Legg-Calve-Perthes disease is a self-limiting condition affecting the femoral head in children. It presents with limp and hip or knee pain in boys aged 4-8 years. Diagnosis is made based on x-ray findings of femoral head collapse and MRI evidence of avascular necrosis. Treatment depends on age and severity but involves containment of the femoral head through casting, bracing or surgery to prevent deformity and allow remodeling. The goal is to achieve a spherical femoral head and prevent osteoarthritis.

1. Legg-Calve-Perthes disease
- Dr. Rajeev kumar
Junior resident
UPUMS,Saifai

2. Definition
• A self limiting disease of the femoral head comprising of necrosis,
collapse, repair and remodeling that presents in the first decade of
life and is commonly seen in boys.

3. Epidemiology
• LCP disease affects 1 in 1200 children
• Boys 4-5 times more commonly affected than girls
• B/L Hip involvement-10%-15%
• Urban>Rural
• Incidence more in Asian/Europian than American/african

4. Etiology
• Idiopathic
• Mutation in type-2 collagen, abnormal; insulin like growth factor 1
and various type of thrombophilia.
• Role of thrombophilia as a cause of LCP disease is controversial. Some
studies reported 50%-75% association while other studies have not
shown any such association.
• Associated factors-
• A family H/O is found in 1.6%-20% of cases
• ADHD
• Pt are commonly skeletally immature with bone age delayed in 89%

5. Pathogenesis
TRUETA’S HYPOTHESIS
• •Infants and children <4 yrs : 2 arterial blood supplies to femoral head- the
retinacular or ascending cervical arteries (Lateral epiphyseal artery) & the
metaphyseal arteries
• •4-12 yrs : single areterial supply by Lateral epiphyseal artery. This solitary
blood supply makes this age period vulnerable to ischaemic necrosis due to
compression by external rotators and extreme movements.
• •After 12 yrs: foveolar arteries of Ligamentum teres contribute blood along
with Lateral epiphyseal arteries & incidence decreases. (In blacks it occurs
earlier)

6. Adult

7. Child

8. Caffey’s hypothesis
• Avascular necrosis of femoral head results from intraepiphyseal
compression of blood supply to the ossification center and not due to
external compression of vessel.

9. Risk factor
Strong
• Male sex
• Age 4to 8 yrs
• Socioeconomic deprivation
• Hypercoagulable states
Weak
• Urban population
• Hip jt effusion
• Passive smoking
• Skeletal dysplasias
• Cong. Anomalies

10. Clinical presentation
• Age-4 to 8 yrs(range-2-12)
• The onset is incidious with limp and pain in groin, hip, thigh or knee
regions(pain may be absent)
• H/O-recent or remote viral illness present

11. Physical examination
• Examination may reveal an abnormal gait (antalgic and/or
Trendelenburg).
• Limitation of hip motion depends on the stage of disease. ROM
testing often reveals decreased abduction and internal rotation. Hip
flexion contractures are seen rarely.
• Limb-length discrepancy, if present, is mild and a result of femoral
head collapse. Hip contractures may make the limb-length
discrepancy seem greater than it actually is.

12. Diagnostic tests
• Bilateral hip x ray-femoral head collapse and fragmentation ,
subchondral #
• CBC-normal
• ESR-may be increased in symptomatic phase
• CRP-may be increased in symptomatic phase
• Bone scientography- cold spot seen in affected hip
• MRI-Femoral head collapse and fragmentation

13. Differential diagnosis
• Septic arthritis
• Transient synovitis of the hip
• Juvenile idiopathic arthritis
• Tuberculosis hip
• Sickle cell disease

14. Septic arthritis
Differentiating sign/symptoms
• Acute onset
• Systemic symptoms present/L
involvement
• Acute pain in hip
• Marked restriction in movement
Differentiating test
• WBC-elevated
• Positive blood culture
• Widened jt space on x ray
• Jt effusion on USG
• Jt aspiration may confirm
infection

15. Transient synovitis of the hip
• Acute onset
• Prior H/O-viral illness
• Mild systemic feature
• U/L involvement
• Restricted motion with minimal
pain
• Spontaneous improvement over
24 to 48 hrs
• Normal WBC count
• Negative blood culture
• Normal x ray
• USG-jt effusion
• Jt aspiration sterile

16. Juvenile idiopathic arthritis
• Acute or chronic presentation
• Single or multiple jt involvement
• Systemic symptoms present
• Effusion of affected jt
• Recurrence of acute episode
• High WBC count in acute phase
• RA factor-positive
• Joint aspirate-shows white cells
• X ray-decreased jt space

17. Sickle cell disease
• Acute abdominal pain
• Systemic symptoms
• U/L involvement with asso. Long
bone osteomyelitis
• Sickling test positive

18. Stages of Perthes disease (waldenstrom)
Initial- Infarction produces a smaller, sclerotic epiphysis with medial joint space
widening
• Radiographs may remain occult for 3-6 months
Fragmentation- Femoral head appears to fragment or dissolve
• Result of a revascularization process and bone resorption producing collapse and
subsequent
increased density
• Hip related symptoms are most prevalent. Lateral pillar classification based on this
stage
Reossification - Ossific nucleus undergoes reossification as new bone appears as
necrotic bone is resorbed
- May last up to 18 months
Healing or remodelling- Femoral head remodels until skeletal maturity.
Begins once ossific nucleus is completely reossified trabecular pattern returns

19. Catterall classification
• Group 1- involvement of the anterior epiphysis only
• Group 2- involvement of the anterior epiphysis with a clear sequestrum
• Group 3- only a small part of the epiphysisis not involved
• Group 4- total head involvement
-Based on degree of head involvement
-At risk signs(indicate a more severe disease course)
1. Gage sign-V shaped radiolucency in the lateral portion of the epiphysis
and/or adjacent metaphysis
2. Calcification lateral to the epiphysis
3. Lateral subluxation of the femoral head
4. Horizontal proximal femoral physis
5. Metaphyseal cyst

21. Gage sign

22. Lateral subluxation

24. Salter-Thompson Classification
Class A ■ Crescent sign involves < 1/2 of femoral head
Class B ■ Crescent sign involves > 1/2 of femoral head
Based on radiographic crescent sign

25. Lateral pillar(herring) classification
Group A - Lateral pillar maintains full height with no density changes
identified
• Uniformly good outcome
Group B -Maintains >50% height
. Poor outcome in patients with bone age > 6 years
Group B/C Border- Lateral pillar is narrowed (2-3 mm) or poorly
ossified with approximately 50% height
• Recently added to increase consistency and prognosis of classification
Group C - Less than 50% of lateral pillar height is maintained
• Poor outcomes in all patients

27. • Determined at the beginning of fragmentation stage
• Usually occurs 6 months after the onset of symptoms
• Based on the height of the lateral pillar of the capital femoral
epiphysis on antero-posterior imaging of the pelvis
• Has best inter-observer agreement
• Designed to provide prognostic information
• Limitation is that final classification is not possible at initial
presentation due to the fact that the patient needs to have entered
into the fragmentation stage radiographically.

28. Imaging evaluation in Perthes disease
• In the past, diagnosis often was delayed because plain radiographic
changes are not apparent until 6 weeks or more from the clinical
onset of Perthes disease.
• Scintigraphy and MRI can establish the diagnosis much earlier.
• MRI seems to be superior to scintigraphy for depicting the extent of
involvement in the early or evolutionary stage of Perthes disease.
• Gadolinium-enhanced subtraction MRI (perfusion MRI) has been used
at the initial fragmentation stage to determine the extent of lateral
pillar involvement.

29. A, Perfusion MRI at initial disease showing lack of perfusion (black area) in most of the epiphysis except in gray area
in lateral aspect (right lower panel). B, Corresponding HipViasc images showing level of perfusion in epiphysis. Blue as
shown on color
scale indicates absence of perfusion.

30. Scintigraphy

31. Treatment
• Treatment of Perthes disease is highly controversial.
• Current treatment are largely based on containing the femoral head
in the acetabulum, which optimizes molding of the soft femoral head
and minimizes deformity of the head.
• The aim of the treatment is to achieve a spherical femoral head and
congruent joint to minimize the risk of osteoarthritis.
• Tt is based on the patient’s age at disease onset and severity of
femoral head involvement(Herring classification).

32. • Current treatment protocol for children age 4 yrs and older begins with
explaining to the parents the natural history and expected duration of the
disease(24 to 36 months)
• Children 2-3 years old can be observed and do not need aggressive
treatment. once synovitis resolves, a daily home physiotherapy including
active and active-assisted range of motion and muscle stretching exercise
to the hip and knee.
• Pt<6yrs of age without complete collapse of the lateral pillar can generally
be treated non-surgically.
• Pt>8yrs of age appear to benefit from surgically provided containment of
the femoral head.(particularly true for Herring B and B/C border grp)
• Pt with lateral pillar group C tend to do poorly regardless of treatment and
age at onset.
• Pt b/w 6 to 8 yrs age evidence is not clear regarding potential advantages
of a specific surgical procedure in lateral pillar classification.

33. Non-surgical treatment
• Containment of femoral head by casting or bracing with hip in
abducted and internal rotation position.
• Petrie casts and a variety of abduction orthoses.
• Protected weight bearing has also been recommended, especially
before the ossification stage.
• Use of bisphosphonates, which inhibit bone resorption and thereby
prevent deformity of the femoral head is under investigation.

36. Surgical treatment
• Surgical containment of the femoral head may be approached from
the femoral side, acetabular side or both, acc. to surgeon’s
preference.
• Containment of femur is achieved with a proximal femoral varus
derotational osteotomy.
• Containment of the acetabulum is achieved with a redirectional
osteotomy (salter, triple innomonate), acetabuloplasty(Dega,
pemberton), or acetabular augmentation procedure(shelf
arthroplasty).
• For improved outcomes, hips must be containable, i.e, they must
have a relatively full ROM with congruency b/w femoral head and
acetabulum.
• Hip arthrodiastasis(via external fixator)for 4 to 5 months has been
described in older children and active/severe disease.

37. Proximal femoral varus osteotomy and fixation

38. Innominate osteotomy

39. Lateral shelf acetabuloplasty

40. Valgus osteotomy

41. Hip arthrodiastasis

42. Salvage treatment
• Salvage is used when the hip has poor congruency or is no longer
containable.
• The goals of treatment are to relieve symptoms and restore stability.
-An abduction-extension proximal femoral osteotomy.
-chiari osteotomy,shelf acetabuloplasty and arthroplasty.

43. Residual deformities
• Deformity of the femoral head may result in femoroacetabular
impingemen t(FAI). FAI may be treated with surgical dislocation and
proximal femoral osteochondroplasty.
• An overriding GT and short femoral neck also may result in FAI. Mx
with intertrochanteric valgus osteotomy and surgical dislocation with
relative lengthening of the femoral neck.
• Possibility of proximal femoral physeal arrest requires monitoring of
leg lengths until skeletal maturity.

44. Outcome
• Prognosis is related to patient age at disease onset.
• Age younger than 6 yrs at disease onset is more predictive of a good
outcome.
• Deformity of the femoral head also correlates with long-term
outcome.
• The severity of this deformity and the degree of hip joint congruence
at maturity(as defined by stulberg) correlate with risk of premature
osteoarthritis.

46. THANK YOU