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Legg-Calvé-Perthes (LCP) Disease
Definition & Epidemiology
Legg-Calvé-Perthes (LCP) disease is idiopathic avascular necrosis (AVN) of the proximal femoral epiphysis in children. Key demographic features:
- Age: Typically 4-10 years; average age at diagnosis ~7 years
- Sex: Boys affected 3-4x more than girls (but girls have worse prognosis)
- Bilaterality: ~10% of cases are bilateral (though usually at different stages)
- Laterality: Rarely truly simultaneous - bilateral presentation should prompt search for alternative diagnoses
Aetiology & Pathogenesis
The exact cause remains unknown. The leading hypothesis involves coagulation disorder promoting thrombotic occlusion of the vascular supply to the femoral head, leading to bone death. Contributing factors include:
- Socioeconomic deprivation
- Passive smoking
- Delayed bone age (skeletal maturity is typically delayed in these children)
- Thrombophilia (some evidence of inherited risk factors)
The disease is self-limiting and follows a predictable sequence:
- Avascular/Necrotic phase: Ischaemia causes bone death in all or part of the femoral epiphysis
- Revascularisation/Fragmentation phase: Blood supply returns; dead bone is resorbed and the ossific nucleus fragments within the cartilaginous femoral head
- Reossification/Healing phase: Bony epiphysis regenerates ("heals")
- Residual phase: Final shape of the head is established
During the collapse and fragmentation phases, the cartilage follows the shape of the reossifying epiphysis - leading to irreversible femoral head deformity if the head is not protected.
Differential Diagnosis
Other causes of femoral head AVN must be excluded, especially in bilateral cases:
| Condition | Key Distinguishing Feature |
|---|
| Steroid-induced AVN | History of steroid use |
| Septic arthritis sequelae | Preceding infection |
| Sickle cell disease | Haemoglobinopathy |
| Hypothyroidism | TFTs, systemic features |
| Multiple epiphyseal dysplasia | Bilateral, other joints involved |
| Meyer dysplasia | Age <4 yrs, bilateral, NO subchondral fracture or subluxation |
Meyer dysplasia in particular can mimic LCP and lead to unnecessary treatment - it shows delayed/smaller ossification centres and cystic changes but lacks condensation, subchondral fractures, and fragmentation.
Clinical Features
- Limp (antalgic or Trendelenburg)
- Pain in hip or referred to knee (intermittent)
- Restricted hip movement - especially abduction and internal rotation
- Symptoms typically present for ~6 weeks before diagnosis is made
- Irritability of the hip joint
Diagnosis & Imaging
Plain Radiography
AP pelvis + "frog-leg" lateral views are the first-line investigation. Radiographic changes lag 6 weeks behind clinical onset. If early presentation with normal X-rays - repeat at 6 weeks if still symptomatic.
Waldenström radiographic stages (modified by International Perthes Study Group - IPSG):
- Initial: Slight widening of joint space, smaller ossific nucleus
- Fragmentation: Dense, fragmented femoral head epiphysis
- Reossification: New bone formation within the epiphysis
- Healed/Residual: Final shape established
AP pelvic radiograph showing avascular necrosis of the left femoral head with destruction, short neck and high greater trochanter
MRI
Superior to scintigraphy for early detection and defining extent of involvement. Gadolinium-enhanced perfusion MRI can show lateral pillar involvement earlier than plain films (at initial fragmentation stage rather than mid-fragmentation), potentially allowing earlier treatment decisions. ~50% of children require sedation/GA. Reperfusion progresses in a horseshoe pattern - posterior to anterior.
Bone Scintigraphy
Can establish early diagnosis but now largely superseded by MRI.
Classification Systems
Herring Lateral Pillar Classification (most commonly used)
Applied during the fragmentation phase - based on height of the lateral pillar of the femoral head on AP radiograph:
| Group | Lateral Pillar Height | Prognosis |
|---|
| A | Fully maintained (no involvement) | Good |
| B | >50% of original height preserved | Intermediate |
| B/C border | Exactly 50% height or narrow pillar | Poorer |
| C | <50% of original height | Poor |
If the anterolateral portion of the head is preserved, prognosis is good.
Catterall Classification (older)
Divides into groups I-IV based on proportion of femoral head involved (25%, 50%, 75%, total).
Salter-Thompson Classification
Groups A and B - based on extent of subchondral fracture.
Stulberg Classification (outcome)
Used at skeletal maturity to grade residual femoral head sphericity (I-V); higher grades predict early osteoarthritis.
Prognosis
Key prognostic factors:
- Age at onset: Younger children (<6 yrs) have better prognosis due to greater remodelling potential
- Sex: Girls have worse prognosis
- Herring grade: Group A > B > C
- Extent of head involvement: Partial involvement better than total
- Maintenance of motion: Loss of hip movement is a poor sign
Management
Principles
The goal is to minimise femoral head deformity and prevent secondary acetabular dysplasia by maintaining a good range of joint movement and ensuring femoral head containment within the acetabulum ("containment principle").
Non-operative
- Analgesia and physiotherapy to maintain ROM
- Avoid crutches/wheelchairs (promote flexion/adduction deformity)
- Bracing: Scottish Rite (Atlanta) orthosis or Petrie casts for abduction - but evidence shows brace management does not alter natural history; mainly used in younger children
- Activity modification, anti-inflammatories
- Observation in: children <6 years, Herring Group A, Herring Group B <8 years
Operative (Containment Surgery)
Indicated in higher-risk cases - older children, Herring B/C or C, loss of containment.
A. Proximal Femoral Varus Derotational Osteotomy (VDRO)
- Procedure of choice when containment needed but bracing not feasible
- Preferred in children 8-10 years, with significant femoral anteversion
- Achieves 0-15° of varus correction
- Advantages: Maximal femoral head coverage, corrects anteversion
- Disadvantages: Limb shortening, risk of gluteal lurch, premature physeal closure, poor results in Group C aged >9 years
B. Innominate (Salter) Osteotomy
- Provides anterolateral femoral head coverage
- Advantages: May slightly lengthen extremity, avoids second operation for plate removal
- Disadvantages: Risk of over-correction, can compress already compromised femoral head, does not accelerate healing
Note: Neither osteotomy has been conclusively shown to be superior to the other - choice depends on surgeon's expertise and individual patient factors.
C. Shelf Acetabuloplasty / Chiari Osteotomy
- Late "salvage" procedures for established deformity limiting movement
D. Arthrodiastasis (Joint Distraction)
- Used in late-onset or severe cases
- External fixator maintains joint space during revascularisation
Late/Salvage Surgery
When deformity is established:
- Surgical hip dislocation with femoral head reshaping
- Relative femoral neck lengthening
- Joint-preserving surgery (improves pain, ROM, and abductor strength)
- Total hip replacement in adults with end-stage osteoarthritis
Treatment Algorithm (Summary)
| Patient Group | Recommended Management |
|---|
| Age <6 years, any Herring group | Observation ± physio |
| Age 6-8 years, Herring A | Observation |
| Age 6-8 years, Herring B | Consider containment (brace or surgery) |
| Age 6-8 years, Herring C | Surgery (osteotomy) |
| Age >8 years, Herring B or C | Surgery (osteotomy) |
| Any age, Herring B/C border | Surgery generally favoured |
Sources:
- Campbell's Operative Orthopaedics 15th Ed 2026, Ch. 34 (Legg-Calvé-Perthes Disease)
- Bailey and Love's Short Practice of Surgery 28th Ed, Ch. 44
Recent evidence note: A 2026 systematic review and meta-analysis (
PMID 41308557) evaluated the diagnostic performance of diffusion-weighted MRI (DWI) in LCP disease, supporting the growing role of advanced MRI techniques in early diagnosis.