Diffuse Idiopathic Skeletal Hyperostosis in a Patient With Shoulder Pain.

A 74-year-old man was referred to physical therapy by his primary care physician for insidious onset of right shoulder pain. Following examination, spinal radiographs and the human leukocyte antigen (HLA-B27) test, which is used to identify common autoimmune disorders, were completed. The radiographs revealed multilevel changes consistent with diffuse idiopathic skeletal hyperostosis, and the HLA-B27 test was positive. J Orthop Sports Phys Ther 2020;50(4):215. doi:10.2519/jospt.2020.9243.

Forestier's syndrome : a rare cause of dysphagia. A case report and review of the literature.

We report a 72-year-old male with Forestier's syndrome suffering of dysphagia due to an anterior cervical calcification, unusually great in both volume and extent. Its resection by anterior approach allowed the immediate restoration of a normal swallowing. A bony resection is sufficient in case of Forestier's syndrome, but it must be associated with fixation in case of degenerative osteophyte with disc instability. Long-term follow-up is necessary because the recurrence of the calcification is slow but frequent.

The controlled study of diffuse idiopathic skeletal hyperostosis for the assessment of physical function in elderly populations.

BACKGROUND: Diffuse idiopathic skeletal hyperostosis (DISH) is associated with increasing age, obesity, and diabetes mellitus. However, little is known about the clinical impacts of DISH on physical function and spinal deformity in elderly populations. The purpose of this study was to elucidate the influence of DISH on physical function, spinal deformity, and health-related quality of life (HRQOL) in elderly populations. METHODS: We enrolled 504 volunteers (203 men and 301 women, mean age 74.0 years). Height, weight, body mass index (BMI), blood pressure, grip strength, one-leg standing time, sit-and-reach, functional reach, and bone mineral density (BMD) were measured. Using whole spine standing X-rays, the prevalence, location, and numbers of fused vertebra of DISH and spinopelvic parameters were measured. HRQOL measures, including the Oswestry Disability Index and the EuroQuol-5D were also obtained. We compared DISH subjects with control subjects of age and sex matching. We compared DISH subjects in the thoracic spine (T-DISH) to those in the thoraco-lumbar spine (TL-DISH). RESULTS: DISH occurred more frequently in men (14.3%) than in women (4.3%). The mean age was significantly higher of subjects with DISH than of those without DISH. The mean number of fused vertebra by DISH was 5.5 ± 1.5, and T-DISH was observed in 57% cases. DISH group showed greater body weights, BMIs, blood pressures, and BMD in the lumbar spine compared to the control group. No inter-group differences were observed in physical function, HRQOL and spinopelvic parameters. Subjects with TL-DISH had significantly lower values of sit-and-reach and functional reach than those with T-DISH. CONCLUSIONS: Subjects with DISH showed greater body weights, BMIs, blood pressures, and BMD compared to age- and sex-matched controls, while physical function, spinal alignment, and HRQOL were comparable between groups.

Severe hip joint motion restriction in a patient with diffuse idiopathic skeletal hyperostosis: A case report.

RATIONALE: Severe limitation of motion (LOM) in the spine occasionally occurs in patients with diffuse idiopathic skeletal hyperostosis (DISH). However, in extraspinal areas, significant LOM has rarely been reported. In this study, we report a patient with DISH who had severe motion restriction within both hip joints. PATIENT CONCERNS: A 57-year-old man presented with a 10-year history of LOM of bilateral hip joints. He had gait difficulty due to shortened stride length induced by LOM. Also, he had mild bilateral hip pain [numeric rating scale (NRS): 2]. DIAGNOSES: Hip joint range of motion was 60.3% of normal. The patient had bridging ossification along the anterolateral borders of 7 contiguous vertebrae (T10 to L4) without the findings of ankylosing spondylitis or degenerative disc disease. In addition, severe hyperostosis was diffusely formed around bilateral hip joints. He was diagnosed with DISH. INTERVENTIONS: No specific treatment was performed for controlling LOM of bilateral hip joints. Meloxicam 15 mg was administered to the patient for the management of mild bilateral hip pain. OUTCOMES: At 2-month follow-up visit, the degree of LOM in the bilateral hip joints was not changed. However, the patient's pain severity was reduced from NRS 2 to 1. LESSONS: We showed that DISH can cause significant motion restriction due to severe hyperostosis in the bilateral hip joints.

Bone mineral density changes over time in diffuse idiopathic skeletal hyperostosis of the thoracic spine.

Diffuse idiopathic skeletal hyperostosis (DISH) is an increasingly prevalent ankylosing condition. Patients with DISH have an increased risk of spinal fractures, hypothetically the result of biomechanical changes in the spine. The aim of this study was to analyze the occurrence of biomechanical stress shielding in patients with DISH. To do this, bone mineral density (BMD) was measured longitudinally in the vertebral bodies of subjects with and without DISH and in the newly formed bone of subjects with DISH. The presence of DISH was evaluated using Resnick criteria on two chest computed tomography (CT) scans taken at least 2.5 years apart from subjects over 50 years of age. Three groups were identified: pre-DISH (individuals who developed DISH after the first CT scan), definite DISH (individuals who had DISH on both CT scans), and controls (individuals with no DISH). Hounsfield units (HU) were measured in the newly formed bone and in predefined anterior and posterior portions of the involved vertebral bodies. Mean BMD of the newly formed bone increased significantly (mean ΔHU 137.5; p < 0.01) during a mean interval of 5 years in the cranial, middle, and caudally involved vertebral segments of both DISH groups. Mean BMD of the vertebral bodies in the ankylotic segments in the DISH groups did not significantly differ from that of the non-ankylotic vertebral bodies of the same subject. In contrast to our hypothesis, the HU value of the vertebral body decreased more in the control group than in the DISH groups; however, statistical significance was only reached at the cranial level in the anterior part of the vertebral body (p = 0.048). Our data suggest that 1) vertebral BMD is not influenced by the presence of DISH and 2) increased spinal stiffness may play a more important role than vertebral BMD in the increased fracture risk of and the typical fracture patterns observed in individuals with DISH.

Anterior longitudinal ligament in diffuse idiopathic skeletal hyperostosis: Ossified or displaced?

Diffuse idiopathic skeletal hyperostosis (DISH) is often theorized to be an ossification of the anterior longitudinal ligament (ALL). Using computed tomography (CT) imaging and cryomacrotome sectioning, we investigated the spatial relationship between the ALL and newly formed bone in DISH to test this hypothesis. In the current study, four human cadaveric spines diagnosed with DISH using CT imaging were frozen and sectioned using a cryomacrotome. Photographs were obtained of the specimen at 125 µm intervals. Manual segmentations of the ALL on cryomacrotome photographs were projected onto the three-dimensional reconstructed CT scans. The presence and location of newly formed bone were assessed in relationship to the location of the ALL. The ALL could be identified and segmented on the photographs at all levels. The ALL was located at the midline at levels where no new bone had formed. At the locations where new bone had abundantly formed, the ALL was displaced towards to the contralateral side and not replaced by bony tissue. The displacement of the-morphologically normal appearing-ALL away from the newly formed bone implies that newly formed bone in DISH may not originate from the ALL. © 2018 The Authors. Journal of Orthopaedic Research® Published by Wiley Periodicals, Inc. on behalf of Orthopaedic Research Society J Orthop Res 36:2491-2496, 2018.

Thoracic spondylolisthesis and spinal cord compression in diffuse idiopathic skeletal hyperostosis: a case report.

BACKGROUND: Diffuse idiopathic skeletal hyperostosis has long been regarded as a benign asymptomatic clinical entity with an innocuous clinical course. Neurological complications are rare in diffuse idiopathic skeletal hyperostosis. However, if they do occur, the consequences are often significant enough to warrant major neurosurgical intervention. Neurological complications occur when the pathological process of ossification in diffuse idiopathic skeletal hyperostosis extends to other vertebral ligaments, causing ossification of the posterior longitudinal ligaments and/or ossification of the ligamentum flavum. Thoracic spondylolisthesis with spinal cord compression in diffuse idiopathic skeletal hyperostosis has not previously been reported in the literature. CASE PRESENTATION: A 78-year-old Japanese man presented with a 6-month history of gait disturbance. A magnetic resonance imaging scan of his cervical and thoracic spine revealed anterior spondylolisthesis and severe cord compression at T3 to T4 and T10 to T11, as well as high signal intensity in a T2-weighted image at T10/11. Computed tomography revealed diffuse idiopathic skeletal hyperostosis at T4 to T10. He underwent partial laminectomy of T10 and posterior fusion of T9 to T12. The postoperative magnetic resonance imaging revealed resolution of the spinal cord compression and an improvement in the high signal intensity on the T2-weighted image. CONCLUSIONS: We report the first case of thoracic spondylolisthesis and spinal cord compression in diffuse idiopathic skeletal hyperostosis. Neurosurgical intervention resulted in a significant improvement of our patient's neurological symptoms.

Disruption of biomineralization pathways in spinal tissues of a mouse model of diffuse idiopathic skeletal hyperostosis.

Equilibrative nucleoside transporter 1 (ENT1) mediates passage of adenosine across the plasma membrane. We reported previously that mice lacking ENT1 (ENT1(-/-)) exhibit progressive ectopic mineralization of spinal tissues resembling diffuse idiopathic skeletal hyperostosis (DISH) in humans. Here, we investigated mechanisms underlying aberrant mineralization in ENT1(-/-) mice. Micro-CT revealed ectopic mineralization of spinal tissues in both male and female ENT1(-/-) mice, involving the annulus fibrosus of the intervertebral discs (IVDs) of older mice. IVDs were isolated from wild-type and ENT1(-/-) mice at 2months of age (prior to disc mineralization), 4, and 6months of age (disc mineralization present) and processed for real-time PCR, cell isolation, or histology. Relative to the expression of ENTs in other tissues, ENT1 was the primary nucleoside transporter expressed in wild-type IVDs and mediated the functional uptake of [(3)H]2-chloroadenosine by annulus fibrosus cells. No differences in candidate gene expression were detected in IVDs from ENT1(-/-) and wild-type mice at 2 or 4months of age. However, at 6months of age, expression of genes that inhibit biomineralization Mgp, Enpp1, Ank, and Spp1 were reduced in IVDs from ENT1(-/-) mice. To assess whether changes detected in ENT1(-/-) mice were cell autonomous, annulus fibrosus cell cultures were established. Compared to wild-type cells, cells isolated from ENT1(-/-) IVDs at 2 or 6months of age demonstrated greater activity of alkaline phosphatase, a promoter of biomineralization. Cells from 2-month-old ENT1(-/-) mice also showed greater mineralization than wild-type. Interestingly, altered localization of alkaline phosphatase activity was detected in the inner annulus fibrosus of ENT1(-/-) mice in vivo. Alkaline phosphatase activity, together with the marked reduction in mineralization inhibitors, is consistent with the mineralization of IVDs seen in ENT1(-/-) mice at older ages. These findings establish that both cell-autonomous and systemic mechanisms contribute to ectopic mineralization in ENT1(-/-) mice.

The prevalence of diffuse idiopathic skeletal hyperostosis in Japan - the first report of measurement by CT and review of the literature.

BACKGROUND: Diffuse idiopathic skeletal hyperostosis (DISH) is prone to be accompanied by a spinal column fracture which is resistant to conservative therapy. This major characteristic of DISH is not recognized adequately by physicians, because the disease's detailed pathological condition has not yet been investigated. Therefore, the purposes of this study were to investigate the prevalence of DISH using computed tomography (CT), and to validate the reliability of CT interpretation. METHODS: Subjects were 558 patients (300 male and 258 female) who underwent both CT of chest to pelvis and x-ray of chest and abdomen from August 2011 to July 2012 at any department other than orthopedic surgery in our institution. The definition of DISH based on x-ray as well as CT was the presence of consecutive fused vertebral bodies according to Resnick's criteria. The prevalence of DISH based on both modalities was calculated in all subjects. For 107 subjects extracted at random, intra- (Cohen kappa) and inter-observer error (Fleiss kappa) were calculated and the levels of fused segments were investigated. RESULTS: Ninety-eight of 558 subjects (17.6%) were diagnosed as DISH by x-ray, and 152 (27.2%) by CT. Among males, 70 of 300 subjects (23.3%) were diagnosed by x-ray, and 116 (38.7%) by CT. Among females, 28 of 258 subjects (10.9%) were diagnosed by x-ray and 36 (14.0%) by CT. The levels of fused segments were presented from thoracic spine to lumbar spine, especially the middle and lower thoracic spine. Cohen kappa of x-ray was 0.587, and that of CT was 0.825. Fleiss kappa of x-ray was 0.552, and that of CT was 0.643. CONCLUSIONS: The prevalence of DISH based on CT was 27.1%, which was higher than that of x-ray. In addition, intra- and inter-observer error by review of CT was less than that of x-ray. CT evaluation would be a better method for precise understanding of the state of DISH.

The impact of concomitant diffuse idiopathic skeletal hyperostosis on the achievement of minimal disease activity in subjects with psoriatic arthritis.

Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by ossification of different entheses. Psoriatic arthritis (PsA) is a seronegative spondyloarthritis associated with psoriasis. Given the possible overlap of the two diseases, we assessed whether DISH presence may affect PsA clinical outcomes. Also, predictors of DISH presence in the cohort were investigated. Consecutive PsA patients from two Italian Rheumatology Research Units were enrolled. Subjects were splitted into two groups, according to the current treatment (TNF-α blockers or traditional DMARDs). All patients underwent a rheumatologic examination, blood sample collections and spine radiographs. Information about traditional vascular risk factors was recorded. In each patient, the presence of minimal disease activity was evaluated and the presence of DISH was established according to the Resnick and Niwayama criteria. Among the 80 enrolled subjects (57.5 % men, mean age 56.5 ± 11.1 years), the overall prevalence of DISH was 30.0 %. Patients with DISH were older, with higher BMI and waist circumference. DISH subjects showed worsen BASMI, HAQ and ESR. In a multivariate regression model, BASMI was a significant predictor of DISH presence (OR 3.027, 95 % CI 1.449-6.325, p = 0.003). The prevalence of MDA was lower in DISH patients than in no-DISH (16.7 vs 41.1 %, p = 0.041), and the presence of DISH was a predictor of not achieving MDA (OR 3.485, 95 % CI 1.051-11.550, p = 0.041). PsA subjects with DISH showed worsen indices of spine mobility and articular function and lower prevalence of minimal disease activity than no-DISH patients.

Proximal Junctional Failure After Long-Segment Instrumentation for Degenerative Lumbar Kyphosis With Ankylosing Spinal Disorder.

STUDY DESIGN: Case report. OBJECTIVE: We report a case of proximal junctional failure at the ankylosed, but not the mobile, junction after segmental instrumented fusion for degenerative lumbar kyphosis with ankylosing spinal disorder. SUMMARY OF BACKGROUND DATA: Proximal junctional failure (PJF) and proximal junctional kyphosis (PJK) are important complications that occur subsequent to long-segment instrumentation for correction of adult spinal deformity. Thus far, most studies have focused on the mobile junction as a site at which PJK/PJF can occur, and little is known about the relationship between PJK/PJF and ankylosing spinal disorders such as diffuse idiopathic skeletal hyperostosis. METHODS: The patient was an 82-year-old female with degenerative lumbar kyphosis. She had abnormal confluent hyperostosis in the anterior longitudinal ligaments from Th5 to Th10. The patient was treated operatively with spinal instrumented fusion from Th10 to the sacrum. RESULTS: Four weeks subsequent to initial surgery, the patient developed progressive lower extremity paresis caused by the uppermost instrumented vertebrae fracture (Th10) and adjacent subluxation (Th9). Extension of fusion to Th5 with decompression at Th9-Th10 was performed. However, the patient showed no improvement in neurological function. CONCLUSION: PJF can occur at the ankylosing site above the uppermost instrumented vertebrae after long-segment instrumentation for adult spinal deformity. PJF in the ankylosed spine may cause severe fracture instability and cord deficit. The ankylosed spine should be integrated into the objective determination of materials contributing to the appropriate selection of fusion levels. LEVEL OF EVIDENCE: 3.

Naturally occurring spinal hyperostosis in dogs as a model for human spinal disorders.

Both spondylosis and diffuse idiopathic skeletal hyperostosis (DISH) are prevalent in humans and are considered distinct entities. Nowadays, the term spondylosis is in the biomedical literature mostly used when concurrently degenerative disc disease is present. In companion animals, many reports on spondylosis, often without intervertebral disc degeneration, are described. The nomenclature and the definitions of both spondylosis and DISH in biomedical and veterinary literature should be more in line to facilitate comparison. Spondylosis and DISH occur in dogs spontaneously and can co-occur in one animal. Specifically, Boxers may serve as translational disease models for the elucidation of the gene(s) involved in the (etio)pathogenesis of spondylosis and DISH or serve as a test population for newly developed treatment options.

Good outcomes of percutaneous fixation of spinal fractures in ankylosing spinal disorders.

INTRODUCTION: The ankylosed spine is prone to trauma even with after application of force at low energy levels. Multi-level vertebral bony fusions produce long lever arms, susceptible to fracture, with an increased risk of neurological injury. Additional problems result from delayed presentation and osteoporosis. These patients are also often at high risk of complications, making conventional open spinal surgery less appealing. We present the outcomes of percutaneous fixation and its advantages in this high risk group of patients. METHODS: A retrospective review of a series of 10 patients with a diagnosis of either ankylosing spondylitis or diffuse idiopathic skeletal hyperostosis (DISH). All patients had sustained a spinal fracture between January 2009 and January 2013 and underwent percutaneous fixation using Medtronic longitude system (Minneapolis, USA) with Polyaxial screws. All were followed up with outcomes, complications and functional scores (Oswestry Disability Index (ODI) and Pain Visual Analogue scores (VAS). RESULTS: The mean patient age was 68. There was a delayed presentation in seven patients, of which two presented with neurological compromise. The neurological deficit did not change with surgery and there were no neurological complications as a result of surgery. The mean length of stay was 24 days, with no direct surgical complications. The mean drop in haemoglobin level was 2.1, with three patients requiring a blood transfusion. The patients were followed up to a mean of 22 months, with a mean ODI of 16 and pain VAS of 1.1. At the time of follow up, two patients had died with no loss to follow up. DISCUSSION: Even minor trauma can result in fracture in the ankylosed spine, requiring a high index of suspicion from the physician. The risks of missing such a fracture are significant neurological injury. The biomechanics of the spine are significantly altered, and treatment is demanding. We propose that minimally invasive spinal surgery can achieve good outcomes, low complication rates and high rates of satisfaction.

Otolaryngologic manifestations of diffuse idiopathic skeletal hyperostosis.

Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by formation of large cervical osteophytes that may compress the posterior wall of the aerodigestive tract. It is a rare cause of dysphagia in the elderly. The aim of this study was to investigate the various otolaryngologic manifestations of DISH. Eleven elderly patients with DISH were included in the study. All patients presented with dysphagia that was graded on the swallowing screening tool (EAT-10), and the diagnosis of DISH was based on computed tomographic criteria. The patients were subjected to otolaryngologic examination and flexible laryngoscopy. Polysomnography was used for patients with excessive daytime sleepiness for detection of obstructive sleep apnea (OSA). In addition to dysphagia of varying severity, OSA was found in nine patients, change of voice in six, globus sensation in seven, aspiration in three, and cervical pain in seven. Flexible laryngoscopy showed bulging of the posterior pharyngeal wall in all patients. DISH may be an unrecognized contributory factor to both dysphagia and OSA in the elderly. Change of voice, aspiration, globus sensation, and cervical pain are other otolaryngologic manifestations that may be encountered symptoms of the disease. An otolaryngologist should be aware of the disease that may be overlooked, and computed tomography is a confirmatory diagnostic method.

Prevalence and progression of radiographic ossification of the posterior longitudinal ligament and associated factors in the Japanese population: a 3-year follow-up of the ROAD study.

SUMMARY: The prevalence of radiographic cervical ossification of the posterior longitudinal ligament (OPLL) in 1,562 Japanese from a population-based cohort was 1.9 %. The presence of OPLL showed a significant association with the femoral neck bone mineral density (BMD), presence of diffuse idiopathic skeletal hyperostosis (DISH) and plasma pentosidine levels. Only one new case of radiographic OPLL was detected, but OPLL progressed in all affected subjects. INTRODUCTION: The purpose of this study was to clarify the prevalence and progression of radiographic OPLL and the associated factors, using the population-based cohort Research on Osteoarthritis/osteoporosis Against Disability (ROAD). METHODS: In the ROAD study, 1,690 participants underwent X-ray examination of the entire spine and both knees. Radiographic OPLL, lumbar spondylosis, knee osteoarthritis and DISH were diagnosed by a single, well-experienced orthopaedic surgeon. An interviewer-administered questionnaire and tests for anthropometric measurements were administered, and the BMDs of the lumbar spine and proximal femur were determined. A new OPLL case was considered if heterotopic ossification in the posterior longitudinal ligament was absent at baseline but present during follow-up. Progression was defined as an increase in the maximum length or width of the ossification at follow-up over that at baseline. RESULTS: Radiographic OPLL was detected in 30 (17 men, 13 women) of 1,562 individuals who underwent X-ray examination of the cervical spine (prevalence = 1.9 %). Its prevalence was significantly higher in men than in women (p = 0.007), but no association with age was observed. In a logistic regression analysis, OPLL showed a significant association with the femoral neck BMD, presence of DISH and plasma pentosidine levels. Only one new case of radiographic OPLL was detected, but OPLL progressed in all affected subjects. CONCLUSION: This population-based study clarified the prevalence of radiographic OPLL in the Japanese population as well as its progression. OPLL showed significant association with plasma pentosidine levels, BMD and DISH.

Diffuse idiopathic skeletal hyperostosis: clinical features and pathogenic mechanisms.

Diffuse idiopathic skeletal hyperostosis (DISH) is a systemic condition characterized by the ossification and calcification of ligaments and entheses. DISH is observed on all continents and in all races, but most commonly in men over 50 years of age. Although DISH is asymptomatic in most individuals, the condition is often an indicator of underlying metabolic disease, and the presence of spinal or extraspinal ossifications can sometimes lead to symptoms including pain, stiffness, a reduced range of articular motion, and dysphagia, as well as increasing the risk of unstable spinal fractures. The aetiology of DISH is poorly understood, and the roles of the many factors that might be involved in the development of excess bone are not well delineated. The study of pathophysiological aspects of DISH is made difficult by the formal diagnosis requiring the presence of multiple contiguous fully formed bridging ossifications, which probably represent advanced stages of DISH. In this Review, the reader is provided with an up-to-date discussion of the epidemiological, aetiological and clinical aspects of DISH. Existing classification criteria (which, in the absence of diagnostic criteria, are used to establish a diagnosis of DISH) are also considered, together with the need for modified criteria that enable timely identification of early phases in the development of DISH.

Pathogenesis of hyperostosis: a key role for mesenchymatous cells?

The similarities between diffuse idiopathic skeletal hyperostosis (DISH) and some forms of ankylosing spondylitis suggest shared pathogenic mechanisms. Entheseal ossification progresses at the same rate in the two conditions, and spondyloarthritis was the first diagnosis considered in several families with genetically determined early-onset DISH. However, DISH may be a heterogeneous condition, as the presence of peripheral calcifications in some families suggests pathogenic similarities with several animal models combining entheseal ossification and peripheral calcifications, as well as with X-linked familial hypophosphatemia and dentin-matrix-protein mutations. In the far more common presentation of hyperostosis without calcifications, entheseal ossification may be related to abnormal osteoblastic differentiation of mesenchymatous stem cells normally found around the intervertebral disks, in the vertebral periosteum, and in the anterior and posterior longitudinal ligaments. The many factors suspected of promoting this abnormal differentiation include bone morphogenetic proteins (BMPs), retinoids, and various hormonal factors; in addition, adipokines such as leptin are the focus of growing interest based on the well-documented association between DISH and obesity. Confirmation of the role for mesenchymatous cells in DISH should encourage investigations of mesenchymatous cells as possible pathogenic contributors to the entheseal abnormalities seen in spondyloarthritis. These cells normally exert immunosuppressive effects, which may be subverted in spondyloarthritis, notably by a T-cell population that homes specifically to the entheses.