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Legg-Calve-Perthes disease; pseudocoxalgia; avascular necrosis of the hip

Epidemiology: - children 3-12 years of age - peaking between 5-7 years of age - male/female ratio = 4 Pathology: - osteonecrosis of the femoral head occurring in a child - interruption of the blood supply to the capital femoral epiphysis is the cause of the osteonecrosis - stage 1: femoral head becomes more dense with possible fracture of supporting bone - stage 2: fragmentation & reabsorption of bone - stage 3: reossification when new bone has regrown Genetics: - associated with defects in COL2A1 Clinical manifestations: - children usually present with a limp or pain in the hip, thigh or knee - examination of the knee is normal - limited range of motion with hip pain on internal rotation & abduction of the ipsilateral hip - may vary, depending on the phase of disease progression through ischemia, revascularization, fracture & collapse, & repair & remodeling of the bone Radiology: - anteroposterior & frog-leg lateral radiographs of the hip - may show evidence of bone necrosis, fragmentation, reossification or remodeling & healing - earliest signs include decreased size or increased density of the proximal femoral epiphyses on an AP view & crescent sign (subchondral fracture that correlates with extent of necrosis) on lateral view [3] Management: - rest, avoid aggravating activities - range of motion exercises - occasionally orthoses or surgery may be required - prognosis - femoral head involement of > 50% predicts poor outcome - age > 6 years confers worse prognosis [3]

General

osteochondrosis avascular osteonecrosis; aseptic necrosis developmental disorder

Database Correlations

OMIM 150600

References

  1. Basic Radiology, Chen MYM, Pope TL & Ott DJ (eds), McGraw Hill, NY 1996
  2. OMIM :accession 150600
  3. Houghton KM. Review for the generalist: evaluation of pediatric hip pain. Pediatr Rheumatol Online J. 2009 May 18;7:10. PMID: 19450281 Free PMC Article
  4. Karkenny AJ, Tauberg BM, Otsuka NY. Pediatric Hip Disorders: Slipped Capital Femoral Epiphysis and Legg-Calve-Perthes Disease. Pediatr Rev. 2018 Sep;39(9):454-463. PMID: 30171056 Review.
  5. Kim HK, Herring JA. Pathophysiology, classifications, and natural history of Perthes disease. Orthop Clin North Am. 2011 Jul;42(3):285-95 PMID: 21742140 Review.