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Duke Orthopaedics
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Wheeless' Textbook of Orthopaedics

Legg Calve Perthes Disease

- Discussion:
    - self limiting hip disorder caused by a varying degree of ischemia and subsequent necrosis of the femoral head;
           - avascular necrosis of nucleus of proximal femoral epiphysis, abnormal growth of the physis, and eventual remodeling of regenerated  bone are the key features of this disorder;
           - loss of blood supply to the epiphysis is thought to be the essential lesion;
    - usually seen in 4 to 8 yr old boy with delayed skeletal maturity;
    - male to female ratio: 4-5 to 1;
    - rare in blacks;
    - increased incidence with a positive family history, low birth wt, and abnormal pregnancy / delivery;
    - up to 12% of cases are bilateral but will be at different stages & are asymmetric (vs. MED)
    - age is the key to the prognosis - after 8 yr represents poor prognosis;
           - ref: Legg-Calve-Perthes disease in patients under 5 years of age does not always result in a good outcome. Personal experience and meta-analysis of the literature.
    - pathogenesis:
           - AVN of femoral epiphysis which results in delayed occific nucleus;
           - articular cartilage is nourished by synovial fluid - continues to grow;
           - cartilage columns become distorted with some loss of their cellular components;
                  - they do not undergoe normal ossification, which results in excess of calcified cartilage in the primary trabecular bone;
           - revascularization procedes from peripheral to central;
           - symptoms occur with subchondral collapse and fracture;
           - references:
                 - Legg-Calvé-Perthes Disease and Thrombophilia.
                 - Protein C Levels in Patients With Legg-Calve-Perthes Disease: Is It a True Deficiency?
    - diff dx:
           - bilateral Perthes: (requires skeletal survey as apart of  work up);
                 - hypothyroidism
                 - multiple epiphyseal dyspasia
                      - ref: Does it Always Have to be Perthes' Disease?: What is Epiphyseal Dysplasia?
                 - spondyloepiphyseal dysplasia tarda
                 - sickle cell
           - unilateral Perthes:
                 - septic arthritis
                 - sickle cell
                 - spondyloepiphyseal dysplasia tarda
                 - gaucher's disease
                 - eosinophilic granuloma
                 - transient synovitis
                        - transient synovitis at one time was thought to lead to LCP, however, it is now believed there is no causal relationship;

- Clinical Presentation:
    - includes pain (often knee pain) effusion (from synovitis) & a limp;
    - early phase:
          - limited abduction of hip & limited internal rotation in both flexion & extension are seen;
          - antalgic gait (due to pain);
    - late phase: Trendelenburg gait;


- Radiographic Evaluation:

  Perthes in 5 yo male;
   


  Perthes in 6 yo male    later at 6 1/2 yrs     later at 7 yrs
   
       

  Perthes in 6 yo female       later at 7 yrs             later at 8 yrs            later at 9 yrs
   
       

   



- Prognosis:
    - at least 50% of involved hips do well with no treatment;
    - many others will do well up until the 5th decade when anatomic asphericity leads to DJD;
    - age is the key to prognosis:
          - less than 6 years of age: outcome is good, regardless of treatment;
          - between 6-8 years of age: results not always satisfactory with containment;
          - greater than 9 years of age: questionable benefit from containment;
                - children older than 8-9 yrs at initial onset will have poor prognosis and may be expected to have significant symptoms and restricted ROM;
    - congruency:
          - a flat topped femoral head which is incongruent w/ the acetabulum has the worst prognosis (Stulberg V)
    - degree of epiphyseal involvement
    - ability to maintain hip motion
    - shape of the femoral head after healing
    - development of subluxation of the joint.
          - felt to be a key radiographic at- risk sign for poor prognosis;
    - w/ lateral margin of capital femoral epiphysis involved;
    - decreased hip range of motion (decreased abduction);
    - collapse of lateral pillar more than 50% (Herring group C)
          - in the study by Ismail and Macincol  MF (1998), non of the hips in Herring group C had a normal appearing hip, irrespective of age;
    - references: 
          - Prognosis in Perthe's disease: a comparison of radiologic predictors
          - Legg-Calve-Perthes disease. The prognostic significance of the subchondral fracture and a two-group classification of the femoral head involvement
          - Legg-Calvé-Perthes Disease Part I: Classification of Radiographs with Use of the Modified Lateral Pillar and Stulberg Classifications.


- Non Operative Treatment


- Surgical Treatment:
    - in the report by Ismail and Macincol MF (1998), surgery is indicated for children greater than age 6 years in Herring group C and for children in Herring group B with loss of spherricity (as noted by arthrography);
          - these authors recommend a proximal varus osteotomy;
    - salvage procedures are reserved for pts with severe impairment;
    - procedures include:
          - excision of extruded portion of head for hinge abduction;
          - acetabular osteotomy:
                  - Chiari osteotomy to cover femoral head;
                  - lateral shelf osteotomy:
                          - in the study by Daly K, et al (1999), good results were found w/ 2 yrs followup in Stulberg stages 1-3;
                                 - as with other studies on this subject, followup was short, there was no control group, and results were best in patients in the earliest stages of the disease (ie those most likely to due well);
          - valgus osteotomy to increase abduction & bring more normal medial femoral  head into wt bearing area;
          - arthrodesis at skeletal maturity in unilateral involvement) for pts  w/ severe functional impairment;
                 - pts > 9 yrs  may benefit from combined innominate & femoral osteotomies if done in early stages;
    - references: 
          - Lateral shelf acetabuloplasty in Perthes' disease. A review of the end of growth.
          - The effect of an incomplete interochanteric osteotomy on Legg-Calve-Perthes disease.
          - Prognosis after conservative and operative treatment in Perthes' disease.
          - Shelf Arthroplasty in Patients Who Have Legg-Calve-Perthes Disease. A Study of Long-Term Results.
          - Results of innominate osteotomy in the treatment of Legg-Calve-Perthes disease.

- Long Term Consequences:
    - coxa magna
    - coxa plana
    - coxa breva
    - hinged abduction:
          - occurs when an enlarged femoral head is laterally extruded and impinges against the acetabular rim when the hip is abducted



 Current Concepts Review. Legg-Calve-Perthes Disease.

 Current Concepts Review. The Treatment of Legg-Calve-Perthes Disease. A Critical Review of the Literature.

 Abnormalities of proximal femoral growth after severe Perthes' disease.

 Comparison of femoral and innomi of the femoral head.

 The arterial supply of the developing proximal end of the human femur.

 The natural history of Legg-Calve-Perthes disease.

 Legg-Calve-Perthes disease. The prognostic significance of the subchondral fracture and a two-group classification of the femoral head involvement.

 A long-term follow-up of Legg-Calve-Perthes disease.

 Growth disturbance in Legg-Calve-Perthes disease and the consequences of surgical treatment.

 Prognosis in Perthes' disease after noncontainment treatment. 106 hips followed for 28-47 years.

 Association of antithrombotic factor deficiencies and hypofibrinolysis with Legg-Perthes disease.

 An evaluation of various methods of treatment for Legg-Calve-Perthes disease.

 Physeal slope in Perthes disease.

 Legg-Calvé-Perthes Disease. Part II: Prospective Multicenter Study of the Effect of Treatment on Outcome. 

 Comorbidities in Perthes’ disease. A case control study using the General Practice Research Database

 A Prospective Multicenter Study of Legg-Calvé-Perthes Disease: Functional and Radiographic Outcomes of Nonoperative Treatment at a Mean Follow-up of Twenty Years

Legg-Calvé-Perthes Disease (Updated August 2004) from the Orthopaedic Care Textbook



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Thursday, January 3, 2013 10:14 am