Musculoskeletal · PANCE / PANRE

Legg-Calvé-Perthes Disease

Idiopathic avascular necrosis of the proximal femoral epiphysis in children aged 4-8.

Also known as: Legg-Calve-Perthes, Perthes disease, LCPD, coxa plana

Overview

Idiopathic osteonecrosis of the femoral head occurring in growing children, characterized by self-limited interruption of vascular supply to the capital femoral epiphysis followed by revascularization, resorption, reossification, and remodeling.

Epidemiology

Incidence 1 in 1,200 to 1 in 12,000 children. Peak age 4-8 years (range 2-12). Male-to-female ratio 4-5:1. Bilateral involvement in 10-15 percent (usually asynchronous).

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Risk factors

  • Male sex
  • Short stature with delayed bone age
  • Low socioeconomic status
  • Exposure to secondhand smoke
  • Family history (modestly increased risk)
  • Thrombophilia (controversial)

Pathophysiology

Disruption of blood supply to the femoral head produces avascular necrosis. Disease evolves through four radiographic stages (Waldenström): initial (sclerosis and apparent enlargement), fragmentation (subchondral fracture and lucency), reossification (new bone formation), and residual (remodeling). The shape achieved at skeletal maturity determines long-term hip function.

Clinical presentation

Symptoms

  • Insidious, painless or mildly painful limp
  • Hip, groin, thigh, or referred knee pain
  • Decreased activity tolerance
  • Symptoms typically persist over weeks to months without an acute event

Signs / physical exam

  • Antalgic and Trendelenburg gait
  • Decreased hip abduction and internal rotation
  • Mild thigh atrophy from disuse
  • Leg length discrepancy in advanced disease

Classic findings

Young boy aged 4-8 with a painless limp, restricted internal rotation and abduction of the hip, and characteristic crescent sign or femoral head flattening on radiographs.

Differential diagnosis

  • Septic arthritis — Fever, refusal to bear weight, elevated WBC/ESR/CRP, joint aspiration
  • Transient synovitis — Recent viral illness, painful limp, afebrile, self-limited; Kocher criteria
  • SCFE — Older children (10-16), obese, externally rotated hip, displaced epiphysis on radiographs
  • Developmental dysplasia of the hip — Identified in infancy; abnormal Ortolani/Barlow or limited abduction
  • Sickle cell osteonecrosis — Known sickle cell disease, often bilateral, MRI confirms
  • Multiple epiphyseal dysplasia / Meyer dysplasia — Bilateral, symmetric epiphyseal changes; not progressive

Diagnostic workup

Diagnostic criteria

Lateral pillar (Herring) classification at the fragmentation stage assesses the height of the lateral one-third of the epiphysis: A — full height preserved (best prognosis); B — at least 50 percent of original height; B/C border — exactly 50 percent or narrow lateral pillar; C — less than 50 percent of height (worst prognosis). Age at onset is the other major prognostic variable — younger children (<6 years) generally do better.

Labs

  • CBC, ESR, CRP normal in LCPD — distinguishes from septic arthritis and osteomyelitis
  • Consider hemoglobin electrophoresis if sickle cell osteonecrosis is in the differential

Imaging

  • AP and frog-leg lateral pelvic radiographs
  • Early findings: increased epiphyseal density, joint space widening, crescent sign (subchondral fracture)
  • Later findings: epiphyseal fragmentation, flattening, lateral extrusion of the femoral head
  • MRI for early disease before radiographic changes appear

Diagnostic algorithm

Lateral Pillar (Herring)Lateral 1/3 Epiphysis HeightPrognosis
AFull original heightExcellent — observation
B≥50% of original heightGood in <8 yr; variable in older
B/C borderExactly 50% or narrowIntermediate — surgery often considered
C<50% of original heightPoor — high risk for deformity
Lateral pillar (Herring) classification of Legg-Calvé-Perthes disease and prognosis.

Treatment

First-line

  • Activity restriction to limit hip joint loading and protect the femoral head during fragmentation
  • NSAIDs for pain
  • Physical therapy emphasizing hip range of motion (abduction and internal rotation)
  • Crutches or partial weight bearing for symptomatic relief
  • Observation alone for children <6 years with lateral pillar A or B disease

Second-line / adjunct

  • Containment treatment (keep the femoral head 'contained' within the acetabulum during healing) — bracing has largely fallen out of favor; surgical containment via femoral varus osteotomy or innominate (Salter) pelvic osteotomy considered for children ≥6-8 years with lateral pillar B or B/C border disease
  • Salvage procedures (femoral osteotomy, hip arthroscopy) for late deformity and impingement
  • Total hip arthroplasty in early adulthood for end-stage post-Perthes arthritis

Complications

  • Coxa magna and coxa plana (large, flattened femoral head)
  • Femoral head deformity with hinge abduction
  • Leg length discrepancy
  • Early osteoarthritis of the hip — accelerated by older age at onset and severe lateral pillar involvement
  • Femoroacetabular impingement

PANCE pearls

  • LCPD typically affects younger children (4-8) and SCFE older adolescents (10-16) — age is the most useful initial differentiator.
  • Knee pain in a child is hip pain until proven otherwise — examine the hip and obtain pelvic radiographs.
  • Age at onset and lateral pillar classification together are the strongest predictors of outcome; children diagnosed before age 6 with intact lateral pillar generally do well without surgery.
  • The natural history of LCPD is healing — the goal of treatment is to optimize the shape of the femoral head at skeletal maturity, not to alter the disease course.

References

  • POSNA — Pediatric Orthopaedic Society of North America clinical resources on Legg-Calvé-Perthes disease
  • AAOS — American Academy of Orthopaedic Surgeons evidence-based guidance on Perthes disease

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