Legg's calve perthes disease

A 7 years old male boy, school going presented with
her mother for pain in right groin region and limping
gait. He had difficulty in walking squatting and
climbing on stairs. Pain was sharp and increasing day
by day. Night pain was also disturbing the sleep.
Response to analgesic was non significant.

 Legg-Calvé-Perthes disease is a condition in which
an avascular event affects the capital epiphysis (head)
of the femur.
OR
 Idiopathic osteonecrosis of the capital femoral
epiphysis of the femoral head of unknown etiology.

 After the avascular event, growth of the ossific
nucleus stops and the bone becomes dense.
 The dense bone is subsequently resorbed and
replaced by new bone, and as this occurs the
mechanical properties of the femoral head are altered
such that the head tends to flatten and enlarge

 Legg-Calvé-Perthes disease was independently
recognized as a distinct entity toward the end of the
first decade of the 20th century by
 Arthur Legg of the United States,
 Jacques Calvé of France,
 Georg Perthes of Germany,
 Henning Waldenström of Sweden

Arthur Legg of
the United States
Jacques Calvé of
France,
Georg Perthes of
Germany
Henning
Waldenström of
Sweden

 In 1910, Leggs described the prominent
characteristics of the disorder: 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 noted that affected individuals had minimal
atrophy of the leg and no palpable hip swelling

 Perthes initially thought the condition was a youthful
variation of adult degenerative arthritis.
 Later, he defined 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; however, in this
early work, he thought the disease was a form of
tuberculosis and not a distinct entity.

Drawings by Georg Perthes, from his 1910
publication, of radiographic changes
describing a disorder he initially thought
was a youthful variation of adult
degenerative arthritis and which he
referred to as “arthritis deformans
juveniles

 In the early years, patients diagnosed with Legg-
Calvé-Perthes disease were treated with bed rest,
immobilization, and weight relief.
 To achieve the latter, the patten-bottom brace was
frequently used.

The patten-bottom brace, designed to
relieve the weight across the hip by
suspending the limb on an ischial seat. The
foot does not touch the end of the brace
and the other shoe is elevated to clear the
ground.

 The involved leg was hung from an ischial weight-
bearing caliper, which necessitated the patient's
wearing an elevated shoe on the other foot.

 Contemporary biomechanical concepts suggest that
the compressive forces of muscles operating across
the hip while the leg and brace are suspended
actually create greater intra-articular pressure than
that produced by ordinary weight bearing.

 It was not unusual for patients to be kept in
hospitals for 5 years or more for treatment,
during which time they would use specially
designed carts and gurneys to move about.

Carts specially designed to
allow children to move
about while in the
nonambulatory Newington
abduction frames

 The Snyder sling was
another popular
treatment device during
the 1950s.

 The disorder is most prevalent in children 4 to 12 years
of age, but it can be seen in children from 18 months of
age to skeletal maturity.
 It is more common in boys than in girls by a ratio of 4 or
5 to 1.
 The etiology of Legg-Calvé-Perthes disease is unknown,
but the disorder may be due to a silent coagulopathy in
some individuals.

 Factors That May Be Etiologic
Trauma
Susceptible child
Hereditary factors
Coagulopathy
Hyperactivity
Passive smoking
 Factors Unlikely To Be Etiologic
Endocrinopathy
Urban environment
Synovitis

 Some recent research has demonstrated a relationship
with a coagulopathy involving proteins C and S and
hypofibrinolysis.
 These studies suggested that abnormal lysis of
intravascular clots may be the primary cause of a
majority of cases of Legg-Calvé-Perthes disease.

 Onset: most prevalent between 4 and 12 years of age
 Male sex prevalence: the disease is four or five
times more likely to develop in boys than in girls.
 Involvement: bilateral in 10% to 12% of patients.

 Symptoms: limp that is exacerbated by activity and
alleviated with rest; pain, which may be located in
the groin, anterior hip region, or laterally around the
greater trochanter; history of antecedent trauma.

 Signs: abductor limp; decreased range of motion of
the hip, especially on abduction and internal rotation
(decreased range of motion transient early in disease,
persistent later on); flexion/extension less affected

 Clinical presentation, physical examination
 RTG- A-P, frog-leg lateral views (every 6 weeks
at the beginning, every 3-6 months later)
 USG- synovitis
 MRI, artrography

1. Ischaemia / Necrosis
2. Fragmentation / Resorption
3. Reossification / Healing
4. Residual stage

 Decreased size of
ossification center
 Lateralization of
femoral head
 Subchondral fracture
 Physeal irregularity

 Fragmented
epiphysis
 More irregular
acetabular contour

 New bone
formation- the
bone density
returns

 Reossified femoral
head
 Remodeling of the
head shape
 Remodeling of the
acetabulum

Stage 1:
 Antero-medial portion of head involved and no collapse,
metaphyseal changes do not occur and the epiphyseal plate is
not involved
 Heal without significant sequelae

Stage 2:
 More head involved and may - fragmentation of the
involved segment
 The involved segment shows increased density and
uninvolved pillars of normal bone prevent significant
collapse - regeneration without much loss of height and
the end result is usually good. Metaphyseal reaction
localised

Stage 3:
 More of the head involved - collapse as uninvolved pillars not
large enough t prevent collapse
 May show head within a head
 The metaphysis is usually diffusely involved - broad neck and
the epiphyseal plate is unprotected and also usually involved -
results poorer

Stage 4:
 Whole head involvement and severe collapse
occurs early and restoration of the femoral
head usually less complete
 The metaphyseal changes may be extensive
 The epiphyseal plate is often involved -
abnormal growth (coxa magna, coxa breva,
coxa vara and coxa valga)

 Lateral pillar
clasification
 Detrmine
treatment and
prognosis

 StageA: - Lateral portion of femoral
capital epiphysis present - less than 50%
head involved
 Stage B: - Lateral portion of femoral
capital epiphysis absent - more than 50%
head involved (Lateral margin of
epiphysis protects epiphysis from stress)

 If head conforms to a
single ring in both X-Ray
planes - good prognosis
 If head varies from
perfect circle by no
more than 2mm - fair
results
 If head varies by more
than 2mm in any plane -
poor results

 5-8 years ~19 degrees
 9-12 years ~25 degrees
 13-20 years 26-30 degrees

 Preservation of the roundness of the femoral
head and prevention of deformity while the
condition runs its course.

 Relieve weight bearing
 Achieve and maintain ROM
 Containment of the femoral epiphysis
within the confines of the acetabulum
(Petrie-style casts, Atlanta /Scottish Rite/
brace,Toronto brace and other orthotic
devices)

 Femoral osteotomy = varus +/-
derotation to reduce the degree of
anteversion & extension.
 Pelvic osteotomy (Salter, Chiari, Shelf) or
Femoral osteotomy have similar results

Legg's calve perthes disease

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Legg's calve perthes disease