3. Blood supply to epiphysis is
interrupted & the epiphysis
undergoes AVN.
Although d/s is self-limiting,
blood supply to epiphysis
gets restored eventually,
the consequences of the
disease can be permanent
& far reaching.
4. History - Legg-Calvé-Perthes Disease
• LCPD was independently recognized as a distinct entity in 1910 by
Arthur Legg of
United States
Jacques Calvé of
France
Georg Clemens
Perthes of Germany
Henning Waldenström
of Sweden
5. AETIOLOGY AND EPIDEMIOLOGY
Cause for the vascular insult is unknown
Genetic & several environmental factors have been
implicated.
Currently, some suggests that Perthes disease pts have a
disorder of clotting mechanism, with deficiency of factors -
protein C or S & factor V Leiden mutation.
6.
7.
8. Classification
• Waldenström classification determines the four stages all cases
follow:
• Initial—sclerotic epiphysis with joint widening (x-rays may not show
changes for 4-6 months)
9. Linear # in subchondral area of
femoral head - Known as Salters
sign or Caffey’s sign
10. • Fragmentation—due to bone
resorption and collapse (lateral
pillar classification based on this
stage)
12. • Healed or remodelling—continued remodeling until maturity
13. Clinical Features
Onset: b/w 18 mts of
age & skeletal
maturity (mostly b/w
4 & 8yrs)
Male prevalence:
4 or 5 times more
likely in boys than
in girls
• Involvement: B/L
in 10% to 12% of
pts
14. Symptoms:
- limp exacerbated by activity & alleviated with rest;
- pain, which may be located in groin,
proximal thigh,
knee, or laterally around GT
• possible history of antecedent trauma
15.
16. Signs:
- abductor limp;
- decreased ROM of hip, especially on
abduction & internal rotation
(decreased ROM transient early in the
disease, persistent later on);
- Flexion and extension less affected
17. Systemic Abnormalities of Growth & Development: The
“Predisposed Child”
• Delay in bone age relative to pt’s chronologic age is the most
commonly observed abnormality & is seen in early yrs of the disorder
The carpal bone
age is frequently 2
years or more
behind
the chronologic age
18. Children < 5 yrs age showed an increase in bone age delay over the following 4 to
5 yrs, whereas those diagnosed after 8 yrs of age had less delay over the ensuing
yrs.
A “radiologic pause” in bone age has also been observed, with particular carpals
affected more than others.
This phenomenon had previously been referred to as skeletal standstill.
Although growth of the triquetrum & lunate is significantly delayed, the capitate &
hamate are not affected.
19. ■ Increased incidence with a positive family history, low birth weight,
and abnormal birth presentation (associated with delayed bone age
and attention deficit hyperactivity disorder [ADHD])
22. Scintigraphy used to classify revascularization as either
recanalization of existing vessels (the A track or pathway) or
neovascularization, meaning new vessel formation (the B track or
pathway)
A track had a favorable prognosis, whereas patients on pathway B
generally had a poorer prognosis and often required surgical
intervention
23. USG
Sometimes used in early stages of LCPD
to demonstrate joint effusion and in
later stages to assess femoral head
shape.
- can 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
24. CT Scan
• CT can provide accurate three-dimensional images of the shape of the
femoral head and acetabulum.
• A classification system was developed based on CT findings
-In group A, only the periphery of the femoral head is affected;
- group B, there is considerable necrosis of the central portion of the
head but no posterior involvement; and
- group C, the entire head is affected.
25. MRI
• MRI is an accurate imaging modality
for the early diagnosis head and
acetabulum .
• The varying modalities of MRI, such
as perfusion and diffusion MRI and
delayed gadolinium-enhanced MRI
(dGEMRIC),may offer new insights into
the pathophysiology and
prognostication of this disorder
26. • Most prognostic classification is Herring classification, or lateral pillar
classification
• Based on involvement of lateral pillar of capital femoral epiphysis
during the fragmentation stage
27. Lateral pillar classification of Legg-Calvé-
Perthes disease
Normal pillars was derived by noting the lines
of demarcation between the central
sequestrum and the remainder of the epiphysis
on the anteroposterior radiograph
28.
29. • Surgical treatment may improve radiographic outcome at skeletal
maturity for older patients (chronologic age > 8 years or bone age > 6
years) with lateral pillar groups B and B/C hips
33. Changes in the Acetabulum
• Osteoporosis of acetabular roof
• Premature fusion of triradiate
cartilage ( bicomparmentalisation)
34. Catterall Classification
most of the
epiphysis is
“sequestrat
ed”
Total head
involvement
Anterior
epiphysis
only
involoved
Involvement of
anterior epiphysis
with central
sequestrum
35. head-at-risk signs
- lateral subluxation of the femoral head,
- A radiolucent V in the lateral aspect of
the epiphysis (the Gage sign),
- calcification lateral to the epiphysis, and
- a horizontal physeal line.
- The presence of these signs increased
the chances of a poor outcome
36. Salter-Thompson Classification
group A has less than half of
femoral head involvement
group B has more than
half of femoral head involvement
Based on extent of
subchondral #
40. Poor Prognostic Risk Factors
• extent of uncovering of the femoral head,
• Catterall group III or IV, lateral calcification,
• lateral head displacement (using the head-to-teardrop Distances)
• widening of the femoral neck in the early stages of the disorder.
41. Saturn phenomenon
Widening of the femoral
head before fragmentation,
the (a sclerotic epiphysis
surrounded by a ring of
lucency
45. Symptomatic Therapy
• The primary means of treating symptoms related to LCPD are 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–
weightbearing treatment.
• Short-term use of NSAIDs drugs for pain and discomfort may also be
of benefit.
• Prolonged use of these drugs is best avoided given that they may
negatively affect new bone formation
46. 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.”
• The use of broomstick plasters reportedly originated in
1929 by Parker marks the earliest therapeutic application of this
concept.
47. Maintains the hips in
approximately 45 degrees
of abduction and 5
to 10 degrees of internal
rotation with the knees
slightly flexed.
The patient walks by using
crutches in front and back
Broomstick plasters/ Petrie casts
49. Toronto brace, with its
universal joints, was
designed to keep the hip
abducted while allowing
hip and knee
flexion. Thus the patient
can both sit and stand
50. Evaluation of the Concept of Containment
Treatment
• To assess the dynamic effects of various containment devices, Rab
conducted gait analyses in children with LCPD.
• Use of Petrie casts resulted in an increase in anterior and lateral
coverage of the femoral head and a reduction in posterior and medial
coverage.
• By establishing hip flexion and external rotation of the limb, the
Atlanta brace provided greater posterior coverage than lateral
coverage.
51. • Using a “containment index,” Rab reported that containment increased
from a normal of 64% to 72% with the Atlanta brace but that the index
was unaffected with Petrie casts & Toronto brace.
58. Salvage
• Valgus femoral osteotomy for hinge abduction
• Chiari and/or shelf pelvic osteotomies for hips that can no longer be
contained
59. Conclusion
Because severity vary significantly from one pt to another, Rx decisions continue to be
difficult, & therapeutic methods applied to the disorder differ greatly among centers.
Some centers prefer to limit Rx only to interventions that maintain joint ROM.
Other centers apply nonsurgical & surgical containment methods to children believed
to be at risk for development of significant deformation of femoral head.
Still others use surgical containment to treat most children in whom the disorder develops
after 6 years of age, in the belief that aggressive Rx provides these pts with best chance of
a good outcome.
Legg described : onset b/w 5 & 8 years of age, a h/o trauma, a painless limp, and minimal or no spasm or shortening of the affected limb.
Calvé - 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.
Perthes observed the disorder as “a self-limiting, noninflammatory 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; however, in this early work, he thought the disease was a form of tuberculosis and not a distinct entity
various theories of causation have been proposed but there is insufficient evidence to prove any of them.
Disruption of blood flow --The ischemic damage produces cessation of growth of the epiphysis and increased calcium content of the necrotic bone, thus making it more brittle to accumulate microdamage with mechanical loading. With initiation of revascularization, a predominance of osteoclastic resorption is observed with delayed bone formation. The net bone loss further weakens the epiphysis and makes it more prone to deformation with loading. The fragmentation stage of the disease is marked by an imbalance of bone resorption and bone formation.
Seen In approx. one third of cases,, usually best seen on the frog-leg lateral view
The extent or breadth of the fracture is used to predict the degree of head involvement according to the classification system of Salter and Thompson.
AP radiograph -The central fragment remains dense and has collapsed relative to the lateral portion (lateral pillar) of the femoral head. The lateral pillar is lucent but has not collapsed, and the hip is classified as group B in the lateral pillar classification system. The joint space has widened further
AP radiograph obtained 17 months after onset shows early reossification of the femoral head (the healing stage). D, A closer view of the femoral head at 22 months after onset of disease. There is still widening of the joint space, and the acetabulum has a bicompartmental appearance.
Press next once
However, in b/l cases involvement is asymmetric & virtually never simultaneous.
• Bilateral involvement may mimic multiple epiphyseal dysplasia (MED) and warrants a skeletal survey.
a combination of an antalgic gait and a Trendelenburg gait. In the stance phase of gait, the patient leans the body over the involved hip to decrease the force of the abductor muscles & the pressure within the hip joint.
Positive Trendelenburg test result. A, As the patient stands with wt on normal hip, the pelvis is maintained in the horizontal position by contraction and tension of the normal hip abductor muscles. B, As the patient stands with weight on the affected hip, the pelvis on the opposite normal side drops as a result of weakness of the hip abductor muscles.
Hip abduction is best examined in extension with the patient supine. Dropping the uninvolved leg over the side of
the examining table helps stabilize the pelvis
a patient whose chronologic age was 6 years 6 months and whose bone age was 4 years. B, the same patient at a chronologic age of 12 years. The bone age was now 11 years 6 months.
Although it is clear that most patients with LCPD have characteristic bone maturation delays, how this delay relates to the pathogenesis of the disease is still uncertain.
The right hip (left) is normal. The anteroposterior view of the left hip (middle) shows lack of uptake in the lateral two thirds of the femoral head, and the frog-leg lateral view (right) shows lack of uptake anteriorly.
Magnetic resonance imaging (MRI) scan of a 9-year-old boy with Legg-Calvé-Perthes disease showing extensive involvement of the left hip. Areas of involvement on MRI often exceed those of plain radiographs and may overestimate diseas severity.
Growth arrest in patient with Legg-Calvé-Perthes disease. A, Anteroposterior (AP) radiograph of a 6-year-old boy with mild
involvement of the left hip. There is a dense area laterally in the metaphysis and epiphysis. B, AP radiograph of the boy at 8 years of age
shows an apparent bridge across the physis and growth disturbance of the femoral neck. C, AP radiograph at 10 years of age shows
further loss of growth of the upper femoral neck with a valgus tilt of the femoral head. D, AP radiograph at 13 years of age shows
trochanteric overgrowth and a short femoral neck with a valgus tilt to the femoral head
On plain xray - Bicompartmental acetabulum appears to be composed of 2 arc partly overlapping each other – interpreted as the subluxated femoral head articulating only with the lateral half of the acetabulum moulding it into 2 compartments
According to Catterall, patients in groups I and II had benign prognoses and
did not need medical intervention.. Patients in groups III and IV required treatment.
Catterall also described four “head-at-risk” factors that
he believed could be used to predict prognosis.
Lateral calcification (lateral to the epiphysis) (implies loss of
lateral support)
Lateral subluxation of the head. (implies loss of lateral support)
A horizontal growth plate. (implies a growth arrest
phenomenon and deformity)
based on the extent of subchondral fracture present in the AP and lateral views of the femoral head. It is a two-category system (group A or B):
There is an almost normal range of
flexion and extension but no available rotation or abduction.
Often the hip externally rotates with flexion and returns to a
neutral position with extension. This motion has been likened
to the movement of a cow’s hip, which has a similar shape.
Stulberg V result. The femoral
head is irregular, with hills and valleys, whereas the acetabulum has a smooth contour.
Stickler syn
A, Frontal view of an Atlanta Scottish Rite brace, which consists of a metal pelvic band, hip hinges, thigh cuffs, and an
extensile bar between the legs that permits abduction but restricts adduction. With the hips abducted, the legs are usually flexed and
externally rotated when the patient walks. B, shows one of the activities possible in the orthosis. The brace allows considerable mobility and enables many patients quickly to regain the ability to walk and run and to return to normal physical activities
Which segment of the head is covered by the acetabulum depends on the phase of gait as the joint moves or the femoral head position in relation to the acetabulum.
a wide abduction brace called an A-frame brace, used at home after school and at night time after the removal Petrie casts to maintain good hip abduction. An in-brace radiograph is obtained at the initiation of bracing to confirm that the affected femoral head is contained. This brace is to be used while the patient is recumbent
It is recommended that the brace is worn for 12 hours per day initially. If a good hip abduction is maintained, use of the brace can be decreased to 8 hours per day and then discontinued.
which has a kneeling bar and a chain to keep the foot off the ground while a specially altered crutch allows the abducted, internally rotated limb to clear the body when the patient walks. B, Side view of the Birmingham brace that shows one of the three padlocks that keep the child in the orthosis and ensure compliance.
The Newington brace incorporates a metal A-frame with a central support for the thighs. It also reproduces the hip position obtained with Petrie casts
