Perthes disease is avascular necrosis of the femoral head in children caused by interrupted blood supply. It typically affects children ages 4-8 years old. Presentation includes limping and hip pain. Treatment depends on the stage and aims to contain the femoral head through casting, bracing, or surgery. Containment redirects forces on the femoral head to allow remodeling. Late treatment focuses on improving range of motion and reshaping deformities through osteotomies or salvage surgery. The long term goal is to produce a normal hip joint and prevent arthritis.

1. PERTHES DISEASE
DR RITESH JAISWAL
M.B.B.S D.Ortho DNB (Ortho) M.N.A.M.S M.Ch (Ortho)
Fellowship in Joint Replacement ( Mumbai )
Fellow AO Trauma ( Switzerland )

2. INTRODUCTION
ETIOLOGY
PATHOLOGY
CLINICAL PRESENTATION
CLASSIFICATION
DIFFERENTIAL DIAGNOSIS
MANAGEMENT

3. INTRODUCTION
- Noninflammatory selflimiting idiopathic
avascular necrosis of the femoral head in a
growing child caused by interruption of its blood
supply in proximal femoral epiphysis.
- SYNONYMS: Coxa plana
Osteochondritis deformans juvenilis
Pseudocoxalgia
Osteochondrosis of hip
Coronary disease of hip.

4. • Arthur Legg ( US )
• Jaque Calvé ( France )
Georg Perthes (Germany )

5. - Incidence - 1 in 10,000 children
- 4-8 years is most common age of presentation
- Male to female ratio is 5:1
( Intracapsular arterial ring has been found to be
incomplete more often in males )
- Higher among lower socioeconomic class
- Higher incidence in high latitude
- Caucasian > East Asian and African
American
- In India it is most prevalent in west coast
especially in udupi district.

6. ETIOLOGY
Exact etiology is Unknown
Many factors related to etiology of this disease have been
mentioned -
- Coagulation disorder
- Arterial status of femoral head
- Abnormal venous drainage
- Abnormal growth and development
- Trauma
- Hyperactivity or attention deficit disorder
- Genetic Component
- Enviornmental factors
- Sequel of Synovitis

7. Coagulation disorder
( Protein C or S deficiency, Thrombophilia,
Increased lipoprotein A, Hypofibrinolysis
Factor 5 leidin mutation )
Arterial status of femoral head
Angiographic studies showed obstruction of
superior capsular arteries and decreased flow
in MCFA in perthes disease

8. Abnormal Venous drainage
Venous drainage normally flows through MCFV
In perthes patients , there is increased venous
pressure in femoral neck and associated
congestion in the metaphysis and venous
outflow obstruction has been found
Abnormal growth and development
Delay in bone age 1.5 to 2 years in children with
perthes disease

9. Other aetiological and associated factors but
their exact roles remain unclear:
1. Trauma, hyperactivity and attention deficit
disorder,
2. Susceptibility (abnormal growth and
development):
a. Low birth weight
b. Low socio-economic class
c. Bone maturation delays
d. Boys > girls (4/1)
3. Hereditary and familial factors
4. Passive smoking
5. Transient synovitis.

11. HYPOTHESIS FOR DEVELOPING AVN
OF FEMORAL HEAD IN PERTHES
TRUETA’S HYPOTHESIS
- Postulates that solitary blood supply during 4-8
yrs makes vulnerable for AVN of head.
- Retinacular vessel enters as lateral epiphyseal
artery which gets compressed by lateral
rotator muscles
CAFFEY’S HYPOTHESIS
Intraepiphyseal compression of Blood supply leads
to osteonecrosis

12. PATHOLOGY

13. PATHOLOGY

18. CLINICAL PRESENTATION
Symptoms
- Insidious onset
- may cause painless limp
- Intermittent knee, hip, groin or thigh
pain
Physical exam
- Hip stiffness with loss of internal
rotation and abduction
- Gait disturbance
- Trendelenburg gait (head collapse leads
to decreased tension of abductors)
- Antalgic limp
- Limb length discrepancy is a late finding
( hip contracture can exacerbate the
apparent LLD )

19. - ACCORDING TO STAGE OF DISEASE
WALDERSTORM CLASSIFICATION
- ACCORDING TO PROGNOSIS
CATTERALL
SALTER AND THOMPSON
HERRING LATERAL PILLAR
- ACCORDING TO OUTCOME
STULBERG CLASSIFICATION
MOSE CLASSIFICATION

20. WALDENSTROM STAGES
based on radiographic changes

21. MODIFIED WALDENSTROM

23. MODIFIED ELIZABETHTOWN
CLASSIFICATION
• Stage I a : Part or
whole of the
epiphysis is sclerotic.
There is no loss of
height of the
epiphysis.
• Stage I b : The
epiphysis is sclerotic
and there is loss of
epiphyseal height.
There is no evidence
of fragmentation of
the epiphysis.

24. Stage II a
sclerotic epiphysis
just begun to fragment.
One or two vertical fissures
are seen in either AP/ lateral
view
Stage II b
Fragmentation is advanced.
No new bone is visible
lateral to the fragmented
epiphysis.

25. Stage IIIa :
Early new bone
formation is visible on
the periphery of the
necrotic epiphysis.
The texture of the
new bone is not
normal; it is “porotic”
and covers less than
a third of the width
of the epiphysis.

26. Stage III b : The new
bone is of normal
texture and has
grown over a third
of the width of the
epiphysis.
Stage IV : Healing is
complete and there is
no radiologically
identifiable avascular
bone.

27. CLASSIFICATIONS
Based on Radiographs to assess the extent of
involvement.
HERRING LATERAL PILLAR
CATTERALL
SALTER-THOMPSON

28. LATERAL PILLAR CLASSIFICATION
(Herring et.al)
This is based on the integrity of the lateral pillar on the AP
radiograph only, at the beginning of the fragmentation
phase ( 6mths after onset of symptoms )

29. CATTERALL CLASSIFICATION
Catterall proposed four groups based on the extent of
head involvement at the fragmentation phase

30. HEAD AT RISK SIGNS
Clinical head at risk signs
1) Age - > 8yrs
- younger age, more time to remodel defect
- Late age , less potential to develop acetabulum
- More age with increase body wt likely to damage
epiphysis
2) Female sex- Girls of same age of boys are more skeletally
mature
3) Obesity
4) Limitation of ROM
5) Adduction contracture
6) Subluxating hips

31. CATTERALL ( RADIOLOGICAL )
HEAD AT RISK SIGNS

33. SCINTIGRAPHIC head at risk signs
1) Failure of revascularization of lateral column
2) Decreased activity of physis
3) Anterolateral extrusion of epiphysis
4) Disappearance of previously present lateral
column
5) Intense metaphyseal activity

34. SALTER- THOMPSON CLASSIFICATION
Based on radiographic crescent sign
Class A - crescent sign involves < 1/2 of femoral
head
Class B - crescent sign involves > 1/2 of femoral
head

35. STULBERG CLASSIFICATION
- Gold standard for rating residual femoral head
deformity and joint congruence
- Recent studies show poor interobserver and
intraobserver reliability

38. MOSE CLASSIFICATION
Uses a concentric circle technique to compare
and classify the final outcome in LCPD at the end
of growth.
The final shape of the head is compared with a
perfect circle using a MOSE TEMPLATE on both
AP and lateral images

40. DIFFERENTIAL DIAGNOSIS
UNILATERAL LCPD
1. Septic arthritis (usually the child is unwell, with a fever and
elevated inflammatory markers)
2. Sickle cell disease (history, sickling test, Hb electrophoresis)
3. Eosinophilic granuloma (other lesions in the skull,
radiological features, biopsy)
4. Transient synovitis (lack of characteristic radiographic
changes).

41. BILATERAL LCPD
uncommon and requires a skeletal survey and blood tests to
exclude:
1. Hypothyroidism (thyroid function test).
2. Multiple epiphyseal dysplasia (usually bilateral
simultaneously, with involvement from other joints
epiphyses).
3. Spondyloepiphyseal dysplasia (involvement of the spine).
4. Meyer’s dysplasia delayed, irregular ossification of the femoral
epiphyseal nucleus. This is more common in boys, usually occurs in
the second year of life, is mostly bilateral and usually disappears
by the end of the sixth year. Bone scans are normal.
5. Sickle cell disease.
6. Mucopolysaccharidoses.

42. MANAGEMENT
• INVESTIGATIONS
• TREATMENT

43. INVESTIGATIONS
PLAIN RADIOGRAPHS (AP of pelvis and frog leg
laterals)
- Critical in diagnosis and prognosis
- Early findings include
- Medial joint space widening (earliest)
- Irregularity of femoral head ossification
- Crescent sign (represents a subchondral
fracture)

44. AP view Frog View

45. Stage of synovitis increase joint space
Stage of AVN Necrotic Bone
Crescent / Caffey’s/ Salter’s Sign

46. Changes in Metaphysis
- Holes of necrosis due to metaphyseal necrosis
- Metaphyseal cysts – cystic changes due to tongue of
fibrillated cartilage stretching deep into neck.
- Sagging Rope Sign
Radiodense line overlying proximal femoral metaphysis
Produced by growth plate damage associated with
metaphyseal response

47. BONE SCAN
- Decreased uptake (cold lesion)
* A bone scan may be helpful in the early
stages of the disease, when the diagnosis is
in question, particularly if the differential
diagnosis is between transient synovitis and
LCPD

48. CONTRAST ENHANCED MRI ( most accurate 97-99% )
- Revealing alterations in the capital femoral
epiphysis and physis
- more sensitive than radiograph
- perfusion studies predict maximum extent of
lateral pillar involvement
ARTHROGRAM
- Dynamic arthrogram can demonstrate
coverage and containment of the femoral head

49. BLOOD TESTS
- helpful in ruling out other conditions like
Hypothyroidism, Sickle cell disease etc
HISTOLOGY
- Femoral epiphysis and physis exhibit areas of
disorganized cartilage with areas hypercellularity
and fibrillation

50. TREATMENT
OBJECTIVES
- To Produce a normal Femoral head and neck
- To Produce a normal Acetabulum
- Fully mobile congruous hip joint
- To Prevent hip arthritis in later life

51. Goals :
Treatment efforts are directed towards
- Relief of symptoms
- Restoration and maintenance of full ROM of Hip
- Containment of Femoral head
- Resumption of weight bearing and full activity as
early as possible

52. The factors to take into consideration to decide
the treatment include:
• The age of the child at the onset of symptoms
• The presence of extrusion of the femoral head
• The range of motion of the hip
• The stage of evolution of the disease.

53. TREATMENT OF PERTHES
EARLY IN THE COURSE OF THE DISEASE
(from the onset to the early
stage of fragmentation )
AIM :
- to prevent the femoral head
from bearing forces across the
acetabular margin by either
preventing or reversing
extrusion of the femoral head
by
“containment.”

54. Containment
term used to describe any intervention that places
the antero-lateral part of the femoral epiphysis
well into the acetabulum thereby protecting the
vulnerable part of the epiphysis from being
subjected to deforming stresses.

55. ROLE OF CONTAINMENT
Containment alters joint mechanics to distribute
forces more evenly across the epiphysis thereby
protecting the weak and fragmented femoral head
until reossification can occur.
Since it is the anterolateral part of the epiphysis that extrudes,
containment attempts to ensure that this part of the epiphysis
remains covered by the acetabulum.

56. • The essence of containment is to equalize the
pressure on the head and subject it to the
molding action of the acetabulum. (biological
plasticity)
Thus remodeling of the femoral head is
expected to occur by a process called
biological plasticity (capability of the femoral
head to remodel to spherical shape when
encal-luped to the spherical acetabulum )

58. HOW TO CONTAIN ?
Containment can be achieved by 2 different methods :
1 ) keeping the hip in abduction and internal rotation or
in abduction and flexion by
- casting
- bracing
- surgery on the femur.
2 ) by an osteotomy of the pelvis that re-orients the
acetabulum such that it covers the antero-lateral part of
the femoral epiphysis or by creating a bony shelf over the
extruded part of the epiphysis
- Salter osteotomy
- triple innominate osteotomy)

59. surgical options for
containment
(a)extruded avascular
femoral epiphysis
(b)proximal femoral varus
osteotomy
(c)innominate osteotomy
(d)shelf procedure

60. OSTEOTOMIES
FEMORAL VARUS OSTEOTOMY
- Varus osteotomy with or without rotation
offers the advantage of deep seating of the
femoral head and positioning of the
vulnerable anterolateral portion of the head
away from the deforming influences of the
acetabular margin.
- It improves disturbed venous drainage and
relieves interosseous venous hypertension,
thus accelerating the healing process.

61. PREREQUISITES
1. Full range of motion
2. Congruency between the head and the
acetabulum
3. Ability to seat the head in abduction and
internal rotation
TIMING
As with all containment treatment modalities, to
have any effect, treatment must be instituted in
the initial or fragmentation stage of the disease.

62. SHORTCOMINGS
- Associated risks and costs of the surgical procedure
- Second surgical procedure for hardware removal.
- The affected limb is also shortened by the procedure.
The varus angulation normally decreases with growth,
but if there has been physeal plate damage by the
disease, this remodeling potential may be lost, leaving
the patient with a permanent varus deformity and limb
shortening.
• varus angulation of 10-15° is sufficient to obtain
adequate containment by a femoral varus osteotomy;

64. INNOMINATE OSTEOTOMY
- Provides containment by redirection of the acetabular
roof, providing better coverage for the anterolateral
portion of the head.
- It places the head in relative flexion, abduction, and
internal rotation with respect to the acetabulum in the
weight-bearing position.
- Any shortening caused by the disease process is corrected.
PREREQUISITES
1. Full range of hip joint motion
2. Joint congruency
3. Ability to seat the head in flexion, abduction, and internal
rotation.

67. Irrespective of the method adopted it is
imperative that containment be achieved
sufficiently early in the course of the disease,
before the femoral head gets irreversibly
deformed. It follows that containment must
be achieved before the late fragmentation
stage.

68. Outline of decision-making for treatment of Perthes’ disease
Early in the course of the disease

69. LATE IN THE COURSE OF THE DISEASE (REMEDIAL SURGERY)
(Late part of the stage of fragmentation or in the early part of the
stage of reconstitution)
AIM :
- Attempts to minimize the effects of early deformation of the
femoral head that has already occurred .
*At this stage, some children have a reduced range of
motion (particularly abduction) and attempted
abduction results in hinging.

70. VALGUS FEMORAL
OSTEOTOMY
INDICATION :
- Hinged Abduction of hip
*It overcomes the hinging and
brings a more congruent
surface of the femoral head
under the acetabulum.

71. Arthrodiatasis and Epiphyseal drilling
Arthrodiatasis or joint distraction with an external
fixator in an attempt to unload the hip and facilitate
the restoration of epiphyseal height.
Arthrodiastasis or hinged hip distraction is an
alternative treatment for the older child in the Herring
C group
The reported results have not been sufficiently encouraging to recommend this as the
procedure of choice.
.

72. Epiphyseal drilling in the hope that this will
hasten re-vascularization .
Reports on the long term outcome of this method of treatment
are awaited

73. TREATMENT OF THE SEQUELAE OF THE DISEASE
(SALVAGE SURGERY)
AIM :
Attempt to re-shape the deformed femoral head
and the acetabulum by safe surgical dislocation of
the hip.
• For Abnormalities such as cam impingement, pincer impingement,
functional retroversion and greater trochanteric and lesser
trochanteric impingement .
• Reduction of pain, increased joint motion and improved strength of
the hip abductors .

74. NONCONTAINABLE HIP
For noncontainable hips, particularly those that demonstrate the hinge
abduction phenomenon on arthrography.
These procedures in an already deformed head must be viewed as
salvage procedures with the limited aims of pain relief, correction of
limb length inequality, and improvement of movement and abductor
weakness.
These salvage procedures include
1. Chiari osteotomy
2 Cheilectomy
3 Abduction extension osteotomy
4 Acetabular shelf procedures alone or in combination with femoral
osteotomies.

75. CHIARI OSTEOTOMY
The Chiari osteotomy improves the lateral
Coverage of the deformed femoral head but
does not reduce the lateral impingement in
abduction and may exacerbate any existing
abductor weakness.
Its role in LCPD is yet to be defined.

77. CHEILECTOMY
Cheilectomy removes the anterolateral
portion of the head that is impinging on the
acetabulum in abduction.
This procedure must only be done after the
physis is closed otherwise, a slipped capital
femoral epiphysis (SCFE) may ensue.
This procedure does not correct any residual
shortening or abductor weakness.

79. SHELF ARTHROPLASTY
In recent years, the shelf arthroplasty has been
gaining in popularity in the patient with a poor
prognosis (Catterall 3, 4, lateral pillar B, C,
children > 8 years of age).
This procedure is aimed at providing coverage for
a femoral head that is certain to enlarge because
of the disease process.
Long-term outcomes of this procedure will
determine its role in LCPD treatment.

80. Operative technique for lateral shelf acetabuloplasty

81. Treatment can be divided into 3 Phases
1) INITIAL PHASE
( Restore & Maintain Mobility )
2) ACTIVE PHASE
( Containment & Maintainence of full mobility )
3) RECONSTRUCTIVE PHASE
( Correct Residual Deformities )

82. INITIAL PHASE
- Rest
- Analgesia
- Anti-inflammatory drugs
- Temporary non-weight-bearing with crutches
- B/L Skin Traction and gradually ABDUCTING over 1-2
weeks till full abduction is regained.
- Physiotherapy – Active and Passive ROM excercises
plays an important role in Restoring motion.
- There is little evidence to suggest that prolonged
non-weight-bearing is effective in preventing femoral
head deformity

83. ROLE OF BISPHOSPHONATES & BMP
Bisphosphonates have increasingly been studied
as a way to stop destruction by delaying
resorption of necrotic bone and preventing
collapse of the femoral head.
Bone morphogenetic proteins (BMPs) are also
being investigated as a possible treatment, by
way of promoting osteoclastic bone resorption
and thereby stimulating the healing process.
Routine use of both these therapies remains
controversial.

84. ACTIVE PHASE
Consists of CONTAINMENT of femoral head within
the acetabulum .
This can be achieved by 2 ways :
- NON OPERATIVE - ORTHOSIS
- OPERATIVE – FEMORAL & ACETABULAR
OSTEOTEMIES

85. ORTHOSIS
‘ literature does not support use of orthotics ,
A) NON AMBULATORY WEIGHT RELIEVING
1) ABDUCTION BROOMSTICK PLASTER CAST
2) HIP SPICA CAST
3) MILGRAM HIP ABDUCTION ORTHOSIS
B) AMBULATORY BOTH LIMBS INCLUDED
1) PETRIE ABDUCTION CAST
2) TORONTO ORTHOSIS
3) NEWINGTON ORTHOSIS
4) BIRMINGHAM BRACE
5) ATLANTA SCOTISH RITE BRACE
c) AMBULATORY UNILATERAL
1) TACHDJIAN TRILATERAL SOCKET ORTHOSIS

86. ATLANTA SCOTISH RITE BRACE
NEWINGTON ORTHOSIS BIRMINGHAM BRACE

87. TORONTO BRACE
TACHDJIAN TRILATERAL
SOCKET ORTHOSIS

88. ROLE OF CASTING / BRACING /ORTHOSIS
- They work by abducting and flexing (or
internally rotating) the hip to reposition the
femoral head deep in the acetabulum and
protect it from collapse until re-ossification.
- Orthotic treatment is discontinued when the
disease enters the reparative phase and
healing is established.

89. - This may take 2 years or more, which makes
compliance a real issue in this age group.
- When there is a severe adduction deformity, a
period of traction or an adductor tenotomy
may be necessary before applying these casts
or braces.
- Prolonged bracing is now less popular, as it
appears to offer uncertain benefit over the
long term.

90. RADIOGRAPHIC EVIDENCE OF
HEALING ARE
1) Appearance of regular ossification in the femoral
head.
2) Increased density of femoral head should
disappear.
3) Metaphyseal rarefaction involving the lateral
cortex of the metaphysis should ossify.
4) There should be intact lateral column
5) There should be normal trabeculae bone in
the epiphysis.

91. - Children younger than 6 years of age at onset
usually have a benign course, and major
treatment is not often necessary because they
have a longer growing time to remodel
abnormalities.
- Children between 6 and 9 years of age at onset
have more symptoms and often benefit from
surgical treatment.
- Children older than 9 years of age have a more
severe course, and their response to treatment is
less predictable.

92. ABDUCTION EXTENSION OSTEOTOMY
Abduction extension osteotomy of the femur is indicated when
arthrography demonstrates joint congruency improved by the
extended, adducted position.
Preliminary results indicate improvement in limb
length, decrease in limp, and improvement in function
and range of motion.
This osteotomy is gaining many advocates because of its early
promising results.
Long-term results will be necessary to determine its role in
the treatment of LCPD.

93. TROCHANTERIC
ADVANCEMENT
Indications :
- Trochanteric overgrowth
- Capital femoral physeal
growth arrest

94. PROGNOSTIC FACTORS
Clinical head at risk signs
1) Age - > 8yrs
- younger age, more time to remodel defect
- Late age , less potential to develop acetabulum
- More age with increase body wt likely to damage
epiphysis
2) Female sex- Girls of same age of boys are more skeletally
mature
3) Obesity
4) Limitation of ROM
5) Adduction contracture
6) Subluxating hips

95. Radiological
- CE angle of weiberg
- Salters extrusion index
- Epipyhseal index
- Epiphyseal quotient
Classification ( Assessing outcome )

96. CE angle of weiberg
Wiberg’s or center edge CE angle is formed at
the juncture of the Perkin line with line drawn
from the center of the femoral head to the outer
edge of the acetabular roof(lateral edge of the
sourcil).
Measured on the Anterior Posterior hip
radiographs .
The center edge angle may distinguish between
acetabular insufficiency, under coverage or
overcoverage of the femoral head by the
acetabulum.
Normal – 20-40 degree ( Avg 36 degree )
 > 25 --- Good
 20-25 ---Fair
 20--- Poor

97. Salter Extrusion angle
Horizontal line at bottom of acetabular
teardrop and perpendicular line at lateral
margin of acetabulum is drawn.
Lines from intersection of these lines
through midpoint of physis , gives
extrusion angle .
Salter Extrusion Index
Width of epiphysis (AB)outside
perpendicular line at lateral margin of
acetabulum(EF)expressed as
percentage of total width of epiphysis
( CD)
If > 20 % --- Poor Prognosis

98. Epiphyseal Index
Greatest height of
epiphysis divided by its
width
Epiphyseal Quotient
Epiphyseal index of
involved hip divided by
index for uninvolved hip
 > 0.6 – Good
 0.4-0.6 – Fair
 < 0.4 - Poor

100. THANKS
FOR YOUR
ATTENTION