Perthes' disease is a condition caused by avascular necrosis of the femoral head in children, typically ages 5-10. It results from a temporary loss of blood supply to the hip bone. On x-rays, there are characteristic stages of necrosis, fragmentation as the bone dies and is reabsorbed, and healing as new bone forms. Classification systems exist to describe the severity and prognosis based on how much of the femoral head is involved. Mild cases affect only part of the head, while more severe cases involve most or all of the head, resulting in deformity and a poorer prognosis.

1. PERTHES ’ DISEASE
PERTHES ’ DISEASE
Dr MANNAN AHMED
Dr MANNAN AHMED

2. LEGG – CALVE – PERTHE’S
LEGG – CALVE – PERTHE’S
DISEASE
DISEASE
 First described by
First described by
Waldenstorm
Waldenstorm in 1909
in 1909
 Legg, Calve
Legg, Calve &
& Perthe’s
Perthe’s in
in
1910
1910

3. PERTHE’S DISEASE
PERTHE’S DISEASE
 Coxa plana
Coxa plana
 Pseudocoxalgia
Pseudocoxalgia
 Osteochondritis deformans coxa juvenilis
Osteochondritis deformans coxa juvenilis
 Osteochondrosis capital femoral epiphysis
Osteochondrosis capital femoral epiphysis

4. Perthe’s Disease
Perthe’s Disease is a condition which is
is a condition which is
pathologically characterized by idiopathic
pathologically characterized by idiopathic
avascular necrosis of the epiphysis of the
avascular necrosis of the epiphysis of the
femoral head in a child.
femoral head in a child.
The avascular epiphysis is almost always completely
The avascular epiphysis is almost always completely
revascularised and replaced but resulting in variable
revascularised and replaced but resulting in variable
degree of deformity of the femoral head and growth
degree of deformity of the femoral head and growth
disturbance.
disturbance.

5. EPIDEMIOLOGY
EPIDEMIOLOGY
 Common in
Common in Central Europe,
Central Europe, less common
less common in
in
blacks, Chinese & Indians
blacks, Chinese & Indians.
.
 Quite frequent in rural
Quite frequent in rural South
South Western coast
Western coast
of India.
of India.
10 times more common in Uduppi area of
10 times more common in Uduppi area of
Karnataka than Vellore in Tamil Nadu.
Karnataka than Vellore in Tamil Nadu.

6. EPIDEMIOLOGY
EPIDEMIOLOGY
 Sex: Males
Sex: Males are affected
are affected 4-5 times
4-5 times
more
more often than females.
often than females.
 Age:
Age: most commonly seen in aged
most commonly seen in aged 5 – 10
5 – 10
yrs
yrs.
.
Mean age is higher in South India.
Mean age is higher in South India.
9.9 yrs – males
9.9 yrs – males
8.7 yrs - females
8.7 yrs - females

7. ETIOLOGY
ETIOLOGY
A temporary and possibly repeated vascular
A temporary and possibly repeated vascular
insult
insult
The precise cause of insult is obscure –
The precise cause of insult is obscure –
IDIOPATHIC
IDIOPATHIC
Both
Both developmental and environmental factors
developmental and environmental factors
which make the child susceptible to disease.
which make the child susceptible to disease.
 Proposed theories
Proposed theories.
.
 Inherited protein C and/or S deficiency.
Inherited protein C and/or S deficiency.
 Venous thrombosis.
Venous thrombosis.
 Arterial occlusion / anomalies.
Arterial occlusion / anomalies.
 Raised intra osseous pressure.
Raised intra osseous pressure.
 Synovitis of hip joint.
Synovitis of hip joint.
 Generalized skeletal disorder.
Generalized skeletal disorder.

8. Pathogenesis
Pathogenesis
 Histologic changes described by 1913
Histologic changes described by 1913
 Secondary ossification center= covered by
Secondary ossification center= covered by
cartilage of 3 zones:
cartilage of 3 zones:
 Superficial
Superficial
 Epiphyseal
Epiphyseal
 Thin cartilage zone
Thin cartilage zone
 Capillaries penetrate thin zone from below
Capillaries penetrate thin zone from below

9. Pathogenesis: cartilage zones
Pathogenesis: cartilage zones

10. Pathogenesis
Pathogenesis
 Epiphyseal cartilage in LCP disease:
Epiphyseal cartilage in LCP disease:
 Superficial zone is normal but thickened
Superficial zone is normal but thickened
 Middle zone has 1)areas of extreme hypercellularity
Middle zone has 1)areas of extreme hypercellularity
in clusters and 2)areas of loose fibrocartilaginous
in clusters and 2)areas of loose fibrocartilaginous
matrix
matrix
 Superficial and middle layers nourished by
Superficial and middle layers nourished by
synovial fluid
synovial fluid
 Deep layer relies on blood supply
Deep layer relies on blood supply

11. Pathogenesis
Pathogenesis
 Physeal plate
Physeal plate: cleft formation, amorphis debris,
: cleft formation, amorphis debris,
blood extravasation
blood extravasation
 Metaphyseal region
Metaphyseal region: normal bone separated by
: normal bone separated by
cartilaginous matrix
cartilaginous matrix
 Epiphyseal changes can be seen also in greater
Epiphyseal changes can be seen also in greater
trochanter, acetabulum
trochanter, acetabulum

12. PATHOGENESIS
PATHOGENESIS
4 stages
4 stages on the basis of evolution of disease
on the basis of evolution of disease
 Stage of Avascular Necrosis
Stage of Avascular Necrosis
 Stage of Revascularization / Fragmentation
Stage of Revascularization / Fragmentation
 Stage of Ossification / Healing
Stage of Ossification / Healing
 Remodeling / Residual stage
Remodeling / Residual stage

13. PATHOGENESIS
PATHOGENESIS
Stage of Avascular Necrosis
Stage of Avascular Necrosis
Ischemia
Ischemia
A part ( anterior) or whole of capital
A part ( anterior) or whole of capital
femoral epiphysis is necrosed.
femoral epiphysis is necrosed.
On X-ray –
On X-ray –
 The ossific nucleus looks
The ossific nucleus looks smaller
smaller
 Classically of Perthes’,
Classically of Perthes’, looks
looks
dense
dense
 The
The articular cartilage remains
articular cartilage remains
viable & becomes thicker than
viable & becomes thicker than
normal
normal
–
– increased joint space.
increased joint space.

14. PATHOGENESIS
PATHOGENESIS
Stage of
Stage of REVASCULARIZATION / FRAGMENTATION
REVASCULARIZATION / FRAGMENTATION
 Ingrowths of highly vascular & cellular connective tissue.
Ingrowths of highly vascular & cellular connective tissue.
 Necrotic trabecular debris is resorbed & replaced by vascular
Necrotic trabecular debris is resorbed & replaced by vascular
fibrous tissue the alternating areas of sclerosis and
fibrous tissue the alternating areas of sclerosis and
fibrosis appear on X- ray as
fibrosis appear on X- ray as fragmentation of epiphysis
fragmentation of epiphysis.
.
 New immature bone laid on intact
New immature bone laid on intact
necrosed trabeculae by creeping
necrosed trabeculae by creeping
substitution further increases
substitution further increases
the density of ossific nucleus on
the density of ossific nucleus on
X-ray.
X-ray.

15. It is at this stage that there is
It is at this stage that there is
collapse and loss of structural
collapse and loss of structural
integrity of the femoral head as
integrity of the femoral head as
it is sort of softened due to bone
it is sort of softened due to bone
resorption, collapse of necrotic
resorption, collapse of necrotic
bone and persistence of
bone and persistence of
fibro-vascular tissue leading to
fibro-vascular tissue leading to
deformation of epiphysis.
deformation of epiphysis.
The femoral head may
The femoral head may extrude
extrude from the acetabulum
from the acetabulum
at this stage.
at this stage.
Stage of
Stage of REVASCULARIZATION / FRAGMENTATION (contd.)
REVASCULARIZATION / FRAGMENTATION (contd.)

16. PATHOGENESIS
PATHOGENESIS
Stage of Ossification / Healing
Stage of Ossification / Healing
New bone starts forming
New bone starts forming
and epiphyseal density
and epiphyseal density
increases in the lucent
increases in the lucent
portions of the femoral head.
portions of the femoral head.

17. PATHOGENESIS
PATHOGENESIS
 Remodeling / Residual stage
Remodeling / Residual stage
This is the stage of remodeling and there is no
This is the stage of remodeling and there is no
additional change in the density of the femoral
additional change in the density of the femoral
head.
head.
Depending on the severity of the disease the
Depending on the severity of the disease the
residual shape of the head may be spherical
residual shape of the head may be spherical
or distorted.
or distorted.

18. CLINICAL PICTURE
CLINICAL PICTURE
 Typically a boy, 5-10 years old.
Typically a boy, 5-10 years old.
 Characteristic presentation is a
Characteristic presentation is a painless limp.
painless limp.
 May present with limp along with pain.
May present with limp along with pain.
 The child appears to be
The child appears to be well
well & not sick.
& not sick.
 The hip looks to be deceptively normal – there may be
The hip looks to be deceptively normal – there may be
little wasting.
little wasting.
 Abduction & Internal rotation are nearly always limited.
Abduction & Internal rotation are nearly always limited.
 Antalgic gait in the irritable phase or Trendelenburg gait.
Antalgic gait in the irritable phase or Trendelenburg gait.

19. DIFFERENTIAL DIAGNOSIS
DIFFERENTIAL DIAGNOSIS
Unilateral
Unilateral
 Tuberculosis hip
Tuberculosis hip
 Synovitis
Synovitis
 Slipped femoral capital
Slipped femoral capital
epiphysis
epiphysis
 Lymphoma
Lymphoma
 Eosinophilic granuloma
Eosinophilic granuloma
Bilateral
Bilateral
 Hypothyroidism
Hypothyroidism
 Multiple epiphyseal
Multiple epiphyseal
dysplasia
dysplasia
 Spondyloepiphyseal
Spondyloepiphyseal
dysplasia
dysplasia
 Sickle cell disease
Sickle cell disease

20. IMAGING STUDIES
IMAGING STUDIES
Perthe’s disease is suspected clinically but
Perthe’s disease is suspected clinically but
diagnosis rests on plain X-rays.
diagnosis rests on plain X-rays.
Pelvis with both hips – AP view
Pelvis with both hips – AP view
Frog leg Lateral view of the hip
Frog leg Lateral view of the hip

21. IMAGING STUDIES
IMAGING STUDIES
 Stages of Avascular necrosis, Fragmentation, Ossification & Residual
Stages of Avascular necrosis, Fragmentation, Ossification & Residual
stage.
stage.
 Other radiological changes
Other radiological changes
Metaphyseal changes –
Metaphyseal changes –
Hyperemia & osteoporosis
Hyperemia & osteoporosis
Cystic changes – poor prognosis
Cystic changes – poor prognosis
Changes in physis –
Changes in physis –
Abnormal growth and premature
Abnormal growth and premature
closure leading to short & wide neck.
closure leading to short & wide neck.
Greater Trochanter –
Greater Trochanter –
Elevated proximally d/to retardation
Elevated proximally d/to retardation
of the longitudinal growth of femoral
of the longitudinal growth of femoral
neck – abductor insufficiency.
neck – abductor insufficiency.

22. IMAGING STUDIES
IMAGING STUDIES
Sagging rope sign
Sagging rope sign
 A rope like radiodense line overlying the proximal
A rope like radiodense line overlying the proximal
femoral metaphysis.( intertrochantric area)
femoral metaphysis.( intertrochantric area)
 Is infact the anterior portion of the overlarge femoral
Is infact the anterior portion of the overlarge femoral
head as it projects on a shortened and wide proximal
head as it projects on a shortened and wide proximal
femoral metaphysis.
femoral metaphysis.

23. IMAGING STUDIES
IMAGING STUDIES
Hinge abduction
Hinge abduction
 The Articular surface of the head and acetabulum are
The Articular surface of the head and acetabulum are
not concentric.
not concentric.
 The
The femoral head hinges
femoral head hinges at the acetabulum when limb is
at the acetabulum when limb is
abducted – the medial joint space is increased.
abducted – the medial joint space is increased.
 Best diagnosed on arthrography.
Best diagnosed on arthrography.

24. IMAGING STUDIES
IMAGING STUDIES
Head In Head Sign

25. RADIOGRAPHIC CLASSIFICATION
RADIOGRAPHIC CLASSIFICATION
 Radiographic picture varies with the stage and
Radiographic picture varies with the stage and
severity of the disease.
severity of the disease.
 Number of classification systems have been
Number of classification systems have been
developed to estimate the severity of the disease
developed to estimate the severity of the disease
based on the radiographic findings .
based on the radiographic findings .
Catterall,
Catterall,
Salter and Thompson,
Salter and Thompson,
Herring
Herring

26. CATTERALL CLASSIFICATION
CATTERALL CLASSIFICATION
 Catterall Group I
Catterall Group I:
: anterior
anterior portion of
portion of epiphysis.
epiphysis.
no collapse.
no collapse.
 Catterall Group II
Catterall Group II: Anterior segment (<50 %).
: Anterior segment (<50 %).
Central
Central segment fragmentation & collapse.
segment fragmentation & collapse.
The lateral weight bearing segment intact .
The lateral weight bearing segment intact .
 Catterall Group III
Catterall Group III: Most of the nucleus is involved.
: Most of the nucleus is involved.
Only a small posterior segment viable.
Only a small posterior segment viable.
Fragmen. & collapse including lateral part.
Fragmen. & collapse including lateral part.
Metaphyseal resorption.
Metaphyseal resorption.
 Catterall Group IV
Catterall Group IV: The
: The entire head
entire head is involved.
is involved.

27. Catterall I Catterall II
GROUP I & II Have A Good Prognosis
GROUP I & II Have A Good Prognosis

28. Groups III and IV have a poor prognosis
Groups III and IV have a poor prognosis
Catterall IV
Catterall III

29. SALTER AND THOMPSON
SALTER AND THOMPSON
 A more simple classification.
A more simple classification.
 Recognized that Catterall first two groups & next
Recognized that Catterall first two groups & next
two groups are distinct with a different prognosis.
two groups are distinct with a different prognosis.
Group A
Group A: < 1/2 head involved
: < 1/2 head involved
favorable prognosis
favorable prognosis
Group B
Group B: > 1/2 head involved
: > 1/2 head involved
unfavorable prognosis
unfavorable prognosis

30. HERRING LATERAL PILLAR CLASSIFICATION
HERRING LATERAL PILLAR CLASSIFICATION
Lays importance on the structural integrity of
Lays importance on the structural integrity of
superolateral – the principal load bearing part of the head.
superolateral – the principal load bearing part of the head.
 Lateral Pillar Group A:
Lateral Pillar Group A: no loss in height of the lateral pillar
no loss in height of the lateral pillar
minimal density change.
minimal density change.
 Lateral Pillar Group B:
Lateral Pillar Group B: There is lucency & < 50% loss of
There is lucency & < 50% loss of
height in the lateral pillar.
height in the lateral pillar.
 Lateral Pillar Group C:
Lateral Pillar Group C: There is > 50% loss in the height of
There is > 50% loss in the height of
the lateral pillar, severe collapse.
the lateral pillar, severe collapse.
Outcome relates strongly to the integrity of the lateral pillar
Group A faring the best & Group C the worst prognosis

31. HERRING LATERAL PILLAR CLASSIFICATION
Group A
Group A Group C
Group C

32. MANAGEMENT
MANAGEMENT
No general agreement on the “correct” course
No general agreement on the “correct” course
of treatment for all cases.
of treatment for all cases.
Aims of treatment :
Aims of treatment :
 Primary aim
Primary aim is to prevent deformation of the
is to prevent deformation of the
femoral head.
femoral head.
 Prevention of stiffness and maintenance of
Prevention of stiffness and maintenance of
good range of movements.
good range of movements.
 Prevent or correct growth disturbances-
Prevent or correct growth disturbances-
greater trochanteric overgrowth
greater trochanteric overgrowth

33. MANAGEMENT
MANAGEMENT
Main cause of deformation
Main cause of deformation -
- extrusion
extrusion of the
of the
femoral head.
femoral head.
The
The treatment
treatment when needed is to try to prevent this
when needed is to try to prevent this
deformation .
deformation .
Containment
Containment of the femoral head within the acetabulum.
of the femoral head within the acetabulum.
The socket, thus, acts as a mould to keep the head
The socket, thus, acts as a mould to keep the head
spherical while still it is in the softened state.
spherical while still it is in the softened state.

34. MANAGEMENT
MANAGEMENT
 Essential that intervention to prevent
Essential that intervention to prevent
deformation of head is instituted before this
deformation of head is instituted before this
complication develops / any irreparable
complication develops / any irreparable
deformation
deformation
 When does deformation occur & till when
When does deformation occur & till when
is it reversible ?
is it reversible ?
Deformation occurs during the phase of
Deformation occurs during the phase of
revascularization (fragmentation) & early
revascularization (fragmentation) & early
regeneration (ossification).
regeneration (ossification).
It would therefore follow that if the containment is
It would therefore follow that if the containment is
to succeed, it would need to be performed
to succeed, it would need to be performed
before the late phase of fragmentation, i.e., in
before the late phase of fragmentation, i.e., in
stages of AVN or early fragmentation.
stages of AVN or early fragmentation.

35.  How long containment?
How long containment?
Needs to be ensured until the healing process
Needs to be ensured until the healing process
and beyond the stage where epiphysis is
and beyond the stage where epiphysis is
vulnerable to deformation that is until the late
vulnerable to deformation that is until the late
stage of
stage of regeneration phase ( 2 yrs)
regeneration phase ( 2 yrs)

36.  Symptomatic treatment
Symptomatic treatment
 CONTAINMENT OF HEAD
CONTAINMENT OF HEAD
(a) Conservative methods
(a) Conservative methods
(b) Surgical methods
(b) Surgical methods
Management
Management

37. CONSERVATIVE METHODS
CONSERVATIVE METHODS
Weight relief & rest
Weight relief & rest
In the past, treatment was primarily directed at avoiding
In the past, treatment was primarily directed at avoiding
weight by bed rest for prolonged period (up to 2 yrs) or
weight by bed rest for prolonged period (up to 2 yrs) or
weight relieving calipers to prevent head deformation.
weight relieving calipers to prevent head deformation.
Little evidence for efficacy.
Little evidence for efficacy.
Containment by bracing & casting
Containment by bracing & casting
Plaster cast in abd. & internal rotation – broomstick casts
Plaster cast in abd. & internal rotation – broomstick casts
Braces to keep hip in desired position.
Braces to keep hip in desired position.
Weight bearing is allowed in braces.
Weight bearing is allowed in braces.
Casts - temporary form of containment till definitive
Casts - temporary form of containment till definitive
treatment undertaken.
treatment undertaken.

38. HIP ABDUCTION BRACE / CASTS
HIP ABDUCTION BRACE / CASTS
Broom stick casts
Scottish Rite orthosis

39. BROOMSTICK CASTS
BROOMSTICK CASTS

40. SURGICAL METHODS
SURGICAL METHODS
Femoral osteotomy
Femoral osteotomy – S/T or I/T.
– S/T or I/T.
Innominate osteotomy
Innominate osteotomy –
– Anterolateral coverage
Anterolateral coverage
Operative reconstruction provides the advantage of
Operative reconstruction provides the advantage of
improved containment & early mobilization and is a
improved containment & early mobilization and is a
preferred method.
preferred method.
No end point for discontinuing the treatment because the
No end point for discontinuing the treatment because the
improved containment is permanent.
improved containment is permanent.
Short term studies suggest an improvement in the natural
Short term studies suggest an improvement in the natural
course of the disease process with femoral osteotomy.
course of the disease process with femoral osteotomy.
(Salter’s )

41. FEMORAL OSTEOTOMY
FEMORAL OSTEOTOMY
 Technically less demanding than innominate osteotomy
Technically less demanding than innominate osteotomy
 Usually 20
Usually 200
0
varus angulation & 20
varus angulation & 200
0
IR appears sufficient.
IR appears sufficient.
 Good to decide abduction, internal rotation or flexion on a
Good to decide abduction, internal rotation or flexion on a
pre-operative arthrogram.
pre-operative arthrogram.

42. FEMORAL OSTEOTOMY
FEMORAL OSTEOTOMY
 Up to 12 years of age an
Up to 12 years of age an open wedge osteotomy
open wedge osteotomy
may be performed without the risk of delayed union /
may be performed without the risk of delayed union /
non-union.
non-union.
Also the amount of shortening is minimized.
Also the amount of shortening is minimized.
 Pre-requisites – near normal hip movements.
Pre-requisites – near normal hip movements.

43. PELVIC OSTEOTOMY
PELVIC OSTEOTOMY
 Redirectional Osteotomy
Redirectional Osteotomy
 Salter’s osteotomy to
Salter’s osteotomy to
reorient the acetabulum
reorient the acetabulum
 Shelf Operation
Shelf Operation
 To create a bony shelf to
To create a bony shelf to
cover the extruded part of
cover the extruded part of
the epiphysis.
the epiphysis.
 Displacement Osteotomy
Displacement Osteotomy
 Chiari osteotomy is
Chiari osteotomy is
another way to improve
another way to improve
the coverage
the coverage.
.

44. Guidelines To Specific Treatment :
Guidelines To Specific Treatment :
Present trend
Present trend
Favorable
Favorable outcome
outcome
< ½ head affected
< ½ head affected
with no extrusion
with no extrusion
(Catterall I & II, Herring A,B)
(Catterall I & II, Herring A,B)
Unfavorable outcome
Unfavorable outcome
whole head affected
whole head affected
with some lateral extrusion
with some lateral extrusion
(Catterall III & IV, Herring C,
(Catterall III & IV, Herring C,
Head at risk signs)
Head at risk signs)
<7 year > 7 year
• Containment with braces
• Periodic review
Surgical
containment
• No specific treatment
No specific treatment other
other
than symptomatic treatment.
than symptomatic treatment.
• Require a periodic
Require a periodic
radiological review.
radiological review.
Grade the patients acc. to likely outcome, of the shape of the femoral
Grade the patients acc. to likely outcome, of the shape of the femoral
head - determined by radiographic features in the early stage
head - determined by radiographic features in the early stage

45. TREATMENT
TREATMENT
Reconstructive procedures
Reconstructive procedures
 Valgus extension osteotomy
Valgus extension osteotomy
indication -hinge abduction of hip
indication -hinge abduction of hip
 Cheilectomy
Cheilectomy
indication – malformed femoral head with lateral
indication – malformed femoral head with lateral
protuberance Coxa plana
protuberance Coxa plana
 Chiari osteotomy
Chiari osteotomy
indication – malformed femoral head with lateral
indication – malformed femoral head with lateral
subluxation
subluxation
 Trochanteric advancement
Trochanteric advancement
indication – premature capital femoral physeal arrest
indication – premature capital femoral physeal arrest
 Greater trochanteric epiphysiodesis
Greater trochanteric epiphysiodesis
indication – premature capital femoral physeal arrest
indication – premature capital femoral physeal arrest
 Shelf augmentation procedure
Shelf augmentation procedure
indication – coxa magna coxa magna & lack of acetabular
indication – coxa magna coxa magna & lack of acetabular
coverage
coverage

46. PROGNOSTIC FACTORS
PROGNOSTIC FACTORS
 Age of the child at presentation.
Age of the child at presentation.
 Sex : girls have poor prognosis.
Sex : girls have poor prognosis.
 Extent of epiphyseal involvement.
Extent of epiphyseal involvement.
 Range of movement at the hip.
Range of movement at the hip.
 Presence of epiphyseal extrusion – most
Presence of epiphyseal extrusion – most
important factor influencing outcome.
important factor influencing outcome.
 Metaphyseal translucencies.
Metaphyseal translucencies.
 Head at risk signs.
Head at risk signs.

47. “
“HEAD AT RISK SIGNS”
HEAD AT RISK SIGNS”
 Gage's sign
Gage's sign :-
:- a V shaped lucency in the lateral epiphysis.
a V shaped lucency in the lateral epiphysis.
 Lateral calcification (lateral to the epiphysis) (implies loss of
Lateral calcification (lateral to the epiphysis) (implies loss of
lateral support)
lateral support)
 Lateral subluxation of the head. (implies loss of lateral support)
Lateral subluxation of the head. (implies loss of lateral support)
 A horizontal growth plate. (implies a growth arrest
A horizontal growth plate. (implies a growth arrest
phenomenon and deformity)
phenomenon and deformity)

48. Lat subluxation / Calcification lat
Lat subluxation / Calcification lat
to epipiphysis – HEAD AT RISK
to epipiphysis – HEAD AT RISK

49. GAGE
GAGE`
`S SIGN
S SIGN

50. Thank You
Thank You