Legg-Calvé-Perthes Disease of hip .pptx

Legg-Calvé-
Perthes
Disease
By: Dr Aman Dev Singh
PG Trainee
Department of
Orthopaedics
MODERATOR: Dr Ujjawal
Pradhan

DEFINITION
• Blood supply of the capital femoral epiphysis is disrupted, resulting in
epiphyseal osteonecrosis and chondronecrosis with cessation of
growth of the epiphysis.
SYNONYMS:
• Legg-Calvé-Perthes Disease
• Legg Stress fracture of femoral head
• Osteochondritis deformans juvenilis
• Osteochondrosis of hip joint
• Coxa plana
• Pseudocoxalgia

INCIDENCE & DEMOGRAPHY
• 1 in 10,000 cases
• Higher altitude.
• High incidence in the western coastal region of South India (14.4 per
100,000)
• 2 to 12 years of age (with a peak between 6 and 8 years).
• Bilateral involvement - 10% to 13%
• Boys to girl ratio - 5:1

HISTORY
• LCPD - distinct entity in 1910
Arthur Legg of the United States, Jacques Calvé of France, George
Perthes of Germany, and Henning Waldenström of Sweden.
• Legg – “onset between 5 and 8 years of age, a history of trauma, a painless
limp, and minimal or no spasm or shortening of the affected
limb”
• Calvé – “affected individuals had minimal atrophy of the leg and no palpable
hip swelling. He thought that the condition was a result of
abnormal or delayed bone formation.”

HISTORY
• Perthes observed the disorder as “a self-
limiting, non-inflammatory condition, affecting
the capital femoral epiphysis with stages of
degeneration and regeneration, leading to
restoration of the bone nucleus.”
• Waldenström reported the radiographic
changes associated with the disorder in 1909;
he thought the disease was a form of
tuberculosis and not a distinct entity

AETIOLOG
Y
• Coagulation Abnormalities
• Arterial Status of the Femoral Head
• Venous Drainage of the Femoral Head and
Neck
• Systemic Abnormalities of Growth and
Development: The “Predisposed Child”
• Hyperactivity or Attention Deficit Disorder
• Trauma
• Hereditary Influences
• Type II Collagenopathy
• Environmental Influences
• Synovitis

AETIOLOGY
Coagulation Abnormalities:
• Hemoglobinopathies such as sickle cell disease and thalassemia.
• Patients with CML, NHL, ITP and hemophilia.
• Deficiencies of proteins C and S and the presence of hypofibrinolysis.
• Resistance to activated protein C was the most common thrombophilic trait.
• Thrombophilia may cause venous thrombosis in the femoral head, with
venous hypertension and hypoxic bone death resulting in LCPD.

1. Vascular supply
2. Increased intra-articular pressure
3. Intraosseous pressure
- the venous drainage in the femoral head is impaired, causing an increase in
intraosseous pressure.
4. Coagulation disorder
- Associated with absence of factor C or S.
- Increase in serum levels of lipoproteins,thrombogenic substance.

5. Growth hormones
• Studies have shown reduced levels of growth hormones, somatomedin A and C.
6. Social conditions
• - Usually belong to lower socioeconomic status, reflects dietary and
environmental factors.
7.Trauma-the lateral epiphyseal artery which courses through a narrow
passage is susceptible to damage.

8. Abnormal growth and development
•Bone age is lower than chronological age by 1-3 yrs (radiological pause)
•- Usually shorter than their peers.
9. Genetic factors
- Inheritance 2-20%;inconsistent pattern.
- More Incidence of low birth weight, abnormal birth presentations.
• - First degree relatives have 35% more risk , 2nd and 3rd degree relatives
are 4 times more prone for perthes disease.

Blood supply
to femoral
head
 Retinacular arteries
 Metaphyseal arteries
 Artery of
ligamentum teres

Blood supply during growth:
 Infants
1. Medial ascending cervical or inferior metaphyseal arteries of trueta.
2. Lat epiphyseal
3. Lig teres – insignificant
 4 months – 4 years
1. Lat epiphyseal
2. Med epiphyseal decrease in
number.

 4 yrs to 7 years
• Epiphyseal plate forms a barrier to metaphyseal vessels.
 Pre-adolescent
• After 7 yrs arteries of lig teres become more prominent
• and anastomose with the lateral epiphyseal vessels

Truetta’s Hypothesis
 He postulated that the solitary blood supply in the age
group 4-8 yrs makes them susceptible to ischemia.
 Compression of Lat epiphyseal arteries by ext.rotators.
Trueta, J. (1957). THE NORMAL VASCULAR ANATOMY OF THE HUMAN FEMORAL HEAD DURING GROWTH.
The Journal of Bone and Joint Surgery. British Volume, 39-B(2), 358–
394. doi:10.1302/0301-620x.39b2.358

PATHOGENESIS
1) INCIPIENT OR SYNOVITIS STAGE
•lasts for 1 to 3 weeks.
•The synovium is swollen, hyperaemic
and edematous.
• Increase in proinflammatory
cytokines IL 6, joint fluid is
increased

•2) STAGE OF AVASCULAR NECROSIS
 lasts for 6 months to 1 year.
 It involves a portion of the ossific nucleus usually situated anteriorly or
involves the entire nucleus.
 The bone architecture remains normal but lacunae are vacant
 Bone trabeculae are crushed into minute fragments and compressed
into a compact mass.
 The gross appearance and contour of the femoral head remains
unchanged

3) ST
AGE OF FRAGMENTA
TION OR RESORPTION
 Lasts for 2 to 3 years and characterized by resorption of the necrotic bone
and replacement by viable bone.
 Subchondral fracture of necrotic bone result in multiple trabecular
fragments being compressed together

4) HEALED OR RESUDIAL STAGE
 The normal bone is forming along side and replacing slowly resorbing bone
 The newly formed bone is immature formed of slender trabeculae and early compressed
together with necrotic fragments
 The entire ossific nucleus may be deformed assuming
• mushroom shaped contour
 Finally, an enlarged femoral head (coxa magna) emerges varying in contour from a
normally spherical and concentrically lodged head to a deformed
• Flattened and eccentrically placed head

CLINICAL FEATURES
Patient’s History:
• Isolated incident of trauma (often a fall or twisting injury) several months
earlier, followed by the onset of a limp and hip pain.
• Initial symptoms normally resolve completely. The patient often goes
through periods of exacerbation and alleviation, with the symptoms waxing
and waning.
• The child may deliberately decrease his or her normal level of activity to
relieve pain.

CLINICAL FEATURES
SYMPTOMS:
 Most childern present with mild and intermittent pain in the thigh or a
limp or both.
 The onset of pain may be acute or insidious
 The classical presentation is described as a “painless limp” the
• child limps but does not complain of discomfort.
 Pain is aggravated by movement of hip and relived by rest.
 H/o of trauma usually a mild is present.

Examination
 Antalgic gait
 Muscle spasm secondary to irritable hip.
 Limitation of abduction and internal rotation
 Short stature
 Flexion deformity may be present
 DIFFERENTIAL ROTATION .
 TRENDELENBERG TEST POSITIVE

APPERANCE OF GREATER
TROCHANTER:-
• GT Large in some cases.
Since longitudinal growth of the
femoral neck may cease completely at 12
-14 years of age , whereas growth of the
greater trochanter continues until 17 -18
years, a discrepancy in growth neck and
the greater trochanter may result.
• The elevation impairs the power of
pelvitrochanteric abductor muscles,
manifested by positive trendelberg
test.

INVESTIGATION
• X-ray radiography
• USG
• MRI
• CT Scan
• Bone scan
• Arthrography

• A linear fracture line is noted in
the subchondral area of the
femoral head known as the
CRESCENT SIGN/ Salter Sign/
Caffey Sign
• Waldenström sign: increased
distance between pelvic
teardrop and femoral head seen

• A modified Waldenström staging divides each stage into early (denoted by letter “a”) and late (denoted by
letter “b”)
• Ia  increased density without loss of epiphyseal height;
• Ib  increased density and some loss of height;
• IIa  earliest fragmentation;
• IIb  more advanced fragmentation;
• IIIa  first appearance of new bone formation;
• IIIb  new bone formation with normal texture over one third or more of the epiphysis.
• IV  hips were fully healed.

• Median durations of the stages were approximately 4 months for Ia, 3 months for Ib, 4
months for IIa, 4 months for IIb, 7 months for IIIa, and 11 months for IIIb. Joseph et al.
found that most femoral head deformity and extrusion, metaphyseal widening, and
acetabular deformation occurred between stages IIb and IIIa.

Changes in the Metaphysis
• Metaphyseal necrosis to tongues of fibrillated cartilage
stretching deep into the femoral neck
• Hips with cystic changes were found to be twice as likely
to have poor outcomes as hips without cysts.
• The “sagging rope” sign: a radiodense line overlying the
proximal femoral metaphysis, edge of the rope the
anterior portion of the overlarge femoral head as it
projects over the metaphysis

• Associated with early closure of the triradiate cartilage.
• Acetabular retroversion is commonly found
• Positive Ischial spine sign.
• Positive cross over sign
• Posterior wall sign

MAGNETIC
RESONANCE
IMAGING
• Non-contrast MRI relies on signal
changes from fat present in the
epiphysis to detect AVN.
• The ischemic area appears as a
widespread absence of
enhancement, allows recognition
of early reperfusion patterns.
• De Sanctis and colleagues
proposed a classification based on
MRI findings of percentage of
head necrosis, lateral extrusion,
and physeal disruption.

ARTHROGRAPHY
o Indicated to know the contour of head and congruity
of articular surface
o Provides reliable information regarding containment.
o We can assess congruity of hip in many different
positions.
o Not routinely used .
o Arthrography is important only in the fragmentatory
and reparative stages
o used in the assessment of loss of hip containment and
hinge abduction of the hip, in which the femoral head
“hinges” out of the acetabulum when the hip is abducted

ULTRASONOGRAPH
Y
• Used in the early stages to demonstrate
joint effusion and in later stages to assess
the shape of the femoral head.
• Provide a good profile of the cartilaginous
femoral head (comparable with that of
arthrography) and allows subsequent
observation of deformation of the head
without the need for radiographs.

Bone Scan
 Indicated to diagnose in early stages and to classify the severity.
 Diagnosis possible months before signs appear on X- Ray.
 Avascular areas show cold spots.
 Revascularization can be detected much before radiographic
evidence.

COMPUTED
TOMOGRAPHY
 Not as sensitive as nuclear
medicine or MRI.
 CT may be used for
follow-up imaging in
patients with LPD.
• Help the clinician
distinguish the cause to be
an area of incomplete
reossification within the
femoral head or a true
osteochondrotic lesion.

Classification
Systems Based on
Radiographic
Findings
CATTERALL
CLASSIFICATION:
• Emphasizes extent of
head involvement and
outcome
• Applied during
fragmentation stage
when the necrotic
segment is demarcated
from the viable portion
GROUP % INVOLVEMENT FEATURES
GROUP I <25% • Only anterior part of epiphysis
involved
• No collapse
• No sequestrum
GROUP II 25-50% • More extensive involvement
• sequestrum
GROUP III 50-75% • Almost entire epiphysis involved
• Sequestered head within a head
• Broadening of femoral neck
GROUP IV >75% • Total epiphyseal collapse Epiphyseal
displacement
• mushroom deformity

Classification Systems Based
on Radiographic Findings
Catterall also described four “head-at-risk” factors to
predict prognosis.
• Lateral subluxation of the femoral head
• Radiolucent V in the lateral aspect of the epiphysis
(the Gage sign)
• Calcification lateral to the epiphysis
• Horizontal physeal line

Classification Systems Based on
Radiographic Findings
Salter-Thompson Classification:
• Based on the extent of subchondral fracture present in the AP and lateral views of
the femoral head correlates with subsequent extent of maximal resorption
• Subchondral fracture nearly always visible in early stages (first 4 months)
• Group A (<50% head involved = Catterall I & II)
• Group B (>50% = III & IV)
• Viable lateral margin (lateral pillar present)
• Good prognosis
• no lat. pillar to shield epiphysis
• Poor Prognosis

Lateral Pillar (HERRING) Classification:
• Based on radiographic changes in the lateral portion of the femoral head when it enters the fragmentation
stage, as seen on the AP view.
• At the beginning of fragmentation, there is frequent separation among the central, medial, and lateral
segments (“pillars”) of the femoral head.
• When the lateral pillar remains intact, it acts as a weight-bearing support to protect the central avascular
segment.

CLASSIFICATION
OF END RESULT
Mose Classification:
• based on fitting the contour of the healed
femoral head to a template of concentric
circle.
• In good outcomes, the shape of the femoral
head deviates no more than 1 mm from a
given circle on both AP and frog-leg lateral
radiographs.
• If the shape falls within 2 mm, it is
considered a fair outcome. If the deviation
is greater than 2 mm, it is a poor outcome.

Stulberg Classification:
• five groups based on the femoral head shape and
acetabular fit.
• In groups III and IV, the contour of the acetabulum
matches that of the femoral head (referred to as
congruous incongruity).
• In group V hips there is collapse of the femoral head but
the acetabular contour does not change (referred to as
incongruous incongruity)
• group I and II hips had a good long-term prognosis,
• group III, IV, and V had evidence of osteoarthritis in 58%,
75%, and 78%, respectively, in late adulthood.
• Patients with group V hips developed painful arthritis in
early adulthood.

POOR PROGNOSTIC FACTORS
These factors include
• Catterall group III or IV
• Lateral calcification
• Lateral head displacement (using the head-to-
teardrop distance)
• Widening of the femoral head before fragmentation,
the Saturn phenomenon (a sclerotic epiphysis
surrounded by a ring of lucency
• Widening of the femoral neck in the early stages of
the disorder

Prognostic Factors
1. Age at diagnosis
2. Extent of involvement
3. Sex
4. Catterall “head at risk” clinical signs
Clinical
5. Progressive loss of hip motion
6. Increasing abduction contracture
7. Obese child

Prognostic Factors
 Uniplanar methods
- CE angle of Weiberg.
- Salters extrusion Index.
- Epiphyseal index.
- Epiphyseal quotient.
 Biplanar methods
- Stulberg classification.

CE angle of Weilberg
 Indicator of acetabular depth It is the angle formed
by a perpendicular lines through the midportion 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

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

TREATMENT
Concept of Containment treatment:
• Harrison and Menon stated that “if the head is contained within the acetabular cup,
then like jelly poured into a mold the head should be the same shape as the cup
when it is allowed to come out after reconstitution.”
• Treatment needed to be started while the femoral head was still soft but before it
became deformed.
• When the hip was maintained in a neutral or adducted position, the femoral head
became deformed.
• When the hip was in flexion and abduction and weight bearing was permitted, the
acetabulum acted as a mold and the femoral head did not deform.
• This was termed as “BIO PLASTICITY"

TREATMENT
Current treatment approach during active disease:
• based on the age of the patient, the stage of the
disease.
• lateral pillar classification system coupled with
the age of the patient at onset of disease
• at the initial stage or early stage of
fragmentation when lateral pillar classification
cannot be assigned accurately, decision based
on the age of the patient and the extent of
femoral head necrosis.
• Categorize patients into four age groups : before
6 years, 6 to 8 years, 8 to 12 years, and after 12
years.

Age at Onset Before 6
Years:
• Focuses on pain relief, with a reduction
in activities and short-term use of anti-
inflammatory medications
• Short periods of bed rest for major
episodes of pain or loss of joint motion.
• Operative treatments (osteotomies)
have been shown to have no added
benefit to the outcome in this age
group.

Age at Onset 6 to 8 Years:
• Symptomatic - 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:
• Operative treatment is superior to non-operative treatment for this age
group.
• Early femoral varus or innominate osteotomy is considered in this age group
if a majority of the femoral head shows hypoperfusion.
• Longer duration of protected weight bearing postoperatively advocated.
• Application of Petrie casts for 6 weeks to contain, the femoral head, followed
by a prolonged use of a wide abduction brace (A-frame brace) at night to
maintain good hip abduction.
• Non–weight bearing on the affected hip is advocated and the bracing is
discontinued when the femoral head enters the stage of reossification.

Age at Onset After 11 Years:
• Success rate is less predictable regardless of the treatment method.
• Poor healing and remodeling potential.
• Multiple epiphyseal drilling, core decompression, and vascularized fibular
grafting, are being tried

Preferred Treatment Approach for the Fragmentation Stage:
• Symptomatic treatment for children with onset after eighth birthday, lateral
pillar group A
• Surgical treatment for children with onset after eighth birthday, if they
present with lateral pillar groups B and B/C border
• Non-operative containment treatment for children with onset after eighth
birthday who present with lateral pillar group C
• Non-operative treatment choices: prolonged non–weight bearing, Petrie
casts, and wide abduction (A-frame) brace
• Surgical choices: femoral varus osteotomy, Salter innominate osteotomy,
both osteotomies combined (onset after age 9 years)

Preferred Treatment Approach for the Healing or Healed Stage:
• Late measures:
1. femoral valgus osteotomy for established head and acetabular flattening,
adducted hip, short leg gait
2. surgical hip dislocation with possible trochanteric advancement and
osteochondroplasty for impingement and labral disorders.
• Mechanical symptoms:
1. hip arthroscopy with removal of osteochondrotic fragment

Symptomatic Therapy:
• Bed rest (with or without traction) or local rest
by non–weight bearing on the affected hip.
• A wheelchair, crutches, or a walker is prescribed
for the non weight bearing treatment.
• Short-term use of nonsteroidal anti
inflammatory drugs for pain and discomfort.
• simple longitudinal slings and traction using 5
lb of weight can be used
• Traction may also be used gradually to abduct
the affected leg.

TREATMENT
• Elevation of intraarticular hip pressure secondary
to synovitis has been reported to be highest
when traction was applied with the hip fully
extended and lowest when the hip was flexed 30
to 45 degrees and rotated slightly externally.
• Toe-touch weight bearing or non–weight bearing
can help alleviate pain and increase range of
motion

Nonsurgical
Containment Using
Orthotic Devices:
• All braces abduct the affected hip,
most allow for hip flexion, and some
control rotation of the limb.

Surgical Containment:
1. FEMORAL OSTEOTOMY-
• Children older than 8 years of age at onset who developed lateral pillar B or
B/C border hip in the fragmentation stage.
• Important to regain a reasonable range of motion before the procedure with
the help of Petrie casts or preoperative traction.
• Performed in the increased density or early fragmentation stage.

FEMORAL OSTEOTOMY-
•COMPLICATIONS
• Excessive postoperative varus or failure of the varus to remodel
• Persistent external rotation of the limb after rotational osteotomy,
• Shortening of the extremity,
• Increased abductor lurch resulting in an abductor limp,
• Trochanteric overgrowth,
• Need to remove the fixation device, fracture after removal of the
plate
• Delayed union or non-union.

TREATMENT
2. INNOMINATE OSTEOTOMY:
• Onset of disease after 6 years of age, a moderately or severely affected head, and
loss of containment.
• PRE-REQUISITES
• COMPLICATION
• Non-irritable hip
• No significant restriction of range of motion. In addition, the hip
had to be able to abduct to 45 degrees
• Femoral head had to be contained with the hip in that position
• Loss of fixation with displacement of the distal fragment
• Lengthening of the leg
• Decreased hip flexion
• Joint stiffness.
• Hinge abduction

TREATMENT
2. INNOMINATE OSTEOTOMY:

TREATMENT
3.Combined Femoral and Innominate Osteotomy:
• recommended for severely affected hips at high risk of a poor outcome.
• Indications:
4. Valgus Osteotomy
• If the head and acetabulum are congruent when the joint is adducted but are
incongruent in a neutral or abducted position.
• Benefits:
• presence of lateral subluxation
• lateral calcification
• considerable changes in the metaphysis.
• improve roundness of the femoral head
• healed central head fragmentation
• improved joint space, joint motion, and leg length
• reduced subluxation
lessened pain

TREATMENT
5. Shelf Arthroplasty:
• used to improve the acetabular coverage of the femoral head.
• Indications:
• Complications:
• lateral subluxation of the femoral head
• insufficient coverage of the femoral head
• hinge abduction of the hip
• loss of hip flexion secondary to an excessively wide
augmentation graft
• inadequate hip coverage because the graft was too thin
• dysesthesia of the lateral femoral cutaneous nerve

TREATMENT
6.Hip Joint Distraction With External Fixator:
• Used for patients who have not responded to other treatment measures.
• Protective effect on the femoral head
• Restorative effect on the femoral head height when applied at the fragmentation
stage.
7. Chiari Osteotomy:
• Reserved for surgically treating the healing femoral head that remains lateralized.
• Recommended for use in older children who present with a painful hip, significant
femoral head deformity, and incongruity between the head and acetabulum as
demonstrated on arthrography

REFERENCES
• TACHDJIAN'S PEDIATRIC ORTHOPAEDICS 5th
edition.
• Turek’s Orthopaedics Principles & Their Applications.
• Apley & Solomon’s System of Orthopaedics and Trauma
• De Sanctis N. Magnetic resonance imaging in Legg-Calvè-Perthes disease: review of
literature. J Pediatr Orthop. 2011 Sep;31(2 Suppl):S163-7. doi:
10.1097/BPO.0b013e318223b575. PMID: 21857432.
• Werner CM, Copeland CE, Ruckstuhl T, Stromberg J, Turen CH, Kalberer F, Zingg PO.
Radiographic markers of acetabular retroversion: correlation of the cross-over sign,
ischial spine sign and posterior wall sign. Acta Orthop Belg. 2010 Apr;76(2):166-73.
PMID: 20503941.

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