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

1. Interesting Case Presentation
Dr. Ankit Raiyani
Medicine Dept.
LTMMC & GH

2. History
A 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. 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 positive
CNS examination-
• Conscious. Oriented, co-operative
• Speech, cognition, mood, memory- normal
• No CN palsy

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 movements
Other systems -NAD

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. 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. • 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. X-rays of Cx spine

9. MRI Cx Spine

10. X-ray LS Spine & SI
Jt

11. X-ray B/L Elbow Jt

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. Dorsal spine in DISH
Lumbar spine with early changes of DISH
Flowing wax app of bridging enthesophytes

14. Radiological features of ankylosing
spondylitis
• sacroiliitis
• Loss of normal lordosis
• Osteitis, reactive sclerosis
• Squaring of vertebral bodies
• Bridging syndesmophytes

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. HLA-B27 prevalence
1. General population-
– North Indian caste/population groups- 2.7-29%13
– Mumbai- 1.48-9.6% 14
2. Ankylosing spondylitis- 90-95% 15,16
3. DISH- 3-8%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. • 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. 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. Diagnosis
• The classification criteria set by Resnick and Niwayama
requires 8
1. 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. • 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. complications attributable to DISH -
• dysphagia, unstable spinal fractures, spinal
stenosis, postsurgical heterotropic
ossifications, difficult intubation, difficult
gastroscopy, aspiration pneumonia,
myelopathy…

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. 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. 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. 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, Klinefelter's
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. References
1. Sharma RR, Mahapatra A, Pawar SJ, Sousa J, Lad SD, Athale SD. Spinal cord and
cauda equina compression in 'DISH'. Neurol India 2001;49:148
2. 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; 136:763–768
4. 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. 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) [Ankylosing
Hyperostosis 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 diffuse
idiopathic skeletal hyperostosis: development and interrater reliability of a scoring system. Semin
Arthritis Rheum. 1998;28:88–96. doi: 10.1016/S0049-0172(98)80041-3
12.Sarzi-Puttini, Piercarlo; Atzeni, Fabiola New developments in our understanding of DISH (diffuse
idiopathic skeletal hyperostosis) Current Opinion in Rheumatology: May 2004 - Volume 16 - Issue 3 - pp
287-292
13.Shankarkumar, U HLA-B27 allele diversity in Indians: impact of ethnic origin and the caste
system. British Journal of Biomedical Science 2003
14.S.U. Chhaya HLA-B27 polymorphism in Mumbai, Western India. Tissue antigens vol 66 issue 1
17. Vertebral ankylosing hyperostosis (Forestier's disease) and HLA antigens in Pima Indians.
Spagnola AM, Bennett PH, Terasaki PI Arthritis Rheum. 1978 May;21(4):467-72.

29. THANK YOU