid spinal structure resulting from DISH increases susceptibility to spinal fracture, and radiographs have low specificity for detecting those fractures.
Lantsman et al in their study of 147 patients with verified DISH who presented to the emergency department after low-energy trauma found that there were significantly more acute fractures on whole-spine CT than on radiographs [48].
The authors also found that in 57% of acute fractures, the site of tenderness was not indicative of the fractured spinal segment. Furthermore, two patients with tenderness at the site of one fracture also had asymptomatic distant fractures [48].
Histopathology
Histologic examination of vertebral specimens from patients with DISH shows partial or complete bone bridges. These bridges consist of cortical haversian bone, accompanied by morphological changes in the adjacent intervertebral disc [49]. Ossified tissues in DISH are composed of normal-appearing haversian bone [50].
Treatment
There is no cure for diffuse idiopathic skeletal hyperostosis. Treatment is symptomatic and empirical. The treatment includes the following [2] :
Anti-inflammatory drugs
Muscle relaxants
Physical therapy
Analgesics
Sedation
Breathing difficulty treated with nighttime continuous positive airway pressure therapy has been reported [51].
Surgery may be indicated in patients with severe symptoms, such as airway obstruction or dysphagia, that have not responded to conservative treatment [2]. Tracheotomy is the treatment of choice in most cases of DISH-related airway obstruction. Yosimatsu et al [52] reported a case of successful treatment of airway obstruction with careful airway management and surgical osteophytectomy [52].
Conclusion
DISH is a common disease that is usually asymptomatic and is characterized by new bone formation. The etiology remains unknown. In patients with DISH, there are extensive proliferative processes in the musculoskeletal system. DISH pathogenesis is polygenic. It is influenced by the interaction of many gene variants and environmental factors. There are potential disturbances in various genes with different chromosomal localizations and expressions. Molecular studies are required to clarify and verify the diagnostic criteria. Studying the molecular mechanisms involved in the formation of bone along with identifying the genetic markers associated with DISH, can help to discover the ossification pathogenesis of ligaments and tendon attachments. This could lead to targeted and effective therapies.
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