Legg-Calve-Perthes Disease

By
Dr.AKSHAY
• FIRST DESCRIBED BY
LEGG, AND
WALDENSTORM IN
1909, AND BY PERTHES
AND CALVE IN 1910
BLOOD SUPPLY OF HEAD OF FEMUR
 DEFINITION

Legg-Calvé-Perthes
disease (LCPD) is the
name given to idiopathic
osteonecrosis of the
capital femoral epiphysis
in a child.
 Definition
• Perthes disease is a syndrome in which an
avascular event affects the capital epiphysis
(head) of the femur.
• Following the avascular event, growth of the
ossific nucleus stopes and the bone become dens.
• Dens bone subsequently resorbed and replaced
by new bone, during this the mechanical
properties of the femoral head are altered such
that the head tends to flatten and enlarge.
• Bilateral in 10-12 percent of the cases.

• Most prevelent in childern ages 4 to 12 years,

can be seen in childern from 18 months of age
to skeletal maturity.
 Increased Joint
space
Normal Smaller head
joint Denser head
 Epidemiology
• Prevalence:
Transient
synovitis
5.3% 2.8% 6.0%
SCFE 8.5%

Infection 77.4%

Perthes'
disease

Other
 Epidemiology
• Race: Caucasians are affected more
frequently than persons of other races.
• Sex: Males are affected 4-5 times more often
than females.
• Age: LCPD most commonly is seen in persons
aged 3-12 years, with a median age of 7 years.
 ETIOLOGY
• Coagulation abnormalities involving protein C
and S (abnormal lysis of intravascular clots is
likely the primary cause in some cases)
• Arterial status of the femoral head
• Abnormal venous drainage of the femoral
head and neck (venous outflow obstruction)
• Abnormal growth and development.
• Trauma particularly in the predisposed child.
• Hyperactivity or attention deficit disorder.
• Hereditary influences(genetic components)
• Environmental influences , particularly
nutritional factors.
• As a sequela of synovitis (synovitis may be the
first manifestation of the diseas but is rarely, if
ever, the cause of the disorder)
Causes
 UNIFYING HYPOTHESIS
• State that lack of thrombolysis in the venous
drainage of the neck increases pressure in the
femoral head circulation , resulting in AVN.
• Antecedent trauma might precipitate the
avascular event.
 Pathophysiology
• The capital femoral epiphysis always is involved. In 15-20% of patients
with LCPD, involvement is bilateral.

• The blood supply to the capital femoral epiphysis is interrupted.

• Bone infarction occurs, especially in the subchondral cortical bone,

while articular cartilage continues to grow. (Articular cartilage grows
because its nutrients come from the synovial fluid.)

• Revascularization occurs, and new bone ossification starts.

 Pathophysiology
• At this point, a percentage of patients develop LCPD,
while other patients have normal bone growth and
development.

• LCPD is present when a subchondral fracture occurs. This

is usually the result of normal physical activity, not direct
trauma to the area

• Changes to the epiphyseal growth plate occur secondary

to the subchondral fracture.
 Pathogenesis
Avascular necrosis

Temporary cessation of growth

Revascularization from periphery

Resumption of ossification and trauma

Pathological fracture

Resorption of underlying bone

Replacement of biologically plastic bone

Sublaxation

Deformity
 CLINICAL PRESENTATION
• Onset:between age 18 months and skeletal
maturity(most prevelent between 4 and 12
years of age)
• Boys 4-5 times more likely to develop the
disease.
• Bilateral in 10-12% of patients.
 SYMPTOMS
• Limp , first noticed by parents.
• Limp is exacerbated by sternous activity and
alleviated with rest.
• Pain , which may be located in the groin,
anterior hip region, or laterally around the
greater trochanter.
• Often there is a reffered pai to knee.
• Child may also be smaller in stature than peers.
 SIGNS
• Limp is normally a combination of an antalgic and
trendelenburg gait.
• Positive trendelenburg test on the involved side.
• Atrophy of gluteus, quadriceps, and hamstring.
• Muscle spasm.
• Loss of motion is noted at maximum abduction and
internal rotation.
• Log roll test will be positive.
• Severe cases adduction contractures develop.
 CLINICAL COURSE
• Waldenstrom defined the stages of the
disease:-4 stages
1. Initial
2. Fragmentation
3. Healing (reossification)
4. Residual phase
• Clinical findings correspond to some degree with the
radiographic stages of the disease.
1. Early initial stage-radiographs show only increased
density of femoral head, and the patient may experience
recurrent aggravation and alleviation of symptoms and
signs.
2. Later initial stage-a subchondral fracture is frequently
noted on radiographs (Salter’s sign)
3. Fragmentation stage- the femoral head starts to collpse
and may extrude from the acetabulum. The patients limp
and pain will be more pronounced , greater loss of range
of motion of the affected limb.Clinical symptoms and
signs progressively worsen throughout the fragmentation
phase.
4. Healing stage-radiographically there is
development of new bone in the subchondral
regions of femoral head. Pain and limp
usually started to resolve, but there is still
some limitation of motion.
Initial Stage
Initial Stage
Fragmentation Stage
Fragmentation Stage
Reossification Stage
Reossification Stage
 Workup
Lab Studies:
• CBC
• Erythrocyte sedimentation rate - May be
elevated if infection present
 Workup
Imaging Studies:
• Plain x-rays of the hip are extremely useful in establishing the
diagnosis.
• Frog leg views of the affected hip are very helpful.
• Plain radiographs have a sensitivity of 97% and a specificity of 78% in
the detection of LCPD
• Multiple radiographic classification systems exist, based on the extent
of abnormality of the capital femoral epiphysis.
– Waldenstrom, Catterall, Salter and Thompson, and Herring are the 4
most common classification systems.
– No agreement has been reached as to the best classification system.
 IMAGING STUDIES
• AP-view pelvis.
• Frog leg lateral.
• View films sequentially over the course of
disease.
• MRI
• Scintigraphy
• USG
• CT
 Radiographic Classifications
• Describe extent of epiphyseal disease
• Catterall classification= most commonly used
– 4 groups based on amount of femoral head
involvement
– Also presence of sequestrum, metaphyseal rxn,
subchondral fx
 Catterall classification

• Catterall Group I: Involvement only of the anterior epiphysis (therefore

seen only on the frog lateral film)
• Catterall Group II: Central segment fragmentation and collapse.
However the lateral rim is intact and thus protects the central involved
area.
• Catterall Group III: The lateral head is also involved or fragmented and
only the medial portion is spared. The loss of lateral support worsens
the prognosis.
• Catterall Group IV: The entire head is involved.
• Catterall's classification has a significant inter and intra observer error.
 Catterall classification
• Groups I and II had a good prognosis (in 90%)
and required no intervention.
• Groups III and IV had a poor prognosis (in 90
%) and required treatment.
• The classification is applied to the frog lateral
and AP film during the fragmentation phase
Group I
Group II
Group III
Group IV
 HEAD AT RISK
• Catterall also described 4 factors that he believed
could be used to predict prognosis.
1. Lateral subluxation of femoral head.
2. A radioluscent “V” in the lateral aspect of the
epiphysis(Gage’s sign).
3. Calcification lateral to the epiphysis.
4. Horizontal physeal line.
 The presence of these signs increased the
chances of a poor outcome.
 (Herring) Lateral Pillar Classification

• Lateral Pillar Group A: There is no loss in height of the lateral 1/3 of

the head and minimal density change. Fragmentation occurs in the
central segment of the head.
• Lateral Pillar Group B: There is lucency and loss of height in the
lateral pillar but not more that 50% of the original (contralateral)
pillar height. there may be some lateral extrusion of the head.
• Lateral Pillar Group C: There is greater than 50% loss in the height of
the lateral pillar. The lateral pillar is lower than the central segment
early on.
 Lateral Pillar Classification
• 3 groups:
– A) no lateral pillar
involvment
– B) >50% lat height
intact
– C) <50% lat height
intact
 Salter-Thompson Classification
• Simplification of Catterall
• Based on status of lateral margin of capital
femoral epiphysis
• Group A (Catterall I & II equivalent)
• Group B (Catterall III & IV equivalent)
 CLASSIFICATION OF END RESULTS
• Developed to address final outcomes. Are best
used to evaluate the skeletally mature patients.
1) The Mose classification:-based on fitting the
contour of the healed femoral head to a
template of concentric circles.
In good outcomes, the shape of femoral head
deviates no more than 1mm from a given circle
on both AP and frog-leg lateral radiograph.
• If the shape falls with in 2mm , it is considered
a fair outcome.
• If the deviation is more than 2mm, it is a poor
outcome.
1) The Stulberg classification:-reproducible
system in which hips are seprated into 5
groups.
• Group-1: shape of femoral head is basically
normal.
• Group-2:loss of head height occurs, but head’s
contour conforms within 2mm to a concentric
circle on AP & frog-leg lateral radiographs.
• Group-3:femoral head is more elliptical and
deviates from a circle by more than 2mm.
Contoure of acetabulum matches that of the
femoral head (congruous incongruity).
• Group-4:femoral head is flattened, with the
flattened area greater than 1cm in length. Contour
of acetabulum matches that of the femoral head.
• Group-5:collapse of femoral head, but
acetabular contour does not change
(incongruous incongruity). Appearance is
similar to that seen in adult avascular necrosis
in which there is collapse of central portion of
head.
 PROGNOSTIC RISK FACTORS ASSOCIATED
WITH A POOR OUTCOME
• Lateralization of the femoral head.
• Extent of uncovering of femoral head.
• The catterall classification.
• Lateral calcification.
• Lateral head displacement(using head to
teardrop distance).
• Widening of the femoral head before
fragmentation.
• Saturn phenomenon (a sclerotic epiphyses
surrounded by a ring of lucency).

• Widening of femoral neck in early stage of

disease.
 DIFFERENTIAL DIAGNOSIS
• Conditions producing AVN, as
haemoglobinopathies, leukemia, lymphoma,
ITP, or haemophilia.
• Hypothyroidism.
• Multiple epiphyseal dysplasia
• Spondyloepiphyseal dysplasia, and morquio’s
diseas if there is stronge family history of hip
abnormalities.
• Traumatic dislocation of the hip resulting in AVN.
• Uncommon conditions in which radiographic
findings are similar to those of perthes disease,
among them Maroteaux-lamy syndrome,
osteochondroma of femoral neck, multipal
osteochondromatosis, synovial
osteochondromatosis, schwartz-jampel
syndrome, and trichorhinophalangeal syndrome.
 TREATMENT
• The lateral pillar classification system coupled
with the age of the patient at onset of the
disease provides usefull prognostic
information.
• Current treatment philosophy is to treat
symptomatically those patients with group A
hips and group B hips in whome onset of the
disease occurred at skeletal age of 6 years or
less.
• Group A hips can be spared treatment as they
rarely have persistent loss of joint motion or
major symptoms.
• Initial management focus on pain relief, with a
reduction in activities and use of antiinflammatory
medications, and short periods of bedrest for
major episodes of pain or loss of joint motion.
• Group B hips in whome disease onset occurred
after skeletal age 6 and all with group C hips are
treated by containment once joint range of
motion has been achieved by symptomatic
means.
 SYMPTOMATIC THERAPY
• It is by bedrest and traction.
• NSAIDs for pain and discomfort and crutches
to reduce weightbearing.
• The benefits of bedrest are greatest around
the time of the subchondral fracture.
• In child’s home, simple longitudinal traction
using 5 pounds of weight can be attached to
bed.
• It may be necessary to use the traction at
night or it may be needed to be applied the
entire day for several days or weeks until
range of motion is regained.
• If the chiled is hospetalised, traction may also
be used to gradually abduct the affected leg.
• Elevated intra-articular hip pressure secondary
to synovitis has been reported to be highest
when traction is applied with the hip fully
extended and lowest when the hip is flexed
30-45 degree and rotated slightly externally.
• Partial weight bearing or nonweightbearing
can help alleviate pain and increase range of
motion. Crutches are used for this purpose.

• It can be difficult to keep a child on crutches

from weightbearing-a problem that lead to
development of the Snyder sling.
Non surgical containment
 Non surgical containment using orthotic
devices
• All braces abduct the affected hip, most allow
for hip flexion and some control rotation of
the limb.
• Before starting containment therapy it is
important to restore range of motion to the
irritable hip.
• Bedrest, traction and reduced weightbearing
are helpful.
• Throughout containment treatment it is vital
that range of motion be preserved, it is
difficult in sever disease.
• Petrie casts:-placing patients in casts with bars
between the legs so that the hips were
abducted to 45 degree and rotated internally
5-10 degree.
This treatment program was achieved through
traction or bedrest, with adductor tenotomy
performed when required.
• Every 3-4 months the casts were changed, with
the patient’s knees and ankles mobilized between
changes.
• Cast containment continued untill the femoral
head is well into the healing stage, average length
of treatment being 19 months.
• Cast treatment particularly employed when other
treatment are not effective.
• Also usefull in older patients whose range of
motion is too restricted and painful to allow
proper positioning of the femoral head for brace
wear.
PETRIE CAST
 Braces for containment
• Toronto brace:- very heavy and complicated,
was created to keep the hip abducted while
allowing hip and knee flexion.
• The Birmingham brace:- was created with the
goal of attaining perfect immobilization.
. The brace incorporated a leather corset that
extended from the nipple line to just above
the knee of the affected leg.
Toronto brace
• A kneeling bar and a chain kept the foot off
the ground, while a specially altered crutche
permitted clearance of the abducted,
internally rotated limb when the patient
walked.
• Tachdjian and jouett created a fairly simple
unilateral orthosis with an ischeal seat, but
the device lacked enough purchase to
maintain the irritable hip in proper position.
• Newington brace:- another unwieldy and
complicated device, incorporates a metal A –
frame with a central support for the thighs.
It also reproduces the hip position obtained
with Petrie casts.
Newington brace
 Atlanta Scottish Rite brace
• commonly used today.
• Consists of a metal pelvic band, hip hinges, thigh cuffs,
and an extensile bar between the thigh cuffs that
permits abduction of the limb but restricts adduction.
• With the hips abducted, the legs are usually flexed and
externally rotated when the patient walks.
• With this brace , many patients quickly regain the
ability to walk and run, and are able to return to
normal physical activities.
• If range of motion of hip is lost during
containment treatment, the patient was put
back in traction.

• The brace was worn 24hrs a day until there

was evidence of new bone formation on both
AP and lateral radiographs.

• Weaned from the orthosis, with average

length of treatment being 19 months.
Scotish Rite abduction brace
 SURGICAL CONTAINMENT
• Femoral Osteotomy:varus derotational femoral osteotomy
has been successfully used to contain the femoral head.
• 2 most common indications are onset of disease after 6
years of age and a hip with poor prognosis based on
radiographic findings.
• Surgery has not been found to improve outcome in
children younger than 6 years at onset of disease.
• Femoral osteotomy is recommended for hips that have ‘at-
risk’ radiographic signs, even if the head is not severly
deformed.
• Before femoral osteotomy is performed, it is
important that the patint regain a resonable
range of motion.
• Femoral osteotomy should be performed before
reossification of the femoral head begins.
• The extent of remodeling of the varus femoral
neck depends on the growth of the capital physis,
a factor difficult to predict.
• Some patients develop an abductor limp
following surgery, and trochantric epiphysiodesis
has been recommended when trochantric
overgrowth is likely.
• A postoperative neck shaft angle of 100 to 110
degree varus has also been recommended, as
femoral neck tends to grow back into valgus.

• The angle should never be less than 105

degree.

• To decrease the amount of trochantric

overgrowth, some surgeons performe an
epiphysiodesis of the greater trochanter at the
time of osteotomy or at plate removal.
Varus Osteotomy
 complications
• Excessive postoperative varus.
• Failure of the varus to remodel.
• Persistent external rotation of the limb.
following rotational osteotomy.
• Shortening of the extremity.
• Increased abductor lurch resulting in an
abductor limp.
• Trochantric overgrowth.
• The need to remove the fixation device.
• Fracture after removal of the plate.
• Delayed union or non union.
• Compared with innominate osteotomy, femoral
osteotomy has been found to cause greater
shortening of the limbs and to more frequently result
in abductor limp.
• Occasionally collapse of femoral head, with sever loss
of range of motion.
• Loss of external rotation and abduction in some cases.
• Sometimes a second osteotomy is required when first
operation does not provide adequate containment.
 Innominate Osteotomy
• First performed by Salter in1962.
• Preoperative prereqisites were minimam
deformity of the femoral head, non irritable
hipand no significant restriction of range of
motion.
• The hip had to be able to abduct to 45 degree
and the femoral head had to be contained in
that position.
 Indications
• Onset of disease after 6 years of age.
• A moderately or severely affected head.
• Loss of containment.
• The capsule should not be opned.

• The iliopsoas should always be lengthened.

• If the adductor muscles are tight, they should

always be lengthened.

• Postoperative casting is not necessary if the

osteotomy is secured with 2 or 3 heavy
threaded pins or screws.
• Kalamchi version of the Salter procedure,in
this the distal pelvic fragment is repositioned
into a notch created posteriorly in the
proximal side of the transected ilium.
• By doing so, the acetabulum is repositioned
without lengethening the pelvis, preventing
increased pressure on femoral head.
 Complication
• Loss of fixation with displacement of the distal
fragment.
• Lengthening of the leg.
• Decreased hip flexion.
• Joint stiffness.
• Hing abduction can also occur postoperatively.
 Combined femoral and innominate
osteotomy
• Indicated for severely affected hips at high risk
of a poor outcom.
• Indications for the procedure are the presence
of lateral subluxation, lateral calcification and
considerable changes in metaphyses.
• Provide greater coverage of the femoral head
than achieved with either procedure alone.
 Valgus osteotomy
• Initially recommended for treating hinge
abduction, in which the flattened femoral
head interferes with abduction. Operation is
performed if the head and acetabulum are
congruent when the joint is adducted but are
incongruent in a neutral or abduction position.
• Leades to improvement in gait.
• Improve roundness of femoral head.
• Healed central head fragmentation.
• Improved joint space, joint motion, and leg
length.
• Reduced subluxation
• Lessened pain.
 Shelf Arthroplasty
• Indications for the operation include lateral
subluxation of the femoral head, insufficient
coverage of the femoral head, or hinge
abduction of the hip.
• In this a deep notch is made above the
acetabulum to permit the acetabular roof to
be ‘pried down’.
 Complications
• Loss of hip flexion due to an excessively wide
augmentation graft.
• Inadequate hip coverage as the graft is too
thin.
• Dysesthesia of the lateral femoral cutaneous
nerve.
 Chiari Osteotomy
• Pelvic osteotomy
• Reserved for surgically treating the healing
femoral head that remains lateralized.
• In older childern with painful hip, significant
femoral head deformity, and incongruity
between the head and acetabulum.
 Cheilectomy
• It surgically removing protruding fragments of
femoral head(usually the anterolateral segment)
• Performed in the past.
• This procedure weakens the attachment of the
femoral head to the neck, which can result in
epiphyseal slippage.
• Postoperative joint stiffness.
• Should always be done after closure of capital
physis to avoid a slipped epiphysis.
 SUMMARY

• For patients less that 6 years old the

prognosis is good for the majority.
• If they are stiff or painful they respond to
bed rest, traction and pain relieving anti-
inflammatory medication.
• There is no evidence that abduction splints
or surgical intervention is warranted in the
majority of these younger patients.
 SUMMARY
• For patients between 6 and 8 years but with a bone age
less than 6 and an intact lateral pillar (Herring A and B)
the prognosis is similar to that for the first group and
observation is as good as surgical intervention for the
majority.
• If they have bone ages greater than 6 years and Herring
lateral pillar classification B then "containment" of the
head within the acetabulum seems to be warranted.
• This may be done by abduction bracing, femoral varus
osteotomy or a pelvic osteotomy.
 SUMMARY
• If they are between 6 and 8 and are in lateral pillar group
C then the result of intervention are equivocal.
• Children presenting with Perthes disease at age 9 or
older often have lateral pillar B or C and a poor
prognosis.
• The trend is towards early containment of these hips
although stiffness can be a problem following early pelvic
(Salter's) osteotomy.
 Prognosis
• The younger the age of onset of LCPD, the
better the prognosis.
• Children older than 10 years have a very high
risk of developing osteoarthritis.
• Most patients have a favorable outcome.
• Prognosis is proportional to the degree of
radiologic involvement.