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Perthes Disease
Definition
 It is a self limiting disorder of the hip produced by
ischemia and varying degrees of necrosis of the femoral
head.
Synonyms
 Coxa Plana
 Pseudocoxalgia (Calve)
 Arthritis deformans juvenilis (Perthes)
 Osteochondroses of the hip.
 Arthur Legg of the
United States
 Jacques Calve of
France
 Georg Perthes of
Germany
Historical aspect
 Parker started the use of broomstick cast in 1929.
 Eyre-Brook introduced traction in bed for 18-24 months.
Blood supply to femoral head
 Retinacular arteries
 Metaphyseal arteries
 Artery of the round
ligament
Blood supply to femoral head
 Infants
1. Metaphyseal arteries .
2. Lat epiphyseal arteries
3. Lig teres – insignificant
 4 mts – 4 years
1. Lat epiphyseal
2. Metaphyseal art. decrease in number
(due to appearance of growth plate).
Blood supply to femoral head
 4 yrs to 7 years
1. Epiphyseal plate forms a barrier to metaphyseal vessels.
 Pre-adolescent
1. After 7 yrs arteries of lig teres become more prominent
and anastomose with the lateral epiphyseal vessels.
Blood supply to femoral head
 Adolescent
After skeletal maturity
metaphyseal vessels again come
into picture
Incidence
 Male : Female = 4-5:1
 2.5:1 in India
 Age of onset earlier in females.
 Age –
Range – 2-13 years.
Most common 4-8 years.
Average – 6 years.
 Bilateral in 10-12 %
 Incidence more in Caucasians as compared to
Negroid, mongoloid.
Etiology
 Etiology not known.
 Coagulation disorders.
 Altered arterial status of femoral head.
 Abnormal venous drainage.
 Abnormal growth and development.
 Trauma.
 Hyperactivity or attention deficit disorder.
 Genetic component.
 Environmental influences.
 As a sequelae to synovitis.
Coagulation disorders
 Protein C or S deficiency
 Thrombophilia
 Hypofibrinolysis
Altered arterial status
 Angiographic studies have shown obstruction of
superior capsular arteries and decreased flow in
medial circumflex femoral arteries .
 The intracapsular ring has been found to be
incomplete.
Abnormal venous drainage
 Increased venous pressure in the
femoral neck
 Congestion in the metaphysis
 Venous outflow exits more distally in the diaphysis.
Abnormal growth and development
 A delay in Bone age of 1.5 to 2 years
 Low birth weight
 Low levels of somatomedin C
Trauma.
 In the developing femur (4 – 7 yrs),the major lateral
epiphyseal vessels must course through a narrow
passage ,which could make it susceptible to trauma.
Hyperactivity or attention
deficit disorder
Genetic component
 Familial association.
 X-Linked recessive inheritance.
Environmental influences
 Low socioeconomic status.
Sequel to synovitis
 Synovitis of the hip occurs early in Perthes disease.
 Increased pressure in synovitis may cause a tamponade
effect on the vasculature
Pathogenesis
 Waldenstrom staged the pathological
process of the disease as
1. Initial or ischaemic stage
2. Resorption or fragmentation stage
3. Reparative stage
4. Remodelling stage
Pathogenesis
 Ischaemic stage
- Necrosis
- Crushing of trabaculae.
- degeneration of basal layer of
articular cartilage
- Thickening of peripheral
cartilagenous cap.
- Shape of head maintained.
Ischaemic stage
Pathogenesis cont…
 Resorption stage
- Invasion of vascular connective tissue.
- Resorption of dead bone by
Osteoclasts.
- loss of epiphyseal height due to
1) Collapse of bony trabaculae.
2) Resorption of dead bone
Resorption stage
Pathogenesis cont…
 Reparative stage
- pathological fracture.
- creeping substitution and
apposition of viable bone in dead
trabaculae.
Reparative stage
Remodelling stage
(replacement by biologically plastic bone)
If treated
 Femoral head is
congrous
If untreated
 Subluxation and deformity
Clinical Features
 Painless limp leads to painful limp
 Pain in the groin,anterior hip
or greater trochanter
 Referred pain to the knee
 Combination of antalgic & trendelenburg
gait.
 Decreased range of motion especially abduction and internal rotation.
 Atrophy of thigh muscles.
 Shortening
Investigation
 X-Ray –AP & Frog leg Lat view (Lowenstein view)
 USG
 Arthrography
 Bone Scan
 MRI
X-Ray
 Ossific nucleus
smaller
X-Ray
 Cresent Sign or
Salters sign or
Caffey’s sign
X-Ray
 Increased Radio
opacity of femoral
head due to collapse,
new bone formation
and calcification of
dead marrow.
X-Ray
 Fragmentation of
epiphysis
X-Ray
 Metaphyseal widening
and cystic changes in
femoral neck
X-Ray
 Lateral extrusion of
femoral head.
 Hinged abduction.
X-Ray
 Sagging rope sign in
adults with history of
perthes
Ultrasound
 Synovial effusion
 Cartilage hypertrophy in early stages
Arthrography
 Shows configuration of the femoral head and its relation
with the acetabulum.
 Containment
 Congruity
 Not routinely used .
Bone Scan
 Diagnosis possible months before signs appear on X-Ray.
 Avascular areas show cold spots.
Bone Scan
 Convay et al
classification
 Stage 1 is total lack
of uptake
Bone Scan( stage 2)
 Revascularisation of a
lateral column
 Failure to revascularise
at lat column is a grave
sign
 Also called
“scintigraphic head at
risk sign”
 Precedes radiographic
head at risk sign by 2-3
mths
Bone Scan( stage 3)
 Gradual filling of
anterolateral part
Bone Scan( stage 4)
 Return to normal
MRI
 Accurate in early diagnosis.
 Shows congruity,containment,synovial hypertrophy well.
Classification
 Waldenstroms classification.
 Catterall classification
 Salter classification
 Herrings lateral pillar classification.
Waldenstroms classification
(Pathological classification)
Catterall classification
(based on x ray AP and Lat view).
 I – only anterior portion of epiphysis
affected.
 II – anterior segment involved central
sequestrum present
 III – most of epiphysis sequestered with
unaffected portions located medial
and lateral to central segment
 IV – all of epiphysis sequestered.
Salter Classification
 Type A = I & II Catterall
 Type B = III & IV Catterall.
Herring Lat Pillar
 Group-A no involvement of the lateral
pillar, with no density changes and no
loss of height of the lateral pillar
 Group-B hips have lucency in the
lateral pillar and may have some loss
of height , but not exceeding 50% of
the original height.
 Group-C hips are those with more
lucency in the lateral pillar and >50%
loss of height
Salters extrusion Index
 If AB is more
 than 20% of CD
 it indicates a
 poor prognosis
Modified Elizabethtown
classification
Stage Ia: Part or
whole of the
epiphysis is
sclerotic. There is
no loss of height of
the epiphysis.
Modified Elizabethtown
classification
Stage Ib: The
epiphysis is
sclerotic and
there is loss of
epiphyseal
height. There is
no evidence of
fragmentation of
the epiphysis.
Modified Elizabethtown
classification
Stage IIa: The
sclerotic epiphysis
has just begun to
fragment. One or
two vertical fissures
are seen in either
the AP or the lateral
view
Modified Elizabethtown
classification
Stage IIb:
Fragmentation is
advanced. No new
bone is visible
lateral to the
fragmented
epiphysis.
Modified Elizabethtown
classification
Stage IIIa: Early
new bone
formation is visible
on the periphery of
the necrotic
epiphysis and
covers less than a
third of the width of
the epiphysis
Modified Elizabethtown
classification
Stage IIIb: The
new bone is of
normal texture and
has grown over a
third of the width of
the epiphysis.
Modified Elizabethtown
classification
Stage IV the healing is complete and there is no
radiologically identifiable avascular bone
Prognostic Factors
1. Age at diagnosis
<6 yrs – good
6 – 9 yrs – fair
>9 yrs - poor
1. Extent of involvement
2. Sex
3. Catterall “head at risk” signs
Catterall “head at risk”
signs
 Clinical
 Radiographic
 Clinical
1. Progressive loss of hip motion more so abduction.
2. Obese child
 Radiographic
1. Gage sign
2. Calcification lateral to epiphysis
3. Diffuse metaphyseal rarefaction
4. Lateral extrusion of femoral head
5. Growth disturbance of physis
Physeal disruption
Metaphyseal rarefaction
Femoral head extrusion
Gage’s sign
 Rarefaction in the
lateral part of the
epiphysis and
subjacent
metaphysis.
Classification of results
 Uniplanar methods
- CE angle of Weiberg.
- Salters extrusion Index.
- Salters extrusion angle.
- Epiphyseal index.
- Epiphyseal quotient.
 Biplanar methods
- Mose classification.
- Stulberg classification.
CE angle of Weiberg
 Indicator of acetabular depth
 It is the angle formed by a
perpendicular line through the
midpoint of the femoral head
and a line from the femoral
head center to the upper outer
acetabular margin.
 Normal = 20 to 40 degrees
 Angle >25 = good, 20-25= fair, <
20 = poor
Salters extrusion Index
 If AB is more
than 20% of CD
it indicates a
poor prognosis
Salters extrusion angle
 Normal is 50
degrees or
more
Epiphyseal index & quotient
 Epiphyseal index = greatest height of the epiphysis
divided by its width.
 Epiphyseal quotient = Epiphyseal index of involved hip
divided by the index for uninvolved hip.
>0.6 = good
0.4-0.6 = fair
<0.4 = poor
Mose Classification
 Good < 1 mm
 Fair < 2 mm
 Poor > 2 mm
Stulberg classificaton
 Class I – Shape of the femoral head was
basically normal.
 Class II – Loss of head height but within 2 mm
to a concentric circle on AP and frog
leg X-Ray
 Class III – Deviates more than 2 mm and
acetabulum contour matches
the head contour
 Class IV – Head Flattened, Flattened area
<1cm. Acetabulum contour matches
the head contour
 Class V – Collapse of femoral head, Acetabular
contour does not change
Stulberg classificaton
 Class I & II – Spherically congruent.
 Class III & IV – Congruous Incongruity
OR
Aspherical congruity.
 Class V – Incongruous incongruity
OR
Aspherically incongruent.
Stulberg classificaton
 Class I
Shape of
the femoral
head is
basically
normal.
Stulberg classificaton
 Class II
Loss of
head
height but
within 2
mm
Stulberg classificaton
 Class III
Deviates
more
than 2
mm
Stulberg classificaton
 Class IV
Head
Flattene
d
Stulberg classificaton
 Class V
Collapse of
femoral
head,
Acetabular
contour
does not
change
Differential diagnosis
 Tuberculosis of the hip
 AVN due to leukemia, lymphoma, gauchers disease,
hemoglobinopathies etc
 Meyers dysplasia
 AVN following dislocation.
 Transient synovitis
Treatment
 Objectives
- To produce a normal femoral
head and neck
- To produce a normal acetabulum
- A congruous hip which is fully
mobile
- To prevent degenerative arthritis
of the hip later in life
Treatment
 Treatment efforts are directed towards
- Restoration and maintenance of
full mobility of the hip
- Containment of the femoral
head.
- Resumption of weight bearing
and full activity as soon as
possible
Treatment
 Caterall group 1 and
group 2 ( < 7 years)
No
 Herring group 1 & Treatment
group 2 (< 6 years)
Treatment
Treatment is divided into 3 phases
 Initial Phase – restore & maintain mobility
 Active Phase – Containment and maintainance of full mobility.
 Reconstructive phase – correct residual deformities.
Treatment ( Initial Phase )
 Physiotherapy – active and passive
range of motion
exercises to restore
motion
 Traction – B/L skin traction and
gradually abducting over 1-2
weeks till full abduction is
regained.
Treatment ( Active Phase )
 Consists of containment of the femoral head within the
acetabulum. This can be achieved by
orthosis
or by
surgery
Treatment (Orthosis)
 Non Ambulatory weight releiving
1. Abduction broomstick plaster cast
2. Hip pica cast
 Ambulatory Both limbs included
1. Petrie Abduction cast
2. Toronto orthosis
3. Newington orthosis
4. Birmingham brace
5. Atlanta Scotish Rite Brace
 Ambulatory unilateral
1. Tachdjian trilateral socket orthosis
Treatment (Orthosis)
 Atlanta Scotish Rite
Brace
Treatment (Orthosis)
 Atlanta Scotish Rite
Brace
Treatment (Orthosis)
 Newington orthosis
Treatment (Orthosis)
 Birmingham brace
Treatment (Orthosis)
 Toronto Brace
Treatment (Orthosis)
 Tachdjian trilateral
socket orthosis
Treatment (Orthosis)
 Orthotic treatment is discontinued when the
disease enters the reparative phase and healing
is established.
 The radiographic evidence of healing are
1. Appearance of irregular ossification in the femoral
head.
2 . Increased density of femoral head should
disappear.
3 . Medial segment of femoral head should increase in
size and height.
4 . Metaphyseal rarefaction involving the lateral cortex
of the metaphysis should ossify.
5 . There should be intact lateral column.
Treatment ( Surgical)
 Femoral varus osteotomy.
 Inominate osteotomy.
 Combined femoral and inominate osteotomy
 Valgus osteotomy
 Shelf arthroplasty
Femoral varus osteotomy
Femoral varus osteotomy
Femoral varus osteotomy
Femoral varus osteotomy
Femoral varus osteotomy
Recent Advances
 Anticoagulant
 Ibadronate
Thank You

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