Perthes Disease

Synonyms
Perthes Disease
Osteochondritis Deformans Juvenilis
Childhood Aseptic Necrosis of Femoral
Head
By:-
Dr. surya Prakash garg
Definition
 Perthes’ disease is a self-limiting form of aseptic
ischaemic necrosis(osteochondrosis) of the
capital femoral epiphysis of unknown
aetiology that develops in children commonly
between the ages of 5 – 12 years.
 More common in India 8 to 11 yrs & south india
 It is a condition of immature hip caused by
necrosis of the femoral epiphysis; the femoral
head subsequently deforms as necrotic bone is
replaced by living bone.
 It is Hip disease occurring during early childhood
and caused by impaired circulation in the
femoral head.
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DR S P GARG January 2
2020
Historical background
 The disease was described almost

simultaneously, in 1910, by –
 G. C. Perthes in Germany,
 J. Calve in France
 A.T. Legg in America.
 Hence name – “Legg Calve Perthes Disease”
 The newly discovered x-ray technique
allowed doctors to differentiate it from
inflammatory forms of hip disease.
10
2020
Etiological Factors that play a
role in development of illness
 Vascular supply
 Increased intra-articular pressure
 Intraosseous pressure
 Coagulation disorder
 Growth hormones
 Growth
 Social conditions
 Genetic factors
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2020
Vascular supply
 Angiograms & laser Doppler flow measurements

 Medial circumflex artery is missing or obliterated in many
cases
 Obturator artery or the lateral epiphyseal artery are also
affected in some cases.
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2020
Increased intra-articular
pressure
 Animal experiments have shown that an ischemia similar
to that in Perthes disease can be generated by increasing
the intra-articular pressure.
 However, the condition of transient synovitis of the hip
does not appear to be a precursor stage of Perthes disease
as the increased pressure resulting from the effusion in
transient synovitis does not lead to vessel closure.
10
2020
Intraosseous pressure
 The measurement of intraosseous pressure in Perthes

patients has shown that the venous drainage in the
femoral head is impaired, causing an increase in
intraosseous pressure.
 In animal studies, the intraosseous injection of fluid, and
the associated increase in pressure, produced a condition
similar to Perthes disease
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2020
Coagulation disorder
 Study have found a coagulation disorder in 75% children

with Perthes disease.
 In most cases the disorder was thrombophilia.
 Rarely the disorder involved elevated serum levels of
lipoprotein, a thrombogenic substance.
 Recent studies have questioned the significance of clotting
factors as an etiological component
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2020
 Growth hormones :
 While earlier studies found reduced levels of the growth
hormone.
 Recent studies have not shown any difference from control
groups in respect of hormone status
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2020
 Growth:
 Children with Perthes disease are shorter, on average,
than their peers of the same age & show a retarded
skeletal age (cartilaginous dysplasia).
 The maturation disorder occurs between the ages of 3 and
5 years.
 Both the trunk and extremities lag behind in terms of
growth.
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2020
 Growth cont…
 The shortening of the extremities is also accompanied by
small feet.
 Since this shortening is offset by excessive growth at a
later age, patients who suffered from Perthes disease as
children are no shorter, as adults, than the population
average.
 More recent experimental studies have shown that the
metaphyseal changes are based on a growth disorder.
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2020
 Social conditions:
 Studies in the UK have shown that Perthes disease is more
common in the lower social status.
 The authors suggest a poorer diet during pregnancy as one
possible explanation for this phenomenon.
 A recent study did not confirm this theory
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2020
 Genetic factors:
 Studies have shown that first degree relatives of children
with Perthes disease are 35 times more likely to suffer
from the condition than the normal population.
 Even second- and third-degree relatives show a fourfold
increased risk.
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2020
 To sum up –
 Genetic factors play an important role in the etiology of
Perthes disease.
 The illness develops as a result of impaired circulation
in the medial circumflex artery in association with a
skeletal maturation disorder with delayed growth in
children aged from 3–5 years.
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2020
Occurrence
 In white population is 10.8 per 1,00,000 children & adolescents aged
from 0–15 year
 In Asians is 3.8 per 1,00,000
 In Mixed-race populations is 1.7 per 1,00,000
 In Blacks is 0.45 per 1,00,000
 The highest reported incidence was in city of Liverpool (UK) early
1980’s, with 15.6 per 1,00,000 individuals under 15 years of age.
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2020
 A decline was subsequently observed in the 1990’s –
possibly as a result of the improved social conditions.
 Similarly high incidence 15.4 per 1,00,000 was recently
reported in a rural area of Southwest Scotland.
 In Sweden an annual incidence of 8.6 per 100,000
people under 15 yrs was Determined.
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2020
Epidemiology
 Disorder of hip in young children

 Usually ages 4-8yr
 As early as 2yr, as late as teenager
 Boys: Girls – 4/5:1
 Bilateral – 10-12%
 No evidence of inheritance
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2020
PATHOGENESIS
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2020
Classification
 All known classifications of Legg-Calvé-Perthes disease

are based on the morphological findings on x-rays.
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2020
Morphological classifications
of the extent of the lesion
 Classification according to Catterall (Common)
 Classification according to Salter & Thompson
 Classification according to Herring
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2020
Classification of extent of lesion - (Acc to
Catterall) its not a progressive staging
Grade Characteristics
I Only anterolateral quadrant affected
Anterior third or half of the femoral
II
head
Up to 3/4 of the femoral head affected,
III
only the most dorsal section is intact
IV Whole femoral head affected

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2020
Grade – I
 Only anterolateral quadrant affected
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2020
Grade - II
 Anterior third or half of the femoral head
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2020
Grade – III
 Up to 3/4 of the femoral head affected,
 only the most dorsal section is intact
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2020
Grade – IV
 Whole femoral head affected
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2020
Classification according to
Salter & Thompson
Group Characteristics
Subchondral # involving <50% of the femoral
A
dome
Subchondral # involving >50% of the femoral
B
dome
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2020
 8-year old boy with subchondral fracture and incipient Legg-Calve-
Perthes disease
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2020
Herring
The lateral pillar classification system is based on radiographic
changes in the lateral portion of the femoral head
when it enters the fragmentation stage, as seen on the AP
view
Grou
Characteristics
p
A Lateral pillar not affected
>50% of height of lateral
B
pillar preserved
<50% of height of lateral
C
DR S P GARG
pillar preserved 10
January 28
2020
Herring “A”
Lateral pillar not affected
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2020
Herring “B”
>50% of height of lateral pillar
preserved
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2020
Herring “C”
<50% of height of lateral pillar
preserved
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2020
10
2020
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2020
Deformation of the femoral
head
 Children femoral head becomes deformed during
revascularization of the epiphysis.
 There is evidence to suggest that irreversible
deformation occurs either in the latter part of the stage
of fragmentation or very early in the stage of
regeneration.
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2020
 Enlargement of the femoral head –
 The femoral head becomes enlarged as the disease
progresses.
 The extent of enlargement is proportional to the degree of
its deformation.
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2020
 Capital physeal growth impairment –
 The avascularity of the epiphysis impairs normal growth at
the capital femoral physis and, as a result of this, in some
older children the femoral neck is foreshortened.
 The trochanter continues to grow normally and as a
consequence the GT outgrows the femoral head and neck.
 This results in altered mechanics of the hip and a
Trendelenburg gait.
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2020
 Secondary degenerative arthritis of the hip –
 All 3 morphological changes in the proximal femur listed
above can contribute independently or collectively to the
development of secondary degenerative arthritis.
 However, the most important factor that predisposes to
the development of degenerative arthritis is deformation
of the shape of the femoral head.
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2020
Stages of Perthe’s Disease
(Waldenström Staging)
1. Avascular stage
2. Fragmentation stage
3. Re-ossification stage
4. Healed stage
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2020
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2020
 Avascular stage.
 The femoral head appears slightly flattened
& denser than normal on the x-ray.
 The joint space is widened (Waldenström
sign).
 Lateralisation of the femoral head.
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2020
 Stage of resorption
(Fragmentation)
- Femoral head breaks up
into fragments
- Lucent areas appear in
the femoral head
- Increased density resolves
- Acetabular contour is
more irregular
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2020
 Stage of Re-ossification
 The femoral head is rebuilt
 New bone formation occurs in the
femoral head
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2020
 Healing stage
 End stage with or without defect healing (normal hip, coxa
magna, flattened head etc.)
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2020
CLINICAL FEATURES
 Limp with Loss of abduction and internal rotation is earliest

presentation because of anterosup part of head involvement
 Sectoral sign decreased int rot in flexion
 Gearstick sign increase abd in flexion than in ext
 EARLY (Necrosis, Fragmentation) –

 Synovitis
 There is pain & limp of insidious onset.
 Pain usually in groin, radiating to thigh or knee.
 Limp is typically antalgic gait.
 LATE (Re-ossification – Remodeling) –
 There is limp (antalgic, short-leg or stiff hip).
 Pain is mild and usually in the hip area.
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2020
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2020
Stages of radiological changes
in Perthe's disease:
 Early Stage –
 Joint space widening (waldenstrom's sign)
 Increased density of femoral epiphysis
 Subchondral fracture, or “crescent sign,” seen on
lateral radiograph
 Mid Stage –
 Fragmentation and flattening of head (Coxa
magna)
 Widening of the physis (waldenstrom's sign)
 Femoral neck cysts
 Extrusion of the femoral head
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2020
 Late Stage–
 Coxa magna
 High-riding trochanter
 Flattened femoral head
 Irregular articular surface
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2020
Physical Therapy
Assessment & Diagnosis
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2020
Clinical Assessment
 A thorough history and examination be completed

to establish an impairment based physical therapy
diagnosis and individualized plan of care (APTA).
 It is recommended initial evaluation, on a monthly
basis or sooner if the pt demonstrates a change in
status, and at discharge:
 Pain and symptoms
 Lower extremity PROM & AROM
 Lower extremity strength
 Gait
 Balance
 Outcome measures
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2020
Pain and symptoms
 It is recommended to assessed using –

 Oucher pain scale
 Numerical Rating Scale (NRS)
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2020
Lower Limb PROM & AROM
 Fluid filled or linear goniometer is recommended to

measure ROM.
 Hip motions to assess include –
 Hip flexion, abduction, extension, internal rotation,
external rotation.
 The knee & ankle ROM be assessed at the initial
evaluation and thereafter if they are significantly
limited.
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2020
Lower Extremity Strength
 Quantitative muscle testing is recommended using a

hand held dynamometer due to its high intra- & inter-
rater reliability.
 MMT also can be used but less reliable.
 Muscle groups to assess include –
 Hip – Flexors, Abductors, Extensors,
Internal Rotators, External Rotators
 Knee – Extensors, Flexors,
 Any Other Muscle Group that is
Significantly Limited
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2020
Gait
 Qualitatively gait assessment is recommended for

common LCP deviations.
 Note 1: Based on limited accessibility and feasibility, the
gold standard for gait analysis of 3-D gait kinematics and
kinetics is not recommended to be used in the clinic.
 Note 2: There is insufficient evidence & lack of reliability
& validity to support use of observational gait assessment
tools with this population.
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2020
Gait cont…
 Commonly observed gait characteristics in LCP include,

but are not limited to:
 Increased hip adduction on stance leg
 Trunk lean outside the normal range
 Trendelenburg (hip drop on unaffected limb while in
swing)
 Compensated trendelenburg/reverse
trendelenburg/duchenne (trunk lean to the affected side
while in stance on the affected limb)
 Toe in or toe out
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2020
Balance
 Balance be assessed on weight bearing status.

 The desired outcome is that the patient maintain
balance for age appropriate times for safe ambulation
and stair negotiation.
 Note: In pts 7 y or older, balance is to be assessed using
the Pediatric Balance Scale.
 If the pt is younger than 7 y old, the test is unavailable
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2020
Outcome measure scores
 The age appropriate Pediatric Quality of Life Inventory

Version 4.0 is recommended.
 Physical Functioning section is administered at the
initial evaluation, on a monthly basis for reassessment
of patient’s reported functional status, and at
discharge.
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2020
Imaging – Radiographic
Feature
 Widening of the joint space and minor subluxation
 Sclerosis
 Fragmentation and focal resorption
 Loss of height
 Metaphyseal cyst formation
 Widening of the femoral neck & head (Coxa
Magna)
 Lateral uncovering of the femoral head
 Sagging rope sign
 Acetabular remodelling
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2020
Frog-lateral View Of The Hips
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2020
Caffey’s sign
 As the disease progresses, a
subchondral # may occur in
the anterolateral aspect of the
femoral capital epiphysis.
 Is an early radiographic
feature best seen on the frog-
lateral projection.
 This produces a crescentic
radiolucency known as the
crescent, Salter’s or Caffey’s
sign
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2020
Fragmentation of the femoral
capital epiphysis
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2020
Sclerosis of epiphysis & widening
of joint space in the early stages
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2020
Metaphyseal cyst formation
within the femoral neck
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2020
‘Sagging Rope Sign’
 This a curvilinear sclerotic line running
horizontally across the femoral neck.
 It is confirmed by 3D CT studies.
 It is a finding in AP radiograph in a
mature hip with Perthes’ disease.
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2020
Ultrasound features
 Effusion, especially if persistent

 Synovial thickening
 Cartilaginous thickening
 Atrophy of the ipsilateral quadriceps muscle
 Flattening, fragmentation, irregularity of
the femoral head
 New bone formation
 Revascularisation with contrast enhanced
power Doppler
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2020
Differential Diagnosis
 It is important to rule out infectious etiology (septic

arthritis, toxic synovitis)
 Others:
 Chondrolysis -Neoplasm
 JRA -Sickle Cell
 Osteomyelitis -Traumatic AVN
 Lymphoma -Medication
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2020
Differential Diagnosis
 D/D unilateral Perthes’  D/D bilateral Perthes’

disease: disease:
 Transient synovitis  Hypothyroidism
 Septic arthritis  Multiple epiphyseal
dysplasia
 Sickle cell disease
 Sickle cell disease
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2020
Diagnosis
 Children with Perthes disease limp and complain of mild

to moderate hip pain.
 This situation can persist for several weeks.
 Clinical examination usually reveals a slight stiff,
protective limp.
 The ROM of the affected hip is usually restricted, in
particular with reduced abduction and internal rotation.
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2020
Classification of Phases of
Rehab
 It is recommended that the Classification
Instrument in Perthes (CLIPer) be used to
place the patient into a rehabilitation
classification phase upon examination.
 The patient should be re-examined using
the CLIPer on a monthly basis to determine
the appropriate progression through the
rehab classification stages
 It is recommended the patient is referred
back to the orthopaedic surgeon if the
patient’s status worsens over two
consecutive PT sessions
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2020
MANAGEMENT
 Most current therapeutic approaches are based on the concept of

containment, which, over the years, has evolved to include
nonoperative as well as operative treatment methods. This
response was referred to as “biologic plasticity.”
 It is notable that only 63% of the femoral head is in contact with
the acetabulum at any one time because the head represents 120%
of a hemisphere, whereas the acetabulum is only 75% of a
hemisphere.
 Age at Onset Before 6 Years- conservative
Our management of this age group focuses on pain relief, with a
reduction in activities and short-term use of anti-inflammatory
medications, and short periods of bed rest for major episodes of
pain or loss of joint motion.
 Age at Onset 6 to 8 Years- conservative=surgical?
The best management for this group remains controversial because
the results of treatment are less clear for this age group.
10
2020
 Treat symptomatically those patients with lateral pillar
group A hips and those with group B hips if the femoral
head is contained within the acetabulum.
Arthrographic assessment of the femoral head and a
containment treatment, Petrie casts with or without hip
adductor tenotomy, are instituted if hip abduction is
decreased and lateral extrusion of the femoral head is
observed radiographically. After 6 weeks of Petrie casting,
a surgical containment procedure such as femoral varus
osteotomy or a wide abduction brace (A-frame brace) is
used to maintain containment. Another option is to resume
symptomatic treatment after the 6 weeks of Petrie casting.
 Age at Onset 8 to 11 Years- surgical
operative treatments in the lateral pillar B and B/C border
groups but not for the lateral pillar C group. Early femoral
varus or innominate osteotomy is considered in this age
group if a majority of the femoral head shows
hypoperfusion. For femoral varus osteotomy, 10 to 15
degrees of varus angulation is applied if the osteotomy is
performed in the early stage because greater angulation
produces greater limb shortening and hip abductor
weakness without producing better results.
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2020
 Age at Onset After 11 Years
The success rate is less predictable in this patient
population regardless of which treatment methods are
applied. Because of the poor healing and remodeling
potential, even patients with partial femoral head
involvement can have a poor outcome in this age group.
The procedures used to treat adult femoral head
osteonecrosis, such as multiple epiphyseal drilling, core
decompression, and vascularized fibular grafting, are
being tried in limited centers, and their efficacy is
unknown at this time
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2020
Symptomatic treatment
 NSAIDS+traction,non weightbearing
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2020
NON SURGICAL CONTAINMENT
1.Broomstick Cast
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2020
2.Petrie Cast
Hips were abducted to 45 degrees and rotated internally 5 to
10 degrees. Every 3 to 4 months the casts were changed, and
the patient’s knees and ankles were mobilized between
changes.
Cast containment continued until the femoral head was well
into the healing stage.
The average length of treatment was 19 months.
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2020
 A wide abduction brace called A-frame has been used in
some centers to maintain femoral head containment
following 6-week Petrie casting
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2020
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2020
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2020
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2020
Surgical Containment
1.Femoral Osteotomy
 The groups with better results after surgery were
children older than 8 years of age at onset who
developed lateral pillar B or B/C border hip in the
fragmentation stage.
 Before femoral osteotomy is performed, it is important
that the patient regain a reasonable range of motion.
Petrie casts can be used to achieve this goal.
 A reduction in articular trochanteric distance associated
with a Trendelenburg gait may occur after surgery,
particularly in older patients. As a result, some surgeons
do not recommend femoral osteotomy for children older
than 8 years of age.
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2020
Varus Osteotomy
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2020
2.Innominate Osteotomy
 The first innominate osteotomy for patients with LCPD

was performed by Salter in 1962.332 His indications for
the procedure included onset of disease after 6 years of
age, a moderately or severely affected head, and loss of
containment.
 Preoperative prerequisites were minimum deformity of
the femoral head (as determined by arthrography), a
nonirritable hip, and no significant restriction of range
of motion. In addition, the hip had to be able to abduct
to 45 degrees and the femoral head had to be contained
with the hip in that position.
 Combined Femoral and Innominate Osteotomy
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2020
SALVAGE PROCEDURE
 Valgus Osteotomy
For LCPD, valgus osteotomy was initially recommended
for treating hinge abduction, in which the flattened
femoral head interferes with abduction.
 Chiari Osteotomy
 Shelf Arthroplasty
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2020
Prognosis
 60% of kids do well without Rx

 AGE is key prognostic factor:
 <6y – good outcome regardless of Rx
 6-8y – not always good results with just containment
 >9y – containment option is questionable, poorer
prognosis, significant residual defect
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2020
Operative Tx
 If non-op Rx cannot maintain containment

 Surgically ideal pt:
 6-9yo
 Catterral II-III
 Good ROM
 <12mos sx
 In collapsing phase
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2020
Surgical Tx
 Surgical options:
 Excise lateral extruding head portion to stop hinging
abduction
 Acetabular osteotomy to cover head
 Varus femoral osteotomy
 Arthrodesis
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2020
Head at risk signs
 Clinical features:  Radiological features:
 Progressive loss of movement  Lateral subluxation of the femoral
head (head partially uncovered)
 Adduction contractures
 horizontal /steep physis
 Flexion in abduction
 Calcification lateral to the epiphysis
 Heavy child
 Metaphyseal cysts
 Gage's sign
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2020
References:
 Lee J, Allen M, Hugentobler K, Kovacs C, Monfreda

J, Nolte B, Woeste E; Evidence-Based Care
Guideline Conservative Management of Legg-
Calve-Perthes Disease In children aged 3 to 12
years, Cincinnati children’s hospital medical
center, 2011
 Benjamin Joseph, Paediatric Orthopaedics, A
System Of Decision-making, 2009
 Fritz Hefti, Pediatric Orthopedics in Practice,
2007
 David Wilson (Ed.), Paediatric Musculoskeletal
Disease With an Emphasis on Ultrasound, 2005
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2020

Perthes Disease

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Perthes Disease

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Synonyms

 The disease was described almost

 Angiograms & laser Doppler flow measurements

 The measurement of intraosseous pressure in Perthes

 Study have found a coagulation disorder in 75% children

 Disorder of hip in young children

 All known classifications of Legg-Calvé-Perthes disease

IV Whole femoral head affected

 Limp with Loss of abduction and internal rotation is earliest

 EARLY (Necrosis, Fragmentation) –

 A thorough history and examination be completed

 It is recommended to assessed using –

 Fluid filled or linear goniometer is recommended to

 Quantitative muscle testing is recommended using a

 Qualitatively gait assessment is recommended for

 Commonly observed gait characteristics in LCP include,

 Balance be assessed on weight bearing status.

 The age appropriate Pediatric Quality of Life Inventory

 Effusion, especially if persistent

 It is important to rule out infectious etiology (septic

 D/D unilateral Perthes’  D/D bilateral Perthes’

 Children with Perthes disease limp and complain of mild

 Most current therapeutic approaches are based on the concept of

 The first innominate osteotomy for patients with LCPD

 60% of kids do well without Rx

 If non-op Rx cannot maintain containment

 Lee J, Allen M, Hugentobler K, Kovacs C, Monfreda

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