Diffuse idiopathic skeletal hyperostosis (DISH) is a systemic condition characterized by characteristic ossification patterns that can occur in the spine and peripheral entheses.[1] DISH most commonly affects the spine and often presents as back pain and stiffness.[2] Resnick et al. originally coined the term DISH in 1975 and is now the most commonly used term to describe this condition in the literature.[3] The term is both inclusive and descriptive of the disorder. Forestier and Rotes-Querol initially described the underlying pathology in pathologic specimens and a series of 200 patients in 1950, who called it “senile ankylosing hyperostosis.”[4]
The ossifications are classically described as in the spine as flowing ossifications along the anterolateral aspect in at least three successive vertebral levels, or four contiguous vertebrae. Although less common, peripheral enthesopathy can occur at the shoulder, elbow, knee, or calcaneus.[2] DISH in the spine most commonly occurs on the right side of the thoracic spinal segment.
While the etiology remains poorly defined, various risk factors have been identified in the literature, including gout, hyperlipidemia, and diabetes. HLA-B8 is common in both DISH and diabetes mellitus.[2] As such, high rates of diabetes mellitus, hyperuricemia, and hyperlipidemia have been present in patients with DISH.[5] Unlike other seronegative spondyloarthropathies, no apparent relationship has been identified between DISH and HLA-B27.
Several recent studies have revealed a significant association between DISH and metabolic disorders, such as diabetes mellitus, hyperinsulinemia, obesity, dyslipidemia, and hyperuricemia.[6][7][8] While these proposed clinical associations have been suggested in the literature, the pathogenesis and proposed mechanism contributing to these characteristic ossification patterns remain debatable. Some authors have attempted to describe underlying causes related to mechanical stress and strain patterns, exposure to various toxic factors, and genetic contributions.[1] Further, angiogenesis remains a relatively popular investigative common denominator that, at least in theory, provides a feasible pathophysiologic linkage in various clinical manifestations of DISH. For example, carotid atherosclerosis and DISH correlate at higher rates in patients with metabolic syndrome. Furthermore, higher prevalence rates of aortic valve sclerosis have been previously identified as an independent risk factor predicting cardiovascular events in patients with DISH.[9]
There have been relatively few publications regarding epidemiologic data for DISH.[10] DISH is rarely reported in patients younger than 50 years of age.[2] The overall incidence in the general population is 6 to 12%. In the general population over 50 years of age, DISH occurs in about 25% of males, and 15% of females. These prevalence rates increase in patients over 80 years of age, with male and female prevalence rates of 28% and 26%, respectively. Current postulations are that the disease course begins between the third and fifth decade of life but manifests clinically at a later age.[2]
In an autopsy study, evidence of DISH was present in approximately one-fourth of the specimens, with a mean age of 65 years (minimum 50 years).[11] Population-based studies have demonstrated an overall prevalence rate ranging from 2.5% and 28%, with relatively increased rates attributable to incremental increases in age, and a gender predilection favoring DISH diagnosis in males compared to females.[12][13][14][15] Additionally, DISH may be more common in the white population compared to black, Asian, and Native-American populations.[14]
A 2016 study out of Japan reported discord between DISH prevalence rates diagnosed via computed tomography (CT) scans compared to radiographic imaging alone. The prevalence in the general population in Japan was 17.6% and 27.2% as reported from radiographs and CT imaging, respectively.[16]
DISH in the spine most commonly occurs on the right side of the thoracic region. The literature supports the original theory of the protective effect and mechanical barrier preventing DISH formation on the contralateral (i.e., left) side of the thoracic spine secondary to the pulsatile descending aorta.[2] Moreover, recent studies have demonstrated that DISH presenting in the cervical and lumbar regions of the spine demonstrate definitively different ossification patterns.
For example, a 2017 study reported that newly formed bone in the cervical spine occurred mainly anterior to the vertebral bodies, which is in contrast to the anterolateral bone deposition pattern seen in DISH of the thoracic spine.[17] A plausible pathophysiologic explanation regarding the varying ossification patterns concerns the regional arterial anatomy. Support for the pulsatile protective mechanical barrier theory is further highlighted by studies noting patients with situs inversus demonstrate DISH on the left side of the thoracic spine.[18] Finally, both the cervical and lumbar spine DISH studies have reported symmetrical non-marginal syndesmophyte ossification patterns.
The traditional diagnostic criteria for DISH had previously included three major elements[19][20][3][21]:
The current widespread definition of DISH by Resnick and Niwayama has recently been challenged as these inclusion criteria may be best applied to the more advanced stages of the condition. Moreover, even dating back to 1985, Utsinger had challenged the contemporary definition and advocated to lower the threshold for spinal involvement to two contiguous while incorporating the presence of peripheral enthesopathies.[22]
There have been recent challenges to the validity and consensus agreement regarding the current and evolving level of support behind these specific criteria. A 2013 Delphi exercise found that consensus agreement and definitive literature support may only be available for the following elements:
Differentiating DISH from AS
Healthcare providers often are inevitably confused regarding the clinical differentiation in discerning a diagnosis of DISH as opposed to AS. In short, the main distinguishing features of DISH compared with AS can be summarized into the following elements[23][24]:
Aside from the current controversial and debated diagnostic criteria, the classic clinical presentation remains that of an older patient with increasing back pain and stiffness. Soft tissue involvement secondary to osteophytes at the cervical segments may result in dysphagia, hoarseness, sleep apnea, and difficult intubation.[25][26] Further evaluation with a swallow study or consultation to otolaryngology or gastroenterology may be warranted. Moreover, heightened clinical suspicion is warranted in the elderly patient presenting with acute on chronic symptoms of back pain, especially in the setting of minor trauma. As in any conditions that entail contiguous osseous fusion of vertebral and spinal elements, a longer lever arm is generated throughout the spine which inevitably predisposes to fractures.[27] Thus, all patients require a thorough, comprehensive neurovascular exam and require imaging of the entire spine to mitigate the risk of overlooking fractures in adjacent spinal regions.
Peripheral joint involvement in DISH has some distinctive features[28]:
Peripheral findings often include hyperostosis and tendonitis.[28] In the pelvis, enthesophytes involving the iliac wing and ischial tuberosity may be present.[29] Periarticular hyperostosis and tendinous ossifications have also been reported in the hip, knee, shoulder, elbow, hand, and wrist.[2]
Laboratory values (erythrocyte sedimentation rate, C-reactive protein, rheumatoid factor, and antinuclear antibody) are often normal in DISH. Radiographic evaluation with AP and lateral spine imaging in patients with DISH demonstrate “flowing candle wax,” which describes the non-marginal syndesmophytes that project horizontally from the vertebrae and form extra-articular ankylosis, this is distinguishable from the vertical “bamboo spine” that forms intra-articular disc space ossification in ankylosing spondylitis (AS).[2]
Increased radiodensity, preservation of facet joints and disc spaces on spine imaging help further distinguish DISH from AS, which may present with osteopenia and degenerative changes.[2] The association between DISH and low bone density remains controversial in the literature.[30] However, the theoretical, clinical association exists regarding the relatively increased risk for vertebral fractures in the setting of low energy mechanisms. While patients with osteoporosis can present with vertebral compression fractures while merely lying in bed in a nursing home,[31][32][33][34][35], vertebral fractures have been reported in the literature in patients with DISH following elective, unrelated surgical procedures. One report from 2012 highlighted postoperative incomplete paraplegia following a routine total hip replacement.[36]
Given that the thoracic spine is most commonly involved, the clinician should have a low threshold for obtaining thoracic spine or chest radiographs even in patients with primary neck or low back pain, stiffness, and diffuse extremity complaints.[37] Establishing a diagnosis of DISH based on thoracic imaging may prevent further unnecessary workup and surgical interventions. Technetium bone scan in DISH may demonstrate increased uptake in the involved regions; however, this finding may be confused with metastatic disease and thus not usually helpful in non-traumatic scenarios.[38] Lumbar spine involvement should be evaluated radiographically at the lumbar spine and the pelvis, as the presence of sacroiliac pathology may direct the diagnostic workup toward other conditions, such as seronegative spondyloarthropathies.
Minor trauma in patients with DISH may result in fracture and instability. These may commonly result in missed injuries that lead to neurologic compromises and delayed treatment. Occult fractures in these patients must be aggressively evaluated using advanced imaging (CT, MRI, or CT myelogram).[39] Extraspinal complaints in patients with DISH may be evaluated accordingly using plain radiographs.
In most patients with isolated back discomfort, the mainstays of treatment include activity modification, physical therapy, bracing, NSAIDs, and bisphosphonates.[2]
Surgical decompression and stabilization may be indicated for specific sequelae of the condition, including fracture, cervical myelopathy, lumbar stenosis, neurologic deficits, infection, or painful deformity.
The differential diagnosis for patients with back pain, stiffness, and spondylophytosis also includes, but is not limited to[40]:
Patients with DISH who sustain spine fractures are at increased risk of instability due to ligamentous calcification and increased deforming forces secondary to vertebral ankylosis. Increased length of instrumentation is often necessary to accommodate the lever arms acting on the fracture site. Meyer demonstrated that surgical treatment of cervical fractures in elderly patients with DISH correlates with a 15% rate of mortality compared to 67% after conservative treatment, highlighting the importance of prompt diagnosis, evaluation, and treatment after trauma in patients with DISH.[15]
Heterotopic ossification (HO) is a frequent complication following total hip arthroplasty (THA) in patients with DISH (30 to 56%).[41][42] In contrast, patients without DISH in the cited series only had a 10 to 22% rate of HO.[41][42] Fahrer et al. reported low rates of pain and functional limitations and recommended no prophylaxis against HO in patients with DISH after undergoing THA.[42]
Patients and family members should receive education regarding the increased susceptibility to significantly morbid (and even fatal) complications even in the setting of low energy traumatic mechanisms and elective procedures.
While semantics exist regarding the absolute and consensus agreement of DISH diagnostic criteria, there remain several critical components to the workup and management of these patients when presenting even in the setting of minor traumas. Emergency medical services (EMS) providers, nurses, advanced practitioners, clinicians, and surgeons require efficient and coordinated care to obtain comprehensive histories, examinations (including mandatory neurovascular examinations), and to have a low threshold to obtain appropriate imaging to ensure underlying fractures do not go undetected. Clinical deterioration warrants urgent evaluation and management modalities. [Level of evidence: I]
[1] | Mader R,Verlaan JJ,Buskila D, Diffuse idiopathic skeletal hyperostosis: clinical features and pathogenic mechanisms. Nature reviews. Rheumatology. 2013 Dec [PubMed PMID: 24189840] |
[2] | Belanger TA,Rowe DE, Diffuse idiopathic skeletal hyperostosis: musculoskeletal manifestations. The Journal of the American Academy of Orthopaedic Surgeons. 2001 Jul-Aug [PubMed PMID: 11476536] |
[3] | Resnick D,Shaul SR,Robins JM, Diffuse idiopathic skeletal hyperostosis (DISH): Forestier's disease with extraspinal manifestations. Radiology. 1975 Jun [PubMed PMID: 1129458] |
[4] | FORESTIER J,ROTES-QUEROL J, Senile ankylosing hyperostosis of the spine. Annals of the rheumatic diseases. 1950 Dec [PubMed PMID: 14800245] |
[5] | Vezyroglou G,Mitropoulos A,Antoniadis C, A metabolic syndrome in diffuse idiopathic skeletal hyperostosis. A controlled study. The Journal of rheumatology. 1996 Apr [PubMed PMID: 8730125] |
[6] | Mader R,Novofastovski I,Schwartz N,Rosner E, Serum adiponectin levels in patients with diffuse idiopathic skeletal hyperostosis (DISH). Clinical rheumatology. 2018 Oct [PubMed PMID: 30121711] |
[7] | Mader R,Novofestovski I,Adawi M,Lavi I, Metabolic syndrome and cardiovascular risk in patients with diffuse idiopathic skeletal hyperostosis. Seminars in arthritis and rheumatism. 2009 Apr [PubMed PMID: 18304611] |
[8] | Pariente-Rodrigo E,Sgaramella GA,Olmos-Martínez JM,Pini-Valdivieso SF,Landeras-Alvaro R,Hernández-Hernández JL, Relationship between diffuse idiopathic skeletal hyperostosis, abdominal aortic calcification and associated metabolic disorders: Data from the Camargo Cohort. Medicina clinica. 2017 Sep 8 [PubMed PMID: 28283270] |
[9] | Orden AO,David JM,Díaz RP,Nardi NN,Ejarque AC,Yöchler AB, Association of diffuse idiopathic skeletal hyperostosis and aortic valve sclerosis. Medicina. 2014 [PubMed PMID: 24918668] |
[10] | Cassim B,Mody GM,Rubin DL, The prevalence of diffuse idiopathic skeletal hyperostosis in African blacks. British journal of rheumatology. 1990 Apr [PubMed PMID: 2322769] |
[11] | Boachie-Adjei O,Bullough PG, Incidence of ankylosing hyperostosis of the spine (Forestier's disease) at autopsy. Spine. 1987 Oct [PubMed PMID: 3500518] |
[12] | Mata S,Wolfe F,Joseph L,Esdaile JM, Absence of an association of rheumatoid arthritis and diffuse idiopathic skeletal hyperostosis: a case-control study. The Journal of rheumatology. 1995 Nov [PubMed PMID: 8596145] |
[13] | Mata S,Chhem RK,Fortin PR,Joseph L,Esdaile JM, Comprehensive radiographic evaluation of diffuse idiopathic skeletal hyperostosis: development and interrater reliability of a scoring system. Seminars in arthritis and rheumatism. 1998 Oct [PubMed PMID: 9806369] |
[14] | Weinfeld RM,Olson PN,Maki DD,Griffiths HJ, The prevalence of diffuse idiopathic skeletal hyperostosis (DISH) in two large American Midwest metropolitan hospital populations. Skeletal radiology. 1997 Apr [PubMed PMID: 9151370] |
[15] | Meyer PR Jr, Diffuse idiopathic skeletal hyperostosis in the cervical spine. Clinical orthopaedics and related research. 1999 Feb [PubMed PMID: 10078128] |
[16] | Hirasawa A,Wakao N,Kamiya M,Takeuchi M,Kawanami K,Murotani K,Matsuo T,Deie M, The prevalence of diffuse idiopathic skeletal hyperostosis in Japan - the first report of measurement by CT and review of the literature. Journal of orthopaedic science : official journal of the Japanese Orthopaedic Association. 2016 May [PubMed PMID: 26948246] |
[17] | Bakker JT,Kuperus JS,Kuijf HJ,Oner FC,de Jong PA,Verlaan JJ, Morphological characteristics of diffuse idiopathic skeletal hyperostosis in the cervical spine. PloS one. 2017 [PubMed PMID: 29155874] |
[18] | Carile L,Verdone F,Aiello A,Buongusto G, Diffuse idiopathic skeletal hyperostosis and situs viscerum inversus. The Journal of rheumatology. 1989 Aug [PubMed PMID: 2585410] |
[19] | Donnally III CJ,Varacallo M, Lumbar Spondylolysis And Spondylolisthesis . 2019 Jan [PubMed PMID: 28846329] |
[20] | Donnally III CJ,Varacallo M, Lumbar Degenerative Disk Disease . 2019 Jan [PubMed PMID: 28846354] |
[21] | Akhondi H,Varacallo M, Rheumatoid Spondylitis . 2019 Jan [PubMed PMID: 30335321] |
[22] | Utsinger PD, Diffuse idiopathic skeletal hyperostosis. Clinics in rheumatic diseases. 1985 Aug [PubMed PMID: 3899489] |
[23] | Mader R,Buskila D,Verlaan JJ,Atzeni F,Olivieri I,Pappone N,Di Girolamo C,Sarzi-Puttini P, Developing new classification criteria for diffuse idiopathic skeletal hyperostosis: back to square one. Rheumatology (Oxford, England). 2013 Feb [PubMed PMID: 23024057] |
[24] | Durback MA,Edelstein G,Schumacher HR Jr, Abnormalities of the sacroiliac joints in diffuse idiopathic skeletal hyperostosis: demonstration by computed tomography. The Journal of rheumatology. 1988 Oct [PubMed PMID: 3264578] |
[25] | Kritzer RO,Rose JE, Diffuse idiopathic skeletal hyperostosis presenting with thoracic outlet syndrome and dysphagia. Neurosurgery. 1988 Jun [PubMed PMID: 3262204] |
[26] | Palmer JH,Ball DR, Awake tracheal intubation with the intubating laryngeal mask in a patient with diffuse idiopathic skeletal hyperostosis. Anaesthesia. 2000 Jan [PubMed PMID: 10594434] |
[27] | Vazan M,Ryang YM,Barz M,Török E,Gempt J,Meyer B, Ankylosing Spinal Disease-Diagnosis and Treatment of Spine Fractures. World neurosurgery. 2019 Mar [PubMed PMID: 30476662] |
[28] | Garber EK,Silver S, Pedal manifestations of DISH. Foot & ankle. 1982 Jul-Aug [PubMed PMID: 7129264] |
[29] | Haller J,Resnick D,Miller CW,Schils JP,Kerr R,Bielecki D,Sartoris DJ,Gundry CR, Diffuse idiopathic skeletal hyperostosis: diagnostic significance of radiographic abnormalities of the pelvis. Radiology. 1989 Sep [PubMed PMID: 2788894] |
[30] | Sohn S,Chung CK,Han I,Park SB,Kim H, Increased Bone Mineral Density in Cervical or Thoracic Diffuse Idiopathic Skeletal Hyperostosis (DISH): A Case-Control Study. Journal of clinical densitometry : the official journal of the International Society for Clinical Densitometry. 2018 Jan - Mar [PubMed PMID: 27712986] |
[31] | Varacallo M,Pizzutillo P, Osteoporosis in Spinal Cord Injuries . 2019 Jan [PubMed PMID: 30252365] |
[32] | Varacallo MA,Fox EJ, Osteoporosis and its complications. The Medical clinics of North America. 2014 Jul [PubMed PMID: 24994054] |
[33] | Porter JL,Varacallo M, Osteoporosis . 2019 Jan [PubMed PMID: 28722930] |
[34] | Varacallo M,Pizzutillo P, Osteopenia . 2019 Jan [PubMed PMID: 29763053] |
[35] | Varacallo MA,Fox EJ,Paul EM,Hassenbein SE,Warlow PM, Patients' response toward an automated orthopedic osteoporosis intervention program. Geriatric orthopaedic surgery & rehabilitation. 2013 Sep [PubMed PMID: 24319621] |
[36] | Königshausen M,Dudda M,Merle C,Schildhauer TA,Fehmer T, Thoracic vertebral body fracture after total hip replacement in diffuse idiopathic skeletal hyperostosis. Orthopedics. 2012 Jun [PubMed PMID: 22691644] |
[37] | Mata S,Hill RO,Joseph L,Kaplan P,Dussault R,Watts CS,Fitzcharles MA,Shiroky JB,Fortin PR,Esdaile JM, Chest radiographs as a screening test for diffuse idiopathic skeletal hyperostosis. The Journal of rheumatology. 1993 Nov [PubMed PMID: 8308777] |
[38] | Lee S,Coel M,Ko J,Edwards J, Diffuse idiopathic skeletal hyperostosis can resemble metastases on bone scan. Clinical nuclear medicine. 1993 Sep [PubMed PMID: 8403726] |
[39] | Paley D,Schwartz M,Cooper P,Harris WR,Levine AM, Fractures of the spine in diffuse idiopathic skeletal hyperostosis. Clinical orthopaedics and related research. 1991 Jun [PubMed PMID: 2044283] |
[40] | Nascimento FA,Gatto LA,Lages RO,Neto HM,Demartini Z,Koppe GL, Diffuse idiopathic skeletal hyperostosis: A review. Surgical neurology international. 2014 [PubMed PMID: 24843807] |
[41] | Guillemin F,Mainard D,Rolland H,Delagoutte JP, Antivitamin K prevents heterotopic ossification after hip arthroplasty in diffuse idiopathic skeletal hyperostosis. A retrospective study in 67 patients. Acta orthopaedica Scandinavica. 1995 Apr [PubMed PMID: 7740940] |
[42] | Fahrer H,Koch P,Ballmer P,Enzler P,Gerber N, Ectopic ossification following total hip arthroplasty: is diffuse idiopathic skeletal hyperostosis a risk factor? British journal of rheumatology. 1988 Jun [PubMed PMID: 3132214] |