Legg-Calve-Perthes disease is a condition characterized by osteonecrosis of the femoral head typically seen in children between the ages of 4-8 years old. It results from disrupted blood supply to the femoral capital epiphysis, leading to bone death and deformity of the femoral head over time. Treatment involves relieving pain, protecting weight bearing, restoring range of motion, and minimizing femoral head deformity. Younger children with milder involvement are often treated conservatively with bracing and activity restriction while older children or those with more severe involvement may require surgical procedures like varus derotation osteotomy to improve containment of the femoral head within the acetabulum. The goals of treatment are to improve mobility

1. LEGG-CALVE PERTHES
DISEASE
PRESENTER : DR. BIJAY MEHTA
MODERATOR : ASSO. PROF. DR. RISHI BISTA

2. CONTENTS
History and definition
Incidence
Etiopathogenesis
Clinical Features
Radiological Features
Classification
Differential Diagnoses
Treatment
Summary

3. Synonyms
Perthes Disease
Pseudo-coxalgia
Osteochondritis deformans
Coxa-juveniles

4. Definition
 A self limiting condition characterized by :
disruption of blood supply of the femoral capital
epiphysis
resulting in epiphyseal osteonecrosis and
chondronecrosis
with cessation of growth of the epiphysis.
Results in deformed femoral head
Coxa plana
Coxa magna

5. HISTORY
 110 years old disease
Identified as a separate entity in 1910
Independently identified by :
Arthur Legg
Jacques Calve
Georg Perthes
Henning Waldenstrom

6. Incidence
Incidence- Approx. 1 in 1000 children
Age Group: Can be seen from 18 months – skeletal maturity
But common between 4-8 years - Why??
Male to Female : Roughly 4:1
Bilateral in 10-12% of cases
More common in female
Metachronous

7. Etiology
 Coagulation Disorders –Deficiency of Protein C and S
Delayed Bone age , Systemic abnormalities of growth and development
Hyperactivity/ADHD
Low Birth Weight
Hereditary Influences
Type II collagenopathy
Environmental influences- socioeconomic status , smoking

8. Pathophysiology

9. Pathophysiology

11. Clinical Presentation : Symptoms
 Insidious onset
Limp- usually painless
Usually deteriorates after physical activity and relieved by rest
Pain
Anterior hip pain
Groin
Around GT
Knee

12. Clinical Presentation
 Family history
Blood coagulation disorder
Use of steroid medication
History of trauma

13.  Varies according to stage of disease
Limp- Combination of antalgic and Trendelenburg gait
Trendelenburg sign- may be present
Atrophy of thigh muscles
ROM-
Loss of Internal Rotation –the earliest sign
Abduction-almost always restricted
Flexion-least affected
Clinical Presentation : Signs

14. Radiographic Features
Vary according to stage of disease
Seen after 3-6 months
Medial joint space widening- Earliest
Cartilage thickening , joint effusion
Lateral subluxation of femoral head

15. Radiographic Features
Subchondral Fracture –Crescent Sign
Horizontal physis
Early closure of acetabulum
Bicompartmentalization of acetabulum
Ischim Varum

16. Waldenstrom staging based on
radiographic features
Four Stages
Initial Stage
Fragmentation Stage
Reossification or Healing stage
Remodelling or Healed Stage
Waldenstrom Staging of the Disease

17. Waldenstrom Staging of the Disease
Stage I : Initial Stage-3-6 months
Clinically silent
Small Ossific nucleus
Crescent Sign, Metaphyseal cyst
Medial joint space widening
Stage II: Fragmentation Stage—6-12 months
a/w clinical symptoms
Necrotic bone resorbed and replaced by fibrous tissue
Alternating area of sclerosis and fibrosis
Head collapse starts

18. Waldenstrom Staging of the Disease
Stage III : Reossification Stage-12-18 months
Reossification starts peripherally and progresses centrally
Epiphysis becomes homogenous in density
Anterocentral region last to reossify
Stage IV: Remodelling Stage- upto skeletal maturity
Ossific nucleus completely reossified
Trabecular pattern reformed
Flattened femoral head remains

19. Modified Elizabethtown Classification

20. Classification Systems
For Disorder Severity
Catterall Classification
Lateral Pillar Classification
Salter Thompson Classification
For End Result Classification
Stulberg classification
Moses Classification

21. Catterall Classification
Group I
<25% of epiphysis involved
Only anterior/anterolateral portion of
epiphysis involved
No Collapse/No sequestrum
Group II- 25-50%
Anterior half/3rd –involved
Central sequestrum
Subchondral fracture in anterior half

22. Catterall Classification
Group III 50-75%
50-75% of epiphysis involved
Posterior subchondral fracture line
Group IV
100% of epiphysis involved
Diffuse metaphyseal involvement

23. Head at Risk Signs
 Lateral Subluxation of Femoral Head
Gage Sign
Speckled Calcification lateral
epiphysis
A Horizontal Physis
Metaphyseal Cyst Formation

24. Lateral Pillar Classification(Herring’s)
 Based on radiographic changes in lateral pillar
Why lateral pillar only??

25. Salter Thompson Classification
 Based on radiographic crescent sign
Class A-Crescent Sign -<1/2 of femoral head
Class B Crescent sign->1/2 of femoral head

26. Stulberg Classification


27. Investigations
 X-Ray
MRI
Ultrasonogram
Arthrogram
Bone Scan

28. MRI
Best for Early Diagnosis
For accurate visualization of femoral head and acetabulum
Mandatory before surgery : To look for exact degree of extrusion and
uncoverage
Perfusion and diffusion MRI- useful prognosis

29. Bone Scan
 Sensitive for early diagnosis
Sometimes overestimates the severity
Arthrogram
 Clearly shows the femoral head configuration and containment –best for
hinged abduction
Can assess hip congruity in various positions
Can assess in which position head best contains

30. Differential Diagnosis
 Transient Synovitis
Epiphyseal Dysplasia
Tuberculosis
Chondroblastoma
Other causes of osteonecrosis of femoral head

31. Prognostic Factors
 Age
Shape of Lateral pillar
Subluxation
Mobility-Hip ROM
Extent of Necrosis

32. Treatment : Goals
Relief of pain : NSAIDs/Bed Rest
Avoid weight bearing
Restore ROM
Minimize femoral head deformity at the completion of healing
Can be
Conservative
Operative

33. Treatment :Conservative
Reserved for
Younger children(usually<6 years)
With Herring A or B hips
Includes:
Protected Weight bearing
Activity restriction
Physiotherapy
Abduction Braces

34. Treatment :Operative
Indicated for
Children usually>6 years with Herring B hips
All children with Herring B/C or C hips
Prerequisites :
Near normal abduction
Arthrogram showing containable congruent hip.
Includes:
<8 years- Proximal femoral varus osteotomy
> 8 years- pelvic osteotomy

35. Treatment : Approach
3 Distinct Time Frames
Early in the course of the disease
Late in the course of the disease
After healing (Sequelae)

36. Early Treatment
Goals :
Improved Mobility
Weight Relief
Improved Containment

37. Improving Mobility
 Extremely important
For joint function
Prerequisite for containment
Methods :
Traction
Physiotherapy
Petrie cast

38. Containment
 Biological plasticity
Like jelly/icecream mold
When to contain??
<6 years – consider containment only if extrusion occurs
6-12 years – consider containment even before extrusion occurs
>12 – do not consider containment
Do not consider containment if hip is stiff

39. Containment : Methods
 Conservative:
Abduction Braces
Petrie Casts
Surgical
Varus Derotation Osteotomies
Innominate Osteotomy(Salter Osteotomy)
Shelf Acetabuloplasty
Chiari Osteotomy
Triple Pelvic Osteotomy

40. Varus Derotation Osteotomy
 Advantages:
Prevents deformity of the femoral
head by preventing extrusion
Accelerates healing
Disadvantages
Residual shortening may be
present
Abductor Limp
Trochanteric prominence

41. Treatment : Late Phase
Goal :
To minimize the extent of femoral head deformation that has already occurred
due to extrusion
Treatment Options :
Remedial
Salvage Surgery
Problem in late phase : Hinged Abduction
Treatment : Valgus osteotomy

42. Treatment after healing (Sequelae)
Goal
Improve function
Relieve Pain
Delay onset of OA
Treatment approach will depend upon specific cause of pain or disability

43. Pathoanatomy of Healed Perthes Disease
Femoroacetabular Impingement(FAI)- Osteoplasty
GT Overgrowth
Acetabular Dysplasia

44. Medical Management
 Bisphosphonates- have been tried but poor results due to poor vascularity
Local bisphosphonates –under trial
Bone Morphogenic Proteins(BMPs)- researches going on

45. TAKE HOME MESSAGE
 Perthes disease is a idiopathic self limiting disease.
Usually presents with a painless limp.
Careful history and examination is necessary to rule out other conditions.
Although Xray is sufficient for diagnosis, MRI and bone scan are important for
early diagnosis and management.
Treatment depends on stage of disease.
Containment of the femoral head in the acetabulum is the mainstay of treatment.

46. REFERENCES :
 Tachdjian’s Pediatric Orthopaedics , 6th Edition
Campbell’s Operative Orthopaedics , 13th Edition
Apley and Solomon’s System of Orthopaedics , 10th Edition
Orthobullets
Benjamin Joseph, Charles T. Price, Principles of Containment Treatment
Aimed at Preventing Femoral Head Deformation in Perthes Disease,Orthopedic
Clinics of North America, Volume 42, Issue 3,2011,Pages 317-327.

47. Thank You