LEGG-CALVE-PERTHES’ DISEASE (Coxa plana, Pseudocoxalgia)

LEGG-CALVE-PERTHES’ DISEASE (Coxa plana, Pseudocoxalgia)


LEGG-CALVE-PERTHES’ DISEASE

The disease also known by different names such as:

  • osteochondritis deformans juvenilis
  • pseudocoxalgia
  • coxa plana
  • Occur in children 5–15 years.
  • Characterized by varying degrees of idiopathic avascular necrosis of the upper femoral epiphysis.
  • Followed by spontaneous revascularization over a period of time.
  • During this period, the head is exposed to a risk of deformation.

Predisposing Factors

  1. Genetic
  2. Environmental factors
  3. Trauma
  4. Transient synovitis
  5. Passive smoking
  6. Abnormal venous drainage
  7. Arterial block and infarction
  8. Increased viscosity of the blood
Pathology
  • 1st stage—Stage of synovitis
  • 2nd stage—Stage of avascular necrosis
  • 3rd stage—Stage of (fragmentation) revascularization
  • 4th stage—Residual/healed stage
Radiological Features
Based on Elizabethtown Staging:
  • Stage I (Initial/Avascular necrosis): Waldenstrom’s sign
  • Stage II (Fragmentation): Shrinkage and fragmentation of the epiphysis
  • Stage III (Regeneration): Fragmentation along with new bone formation
  • Stage IV (Healed): Complete re-ossification
Based on Catterall Grouping:

  • The disease is grouped into 4 groups depending on:
  1. Extent of epiphyseal involvement.
  2. Presence of sequestrum.
  3. Presence of collapse.‘Head within the Head’
  4. Metaphyseal abnormality  

Clinical Features
  • Onset is insidious.
  • Limp with or without dull pain.
  • Pain in the hip region.
  • Pain is exaggerated by activity, relieved by rest.
  • Classical limitation of abduction and internal rotation.
  • Trendelenburg gait may be present.
Head at Risk Signs

  • Clinical “Head at Risk” Signs
  1. Older child
  2. Heavy child
  3. Progressive loss of movement
  4. Adduction contracture
  5. Flexion with adduction deformity.
Radiological “Head at Risk” Signs

1. Gage sign

  • ‘V’ shaped radiolucent defect on the lateral side of the epiphysis.

2. Calcification lateral to the epiphysis.

  • Occurs due to thickened and extruded epiphysis.
  • Indicates epiphyseal enlargement.

3. Lateral subluxation.
4. Horizontal growth plate.
5. Diffuse metaphyseal lesion. 

 

Investigations
1. X-ray:

  • Avascular changes as per stage of the disease.

2. Bone scan:

  • Can diagnose the avascularity much before the radiological changes develop.
  • Also useful in differentiating septic from aseptic inflammation.

3. MRI:

  • Delineates the changes in the cartilaginous epiphysis and the joint much better than X-ray.
  • Also useful to assess vascularity of the epiphysis.

4. Arthrography:

  • Useful in the assessment of joint congruity
  • Helps in planning the containment procedure.

Exam Important

  • Perthe’s disease is Osteochondritis of the epiphysis of the Femoral head.
  • Abduction & internal rotation movements is restricted in Perthe’s disease.
  • Investigation of choice for evaluation of suspected Perthes’ disease is Magnetic Resonance Imaging (MRI).
  • Radiological sign in case of Perthe’s disease is Organized calcification.
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