common yet neglected specially in indian populations.here presenting you all details regarding perthes disease in children & treatment modalities

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2. Introduction
 Legg-Calve- Perthes disease is a self
limiting disease of hip in children
produced by osteonecrosis of the
capital femoral epiphysis
 Also known as osteochondritis that was
independently identified by AT Legg
(US) as vascular disruption, J Calve
(france) as arise from rickets, & GC
Perthes (Germany) as secondary to
degenerative arthritis in 1910

3. Other names
 Coxa plana
 Pseudocoxalgia
 Osteochondritis deformans
 Waldenstrom disease

4. Epidemiology
 Common age group is 2-12 years
 Male to female ratio 4-5: 1
 Onset of disease in indian is late
 Bilateral in 10%
 Common in central europe
 Disease is rare in african-american &
chinese people

5. Etiology
 Most probable cause is some sort of vascular disruption
due to trauma or deficient anterior vascular network
 Following factors contribute in disease:
1. Deprived populations… more common in white
2. Delayed & disproportionate growth
3. Exposure to smoke (cigarette & chulah smoke)
4. Local anatomical abnormalities (increased anteversion of
affected hip)
5. Developmental anomaly like association of inguinal
hernia, undescended testis, congenital heart disease
6. Skeletal dysplasia, genetic, malnutrition
7. Third bone child or older parents
8. Factor V Leiden deficiency

6. Stages of progression
 Waldenstrom described 4 stages
1. Stage of avascular necrosis
2. Stage of fragmentation
3. Stage of reossification or healing
4. Residual/remodelling stage

7. Pathogenesis
 Disruption of normal blood supply, &
hence nutrients and oxygen to the
femoral head is the key pathogenic
event that affects articular cartilage ,
epiphysis & in some pt. metaphysis
 This may be due to temponade effect on
retinacular vessels or transient synovitis
temporarily causing obstruction to
retinacular blood flow

8. Blood supply of femoral
head
 <3 years: 2 major arteries supplying head
metaphyseal system
retinacular system
 4-8 years : only retinacular system is
predominant blood supply as
lateral epiphyseal artery
Obliteration of posterosuperior system of
lateral epiphyseal artery is responsible for
osteonecrosis of anterolateral aspect of
femoral head
posterosuperior
posteroinferior

9. ..
 >8 years : development of foveolar
system
so dual supply again
(foveolar &
retinacular)
 16-18 yrs : growth plate disappears so
all 3
systems re-established

10. ..
 Necrotic changes r mainly observed in
deeper layer of cartilage which is
responsible for circumferential growth of
epiphysis
 Pathological Events occuring in perthes
can described as follows:

11. ..
ischemia
• Cessation of enchondral ossification at cartilage-
subchondral junction
• Separation of cartilage from bone & vascular invasion of
cartilage by accessory ossification centers
necrosis
• Bone resorption & deposition of reactive fibrous tissue
• Bony epiphysis shows necrotic changes
Biological
plasticity
• Fracture of trabeculae & thickening of it due to fracture
healing
• Deposition of fibrous tissue & formation of immature bone
are responsible for deformation of femoral head due to
biological plasticity

12. ..
 Osteonecrosis causes mechanical
weakening of femoral head so it begain
to deform under load of normal
activities.
 Microdamage in various portion results
in subchondral fracture in superior
region of epiphysis
 Which again lead to resorption &
formation

13. Morphological changes
 Small capital femoral physis
 Loss of sphericity of femoral head
 Increased width of epiphysis
 Broadening & shortening of femoral neck
 Decreased neck shaft angle
 Increased anteversion on both side
 Coxa magna
 Overgrowth of greater trochanter
 Tear of acetabular labrum
 Short stature & delayed bone age

14. Clinical features
 Limp while walking (earliest finding)
 Pain at groin region & increased by activity
 O/E limitation of internal rotation &
abduction
 Limitation of abduction in flexion is
earliest clinical sign
 Gait is antalgic
 Hernia & undescended testis should be
ruled out

15. Differential diagnosis
 Juvenile idiopathic arthritis
 Osteomyelitis
 synovitis
 Septic arthritis
 Tb hip
 hypothyroidism
 Multiple dysplasia
 Steroid induced osteonecrosis

16. Investigations
 Radiography remains mainstay in
treatment & prognosis.
 Radiological classification is used to
assess severity & prognosis
 USG & MRI is very useful but are not
standard investigations
 Arthrography is very useful & should
perform before surgery

17. Radiological signs
 Smaller sized ossific nucleus of capital femoral
epiphysis with widened joint space [earliest
radiological sign]
 Subchondral fracture (crescent sign)
radiolucent line in anterolateral region
anterolateral region is more affected due to
maximum weight bearing stresses &
supplies by posterosuperior system
 Metaphyseal cyst & expansion
 Gage sign : V shaped defect in lateral part of
epiphysis. it indicates excess cartilage growth
 Lateral subluxation
 Head within head appearance

18. Staging & Classification
 Caterall classification :
 Lateral pillar classification (lateral pillar =
lateral 15-30% of
epiphysis)
> stage A : no loss of lateral pillar
height
> stage B : less than 50% loss of
lateral
pillar height
> stage C : more than 50% loss of
height

19. Catterall classification
Stage 1 (25%
involvement)
Stage 2 (50%
involvement)
Stage 3 (75%
involvement)
Stage 4
(100%
involvement)
Affects anterior
part of femoral
head
Anterolateral or
half femoral
head affected
Affects 3/4th
femoral head
Affects entire
femoral head
No sequestrum Sequestrum
formed
Sequestrum is
large
Dense well
marked
sequestrum
No subchondral
#
Subchondral #
not extend
posterior half of
head
Subchondral #
extends to
posterior half
Subchondral #
extends to entire
head
No metaphysis Anterolateral
metaphyseal
rarefaction
Diffuse
metaphyseal
rarefaction
Central
metaphyseal
rarefaction
Epiphyseal Lateral pillar Lateral pillar not Posterior

20. Head at risk signs
Clinical signs Radiological signs
Onset of disease >8 yr. outcome is
poor
<5 yr. best
prognosis
Metaphyseal abnormalities like
osteoporosis, metaphyseal cyst &
widening of central femoral
metaphysis
Are poor prognostic factors
Girls more severely affected Altered centres of acetabulum
Extent of epiphyseal involvement &
extent of epiphyseal collapse
determine prognosis
Diffused metaphyseal reaction
Obese patient Gage’s sign
Pt. with limited range of motion Lateral extrusion of epiphysis
Increasing abduction contracture Calcification over lateral epiphysis
Subluxating hips on clinical
examination
Acetabular head index & Reimer’s migration index

21. Treatment
 Goals : to prevent deformity of femoral
head
& avoid of late onset arthritis &
improve mobility of joint
 Principles : to prevent lateral subluxation of
the head by containment,
regain a
spherical head & resumption of
weight bearing

22. ..
 Treatment done by 2 approaches
1. Containment : which ensures that anterolateral part
of epiphysis is positioned within acetabulum which
is achieved by abduction & internal rotation or
flexion by either casting or bracing but more
reliably through surgery.
 All children above 8 yr at onset of disease must
undergo containment surgery
 Good range of motion is prerequisite for achieving
containment.
2. Weight bearing or weight sharing braces : not
commonly used now a days

23. Surgical treatment
 Femoral side
1. Proximal femur varus osteotomy (20
degree)
 Pelvic osteotomy
1. Salters osteotomy
2. Shelf operation (enlarge acetabular
volume)
3. Chiari osteotomy (capsular interposional
arthoplasty) deepens deficient acetabulum
4. Trochenteric advancement

24. Collapse head

25. Sagging rope sign

26. Gage sign

27. Coxa plana

28. Coxa magna

29. Lateral pillar severly
affected

30. Case
 4 yr old girl came with c/o limping of lt
hip with pain more at knee with fever.
 no h/o trauma
 On examination there was restriction of
abduction and internal rotation
 X-ray pbh advised
 Based on x-ray as follows perthes
suspected & pt. advised for routine
follow up

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32. .

33. After 6 months

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35. After 1 year

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37. Conclusion
 Perthes is a self limiting disease with no
direct known cause but require early
diagnosis & a regular follow-up
 Extrusion of femoral epiphysis is a
troublesome complication
 More than 20% extrusion required
coverage of epiphysis by surgical
measures preferably

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