Legg–calvé–perthes Disease

Legg–calvé–perthes Disease is a topic covered in the 5-Minute Emergency Consult.

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Basics

Description

  • Idiopathic avascular necrosis of the femoral head in children 4–12 yr
  • Genetics:
    • Increased frequency with factor V Leiden and anticardiolipin antibodies
    • Usually sporadic but small percentage associated with type 2 collagen COL2A1 gene mutations

Pediatric Considerations
Exclusively a pediatric disease

Etiology

  • Idiopathic avascular necrosis from interruption of blood supply to the femoral head from ischemia/occlusion, followed by reossification, deformity, and mechanically weakened bone
  • May be multifactorial
  • Risk factors include secondhand exposure to tobacco smoke, wood smoke, low birth weight, birth length <50 cm, delayed skeletal maturity, trauma, coagulation disorders
  • Gradual progression through 4 stages of disease:
    • Initial stage: Dense femoral head causing intermittent synovitis
    • Fragmentation: Femoral head becomes soft and deforms causing loss of motion
    • Healing: New bone grows into femoral head
    • Residual: Healed femoral head with some deformity
  • Most commonly occurs between ages 3–7; male > female, 4:1; Caucasians
  • Typically unilateral, but bilateral in 10–15% of cases
  • Associated with short stature, delayed and disproportionate growth

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Basics

Description

  • Idiopathic avascular necrosis of the femoral head in children 4–12 yr
  • Genetics:
    • Increased frequency with factor V Leiden and anticardiolipin antibodies
    • Usually sporadic but small percentage associated with type 2 collagen COL2A1 gene mutations

Pediatric Considerations
Exclusively a pediatric disease

Etiology

  • Idiopathic avascular necrosis from interruption of blood supply to the femoral head from ischemia/occlusion, followed by reossification, deformity, and mechanically weakened bone
  • May be multifactorial
  • Risk factors include secondhand exposure to tobacco smoke, wood smoke, low birth weight, birth length <50 cm, delayed skeletal maturity, trauma, coagulation disorders
  • Gradual progression through 4 stages of disease:
    • Initial stage: Dense femoral head causing intermittent synovitis
    • Fragmentation: Femoral head becomes soft and deforms causing loss of motion
    • Healing: New bone grows into femoral head
    • Residual: Healed femoral head with some deformity
  • Most commonly occurs between ages 3–7; male > female, 4:1; Caucasians
  • Typically unilateral, but bilateral in 10–15% of cases
  • Associated with short stature, delayed and disproportionate growth

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