• Clinical science

Legg-Calvé-Perthes disease

Abstract

Legg-Calvé-Perthes disease (LCPD, or Perthes disease) refers to an idiopathic, avascular necrosis of the femoral head. It may occur unilaterally or bilaterally and typically manifests between the ages of four and ten (the younger the patient at the time of diagnosis, the better the prognosis). Children experience hip pain on weight bearing, which often projects to the ipsilateral knee and causes an antalgic gait. Early stages are only detectable on MRI but progress of the disease can be tracked and graded using conventional x-ray. Surgery is performed if x-ray reveals signs that indicate an unfavorable prognosis. The aim of surgical intervention is to cover the femoral head as completely as possible with the hip socket, thus retaining its anatomical position. In mild forms of the disease, reduced weight-bearing and physical therapy are indicated.

Epidemiology

  • Sex: > (4:1)
  • Peak incidence: 4–10 years
  • Incidence (in children < 15 years): 0.4–29/100,000

References:[1][2]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

  • Unknown although there are a number of hypotheses.
    • Repetitive microtrauma
      • Hyperactivity
    • Bleeding disorders
      • Excess factor VII, protein C and S deficiencies, as well as factor V Leiden thrombophilia, are associated with LCPD.
    • Genetic factors
      • Approximately 10% of cases are familial, although the current theory states that afflicted patients may have a genetic susceptibility which is then triggered by environmental factors.
  • Associations with LCPD
    • Environmental factors: association with lower socioeconomic status
    • Frequently associated with delayed bone age and short stature

Classification

Lateral pillar classification

This classification possesses the highest clinical relevance because it correlates best with long-term outcome. The crucial criterion in this classification is the height of the lateral third (“lateral pillar”) of the femoral head.

Modified (Herring) Lateral pillar classification
Group A Height of the lateral pillar is 100% (no involvement)
Group B Height of the lateral pillar is > 50%
Group C Height of the lateral pillar is < 50%

Other classifications

  • Catterall classification
  • Salter and Thompson classification
  • Stulberg classification

References:[3]

Pathophysiology

Avascular necrosis of the femoral head is due to a mismatch between the rapid growth of the femoral epiphyses and the slower development of adequate blood supply to the area

References:[1]

Clinical features

  • Antalgic gait (on weight-bearing leg)
  • Pain in the hip or the upper leg, sometimes projecting to the knee
    • Insidious onset; , pain may fluctuate depending on physical activity
    • Often exacerbated by internal rotation
    • FABER (Flexion, ABduction, and External Rotation) test elicits pain.
    • Groin tender on palpation
  • Restricted range of movement is always present, especially regarding internal rotation and abduction.
  • Hinge abduction : refers to the lateral femoral head bumping into the ventrolateral acetabulum when the leg is abducted, possibly involving pain, a palpable clunk, and restriction in the range of movement.
  • Contralateral involvement in ∼ 15% of cases

If children complain of pain in the knee(s), Legg-Calvé-Perthes disease should be considered!

References:[1][3][4]

Diagnostics

Initial imaging

  • X-ray (anterior-posterior and frog leg positions ;)
    • Frequently unremarkable during the first 3–6 months!
    • Diagnostic criteria
      • Radiographic classification: see lateral pillar classification above
      • “Head-at-risk” signs; (as defined by the complementary Catterall classification) → prognostically unfavorable radiographic signs such as increased lucency of the femoral head
        • Lateral calcification
        • Lateral subluxation of the femoral head
        • Lesions extending to the metaphysis
        • Horizontal alignment of the epiphyseal plate
        • Gage sign: triangle-shaped osteoporotic area of increased radiolucency of the lateral femoral head
        • Crescent sign: subchondral lucency representing a fracture
      • Joint space widening
  • MRI: Indicated in case of unremarkable initial imaging (but persisting clinical suspicion)

References:[1][3][4]

Differential diagnoses

Differential diagnosis of pediatric hip pain

Disease Pathophysiology Clinical features Diagnostic findings
Transient synovitis
  • 4–10 years of age
  • Sex: > (2:1)
  • URI in recent weeks
  • Afebrile
  • Transient hip pain
  • Laboratory: normal
  • Imaging
    • Normal x-ray
    • Effusion on ultrasound that should improve over the course of several days (whereas it remains in LCPD)
Septic arthritis
  • Infection either by hematogenous spread of bacteria or direct contamination
  • Any age
  • Acute onset of pain and fever.
  • Fever > 38.5° C
  • Child may refuse to bear weight
  • Laboratory
  • Imaging
    • Effusion on ultrasound
    • Widened joint space on x-ray though unremarkable in early stages
Slipped capital epiphysis
  • 10–16 years of age
  • Acute on chronic dull pain with antalgic gait
  • Afebrile
  • Vague hip and knee pain
  • Laboratory: normal
  • Imaging: x-ray shows displacement of the femoral head
Legg-Calve-Perthes
  • Mismatch between the rapid growth of the femoral epiphyses and the slower development of adequate blood supply to the area
  • 4–10 years of age
  • Insidious onset, pain may fluctuate with activity
  • Afebrile
  • Vague hip and knee pain
  • Laboratory: normal
  • Imaging: x-ray may be unremarkable in early stages and as the disease progresses increased lucency of the femoral head may be seen
Developmental dysplasia of the hip
  • Not clear but breech delivery, family history, and oligohydramnios seem to play a role
  • Asymptomatic during infancy
  • Inability to abduct the hip, hip pain, and limp later develop if uncorrected
  • Laboratory: normal
  • Imaging: Ultrasound before 4 months and x-ray after 4 months are diagnostic

Transient synovitis (also known as toxic synovitis)

  • Epidemiology
    • Common cause of acute hip pain among children
    • Peak incidence: 4–10 years of age
    • Sex: > (2:1)
  • Pathophysiology
  • Clinical features
    • Unilateral and transient hip or groin pain
    • Recent upper respiratory tract infection in up to 50% of the patients
  • Diagnosis
    • Laboratory: mostly normal (but some cases present with mild leukocytosis and ESR elevation)
    • Imaging
      • Normal x-ray
      • Effusion on ultrasound that should improve over the course of several days
  • Treatment: conservative (i.e., NSAIDs)

References:[5]

The differential diagnoses listed here are not exhaustive.

Treatment

  • Conservative treatment: limited weight bearing, physical therapy
    • Young children (< 6y of age)
    • Lateral pillar A classification
    • Femoral head mostly undamaged
    • Consider casting and bracing before surgery if femoral head deformity develops or range of motion worsens
  • Surgery: femoral osteotomy
    • Older children (> 8y of age)
    • Lateral pillar B/C classification
    • Extensive damage to the femoral head

References:[3]

Complications

  • Early osteoarthritis of the hip joint due to any of the following
    • Incongruence between the femoral head and acetabulum
    • Shortening of the femoral neck featuring trochanteric elevation, which can manifest as a Trendelenburg's sign (waddling gait due to insufficiency of the gluteal muscles)
    • Lateralization and coxa magna (broadening of the femoral head), which can manifest as hinge abduction

We list the most important complications. The selection is not exhaustive.

Prognosis

  • Age at onset is the most important prognostic factor: the younger the patient, the better the prognosis!
  • Girls with LCPD have a less favorable prognosis than boys.

References:[3]