Perthes Disease
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Basics
Description
Childhood femoral head osteonecrosis of unknown etiology, which can weaken the femoral head and produce a permanent femoral head deformity in some patients, predisposing them to early arthritis
Epidemiology
- Prevalence varies depending on the region: 0–15 per 100,000 children <15 years of age.
- In the United States and Canada, about 5 per 100,000
- Rare in African Americans
- Most frequent in children 4–8 years old
- 3–5 times more common in boys than girls
- 10–15% develop bilateral disease in asynchronous fashion.
Pathophysiology
- A partial or complete disruption of blood supply to the femoral head produces a partial or total femoral head osteonecrosis.
- The greater the femoral head involvement, the worse the prognosis.
- Bone necrosis and subsequent resorption of necrotic bone weaken the femoral head.
- Weight bearing worsens the femoral head deformity.
- Chronic hip joint synovitis also develops producing pain and restriction of motion.
- Necrotic femoral head goes through 4 stages of healing over 3–5 years:
- 1. Stage of avascular necrosis
- Smaller femoral head epiphysis with increased radiodensity
- 2. Stage of fragmentation
- Necrotic epiphysis shows fragmentation.
- Necrotic bone is resorbed, weakening the head.
- Most deformity occurs during this stage, which lasts 1–2 years.
- 3. Stage of reossification
- New bone begins to fill the epiphysis.
- Longest stage, lasting up to 3 years
- 4. Healed
- Femoral head is completely reossified.
- Not all heal back in round shape, and deformed femoral heads are at risk for developing arthritis later.
- 1. Stage of avascular necrosis
Etiology
- Unknown
- Unlikely genetic transmission, as <5% have family history
- Many theories:
- Multifactorial (genetic predisposition with environmental trigger)
- Hyperactivity and subclinical trauma
- Type II collagenopathy
- Thrombophilia (factor V Leiden)
- Tobacco smoke exposure
Commonly Associated Conditions
- Delayed bone age
- Hyperactivity
- Genitourinary anomalies (hypospadias, undescended testis, and inguinal hernia)
-- To view the remaining sections of this topic, please log in or purchase a subscription --
Basics
Description
Childhood femoral head osteonecrosis of unknown etiology, which can weaken the femoral head and produce a permanent femoral head deformity in some patients, predisposing them to early arthritis
Epidemiology
- Prevalence varies depending on the region: 0–15 per 100,000 children <15 years of age.
- In the United States and Canada, about 5 per 100,000
- Rare in African Americans
- Most frequent in children 4–8 years old
- 3–5 times more common in boys than girls
- 10–15% develop bilateral disease in asynchronous fashion.
Pathophysiology
- A partial or complete disruption of blood supply to the femoral head produces a partial or total femoral head osteonecrosis.
- The greater the femoral head involvement, the worse the prognosis.
- Bone necrosis and subsequent resorption of necrotic bone weaken the femoral head.
- Weight bearing worsens the femoral head deformity.
- Chronic hip joint synovitis also develops producing pain and restriction of motion.
- Necrotic femoral head goes through 4 stages of healing over 3–5 years:
- 1. Stage of avascular necrosis
- Smaller femoral head epiphysis with increased radiodensity
- 2. Stage of fragmentation
- Necrotic epiphysis shows fragmentation.
- Necrotic bone is resorbed, weakening the head.
- Most deformity occurs during this stage, which lasts 1–2 years.
- 3. Stage of reossification
- New bone begins to fill the epiphysis.
- Longest stage, lasting up to 3 years
- 4. Healed
- Femoral head is completely reossified.
- Not all heal back in round shape, and deformed femoral heads are at risk for developing arthritis later.
- 1. Stage of avascular necrosis
Etiology
- Unknown
- Unlikely genetic transmission, as <5% have family history
- Many theories:
- Multifactorial (genetic predisposition with environmental trigger)
- Hyperactivity and subclinical trauma
- Type II collagenopathy
- Thrombophilia (factor V Leiden)
- Tobacco smoke exposure
Commonly Associated Conditions
- Delayed bone age
- Hyperactivity
- Genitourinary anomalies (hypospadias, undescended testis, and inguinal hernia)
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