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  1. 1. Legg Calve Perthes Disease
  2. 2. History <ul><li>FIRST DESCRIBED BY LEGG AND WALDENSTORM </li></ul><ul><li>IN 1909 AND BY PERTHES AND </li></ul><ul><li>CALVE IN 1910 </li></ul>
  3. 3. Definition <ul><li>Legg-Calvé-Perthes disease (LCPD) is the name given to idiopathic osteonecrosis of the capital femoral epiphysis in a child. </li></ul>
  4. 4. Epidemiology <ul><li>Disorder of the hip in young children </li></ul><ul><li>Usually ages 4-8yo </li></ul><ul><li>As early as 2yo, as late as teens </li></ul><ul><li>Boys:Girls= 4-5:1 </li></ul><ul><li>Bilateral 10-12% </li></ul><ul><li>No evidence of inheritance </li></ul>
  5. 5. <ul><li>Prevalence : </li></ul>
  6. 6. Etiology <ul><li>Unknown </li></ul><ul><li>Past theories: infection, inflammation, trauma, congenital </li></ul><ul><li>Most current theories involve vascular compromise </li></ul><ul><ul><li>Sanches 1973: “second infarction theory” </li></ul></ul>
  7. 7. Causes <ul><li>Proposed theories . </li></ul><ul><ul><li>Excessive femoral antiversion. </li></ul></ul><ul><ul><li>Synovitis. </li></ul></ul><ul><ul><li>Generalized skeletal disorder. </li></ul></ul><ul><ul><li>Arterial anomalies. </li></ul></ul>
  8. 9. Pathogenesis <ul><li>Histologic changes described by 1913 </li></ul><ul><li>Secondary ossification center= covered by cartilage of 3 zones: </li></ul><ul><ul><li>Superficial </li></ul></ul><ul><ul><li>Epiphyseal </li></ul></ul><ul><ul><li>Thin cartilage zone </li></ul></ul><ul><li>Capillaries penetrate thin zone from below </li></ul>
  9. 10. <ul><li>Epiphyseal cartilage in LCP disease: </li></ul><ul><ul><li>Superficial zone is normal but thickened </li></ul></ul><ul><ul><li>Middle zone has </li></ul></ul><ul><ul><li>1) areas of extreme hypercellularity in clusters and 2) areas of loose fibrocartilaginous matrix </li></ul></ul><ul><li>Superficial and middle layers nourished by synovial fluid </li></ul><ul><li>Deep layer relies on blood supply </li></ul>
  10. 11. <ul><li>Physeal plate : cleft formation, amorphis debris, blood extravasation </li></ul><ul><li>Metaphyseal region : normal bone separated by cartilaginous matrix </li></ul><ul><li>Epiphyseal changes can be seen also in greater trochanter, acetabulum </li></ul>
  11. 12. Blood Supply
  12. 13. Radiographic Stages <ul><li>Four Waldenstrom stages: </li></ul><ul><ul><li>1) Initial stage </li></ul></ul><ul><ul><li>2) Fragmentation stage </li></ul></ul><ul><ul><li>3) Reossification stage </li></ul></ul><ul><ul><li>4) Healed stage </li></ul></ul>
  13. 14. Initial Stage <ul><li>Early radiographic signs: </li></ul><ul><ul><li>Failure of femoral ossific nucleus to grow </li></ul></ul><ul><ul><li>Widening of medial joint space </li></ul></ul><ul><ul><li>“ Crescent sign” </li></ul></ul><ul><ul><li>Irregular physeal plate </li></ul></ul><ul><ul><li>Blurry/ radiolucent metaphysis </li></ul></ul>
  14. 15. Fragmentation Stage <ul><li>Bony epiphysis begins to fragment </li></ul><ul><li>Areas of increased lucency and density </li></ul><ul><li>Evidence of repair aspects of disease </li></ul>
  15. 16. Reossification Stage <ul><li>Normal bone density returns </li></ul><ul><li>Alterations in shape of femoral head and neck evident </li></ul>
  16. 17. Healed Stage <ul><li>Left with residual deformity from disease and repair process </li></ul><ul><li>Differs from AVN following Fx or dislocation </li></ul>
  17. 18. Group I
  18. 19. Group II
  19. 20. Group III
  20. 21. Group IV
  21. 22. <ul><li>3 groups: </li></ul><ul><ul><li>A) no lateral pillar involvment </li></ul></ul><ul><ul><li>B) >50% lat height intact </li></ul></ul><ul><ul><li>C) <50% lat height intact </li></ul></ul>Lateral Pillar Classification
  22. 23. Salter-Thompson Classification <ul><li>Simplification of Catterall </li></ul><ul><li>Based on status of lateral margin of capital femoral epiphysis </li></ul><ul><li>Group A (Catterall I & II equivalent) </li></ul><ul><li>Group B (Catterall III & IV equivalent) </li></ul>
  23. 24. Clinical Features <ul><li>Stature usually shorter than peers </li></ul><ul><li>Quadriceps and gluteal muscle wasting is common, Trandelenburg test positive (drop of the hip on the unsupported side) </li></ul><ul><li>Acute phase; range of motion at the hip joint is limited due to muscle spasms </li></ul><ul><li>Progressively; limited internal rotation and abduction is likely due to impingement lesions (hence the Roll test, guarding on affected side) </li></ul><ul><li>Later stage; global reduction in all ranges of motion assoc. with pain, indicating joint arthritis </li></ul>
  24. 25. <ul><li>Age- 4 to 10 years, with peak incidence at 7 </li></ul><ul><li>Gender- Boys (5:1 ratio) but it tends to be more severe in girls </li></ul><ul><li>Height </li></ul><ul><li>Passive smoking or maternal smoking at pregnancy </li></ul><ul><li>ADHD? Increased physical activity </li></ul><ul><li>Family Hx of; skeletal dysplasias or thrombotic disease </li></ul><ul><li>Ethnicity; more common in Whites, Eskimos, Japanese </li></ul><ul><li>Social Hx- associated with low socio-economic status </li></ul>Risk Factors
  25. 26. Differential Diagnosis
  26. 27. Workup <ul><li>Technetium 99 bone scan - Helpful in delineating the extent of avascular changes before they are evident on plain radiographs. </li></ul><ul><ul><li>The sensitivity of radionuclide scanning in the diagnosis of LPD is 98%, and the specificity is 95%. </li></ul></ul><ul><li>Dynamic arthrography - Assesses sphericity of the head of the femur. </li></ul>
  27. 28. <ul><li>Ultrasonography may provide significant diagnostic clues to differentiate early Perthes' from transient synovitis. </li></ul><ul><ul><li>T Futami, Y Kasahara, S Suzuki, S Ushikubo and T Tsuchiya </li></ul></ul><ul><ul><li>Journal of Bone and Joint Surgery - British Volume, Vol 73-B, Issue 4, 635-639 </li></ul></ul>Ultrasonography in transient synvitis and early Perthes’ disease
  28. 29. CT Scan <ul><li>Staging determined by using plain radiographic findings is upgraded in 30% of patients. </li></ul><ul><li>Not as sensitive as nuclear medicine or MRI. </li></ul><ul><li>CT may be used for follow-up imaging in patients with LPD. </li></ul>
  29. 30. MRI <ul><li>It allows more precise localization of involvement than conventional radiography. </li></ul><ul><li>MRI is preferred for evaluating the position, form, and size of the femoral head and surrounding soft tissues. </li></ul><ul><li>MRI is as sensitive as isotopic bone scanning. </li></ul>
  30. 31. Outcome variables <ul><li>Age </li></ul><ul><li>Extent of involvement </li></ul><ul><li>Duration </li></ul><ul><li>Remodeling potential </li></ul><ul><li>Premature physeal closure </li></ul><ul><li>Type of treatment </li></ul><ul><li>Stage of disease at treatment. </li></ul>
  31. 32. Treatment Options <ul><li>Overall goal of treatment </li></ul><ul><li>Reduce hip irritability and pain </li></ul><ul><li>Restore/maintain hip mobility </li></ul><ul><li>Prevent femoral head from extruding or collapsing “CONTAINMENT ” </li></ul><ul><li>Regain spherical shape of femoral head </li></ul>
  32. 33. <ul><li>Below 6 years and Herring A/B </li></ul><ul><li>Mainstay of treatment would be to OBSERVE with 6-12 month reassessment. </li></ul><ul><li>Patients in this age group need bed rest and anti inflammatory medication at most. NO evidence that abduction splints or surgery beneficial </li></ul><ul><li>Prognosis is good for the majority </li></ul>
  33. 34. <ul><li>Non Surgical treatment </li></ul><ul><li>NSAIDS </li></ul><ul><li>Traction </li></ul><ul><li>Casts and braces </li></ul><ul><li>(Scottish Rite Orthosis) </li></ul>
  34. 35. <ul><li>Above 6 and Herring class B </li></ul><ul><li>Containment of the head within the acetabulum is warranted </li></ul><ul><li>This is achieved by; </li></ul><ul><li>Abduction bracing </li></ul><ul><li>Femoral varus osteotomy </li></ul><ul><li>Pelvic ostotomy </li></ul>
  35. 36. <ul><li>Age between 6-8 and Herring class C </li></ul><ul><li>Results of intervention have been equivocal. </li></ul><ul><li>Above 9 years </li></ul><ul><li>Often have Herring class B or C </li></ul><ul><li>Prognosis is poor </li></ul><ul><li>Early containment is key, by pelvic osteotomy and internal fixation </li></ul>
  36. 37. Osteotomies
  37. 38. Summary <ul><li>For patients less that 6 years old the prognosis is good for the majority. </li></ul><ul><li>If they are stiff or painful they respond to bed rest, traction and pain relieving anti-inflammatory medication. </li></ul><ul><li>There is no evidence that abduction splints or surgical intervention is warranted in the majority of these younger patients. </li></ul>
  38. 39. <ul><li>For patients between 6 and 8 years but with a bone age less than 6 and an intact lateral pillar (Herring A and B) the prognosis is similar to that for the first group and observation is as good as surgical intervention for the majority. </li></ul><ul><li>If they have bone ages greater than 6 years and Herring lateral pillar classification B then &quot;containment&quot; of the head within the acetabulum seems to be warranted. </li></ul><ul><li>This may be done by abduction bracing, femoral varus osteotomy or a pelvic osteotomy. </li></ul>
  39. 40. <ul><li>If they are between 6 and 8 and are in lateral pillar group C then the result of intervention are equivocal. </li></ul><ul><li>Children presenting with Perthes disease at age 9 or older often have lateral pillar B or C and a poor prognosis. </li></ul><ul><li>The trend is towards early containment of these hips although stiffness can be a problem following early pelvic (Salter's) osteotomy. </li></ul>
  40. 41. Follow-up <ul><li>Initially, close follow-up is required to determine the extent of necrosis. </li></ul><ul><li>Once the healing phase has been entered, follow-up can be every 6 months. </li></ul><ul><li>Long-term follow-up is necessary to determine the final outcome. </li></ul>
  41. 42. Complications <ul><li>Femoral </li></ul><ul><ul><li>Shortening </li></ul></ul><ul><ul><li>stiffness </li></ul></ul><ul><ul><li>Malrotation </li></ul></ul><ul><ul><li>Limp </li></ul></ul><ul><ul><li>Positive trendelenburg </li></ul></ul><ul><li>Pelvic </li></ul><ul><ul><li>Lenghtening </li></ul></ul><ul><ul><li>Stiffness </li></ul></ul><ul><ul><li>Chondrolysis </li></ul></ul><ul><ul><li>Failure of containment </li></ul></ul>
  42. 43. Prognosis <ul><li>The younger the age of onset of LCPD, the better the prognosis. </li></ul><ul><li>Children older than 10 years have a very high risk of developing osteoarthritis. </li></ul><ul><li>Most patients have a favorable outcome. </li></ul><ul><li>Prognosis is proportional to the degree of radiologic involvement. </li></ul>