Legg calve perthes disease

Synonyms
– Perthes Disease
– Osteochondritis Deformans Juvenilis
– Childhood Aseptic Necrosis of Femoral Head

Dr. P. Ratan khuman (PT)
M.P.T., (Ortho & Sports)

Definition
Perthes‟ disease is a self-limiting form of
osteochondrosis of the capital femoral epiphysis
of unknown aetiology that develops in children
commonly between the ages of 5 – 12 years.
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.
22 June 2012 Dr.Ratan M.P.T.,(Ortho & Sports) 2

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.


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


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.


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.


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


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


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


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.


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.

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


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.


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.


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.


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.


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


Classification
All known classifications of Legg-Calvé-
Perthes disease are based on the
morphological findings on x-rays.


Morphological classifications of
the extent of the lesion
Classification according to Catterall (Common)
Classification according to Salter & Thompson
Classification according to Herring


Classification of extent of lesion - (Acc to Catterall)
Grade Characteristics
I Only anterolateral quadrant affected
II Anterior third or half of the femoral head

Up to 3/4 of the femoral head affected,
III
only the most dorsal section is intact

IV Whole femoral head affected


Grade – I
Only anterolateral quadrant affected


Grade - II
Anterior third or half of the femoral head


Grade – III
Up to 3/4 of the femoral head affected,
only the most dorsal section is intact


Grade – IV
Whole femoral head affected


Classification according to Salter
& Thompson
Group Characteristics
A Subchondral # involving <50% of the femoral dome
B Subchondral # involving >50% of the femoral dome


8-year old boy with subchondral fracture and
incipient Legg-Calve- Perthes disease



Group Characteristics

A Lateral pillar not affected

>50% of height of lateral
B
pillar preserved

<50% of height of lateral
C
pillar preserved


“A”
Lateral pillar not affected


“B”
>50% of height of lateral
pillar preserved


“C”
<50% of height of lateral
pillar preserved


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.


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.


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.

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.


Stages of Perthe‟s Disease
(Waldenström Staging)
1. Avascular stage
2. Fragmentation stage
3. Re-ossification stage
4. Healed stage


Stage and Characteristics
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.


Stage and Characteristics cont…
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


Stage of Re-ossification
– The femoral head is rebuilt
– New bone formation
occurs in the femoral head


Healing stage
– End stage with or without defect healing (normal
hip, coxa magna, flattened head etc.)


CLINICAL FEATURES
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.


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


Stages of radiological changes in
Perthe's disease: cont…
Late Stage–
Coxa magna
High-riding trochanter
Flattened femoral head
Irregular articular surface


Physical Therapy
Assessment & Diagnosis


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


Pain and symptoms
It is recommended to assessed using –
– Oucher pain scale
– Numerical Rating Scale (NRS)


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.


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


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.

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

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


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.


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


Frog-lateral View Of The Hips


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


Fragmentation of the femoral
capital epiphysis


Sclerosis of epiphysis & widening
of joint space in the early stages


Metaphyseal cyst formation within
the femoral neck


„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.


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

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


Differential Diagnosis
D/D unilateral Perthes‟ D/D bilateral Perthes‟
disease: disease:
– Transient synovitis – Hypothyroidism
– Septic arthritis – Multiple epiphyseal
– Sickle cell disease dysplasia
– Sickle cell disease


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.


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


Classification Instrument in Perthes (CLIPer)
Domains Description Score
7 to10/10 4
Pain with
4 to 6/10 2
ADL
0 to 3/10 0
Less than 50% of uninvolved side for the majority of directions 6
Hip ROM 50 to 75% of uninvolved side for the majority of directions 3
76 to 100% of uninvolved side for the majority of directions 0
Less than 50% of uninvolved side for the majority of muscle groups 6
Hip Strength 50 to 75% of uninvolved side for the majority of muscle groups 3
76 to 100% of uninvolved side for the majority of muscle groups 0
Pediatric balance score less than 50% of best score (best score=56)
4
OR SLS with eyes open less than 50% of time on uninvolved side
Pediatric balance score 50 to 75% of best score (best score=56)
Balance 2
OR SLS with EO of uninvolved side 50 to 75% length of time
Pediatric balance score 76 to 100% of best score (best score=56)
0
OR SLS with EO 76 to 100% of uninvolved side
NWB and uses an assistive device and without AD, displays excessive gait
4
deficits with decreased efficiency
Gait No assistive device & displays excessive deficits without a decrease in
2
efficiency. Uses step to pattern on stairs
Non-painful limp Able to perform reciprocal pattern on stairs 0
Total:

Rehabilitation Classification Phase
Score total 14 to 24: Severe Involvement
Score total 6 to 13: Moderate Involvement
Score total 0 to 5: Mild Involvement


Instructions for use
Upon examination, assess pain with ADL‟s,
hip range of motion, hip strength, balance,
and gait.
Assign a correlating score for each domain of
assessment based on examination results and
total the sum.
Place the patient in a rehabilitation
classification phase based on the total score
to guide physical therapy treatment.

Physical Therapy Management
Supervised PT with a customized written
home ex program in all phases of rehab.
It is recommended that the PT engage in
ongoing communication with the patient,
family, and referring physician regarding the
disease process & plan of care.
It is recommended to progress through the
phases of rehabilitation follow a goal based
rather than a time based progression.

Phases of Rehabilitation
Severe Involvement Phase (CLIPer score 14 to 24)
Moderate Involvement Phase (CLIPer score 6 to 13)
Mild Involvement Phase (CLIPer score 0 to 5)


Goals for Management
CLIPer score 14 to 24 CLIPer score 6 to 13 CLIPer score 0 to 5
• Reduce pain to < 7/10 • Reduce pain to < 4/10 • Reduce pain to 1/10 or
• Increase ROM to >50% • Increase ROM to > 75% of the less
of the uninvolved side uninvolved side • Increase ROM to
• Increase strength to • Increase strength to > 75% of >90% of the
>50% of the the uninvolved side uninvolved side
uninvolved side • Progress from use of an • Increase strength to >
• Patient to be assistive device if approved by 90% of the uninvolved
independent with the physician and without adverse side
appropriate assistive effects • Improve balance to
device and weight • Independence with a step to >90% of the maximum
bearing precautions pattern on stairs without UE Pediatric Balance Scale
• Improve balance to support score or single limb
>50% of the maximum • Improved efficiency in walking stance of the uninvolved
Pediatric Balance Scale • Improved balance to > 75% of side
score or single limb the maximum Pediatric Balance • Ambulation with a non-
stance of the uninvolved Scale score or single limb stance painful limp with
side. of the uninvolved side normal efficiency


Pain Management

CLIPer • Hot pack with stretching
score • Cryotherapy
14 to 24 • Medications as prescribed by the referring physician for pain

CLIPer
• Hot pack with stretching
score
• Cryotherapy
6 to 13
• Medications as prescribed by the referring physician for pain

CLIPer
• Hot pack with stretching
score
• Cryotherapy
0 to 5
• Medications as prescribed by the referring physician for pain


ROM Management
• Static stretch for LE musculature with or without hot pack
• Dynamic ROM & AAROM if muscle guarding due to pain
CLIPer and is unable to achieve end ROM with static stretch.
score • Perform AROM and AAROM following passive stretching to
14 to 24 maintain newly gained ROM .
• Stretching for hip – IR, ER, Abd, Extensor, & any other lower
extremity motion that is significantly limited
CLIPer
score • Same as above
6 to 13 • Dosage of may differ based on patient preference & comfort.

CLIPer
score • Same as above
0 to 5 • Dosage of may differ based on patient preference & comfort.


ROM cont…
Static Stretching Parameters –
– 2 minutes of stretching/day/muscle group
– 30 second hold time
– 4 repetitions per muscle group
– If not tolerated, may do 10 to 30 second hold
time with repetitions adjusted to meet 2 minute
requirement
• e.g. if holding 15 seconds, would do 8 stretches


ROM cont…
Dynamic Stretch Parameters –
– 5 second hold
– 24 repetitions per muscle group per day to meet
2 minute stretching time required

Done if patient does not tolerate static stretch


Strengthening Ex (CLIPer score 14 to 24)
Isometric Ex -> Isotonic Ex in gravity
lessened -> Isotonic Ex against gravity.
It is appropriate to include concentric and
eccentric contractions.
Begin with 2 sets of 10 to 15 rep of each ex,
progression to 3 sets of each exs.
– Note: If the patient is unable to perform 2 sets
of 10 rep, the difficulty of the ex is to be
decreased either through weight or type of ex.


Focus on strengthening of HIP (Abd + Flexors +
ER + IR + Extensors + or any other LE muscle
group that displays significant strength deficits).
Special attention to gluteus medius to min intra-
articular pain & for pelvic control during single
leg activities and ambulation .
Weight bearing Vs Non-weight bearing ex is
based on patient‟s tolerance to weight bearing
positions, and safety.


Closed chain double limb exercises with light
resistance (less than full body weight)
It is not recommended to perform single limb
closed chain ex on the involved side due to
increased intra-articular pressure in the hip
joint.


Isotonic Ex in gravity lessened -> Isotonic
Ex against gravity.
Include concentric & eccentric contractions.
Weight bearing and non-weight bearing
activities can be used in combination based
on the patient‟s ability and goals of the
treatment session.


Upper extremity supported functional
dynamic single limb activities may be
performed.
– e.g. step ups, side steps
Double limb closed chain ex may be used
with light resistance if weight bearing allows.
– e.g. mini-squats


Isotonic Ex in gravity lessened -> Isotonic Ex
against gravity.
Include concentric & eccentric contractions.
Functional dynamic single limb activities with
UE support as needed for patient safety may be
performed.
– e.g. step ups, sidesteps
Closed kinetic chain single limb exercises with
light resistance may be performed.
– E.g. leg press

Strengthening Ex Prescription
Special attention should be given to:
– Hip abductors (especially gluteus medius)
– Hip internal rotators
– Hip external rotators
– Hip flexors
– Hip extensors


Isometric Strengthening
Parameters –
– 10 sec hold + 10 rep/muscle gr, total = 100 sec.
– Can adjust hold time to 5 sec + 20 rep to meet
100 sec requirement
Intensity –
– Performed at approx. 75% maximal contraction
Performed with hip in neutral position


Isotonic Strengthening
Parameters –
– High repetitions (10 to 15 reps) and 2 to 3 sets
– Perform both concentric & eccentric contraction
– Low resistance
• Rest 1 to 3 minutes between sets
• Rest can include exercise of a different muscle group
or cessation of activity
If pt is unable to perform 2 sets of 10 rep, exercise
intensity should be decreased either through weight
or type of exercise

Balance training (CLIPer score 14 – 24)
If weight bearing status & symptoms allow –
– Activities that include double limb stance and a
narrowed base of support on stable surfaces may
be performed.
It is not recommended to perform single
limb activities due to increased intra-articular
pressure in the hip joint.


Balance training
(CLIPer score 6 – 13 & 0 – 5)
Same as previous stage
Limit prolonged single limb activities due to
excessive joint compressive forces


Gait training (CLIPer Score 14 – 24)
Follow the referring physician‟s guidelines for
weight bearing status.
Begin gait training with –
– Appropriate assistive device
– Weight bearing status as determined by the
referring physician or
– Based on the patient‟s tolerance to full weight
bearing due to pain or safety


Continue to follow the referring physician‟s
guidelines for weight bearing status.
Progress to gait training without use of an
assistive device as appropriate, focusing on
minimizing deficits and improving efficiency
of walking.
Stair negotiation and other functional
mobility.


Continue to follow the referring physician‟s
guidelines for weight bearing status
Progress to gait training without the use of an
assistive device as appropriate, focusing on
minimizing deficits and improving the
efficiency of walking.
Stair negotiation & other functional mobility.
Progress to walking on uneven surfaces with
an emphasis on safety.

Weight Relief
The load on the hip can basically be relieved
by the following methods:
– Bed rest
– Wheelchair
– Walking with crutches,
– Bracing devices (Thomas splint , Mainz orthosis,
etc.).


Petrie Cast


Broomstick Cast


Bracing


Discharge Criteria
Children may be discharged when 4 of the 5
following criteria have been met:
– Pain rating 0 to 1/10
– ROM 90 to 100% of the uninvolved side
– Strength 90 to 100% of the uninvolved side
– Balance 90 to 100% of the max score on the
Pediatric Balance Scale or maintaining balance with
SLS 90 to 100% of the uninvolved side
– Gait presents with a non-painful limp and uses a
reciprocal pattern on the stairs.

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


Operative Tx
If non-op Rx cannot maintain containment
Surgically ideal pt:
– 6-9yo
– Catterral II-III
– Good ROM
– <12mos sx
– In collapsing phase


Surgical Tx
Surgical options:
– Excise lateral extruding head portion to stop
hinging abduction
– Acetabular osteotomy to cover head
– Varus femoral osteotomy
– Arthrodesis


Varus Osteotomy


Head at risk signs
Clinical features: Radiological features:
– Progressive loss of – Lateral subluxation of the
movement femoral head (head partially
– Adduction contractures uncovered)
– Flexion in abduction – Entire femoral head
– Heavy child involved
– Calcification lateral to the
epiphysis
– Metaphyseal cysts
– Gage's sign


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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