Perthes Disease
Basics
Description
Childhood femoral head osteonecrosis of unknown etiology, which can weaken the femoral head and produce a permanent deformity in some patients, predisposing them to early arthritis
Epidemiology
- Incidence varies depending on the region: 0 to 15 per 100,000 children <15 years of age
- In United States and Canada, about 5 per 100,000
- Rare in persons of African descent
- Most frequent in children 4 to 8 years old
- 3 to 5 times more common in boys than girls
- 10–15% develop bilateral disease in asynchronous fashion.
- Associated factors: delayed bone age, hyperactivity, exposure to smoking, genitourinary anomalies (hypospadias, undescended testis, and inguinal hernia)
Pathophysiology
- A partial or complete disruption of femoral head blood supply produces a partial or total femoral head osteonecrosis.
- Greater the head involvement, worse 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 head goes through four stages of healing over 3 to 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 to 2 years.
- 3. Stage of reossification: New bone begins to fill the epiphysis; longest stage, lasting up to 3 years
- 4. Healed: Head is completely reossified. Not all heads heal back in round shape, and deformed heads are at risk for developing arthritis later.
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)
- Smoke exposure
There's more to see -- the rest of this topic is available only to subscribers.
Citation
Cabana, Michael D., editor. "Perthes Disease." 5-Minute Pediatric Consult, 8th ed., Wolters Kluwer, 2019. Pediatrics Central, peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617545/all/Perthes_Disease.
Perthes Disease. In: Cabana MDM, ed. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617545/all/Perthes_Disease. Accessed December 10, 2024.
Perthes Disease. (2019). In Cabana, M. D. (Ed.), 5-Minute Pediatric Consult (8th ed.). Wolters Kluwer. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617545/all/Perthes_Disease
Perthes Disease [Internet]. In: Cabana MDM, editors. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. [cited 2024 December 10]. Available from: https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617545/all/Perthes_Disease.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC
T1 - Perthes Disease
ID - 617545
ED - Cabana,Michael D,
BT - 5-Minute Pediatric Consult
UR - https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617545/all/Perthes_Disease
PB - Wolters Kluwer
ET - 8
DB - Pediatrics Central
DP - Unbound Medicine
ER -