Systemic conditions Flashcards

DISH Ankylosing spondylitis Rheumatoid spondylitis

1
Q

What is DISH?

A
  • Diffuse idopathic skeletal hyperostosis
  • A very common disorder
  • Unknown aetiology
  • characterised by presence of non marginal syndesmophytes at 3 successive levels involving 4 contiguous vertebrae
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2
Q

What is DISH aka ?

A
  • FORRESTIER DISEASE
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3
Q

What is DISH define by?

A
  • By the PRESENCE of NON MARGINAL SYNDESMOPHYTES AT 4 CONTIGUOUS VERTEBRA, 3 successive levels
  • ( different from Ankylosing spondylitis = MARGINAL SYNDESMOPHYTES)
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4
Q

What are syndesmophytes?

A
  • Vertical outgrowths that extend across the disc space
  • Represent CALCIFATION OF THE ANNULUS FIBROSIS and ALL + PLL
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5
Q

Where do syndesmophytes occur?

A
  • Anywhere in the spine
  • usually THORACIC T7-11 region, > on RIGHT side thought to be due to the protective effect of the pulsatile aorta on the left thoracic spine!!!
  • Symmetrical ( R+L) in CERVICAL and LUMBAR SPINE
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6
Q

What is the epidemiology of difuse idiopathic skeletal hyperostosis?

A
  • Overall incidence 6-12%
  • uncommon before 50 years
  • less common in africans, asians

location

  • anywhere in spine
  • most common Thoracic spine - Right side >cervical >lumbar
    • thought due to protective nature of pulsatile aorta on left thoracic spine
    • symmetrical sides in cervical/lumbar
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7
Q

What are the risk factors for developing diffuse idiopathic skeletal hyperostosis?

A
  • Gout
  • Hyperlipidaemia
  • diabetes
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8
Q

What is DISH associated with?

A
  • Lumbar spine stenosis
  • Cervical spine
    • hoarness
    • sleep apnoea
    • cervical myelopathy
    • dysphagia and stridor
  • Spine fx and instablity
    • Ankylosis creates long lever arm-> displacement with minimal force
    • hyperextension injuries are common
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9
Q

What are the symptoms and signs of diffuse idopathix skeletal hyperextosis?

A

Symptoms

  • Often asymptomatic & discovered incidentally
  • thoracic/lumbar spine
    • mild chronic pain
    • stiffness esp in mane
      • aggrevated cold weather
  • cervical
    • pain & stiffness
    • dysphagia
    • stridor
    • hoarness
    • sleep apnoea
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10
Q

What investigaitons are useful in dx of DISH?

A
  • xrays
    • ap and latera spine
    • non marginal syndesmphytes at 3 successive levels ( 4 continous vertebrae)
    • thoracic syndesmophytes on right side
    • c spine ant bone formation with preservation of disc space ( unlike ank spon)
    • lumbar spine- symmetrical syndesmophytes
    • other joint involvement -elbow
  • Technetium bone scan
    • increased uptake in areas of involvement
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11
Q

Is Diffuse idiopathic skeletal hyperextosis related to HLA-B27?

A
  • NO associated with HLA-B28 -pt with DISH and DM
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12
Q

what are the radiographic findings of DISH

A
  • NON MARGINAL SYNDESMOPHYTES at 3 successive levels ( 4 contiguous vertebra)
  • Flowing candlewax
  • preservation of disc space
  • no ostepenia
  • no assoc with HLA- B27
  • age - older middle age
  • No involvement in SI JOINT

cf ankl spon

marginal syndesmophytes, bamboo spine, ossificiation of disc space, osteopenia, strong assoc wiht HLA b27, younger pts, bilateral sacroilitis

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13
Q

What is the tx of someone with Diffuse Idopathic Skeletal Hyperextosis?

A

non op

  • activity modification. physical therapy, brace wear, NSAIDS, BISPHOSPHONATES
    • most cases

OPERATIVE

  • SPINAL DECOMPRESSION and STABILISATION
    • for lumbar stenosis, cervical myelopathy, adult spinal deformity
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14
Q

What are the complications of DISH?

A
  • Mortality
    • C spine trauma in DISH
      • 15% tx operatively
      • 67% tx non operatively
  • Heterotrophic ossification
    • increased risk of HO after THR
    • 30-50% for THR with DISH cf <20% wout
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15
Q

Define ankylosing spondylitis?

A

A chronic systemic autoimmune spondyloarthropathy characterised by

  • HLA- B27 histocompatablity complex positive
  • RH negative- seronegative
  • primarily affecting spine
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16
Q

What is the pathoanatomy of ankylosing spondylitis?

A
  • exact mechanism unknown
  • likely autoimmune reaction to an envirnomental pathogen to gentically susceptible individual
  • theories in relation to HLA- B27
    • HLA- B27 aggregates with peptides in joint -> degenerative cascade
  • Enthesitis
    • entheses inflammation -> bony erosions, surrounding soft tissue ossification and joint ankylosis (diff from RA- synovial)
  • Disc space involvement
    • Inflammation of the annulus -> bridging of osteophyte formation- syndemosphyte
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17
Q

What is the epidemiology of ankylosing spondylitis?

A
  • 4:1 Male : female
  • affects 0.2% caucasians population
  • usually presents at 30 years
    • juvenile form <16 yrs inc enthesitis
    • fewer than 10% HLA-B27 positive pt have symptoms
  • Genetics
    • HLA- B27 on 6th chromosome, B locus
    • genetic predisposition but mode of inheritance is unknown
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18
Q

What are the dx criteria?

A
  • Bilateral sacroilitis
  • +/- uveitis
  • HLA- B27
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19
Q

What are the systemic manifestations?

A
  • Acute anterior uveitis/ iritis
  • Heart disease
  • pulmonary fibrosis
  • renal amyloidosis
  • ascending aortic conditions- regurge, stenosis
  • Klebsilella peumoniae synovitis
    • HLA-B27 more susceptible to these
      *
20
Q

What are the orthopaedic manifestations of ank spon?

A
  • Bilateral sacroilitis
  • progressive spinal kyphotic deformity
  • cervical spine fractures
  • large joint arthritis
21
Q

What is enthesis?

A
  • Insertion of tendon, ligaments or muscle to bone
22
Q

What are the symptoms of ank spon?

A
  • Lumbosacral pain and stiffness
    • most pts
    • worse in mane
    • insidious onset 3rd decade
  • Neck and upper thoracic pain
    • late in life
    • acute neck pain- raise suspicion of fracture
  • Sciatic
  • loss of horizontal gaze
  • shortness of breath
    • costovertebral joint involvement-> reduced chest expansion
23
Q

what are the signs of ank spon?

A
  • Limited chest wall expansion
    • <2cm more specific dx HLA- B27
  • Decreased spine motion
    • Schober test- used to elevate stiffness
  • Kyphotic spine deformity
    • chin on chest flexion- see pic
    • caused by multiple microfx over time
    • Chin brow to vertical angle
      • measured on lateral
      • correction of this angle- improved surgical outcome
  • Hip flexion contraction
    • exam in supine and sitting position helps differentiate sagittal plane imbalance due to hip flexion contractures or kyphotic spinal deformity
  • Sacroiliac provocation test
    • faber test
    • flexion/abduction/ ext rotatation of ipislateral hip causes pain
24
Q

What imaging is useful in dx ank spon?

A
  • Xrays spine
    • full length AP, lateral axial spine
    • negative in 50% with spinal Fx
    • Squaring of vertebra with vertical Marginal syndemosphytes- see pic
    • Late vertebral scalloping - bamboo spine
    • measure chin to brow vertical angle
    • xray of pelvis
      • ​ferguson pelvic tilt view
      • xray beam 10-15 degree cephalad-see SI
      • Bilateral symmetrical sacroiliac erosions

​​​CT

  • Will show bony changes but no active inflammation
  • best for dx of Cervical Fx

MRI

  • Will detect Inflammation - detect early AS
25
What is the tx of ank Spon?
Non operative * **NSAIDS, COX-2 inhibitors and PT** * first line of tx for pain and stiffness * oral steriods not recommended * TNF alpha blocking agents * 2nd line * infliximab, etancerpt, adalimumab * improvement in symptoms Surgery * depends on cervicothoracic fx * spinal deformity * large joint arthritis
26
What is the epidemiology of cervicothoracic fractures in AS?
* Most occur **midcervical and cervicothoracic junction** * Often _extension type- involve all 3 columns_ * maybe occult- consider CT scan * **High mortality rate 2ary to epidural haemorrhage** * _75% neurologic involvement_ * neurologic symtpoms often present late
27
What is the tx of cervicothoracic Fx?
Non operative * **Traction, orthotic, halo immobilisation** * stable spine fractures with no neurological deficits * low weight traction may faciliate reduction Operative * **Spinal decompression with instrumented fusion** * Progressive neurological deficit * epidural haematoma with neurologic compromise * unstable fx pattern * decision to go ant vs post depends on Fx level, presence & location of haematoma & osteoporosis * Outcomes * _high rate of complications_ * progressive deformity * nonunion * hardware failure * infection
28
What are the tx for spinal deformity with AS?
* Kyphotic deformity * elliminate hip conttractures as reason for deformity Surgery * _Lumbar osteotomy_ * thoracolumbar kyphotic deformity * goal to restore sagittal balance & horizontal gaze * **closing wedge (pedicle subtracting) osteotomy** * **hinge located on ant spine** * greater deformity correction 30-40o per level * better fusion & stability due to direct bony apposition * **Vertebral body resection** * entire vertebral body resected and replaced by cage * **Single-level opening wedge osteotomy** * ​hinge on post edde vertebral body * requires rupture of ALL * _C7-T1 cervicothoracic osteotomy_ * for chin to chest deformity * slight under correction with final chin to brow angle 10o * adv vertebral art ext to transv foramen, larger canal diameter * post op halo immobilisation used as pt poor bone quality
29
What is the tx for large joint arthritis in AS?
* Asymmetric involvement of large joints * shoulder and hip commonly involved TX * **Total hip replacement** * pt with severe arthritis hip 2ary to AS * pts have more **VERTICAL/ ANTEVERTED Acetabulum** * may lead to **ANTERIOR DISLOCATIONS** after THR * **Bilateral total hip arthroplasty** * kyphotic deformity due to hip flexion contracture deformity * at risk of dislocation
30
What is the epidemiology of cervical spondylitis?
* Present in **90% of pts with Rheumatoid Arthritis** * Diagnosis often missed * included 3 main types * **Atlantoaxial Subluxation** * **Basiliar Invagination** * **Subaxial Subluxation**
31
What is the classification of Rheumatoid cervical spondylitis?
* **Ranawat** **​** * **Class 1- Pain , no neurologic deficit** * **Class 2-** **Subjective weakness, hyperreflexia, dyssthesias** * **Class 3A-** Objective weakness, long tract signs, **ambulatory** * **Class 3B-** Objective weakness, long tract signs **, non ambulatory**
32
What are the signs and symptoms of rhematoid cervical spondylitis?
Symptoms * Similar to cervical myelopathy * neck pain * neck stiffness * occipital headaches * gradual onset of weakness and loss of sensation Signs * Hyperreflexia * upper & lower extremity weakness * Ataxia- gait instability/loss of hand dexterity
33
What imaging is useful in dx of cevical spondylitis?
* Xrays * Flexion-extension * obtain before elective surgery * CT * better for bone anatomy & surgical planning * MRI * identify degree of spinal cord compression
34
What is the general tx of cervical spondylitis?
Non op * Pharmacology Operative * Spinal decompression and stabilisation * goal is to prevent further neuroloigcal progression and surgery may no reverse existing deficit
35
What is atlantoaxial subluxation?
* Presents in **50-80% pts with RA** * most common to have * **Anterior Subluxation of C1 on C2** ( can have lat/post)
36
what is the mechanism for atlanoaxial subluxation?
* **A pannus forms between the dens and C1** * Leads to **destruction** of **Transverse Ligament & Dens**
37
What measurements are used to define stability on lateral extension-flexion views?
* **Atlanto-Dens Interval = ADI** * **instability = \>3.5mm of motion between flexion and extension views** * instability alone is not an indication for surgery * \> 7mm = alar ligament disruption * **\>10mm** associated with increased risk of neurological injury= **surgery** * **​Space available for cord (SAC) & Posterior atanto-dens interval =PADI** same thing * **\<14 mm** assoc with increaed risk of neurological injury = **surgery** * **\>13 mm-most important radiological finding that may predict complete neural recovery after decompression surgery**
38
What is the tx of atantoaxial subluxations?
Non operative * IN stable atlantoaxial subluxations Operative * **Posterior C1-2 Fusion** * if **ADI \>10mm** (even if no neuro) * **SAC/PADI \<14mm** (even if no neuro) * Progressive myelopathy * indication- must be able to reduce C1 onto C2 - no need to remove C2 post arch * add transarticular screws avoid use of halo * Post op ct to identify location of vertebral arteries * **Occiput -C2 fusion** * if atlantoaxial subluxation is combined with **basilar invagination** * resection of C1 post arch required for complete decompression * **Odontoidectomy** * rarely indicated * used as a secondary proceedure when there is redisual anterior cord compression due to pannus formation that fails to resolve with time following a posterior spinal fusion
39
What is Basilar invagination?
* **Superior migration of Odontoid** * **Tip of dens migrates above foramen magnum** * present in **40% RA pts** * often seen in combination with fixed atlantoaxial subluxation
40
Describe the mechanism of basilar invagination?
* Cranial migration of dens from **EROSION and BONE loss** between **occiput and C1/C2**
41
What lines are useful on lateral xray to dx basilar invagination?
* **Ranawat C1-C2 index** * Most reproducible measurement * centre of C2 pedicle to line conecting the anterior & posterior C1 arches * normal men 17mm, women 15mm * **distance of \<13 mm consistent with impaction**
42
What is the tx of basilar invagination?
* Operative * **C2 to Occiput fusion** * for progressive canal migration \>5mm * neurological compromise * cervicomedullary angle \<135o on MRI * **Transoral/ anterior retropharyngeal odontoid resection** * ​for brainstem compromise
43
What is the epidemioogy of subaxial subluxation?
* Present in 20% pt wth RA * Often occurs mutiple levels * combined upper c spine instability * lower spine involvment more common with * steriod use * males * seropositive RA * nodules present
44
What is the pathophysiology of subaxial subluxation?
* Pannus formation and soft tissue instability of facet joints and Luschka joints
45
What is seen of subaxial subluxation on radiographs?
* **Subaxial subluxation of vertebral body of \>4mm or 20% = cord compression** * **Cervical height index ( body height/width) \<2.0** almost 100% sensitive and specific for predicting neurologic compromise
46
What are the tx of subaxial subluxation?
* Operative * **Posterior fusion & wiring** * for \>4mm subaxial subluxation in intractable pain and neurological symptoms
47
What are the operative complications are tx a pt with rheuamatoid cervical spondylitis?
* **Failure to improve symptoms** * outcomes less reliable with Ranawat Grade 3B * **Pseudoarthrosis** * 10-20% pseudoarthrosis rate * decreased by extension to occiput * **Adjacent Level degeneration**