LCPD or Perthes disease - idiopathic avascular necrosis of femoral head, characterized mainly in child age 4-7 years - with a feature of limping and pain in the hip or groin

1. Legg–Calvé–Perthes disease

2. Learning objective
• Anatomy of femoral head
• Pathogenesis
• Pathological process
• Clinical features
• Classification
• Prognostic
• Managment

3. Endochondral ossification
Anatomy

6. Introduction
Osteonecrosis of epiphysis femoral head
Common in 4-8 years

7. Epidemiology
• Incidence : 1:1000
• Usual age : 4-8 years
• Boys:girls – 5:1
• Higher incidence in Caucasian, Chinese,
Japanese, Inuits, Northern Europe

8. • Etiology remains unknown .
• But some of the contributing factors are ;
– Antithrombotic factors deficiencies
– hypofibrinolysis

9. • Ischemia of femoral head
Pathogenesis

10. Up to 4 months 1. Metaphyseal vessels
2. Lateral epiphyseal
3. Scanty vessels in ligamentum
teres
4-7 years 1. Lateral epiphyseal vessels
2. Metaphyseal supply DISSAPEAR
7 years 1. Vessels in ligamentum teres have
developed
Susceptible to ischemia,
as it depend entirely on
lateral epiphyseal vessel.

11. Pathology
Stage 1 – ischemia and bone death
• All/part of bony nucleus of femoral head is dead
• Cartilaginous part – remains viable and thicker
• Thickening and edema of synovium and capsule
Pathological process  3-4 years

13. Stage 2 – revascularization and repair

14. Stage 3 – Distortion and remodeling
• Repair process
- Rapid and complete :
shape is restored
- Tardy : bony collapse
and growth distortion

15. Symptoms
• Typically boy – 4-8 years
• Painless limping – continues for week or recur
intermittently
• Pain in groin, thigh and knee – activity related,
relieved by rest
• Urogenital anomaly (4% cases)
Clinical feature

16. Signs
• Hip pain with passive range of movement
• Reduced range of movement (abduction &
internal rotation)
• Hip flexion contracture
• Leg length discrepancy
• Mild muscle wasting – thigh, calf,
buttock
• Tredenlenburg test ; positive

17. • X-ray of both hip (AP & Frog lateral view)
• Bone scan
• Ultrasound – joint effusion
• CT scan – follow up
• Arthrography : to see congruity, head deformity
and determine method of treatment
• Blood inflammatory marker
- FBC
- ESR
- CRP
Investigation

18. • Widening of joint space
• Sclerosis
• Necrotic phase : increase density of ossific nucleus
• Fragmentation : alternating patches of density and
lucency
• Lateral uncovering of femoral head
• Sagging rope sign
• Acetabular remodelling
X-ray

19. Waldenström classification based on
radiographic changes
Stage 1 ( increased density)
- ossific nucleus smaller and denser
- Gage’s sign
- subchondral fracture
- radiolucencies in the metaphysis
Stage 2 (fragmentation and revascularization)
- lucency in epiphysis
- pillars are demarcated
- metaphyseal changes resolve
-acetabular contour change

20. Stage 3 (healing or reossification stage)
- new bone formation
- homogenous epiphysis
Stage 4 (remodelling)
- femoral head is reossified and remodels
- acetabular remodelling

21. Sclerosis of epiphysis and widening of joint space
in the early stages

22. V shape defect
laterally
Slight lateral
displacement of
femoral head

23. Fragmentation of the femoral capital epiphysis

24. Eight-year-old boy with right hip pain for 5 months.
(a) AP pelvis radiograph showing a smaller epiphysis due to growth arrest of the
epiphysis. Note the increased radiodensity of the epiphysis. The femoral head is in
the initial stage of LCPD.
(b) AP pelvis radiograph obtained 5 months later showing fragmented and resorptive
changes in the affected epiphysis with further flattening of the femoral head. Most
of the deformity occurs during the stage of fragmentation

25. Caffey’s sign
Subchondral fracture
in the anterolateral
aspect of the femoral
capital epiphysis
Produces crescentic
radiolucency

26. Sagging rope sign
Curvilinera sclerotic line
running horizontally across
femoral neck
In a mature hip with
Perthe’s

27. According to stage of disease –
Waldenström classification
According to prognostic outcome –
– Caterall classification
– Salter and Thompson
– Herring lateral pillar
According to defining outcome –
Stulberg classification
Classification

28. The Catterall (1982)

29. Catterall group 1

30. Catterall group 2
Note the
intact lateral
pillar

31. Catterall group 3
• involvement of
the lateral pillar as
well as the
subchondral
radiolucent zone
taken 8 months
after onset of
symptoms

32. Catterall group 4
• 14 months—
fragmentation
18 months—early
reossification
25 months—late
reossification
52 months—healed.
• Note also the
growth arrest line
and evidence of
reactivation of the
growth plate along
the femoral neck.
5225181412

33. The Herring – based on severity of structural
lateral pillar not
affected
>50% of height of
lateral pillar preserved
<50% of height of
lateral pillar preserved

34. Salter Thompson – extent of subchondral fracture
<50 % of femoral head
= Caterall I & II
>50% of fermoral head
= Caterall III & IV

35. • Child under 6 years – excellent
• Older – less good
• Female
• Femoral head involvement (head at risk signs)
Prognostic features

36. Caterall ‘head at risk’ –
Clinical
– Progressive loss of hip motion more so abduction
– Fixed flexion deformity and adduction deformities
of hip
– Obese child
– Age on higher side

37. Radiographical
– Progressive uncovering of the epiphysis
– Calcification in the cartilage lateral to ossific
nucleus
– Radiolucent area at the lateral edge of bony
epiphysis (gage ‘s sign)
– Severe metaphyseal resorption

38. • Transient synovitis
• Juvenile rheumatoid arthritis
• Sickle-cell disease
• Septic arthritis
• Morquio disease, cretinism, Gauchers’ disease
• Old perthes deformities in adult :
– Multiple epiphyseal dysplasia
Differential diagnosis

39. Perthes Transient synovitis
Average duration of symptom is 6-8 weeks days
Synovitis thickening Synovitis with capsular distension
Bony changes No bony changes
Perthes Epiphyseal dysplasia
Unilateral Bilateral
No involvement of other joints Involvement of othe joints or spine
Acetabulum not involved Involved

40. Principle
1. Prevent deformity to femoral head before
remodelling phase
2. Restore and maintain ROM
3. Concept of containment
4. Relief of symptoms
Management

41. Guidelines to treatment
• Decision are based on :
– Stage of disease
– Prognostic x-ray classification
– Age and clinical feature particularly range of
abduction and extension

42. Guidelines by Herring (1994)
– Child <6 years : symptomatic treatment
– Bone age at/or below 6 years
• Group A and B – symptomatic
• Group C – abduction brace
– Bone age over 6 years
• Group A and B – abduction brace or osteotomy
• Group C – outcome probably unnaffected by treatment
– Child 9 years and older : operative contaiment

43. Symptomatic
• Pain control
• Hospitalization for bed rest and short period
traction
• Gentle exercise to maintain movement and
regular reassessment
• Preservation of abduction, with formal
stretching

44. Containment
Harrison and Menon stated ;
‘if the head is contained within the acetabular
cup, then like jelly poured into a mold the head
should be the same as the cup when it is
allowed to come out after reconsitution ‘

45. Containment – non operative
• >60% does not require surgery
• NSAIDS
• Nightime abduction splinting
• Physiotherapy
• Monitoring Indication
- <6 years age
-Herring A and B

46. – Orthosis
– Biphosphonate
(delay resorption,
prevent collapse)
– Bone
morphogenetic
protein - 2

47. Petrie cast and A frame orthosis

48. Containment – surgical
• Done before irreversible deformation of femoral
head occurs (early in fragmentation stage )
Ideal patient  6-9 years, Caterall II-III,
good ROM,
Surgical
containment
Femoral VDRO
osteotomies
Varus 20
Derotation 20-30
Pelvic
osteotomies
Salters
osteotomies

49. Femoral varus derotational osteotomy

51. Original technique
of Pauwel
Modified Mullers
Technique
Original Mullers
technique

52. • Advantage
– Seats the head deeply in acetabulum
– Removes vulnerable portion from acetabular edge
– Decrease joint reaction forces
• Disadvantage
– Excessive varus angulation
– Limb shortening
– Non union
– Tredelenburg gait

53. Pelvic osteotomy

54. • Advantage
– Anterolateral coverage of the femoral head
– Lengthening of extremity
– Avoidance of second operation
• Disadvantage
– Increase in acetabular and hip joint pressure
– Increase in leg length on operated side

55. Summary

56. Questions
• What are the blood supply of head in child?
• What are the head at risk sign ?
• How do you classify Perthe’s ?

57. REFERENCES
• Apley ‘s System of Orthopedics and Fractures 9th Edition
• Turek’s Orthopedic, Principle and their Application, 6th
Edition.
• Lovell and Winter's Pediatric Orthopaedics, 5th Edition
• https://www.researchgate.net/publication/224929158_
Bone_Structure_Development_and_Bone_Biology_Bone
_Pathology
• https://radiologykey.com/legg-calve-perthes-disease-
pathology-pathophysiology-and-pathogenesis-of-
deformity/