Legg-Calvé-Perthes Disease


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Overview of Legg-Calvé-Perthes disease including pathology, classification, management, prognosis

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Legg-Calvé-Perthes Disease

  1. 1. Legg-Calvé-Perthes By Hiren M Divecha CT2 T&O 28/4/2010
  2. 2. Background Described independently in 1910 by Legg (USA), Calvé (France) and Perthes (Germany) Definition  Self limiting  Idiopathic  Varying degree of ischemia  Osteonecrosis of the capital femoral epiphysis Loss of blood supply to the epiphysis is thought to be the essential lesion Also occurs in dogs  Toy Poodles, Yorkshire Terriers, Pugs, Jack Russell Terriers, and Dachshunds can be affected
  3. 3. Background Frequency  US 1 in 1200 children <15 yrs  higher in the UK esp. Ireland Age  3-12 yrs (median age of 7 yrs) Race  Caucasians. Rare in black races Sex  M:F = 4-5: 1 Family history:  1 in 100 male children of adults with Legg–Calvé–Perthes 15-20% of cases are bilateral but will be at different stages & are asymmetric (vs. MED)
  4. 4. Aetio-pathogenesis Unclear Interrupted blood supply to capital femoral epiphysis  Abnormal venous drainage increases pressure (Heikkenen 1980)  ? Early closure of ligamentum teres artery thus reduced metaphyseal supply (med fem circum not fully developed) retinacular vessels only (Salter 1984)  Retinacular vessels susceptible to pressure. Increased risk with effusions, 4% pt with synovitis develop Perthes. (Mukamel 1986)  Angiographic studies reveal decreased flow in medial circumflex femoral and superior retinacular vessels  ? Assoc with thrombophilia (Vosamer JBJS Am 2010)  Factor V Leiden, protein S deficiency, prothrombin mutation
  5. 5. Pathology Catterall A, Pringle J, Byers PD, et al: A review of the morphology of Perthes disease. JBJS Br 1982;64:269-275  11 cases necropsy specimens and in-vivo core biopsies  Initial stage  Subchondral bone necrosis  Femoral head ossification stops  Articular cartilage continues to grow (nourished by synovial fluid)  XR appearance of small ossific nucleus & wide cartilage space  Second stage (1-3yrs)  Resorption of necrotic bone and creeping substitution  Third stage  Osteoblasts, new-bone formation and healing
  6. 6. History Usually no history of trauma Limping child, often painless Mild – moderate hip pain  May refer to thigh/ knee Incidental finding  In childhood  As adult
  7. 7. Examination Short stature – delayed bone age Early  Decreased ROM – esp. internal rotation and abduction (synovitis + muscle spasm)  Antalgic gait Late  Decreased ROM from acetabular impingment  Disuse atrophy of thigh muscles  Leg length inequality due to collapse  Trendelenburg gait
  8. 8. DifferentialUnilateral Perthes Bilateral Perthes Septic arthritis  Hypothyroidism Fracture  Sickle cell SUFE  Multiple epiphyseal Transient synovitis dyspasia (initially thought to lead  Spondyloepiphyseal to LCPD) dysplasia tarda Sickle cell Spondyloepiphyseal dysplasia tarda Gauchers disease
  9. 9. Investigations Plain x-rays  Pelvis AP + frog leg views Blood tests  FBC, CRP, ESR Hip USS +/- aspiration if a septic joint is suspected Technetium 99 bone scan  Extent of avascularity. Cold spots  Increased uptake (recanalisation/ neovascularisation) Dynamic arthrography  Assesses sphericity of femoral head  Hinge abduction Bilateral Perthes  requires skeletal survey as apart of work-up
  10. 10. Classification Many radiographic classification systems exist Based on the extent of abnormality of the capital femoral epiphysis  Waldenstrom  Catterall  Herring  Salter and Thompson
  11. 11. Waldenstrom (1922) Demonstrates which stage of the disease is present, but has no predictive value for long-term outcome or treatment Initial (necrosis)  Femoral head is radiodense and smaller, while the cartilage space of the hip is wider  The increased radiodensity occurs because the surrounding bone has a normal blood supply, thus appearing osteopenic compared with the avascular segment Fragmentation  Subchondral fracture, bone resorption and cyst formation Healing phase  Reossification occurs peripheral to central and radiodensity becomes normal Remodelling  Shape may be maintained or further flatten  Residual deformity may be coxa magna, coxa plana, or coxa breva
  12. 12. Catterall (1971) Based on % involvement on AP and frog lateral (poor inter/ intra observer error) Group 1  Anteromedial proportion only, physis and metaphysis OK  heal without sequelae Group 2  nearly 50% involvement  fragmentation without significant collapse  minimal metaphyseal involvement  good result Group 3  nearly 75%  triangle of normal bone postermedially, lateral collapse  diffuse metaphyseal involvement  poorer result Group 4  100% involvement  widespread collapse diffuse metaphyseal +/- physis  sequestra formation  poor result
  13. 13. Herring (1992/2004) Based on degree of collapse of lateral pillar involvement during fragmentation stage Group A  no collapse. No progressive flattening Group B  < 50% collapse Group B/C border  ≤ 50% collapse. Narrow (2-3mm). Little ossification Group C  > 50% collapse. 17% develop progressive flattening Ritterbusch 1993  Greatest predictive value & interobserver reliability
  14. 14. Poor Prognosis Age  <6 yrs – good regardless of treatment  6-8 yrs – not always satisfactory with containment  >10yrs – questionable benefit from containment. poor prognosis. significant symptoms and restricted ROM Lateral pillar stage  >50% lateral pillar collapse Herring JA et al J Pediatr Orthop 13:281-285, 1993  Ismail and MF Macincol JBJS Br 1998 – none group C had normal hip, irrespective of age Sex = F Radiological morphology at completion (Stuhlberg 1981)  flat topped femoral head which is incongruent w/ the acetabulum has the worst prognosis Decreased ROM, adduction contracture, flexion with abduction, heavy child Catterall head at risk signs  lateral subluxation  100% involvement  calcification lateral to physis  metaphyseal cysts  Gage sign (lateral V shaped defect)  horizontal physis
  15. 15. Treatment Goals of treatment  Maintain femoral head sphericity/ containment  Avoid severe degenerative arthritis Guided by  Age of onset  Severity of involvement  Limitation in ROM
  16. 16. Conservative NSAIDs + rest till acute pain subsides ? traction Physiotherapy ROM Containment  Lat sublux, lat pillar collapse  Petrie cast or brace  Exclude hinge abduction  Wean off when reossification starts  Spherical remodelling
  17. 17. Intervention Herring 2004  A – good outcome regardless  B <8yrs – good outcome  B/C 6-8yrs – no benefit from surgery  B & B/C >8yrs benefit from surgery Containment by surgery (before reossification)  Femoral varus osteotomy +/- derotation  Salter osteotomy  Combination  Femoral neck lengthening  Triple pelvic osteotomy
  18. 18. <6yrs 6-8yrs >8yrs A, B B/C, C Contained A, B B/C , C Uncontained Contained Uncontained Coxa vara/Good mobility Poor Mobility Coxa magna Good mobility Poor mobility magna Triple pelvic Varus / Salter Varus / Salter VITO, femoral osteotomy,Conservative Conservative osteotomy osteotomy neck lengthening femoral neck legthening
  19. 19. Varus osteotomy Salter Osteotomy
  20. 20. Femoral necklengthening Triple pelvic osteotomy
  21. 21. Follow-up Initially, close follow-up is required to determine the extent of necrosis Once the healing phase has been entered, follow-up can be every 6 months Long-term follow-up is necessary to determine the final outcome  Gower & Johnston – mid 30s. 86% good outcome. 8% arthroplasty  McAndrew & Weinstein – mid 50s. 40% good outcome. 40% arthroplasty Long Term Consequences  coxa magna  coxa breva  hinged abduction  Enlarged, laterally extruded femoral head impinges against acetabular rim  degenerative changes
  22. 22. When it goes wrong Valgus osteotomy Hip arthroplasty!
  23. 23. References Skaggs DL. Legg-Calve-Perthes Disease. JAAOS 1996;4:9-16 Catterall A. The natural history of Perthes disease. JBJS Br 1971;53:37-53 Catterall A. A review of the morphology of Perthes disease. JBJS Br 1982;64:269-275 Herring JA. The lateral pillar classification of Legg-Calve- Perthes disease. JPO 1992; 12:143-150 Ritterbusch JF, Shantharam SS, Gelinas C. Comparison of lateral pillar classification and Catterall classification of Legg- CalveŽ -Perthes disease. JPO 1993;13:200-202 Herring. Legg-Calvé-Perthes Disease. Part I: Classification of Radiographs with Use of the Modified Lateral Pillar and Stulberg Classifications. JBJS Am 2004; 86:2103-2120 Herring. Legg-Calvé-Perthes Disease Part II: Prospective Multicenter Study of the Effect of Treatment on Outcome. JBJS Am 2004; 86:2121-4