Diffuse idiopathic skeletal hyperostosis

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Diffuse idiopathic skeletal hyperostosis (DISH) is a common skeletal process of uncertain etiology found in 12 to 18% of Indian populations above 50 years. The primary manifestations of DISH are calcification and ossification of the spinal ligaments, as well as entheseal ossification within extraspinal sites

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Diffuse idiopathic skeletal hyperostosis

  1. 1. Interesting Case Presentation Dr. Ankit Raiyani Medicine Dept. LTMMC & GH
  2. 2. HistoryA 52 year male clerk presented with c/o• Tingling sensation and numbness in both UL along anterior aspect of arm since 2 months• Inability to turn head to either side since 6 months• Inability to supinate both forearms since childhood• No c/o backache, neck pain• No c/o joint pain or swelling• Not a known case of Diabetes, hypertension• Nonaddict
  3. 3. Examination• Central obesity +• Vitals- stable• Short neck with restriction of neck movements• Inability to supinate both forearms, active and passive• No kyphoscoliosis, Lhermitte’s sign positiveCNS examination-• Conscious. Oriented, co-operative• Speech, cognition, mood, memory- normal• No CN palsy
  4. 4. • Motor system- – no wasting, fasciculations UL and LL – Tone- spasticity in both LL, normal in UL – Power- 5/5 in all four limbs – Reflexes- DTR – brisk in both LL, 1+ in UL; abdominal- absent, Plantar B/L extensor• Sensory system- B/L fine touch, pain, temp loss in c5-c6 dermatomes. vibration, jt position intact, Rhomberg’s negative• No cerebellar signs• Gait normal• No involuntary movementsOther systems -NAD
  5. 5. Clinical diagnosis• Cervical radiculomyelopathy with longitudinal level in high cervical cord (C5-C6 level) and transverse localization to lateral and posterior segments• Possible etiology 1. Upper Cervical vertebral involvement 2. CV junction anomaly• with B/L radioulnar joint synostosis
  6. 6. Investigations• Hb- 12.6 gm/dl• WBC- 6500/cmm• ESR- 18 mm/1 hr• CRP- 6 mg/L• Creat- 0.9 mg/dl• T. bili- 0.7 mg/dl• FBS- 96mg/dl• PLBS- 124 mg/dl• Ca- 8.9 mg/dl• PO4- 3.8 mg/dl• ALP- 158• HLA B27- POSITIVE
  7. 7. • Radiological features – X-ray Cx spine(2006)- incomplete bridging enthesophytes involving c4-c7 with preserved disk – X-ray Cx spine(2011)- completely bridging enthesophytes. Fusion of c1-c3 vertebrae – X-ray DL and LS spine(2006 & 2011)- bridging enthesophytes with preserved disk space – MRI Cx spine with screening of SI jt- s/o cervical myeloradiculopathy, no SI jt involvement – X-ray b/l elbow Jt (2006 & 2011)- proximal radioulnar synostosis
  8. 8. X-rays of Cx spine
  9. 9. MRI Cx Spine
  10. 10. X-ray LS Spine & SI Jt
  11. 11. X-ray B/L Elbow Jt
  12. 12. X-ray features in DISH• Flowing calcifications and ossifications along the anterolateral aspect of vertebral bodies• Preservation of disk height(absence of excessive disk disease)• Absence of bony ankylosis of facet joints and absence of sacroiliac erosion, sclerosis, or bony fusion,
  13. 13. Dorsal spine in DISH Lumbar spine with early changes of DISH Flowing wax app of bridging enthesophytes
  14. 14. Radiological features of ankylosing spondylitis• sacroiliitis• Loss of normal lordosis• Osteitis, reactive sclerosis• Squaring of vertebral bodies• Bridging syndesmophytes
  15. 15. DISH vs Ankylosing spondylitis ??• Points favoring DISH- – No backache, morning stiffness – low ESR & CRP – Typical “flowing wax” appearance of osteophytes – No sacroiliitis, – Intervertebral disc relatively preserved• Points favoring Ankylosing spondylitis – HLA B27 positivity
  16. 16. HLA-B27 prevalence1. General population- – North Indian caste/population groups- 2.7-29%13 – Mumbai- 1.48-9.6% 142. Ankylosing spondylitis- 90-95% 15,163. DISH- 3-8%17
  17. 17. Review• Diffuse idiopathic skeletal hyperostosis (DISH) is a common skeletal process of uncertain etiology found in 12 to 18% of Indian populations above 50 years1.• The primary manifestations of DISH are calcification and ossification of the spinal ligaments, as well as entheseal ossification within extraspinal sites 2,3• DISH was first described in the more recent literature by Meyer and Forester in 1938 4. They named this entity “moniliform hyperostosis.”• In 1942, Oppenheimer 5 described ossification of the vertebral ligaments and named this entity “spondylitis ossificans ligamentosa.” In 1950 Forestier 6 systematically studied the abnormalities, which he referred to as “senile ankylosing hyperostosis.”
  18. 18. • In a number of papers published between 1975 and 1978, Resnick and others (2,3,7) reviewed the literature and noted the radiologic findings of DISH were always consistent, although the name given to this entity was not.• Resnick et al.7 , described a large number of extraspinal manifestations which occurred in these patients, often without spinal involvement, and this led to the introduction of the more appropriate term of “diffuse idiopathic skeletal hyperostosis”, emphasizing the generalized nature of this disorder.• Peripheral involvement in DISH- Enthesopathies with subsequent new bone formation, and stiffening of peripheral joints such as metacarpophalangeal joints, elbows, and shoulders.
  19. 19. Etiology• The etiology and pathogenesis of DISH is uncertain.• Research has focused on many possible risk factors, which include: – trauma or occupational stress, – Endocrine abnormalities including acromegaly, hypoparathyroidism, diabetes, growth hormone and somatomedin levels, – Fluorosis, ankylosing spondylitis, spondylitic variants, – Hypervitaminosis A – Genetic factors including HLA-B27, HLA-B5 and HLA-A11.
  20. 20. Diagnosis• The classification criteria set by Resnick and Niwayama requires 81. Involvement of at least 4 contiguous vertebrae of the thoracic spine,2. Preservation of the intervertebral disc space,3. Absence of apophyseal joints or sacroiliac inflammatory changes• Another set of criteria, defined by Utsinger 9 as probable DISH, lowered the threshold for spinal involvement to 3 contiguous vertebral bodies, but added the presence of peripheral enthesopathies to the diagnostic measures.
  21. 21. • Recent studies confirm that patients with DISH have a greater body mass index, higher serum uric acid levels and are more likely to have diabetes mellitus. In addition, DISH is most probably related to abnormal bone cell growth/activity reflecting the influence of metabolic factors that lead to new bone formation.• Serum matrix Gla protein may be a marker of osteometabolic syndromes, such as DISH, that cause hyperostosis.11
  22. 22. complications attributable to DISH -• dysphagia, unstable spinal fractures, spinal stenosis, postsurgical heterotropic ossifications, difficult intubation, difficult gastroscopy, aspiration pneumonia, myelopathy…
  23. 23. • Neurological manifestations of DISH is most common due to involvement of Cx spine.• Possible mechanisms- 1. Spinal canal narrowing leads to compressive myelopathy/radiculopathy 2. Reduced flexibility allows fracture through ossified ligaments following a trivial trauma. This compromises spinal canal lumen 3. Atlantoaxial subluxation may occur more frequently
  24. 24. Management• Treatment should be aimed at the symptomatic relief of pain and stiffness; the prevention, retardation or arrest of progression; the treatment of associated metabolic disorders and the prevention of spontaneous or induced complications.• Change of lifestyle, nutrition and therapeutic options to alleviate pain and stiffness are measures that might improve quality of life in patients affected by DISH• Control of associated metabolic disorders such as hypertension, hyperinsulinaemia with or without hyperglycaemia, hyperlipidaemia and hyperuricaemia may reduce the morbidities associated with these disorders and prevent further progression of the condition
  25. 25. Surgical management-• DISH is considered as benign condition not requiring any aggressive management• However many recent reports suggest that patient with neurological symptoms may require decompression.12,13• Indications for surgery- – Acute complication- post traumatic – Progressive neurological symptoms – Predisposition to falls/neck injury- occupational, other medical conditions – Patient’s decision• Outcome- most studies report favorable outcome with rare cases of recurrence as was feared before12,13
  26. 26. Relation of proximal radioulnar synostosis and Klippel-Feil syndrome to axial skeleton• Proximal radioulnar synostosis- – a/w DDH, club feet, missing or diminutive thumb, coalescence of carpal bones, Symphalangism, dislocation of radius – Seen in Apert’s syndrome, Williams syndrome, Klinefelters syndrome• Klippel-Feil syndrome not associated with DISH or Ankylosing spondylitis• Congenital radioulnar synostosis not reported in association of either DISH or ankylosing spondylitis. May occur as acquired form with DISH8• Congenital radioulnar synostosis may occur in association with Klippel-Feil syndrome in fetal alcohol syndrome
  27. 27. References1. Sharma RR, Mahapatra A, Pawar SJ, Sousa J, Lad SD, Athale SD. Spinal cord and cauda equina compression in DISH. Neurol India 2001;49:1482. Boachie-Adjei O, Bullough PG. Incidence of ankylosing hyperostosis of the spine (Forestier’s disease) at autopsy. Spine 1987; 12:739–743.3. Utsinger PD, Resnick D, Shapiro R. Diffuse skeletal abnormalities in Forestier’s disease. Arch Intern Med 1976; 136:763–7684. Resnick D, Shaul SR, Robins JM. Diffuse idiopathic skeletal hyperostosis: Forestier’s disease with extra-spinal manifestations. Radiology 1975; 115:513– 524.5. Meyer M, Forester E. Considerations pathogeniques sur l’hyperostose moniliforme du flanc droit de la colonne dorsale. Rev Rhum Maladies Ostéoarticulaires 1938; 5:286–293. Meyer M, Forester E. Considerations pathogeniques sur l’hyperostose moniliforme du flanc droit de la colonne dorsale. Rev Rhum Maladies Ostéoarticulaires 1938; 5:286–293.6. Oppenheimer A. Calcification and ossification of vertebral ligaments (spondylitis ossificans ligamentosa). Roentgen study of pathogenesis and clinical significance. Radiology 1942; 38:160–164.
  28. 28. 7.Forestier J, Rotés-Querol J. Senile Ankylosing hyperostosis of the spine. Ann Rheum Dis 1950, 9:321–330.8.Resnick D, Shapiro RF, Wiesner KB, et al. Diffuse idiopathic skeletal hyperostosis (DISH) [AnkylosingHyperostosis of Forestier and Rotés-Querol]. Semin Arthritis Rheum 1978; 7:153–187.9.Resnick D, Niwayama G. Diagnosis of bone and joint disorders. 2nd ed. Philadelphia: WB Saunders;1988:1563-615.10.Utsinger PD. Diffuse idiopathic skeletal hyperostosis. Clin Rheum Dis 1985;11:325-51.11.Mata S, Chhem RK, Fortin PR, Joseph L, Esdaile JM. Comprehensive radiographic evaluation of diffuseidiopathic skeletal hyperostosis: development and interrater reliability of a scoring system. SeminArthritis Rheum. 1998;28:88–96. doi: 10.1016/S0049-0172(98)80041-312.Sarzi-Puttini, Piercarlo; Atzeni, Fabiola New developments in our understanding of DISH (diffuseidiopathic skeletal hyperostosis) Current Opinion in Rheumatology: May 2004 - Volume 16 - Issue 3 - pp287-29213.Shankarkumar, U HLA-B27 allele diversity in Indians: impact of ethnic origin and the castesystem. British Journal of Biomedical Science 200314.S.U. Chhaya HLA-B27 polymorphism in Mumbai, Western India. Tissue antigens vol 66 issue 117. Vertebral ankylosing hyperostosis (Forestiers disease) and HLA antigens in Pima Indians. Spagnola AM, Bennett PH, Terasaki PI Arthritis Rheum. 1978 May;21(4):467-72.
  29. 29. THANK YOU

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