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Legg-Calvé-Perthes Disease (Perthes Disease)
10-Mark Structured Answer — 2026 MS Orthopaedics Theory Examination
1. Definition
Legg-Calvé-Perthes (LCP) disease is idiopathic avascular necrosis (AVN) of the proximal femoral epiphysis in children, leading to a self-limiting cycle of necrosis, collapse, revascularisation, and reossification of the femoral head. The final shape of the healed femoral head determines long-term hip function.
2. Epidemiology
| Feature | Details |
|---|
| Sex | Boys : Girls = 4:1 |
| Age | 4-10 years (average 7 years) |
| Laterality | Unilateral in 90%; bilateral in 10% (sequential, not simultaneous) |
| Socioeconomic | More common in lower socioeconomic groups |
| Associated | Passive smoking, low birth weight, skeletal maturity delay |
3. Aetiology / Pathophysiology
The exact cause is unknown but a vascular interruption to the capital femoral epiphysis is the final common pathway.
Proposed mechanisms:
- Coagulation disorder / thrombophilia promoting thrombotic occlusion of the lateral epiphyseal arteries
- Increased intracapsular pressure from synovitis compromising blood supply
- Repetitive micro-trauma
- Elevated venous pressure
The capital femoral epiphysis in children is supplied by the lateral epiphyseal arteries (branches of the medial femoral circumflex artery) — which run along the femoral neck and are vulnerable to compression and injury. Once ischaemia occurs, the process follows a predictable course.
4. Stages (Waldenström's Radiological Stages)
DIAGRAM 1: Four Stages of Perthes Disease
Diagram 1: Sequential radiological stages of Perthes disease from initial necrosis to healed coxa plana.
| Stage | Radiological Findings | Duration | Clinical |
|---|
| 1. Initial (Necrosis) | Smaller, dense sclerotic epiphysis; medial joint space widening; subchondral fracture (crescent sign); X-ray may be normal for 3-6 months | Months | Hip pain, limp |
| 2. Fragmentation | Femoral head appears fragmented and irregular; resorption of dead bone; lateral extrusion may begin | 1-3 years | Symptoms most severe |
| 3. Reossification | New bone lays down in the femoral head; irregular density; head re-forming | Up to 18 months | Symptoms improving |
| 4. Healing / Remodelling | Trabecular pattern returns; head remodels until skeletal maturity; may result in coxa plana, coxa magna | Until maturity | Minimal symptoms |
5. Clinical Features
History:
- Intermittent pain in the hip or referred to the knee (obturator nerve)
- Insidious onset limp
- Symptoms often present for 6 weeks before diagnosis
Examination:
| Sign | Description |
|---|
| Antalgic gait | Short-stride limp away from painful side |
| Trendelenburg sign | Positive if abductor weakness |
| Restricted internal rotation + abduction | Most consistent finding; due to spasm and synovitis |
| Flexion contracture | In severe/late cases |
| Muscle wasting | Thigh atrophy from disuse |
| Leg length discrepancy | Mild shortening in severe cases |
6. Investigations
X-ray (AP + Frog-lateral pelvis): First line; may be normal in early disease
Textbook radiograph — Perthes disease (whole head involvement):
Fig. AP pelvis X-ray showing early Perthes disease of right hip: dense necrotic femoral head epiphysis with sclerosis. (Bailey & Love's Surgery 28th Ed)
MRI: Most sensitive early investigation; shows extent of ischaemia before X-ray changes appear; used for pretreatment planning
Bone scan (Tc-99m): Shows photon deficient area early ("cold spot"); useful when X-ray normal
Arthrogram: Assesses true cartilaginous femoral head shape; guides surgical planning
7. Classification
A. Catterall Classification (Amount of head involvement)
| Group | Involvement | At-risk signs |
|---|
| I | Anterior epiphysis only (<25%) | None |
| II | Anterior with sequestrum (~50%) | May be present |
| III | Most of head involved (~75%) | Usually present |
| IV | Total head involvement (100%) | Present |
Catterall "Head-at-Risk" Signs (indicate poor prognosis):
- Gage sign - V-shaped radiolucency at lateral epiphysis/metaphysis
- Calcification lateral to epiphysis
- Lateral subluxation of femoral head
- Horizontal (oblique) proximal femoral physis
- Metaphyseal cyst
B. Herring Lateral Pillar Classification (Most widely used - applied in fragmentation stage)
DIAGRAM 2: Herring Lateral Pillar Classification
Diagram 2: Herring Lateral Pillar Classification. The femoral head is divided into medial, central and lateral thirds. Lateral pillar height determines group and prognosis.
| Group | Lateral Pillar Height | Prognosis |
|---|
| A | Fully maintained (no loss) | Uniformly good regardless of age |
| B | >50% of original height maintained | Good if age <9 yr; poor if >9 yr |
| B/C border | Exactly 50% or thin lateral pillar | Intermediate |
| C | <50% original height | Worst; aspherical head regardless of age/treatment |
C. Stulberg Classification (Outcome / healed stage)
| Class | Description | Long-term |
|---|
| I | Normal spherical head, normal hip | No arthritis |
| II | Spherical head, coxa magna/plana/short neck | No arthritis |
| III | Aspherical femoral head (ovoid/mushroom), congruent | Mild arthritis |
| IV | Aspherical head, congruent flat acetabulum | Moderate arthritis |
| V | Aspherical head, incongruent acetabulum | Severe arthritis |
8. Management
Principle of Treatment — CONTAINMENT
The containment principle states that the soft, plastic femoral head during fragmentation, if held concentrically within the acetabulum, will be moulded into a spherical shape by the acetabulum acting as a biological mould. This reduces deformity and improves long-term outcome.
DIAGRAM 3: Containment Concept and Treatment Algorithm
Diagram 3: Containment concept - the acetabulum molds the plastic femoral head. Age-based treatment decision flowchart.
Treatment Decision (Age + Herring Grade)
| Age at Onset | Herring Grade | Treatment |
|---|
| <6 years | Any | Conservative (excellent remodelling potential) |
| 6-8 years | A | Conservative |
| 6-8 years | B or B/C | Consider surgical containment |
| >8 years | A | Conservative |
| >8 years | B, B/C, C | Surgical containment strongly recommended |
A. Conservative (Non-Operative) Treatment
- Goal: maintain range of motion, prevent deformity
- Physiotherapy: stretching exercises, maintain abduction and internal rotation
- NSAIDs / analgesics: for pain and synovitis
- Crutches if severe pain (avoid prolonged non-weight-bearing)
- Avoid wheelchairs and prolonged bed rest (promote hip flexion-adduction deformity)
- Bracing (Petrie cast / Atlanta brace): maintains abduction-internal rotation but evidence does not support altering natural history; rarely used now
- Serial X-rays every 3-6 months to monitor stage and Herring grade
B. Surgical Containment
Indicated when conservative treatment fails to achieve containment or in high-risk groups
1. Proximal Femoral Varus Osteotomy (PFVO):
- Redirects femoral head deeper into acetabulum
- Corrects any coxa valga
- Most common surgical procedure for Perthes
- Risk: excessive varus, limb shortening, Trendelenburg gait
2. Salter Innominate Osteotomy:
- Redirects acetabulum anterolaterally to cover femoral head
- Preferred when femoral head is in good position but acetabular coverage insufficient
- Can be combined with PFVO (triple containment)
3. Combined (Femoral + Pelvic) osteotomy: for severe cases in older children
C. Salvage Procedures (for healed deformed hip)
- Shelf acetabuloplasty: extends acetabular cover over enlarged femoral head
- Chiari osteotomy: pelvic medialisation osteotomy
- Trochanteric advancement / greater trochanter epiphyseodesis: corrects high riding trochanter
- Hip distraction (arthrodiastasis): Ilizarov frame; used in late onset severe disease
- Total hip arthroplasty: in adults with painful end-stage deformity
9. Prognosis
Favourable prognostic factors:
- Young age at onset (<6 years) — more remodelling time
- Male sex (greater remodelling potential)
- Herring Group A (or B at young age)
- Spherical femoral head at healing
Poor prognostic factors:
- Age >9 years at onset
- Herring Group B/C or C
- Head-at-risk signs present
- Lateral subluxation of femoral head
- Stulberg Class III-V at skeletal maturity → premature osteoarthritis
The overall time course of the disease is 2-4 years from onset to healing.
10. Complications
| Complication | Notes |
|---|
| Coxa magna | Enlarged femoral head due to hyperaemia during revascularisation |
| Coxa plana | Flattened femoral head from collapse - most common deformity |
| Premature osteoarthritis | Result of aspherical incongruent joint; may need THA in 40s-50s |
| Hinge abduction | Extruded lateral head "hinges" against acetabular rim on abduction - causes pain |
| Leg length discrepancy | From growth disturbance of proximal femoral physis |
| Short femoral neck | With high-riding greater trochanter → abductor weakness |
| Trochanteric overgrowth | Relative lengthening → Trendelenburg gait |
Summary — High-Yield Exam Points
| Feature | Key Point |
|---|
| Disease | Self-limiting AVN of femoral head in children |
| Incidence | Boys 4:1, age 4-10 yr, peak 7 yr, 10% bilateral |
| Stages | Initial → Fragmentation → Reossification → Healing |
| Most used classification | Herring Lateral Pillar (A/B/B/C border/C) — applied in fragmentation stage |
| Outcome classification | Stulberg (I-V) — applied at skeletal maturity |
| Treatment principle | Containment — acetabulum molds femoral head |
| Age <6 | Conservative — excellent prognosis |
| Age >8 + Herring B/C or C | Surgical containment (PFVO or Salter osteotomy) |
| Worst prognostic sign | Lateral subluxation + Herring C + age >9 years |
| Long-term risk | Premature OA → THA in adult life |
Sources: Campbell's Operative Orthopaedics 15th Ed 2026 (p. 1474) | Bailey & Love's Short Practice of Surgery 28th Ed | Rosen's Emergency Medicine