II. Epidemiology

  1. Children age 4-9 years old typically affected (may affect ages 3-12 years old)
  2. Boys affected 4-5 times more often than girls
  3. Familial in 20% of cases
  4. Incidence 1:1000 to 1:5000
  5. Caucasian more often affected than african american
  6. Typical body habitus
    1. Short to average height
    2. Average weight to Overweight (but not typically obese as seen in Slipped Capital Femoral Epiphysis)

III. Risk Factors

  1. Boys
  2. Low birth weight
  3. Abnormal birth presentation (e.g. Breech Presentation)
  4. Family History
  5. Higher birth order
  6. Lower socioeconomic status

IV. Pathophysiology

  1. Juvenile idiopathic avascular necrosis of femoral head
  2. Results from a partial interruption of the blood supply to the femoral head
    1. Repeat episodes result in infarction and necrosis of the femoral head
  3. Subchondral Stress Fractures with remodeling over ensuing 2-4 years
    1. Femoral head flattens and subluxes

V. Etiology

  1. Idiopathic Osteochondrosis

VI. Symptoms

  1. Pain in area of hip and may be referred to knee
  2. Unilateral Hip Pain in 84-90% of cases
  3. Insidious onset of pain

VII. Signs

  1. Child walks with a limp
  2. Decreased Hip Range of Motion
    1. Limited hip abduction
    2. Limited internal rotation
  3. Leg Length Discrepancy

VIII. Imaging: XRay

    1. Anteroposterior Pelvis
    2. Frog-leg lateral hip
  2. Findings
    1. XRay may appear normal early in condition
    2. Joint space widening
    3. Proximal femur growth center with fragmentation, flattening, and sclerosis
      1. May demonstrate a subchondral Stress Fracture line
    4. Crescent sign
      1. Subcortical lucency represents bony destruction
  3. Non-diagnostic results
    1. Consider MRI Hip (gold standard)

IX. Differential Diagnosis

  1. Septic Hip
  2. Transient Hip Synovitis
  3. Proximal femur Osteomyelitis

X. Management

  1. Orthopedic Consultation in all cases (within 1 week)
  2. NSAIDs may be used for pain
  3. Difficult management
    1. Long-term treatment
    2. Limited activity
  4. Bracing and Casting for up to 1-2 years
  5. Surgical reconstruction to improve Hip Joint congruity
    1. Allows child back to activity in 4-6 months

XI. Complications

  1. Severe degenerative hip disease
    1. Requires hip replacement by middle age in 50% cases
  2. Proximal thigh atrophy and limb shortening

XII. Prognosis

  1. Fair at best to avoid longterm Arthritis (see complications above)
  2. Best prognosis for optimal range of motion is with early treatment at young age
  3. Predictors of worse prognosis
    1. Age over 6 years at onset of condition
    2. More severely affected femoral head deformity
    3. Hip Joint incongruity
    4. Decreased Hip Range of Motion

XIII. References

  1. Jhun and Raam in Herbert (2016) EM:Rap 16(2):15-6
  2. Claudius and Behar in Majoewsky (2012) EM:RAP-C3 2(8): 1
  3. Atanda (2011) Am Fam Physician 83(3): 285-91 [PubMed]
  4. Wiig (2008) J Bone Joint Surg Br 90(10): 1364-71 [PubMed]

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Ontology: Legg-Calve-Perthes Disease (C0023234)

Definition (MSHCZE) Aseptická nekróza hlavice kosti kyčelní, nejč. je mezi 3 až 10 lety věku, převažuje postižení chlapců. Projevuje se bolestmi v kyčli, později kulháním; bolest se může někdy přenášet do kolena. Mohou vzniknout svalové kontraktury a atrofie, změny jsou patrné rentgenologicky od počátečního rozšíření kloubní štěrbiny k oploštění a fragmentaci; následně se při nedostatečné léčbě zkracuje krček kosti stehenní a může dojít k deformitám hlavice a sekundární koxartróze. Imobilizace se provádí např. sádrou v abdukci, flexi a mírné zevní rotaci, možné je použití dlah. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) A very rare disorder of unknown etiology affecting children. It is characterized by avascular necrosis of the femoral head.
Definition (MSH) A particular type of FEMUR HEAD NECROSIS occurring in children, mainly male, with a course of four years or so.
Concepts Disease or Syndrome (T047)
MSH D007873
ICD10 M91.2 , M91.3
SnomedCT 111255008, 203371007, 270545000, 203372000, 203367009, 240169001, 156817009, 240241003, 15739006
English Coxa Plana, Disease, Perthes, Legg Perthes Disease, Perthes Disease, Disease, Legg-Perthes, Perthes' disease, Perthe's disease, Perthe's disease of hip, Perthes disease - osteochondritis of the femoral head, coxa plana, LEGG-PERTHES DISEASE, LEGG-CALVE-PERTHES DISEASE, PERTHES DISEASE, LCP, LEGG PERTHES DIS, PERTHES DIS, Perthes' dis. (femoral head), Perthes' disease - osteochondritis of the femoral head, Legg Calve Perthes disease, Legg Perthes disease, Legg-Perthe's disease (diagnosis), Legg-Perthe's disease, Legg Calvé Perthes Syndrome, Legg Calve Perthes Disease, Legg-Calvé-Perthes Syndrome, Syndrome, Legg-Calvé-Perthes, Disease, Legg-Calve-Perthes, disease legg calve perthe, perthes' disease, disease legg perthe, disease leggs perthes, disease perthes's, legg-perthes disease, Disease;Calve-Perthes, disease legg calves perthe, legg calve perthe disease, legg calve perthes disease, perthes legg disease, calve disease legg perthes, disease legg calves perthes, legg perthe disease, legg perthes disease, calve diseases legg perthes, disease legg calve perthes, legg perthes calve disease, legg-calve-perthes disease, perthe's disease, calve disease legg perthe, disease perthes, legg-calve-perthes syndrome, perthes disease, disease legg-calve-perthes, perthe disease, Osteochondritis Deformans, Legg-Calvé-Perthes Disease, Syndrome, Legg-Calve-Perthes, Disease, Legg-Calvé-Perthes, Legg Calve Perthes Syndrome, Legg-Perthes Disease, Legg-Calve-Perthes Syndrome, Osteochondritis Deforman, Legg Calvé Perthes Disease, Legg-Calve-Perthes Disease, Legg-Calve-Perthes Disease [Disease/Finding], LCPD, pseudocoxalgia (diagnosis), Calve - Perthes' disease, (Perthes' disease) or (Coxa plana), Perthe's disease (disorder), (Perthes' disease) or (Coxa plana) (disorder), Coxa plana, Legg-Calve-Perthes disease, Pseudocoxalgia, Coxa plana (disorder), pseudocoxalgia, Legg-Perthes, coxa; plana, Perthes, epiphysis; juvenile osteochondrosis head of femur, femoral capital epiphysis; osteochondrosis, head of femur; juvenile osteochondrosis, head of femur; osteochondrosis, juvenile, Calvé-Perthes, osteochondrosis; femoral capital epiphysis, osteochondrosis; juvenile, head of femur, plana; coxa, Legg-Calvé-Perthes disease, Juvenile osteochondrosis of head of femur, Osteochondrosis of Legg-Calve-Perthes, Calve-Perthes disease
Dutch ziekte van Perthes, coxa; plana, epifyse; juveniele osteochondrose femurkop, femurkop-epifyse; osteochondrose, femurkop; juveniele osteochondrose, femurkop; osteochondrose, juveniel, osteochondrose; femurkop-epifyse, osteochondrose; juveniel, femurkop, plana; coxa, Coxa plana, Pseudocoxalgie
French Maladie de Perthe, Syndrome de Legg-Calve-Perthes, Maladie de Legg-Calvé-Perthes, Maladie de Legg-Calve-Perthes, Syndrome de Legg-Calvé-Perthes, Coxa plana, Maladie de Legg-Perthes, Maladie de Perthes
German Morbus Perthes, Pseudokoxalgie, Coxa plana, Legg-Perthes-Krankheit, Perthes-Krankheit
Italian Malattia di Perthes, Malattia di Legg-Calve-Perthes, Malattia di Legg-Perthes, Osteocondrite deformante, Morbo di Perthes
Swedish Legg-Perthes sjukdom
Japanese ペルテスビョウ, レッグ・カルベ・ペルテス病, Perthes病, Legg-Perthes病, ペルテス病, レッグ-ペルテス病, 扁平股, 若年性変形性骨軟骨炎, レッグ-カルヴェ-ペルテス病
Czech Calvého-Leggova-Perthesova nemoc, coxa plana, coxa plana idiopathica, malum coxae juvenile, Perthesova nemoc, Leggova-Calvého-Waldenströmova-Perthesova nemoc, Leggova-Perthesova nemoc, Maydlova nemoc
Finnish Legg-Calvé-Perthesin tauti
Korean 납작엉덩관절(편평고), 가성 엉덩관절통(증)
Polish Choroba Calvego Legga i Perthesa, Samoistna martwica głowy kości udowej, Osteochondroza młodzieńcza głowy kości udowej, Jałowa młodzieńcza martwica głowy kości udowej, Choroba Legga-Calvego-Perthesa, Choroba Perthesa
Hungarian Perthes-betegség
Portuguese Doença de Legg-Calve-Perthes, Doença de Legg-Perthes, Coxa Plana, Doença de Perthes
Spanish Enfermedad de Legg-Perthes, Enfermedad de Legg-Calve-Perthes, coxa plana (trastorno), coxa plana, enfermedad de Legg - Calvé - Perthes, enfermedad de Perthes, osteocondritis deformante juvenil, seudocoxalgia, Enfermedad de Perthes, Coxa Plana
Norwegian Calvé Legg Perthes sykdom, Perthes sykdom, Juvenil osetokondrose av lårbenet, Coxa Plana, Juvenil osetokondrose av lårbeinet

Ontology: Avascular necrosis of the capital femoral epiphysis (C1442965)

Concepts Disease or Syndrome (T047)
SnomedCT 111255008, 15739006
English Perthes disease of hip, Perthes disease, Avascular necrosis of the capital femoral epiphysis, Avascular necrosis of the capital femoral epiphysis (disorder)
Spanish necrosis aséptica de la epífisis de la cabeza del fémur, necrosis avascular de la epífisis de la cabeza femoral (trastorno), necrosis avascular de la epífisis de la cabeza femoral