Legg+Calve+Perthes+Disease
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Legg Calve Perthes Disease
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History• FIRST DESCRIBED
BY LEGG AND WALDENSTORM IN 1909 AND BY PERTHES ANDCALVE IN 1910
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Definition• Legg-Calvé-Perthes
disease (LCPD) is the name given to idiopathic osteonecrosis of the capital femoral epiphysis in a child.
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Epidemiology• Disorder of the hip in young children• Usually ages 4-8yo• As early as 2yo, as late as teens• Boys:Girls= 4-5:1• Bilateral 10-12%• No evidence of inheritance
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• Prevalence:
77.4%
6.0%2.8%5.3%8.5%
Transientsynovitis
SCFE
Infection
Perthes'disease
Other
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Etiology• Unknown• Past theories: infection, inflammation,
trauma, congenital• Most current theories involve vascular
compromise▫Sanches 1973: “second infarction theory”
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Causes• Proposed theories.
▫Excessive femoral antiversion.▫Synovitis.▫Generalized skeletal disorder.▫Arterial anomalies.
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Pathogenesis• Histologic changes described by 1913• Secondary ossification center= covered by
cartilage of 3 zones:▫Superficial▫Epiphyseal▫Thin cartilage zone
• Capillaries penetrate thin zone from below
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• Epiphyseal cartilage in LCP disease:▫Superficial zone is normal but thickened▫Middle zone has
1) areas of extreme hypercellularity in clusters and 2) areas of loose fibrocartilaginous matrix
• Superficial and middle layers nourished by synovial fluid
• Deep layer relies on blood supply
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• Physeal plate: cleft formation, amorphis debris, blood extravasation
• Metaphyseal region: normal bone separated by cartilaginous matrix
• Epiphyseal changes can be seen also in greater trochanter, acetabulum
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Blood Supply
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Radiographic Stages• Four Waldenstrom stages:
▫1) Initial stage▫2) Fragmentation stage▫3) Reossification stage▫4) Healed stage
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Initial Stage• Early radiographic signs:
▫Failure of femoral ossific nucleus to grow▫Widening of medial joint space▫“Crescent sign”▫Irregular physeal plate▫Blurry/ radiolucent metaphysis
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Fragmentation Stage• Bony epiphysis begins to fragment• Areas of increased lucency and density• Evidence of repair aspects of disease
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Reossification Stage
•Normal bone density returns•Alterations in shape of femoral head and
neck evident
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Healed Stage• Left with residual deformity from disease and
repair process• Differs from AVN following Fx or dislocation
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Group I
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Group II
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Group III
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Group IV
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Lateral Pillar Classification• 3 groups:
▫A) no lateral pillar involvment
▫B) >50% lat height intact
▫C) <50% lat height intact
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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)
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Clinical Features• Stature usually shorter than peers• Quadriceps and gluteal muscle wasting is
common, Trandelenburg test positive (drop of the hip on the unsupported side)
• Acute phase; range of motion at the hip joint is limited due to muscle spasms
• Progressively; limited internal rotation and abduction is likely due to impingement lesions (hence the Roll test, guarding on affected side)
• Later stage; global reduction in all ranges of motion assoc. with pain, indicating joint arthritis
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• Age- 4 to 10 years, with peak incidence at 7• Gender- Boys (5:1 ratio) but it tends to be more
severe in girls• Height• Passive smoking or maternal smoking at pregnancy• ADHD? Increased physical activity• Family Hx of; skeletal dysplasias or thrombotic
disease• Ethnicity; more common in Whites, Eskimos,
Japanese• Social Hx- associated with low socio-economic status
Risk Factors
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Differential Diagnosis
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Workup• Technetium 99 bone scan -
Helpful in delineating the extent of avascular changes before they are evident on plain radiographs.
▫The sensitivity of radionuclide scanning in the diagnosis of LPD is 98%, and the specificity is 95%.
• Dynamic arthrography - Assesses sphericity of the head of the femur.
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• Ultrasonography may provide significant diagnostic clues to differentiate early Perthes' from transient synovitis.
T Futami, Y Kasahara, S Suzuki, S Ushikubo and T Tsuchiya Journal of Bone and Joint Surgery - British Volume, Vol 73-B, Issue 4, 635-
639
Ultrasonography in transient synvitis and early Perthes’ disease
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CT Scan
• Staging determined by using plain radiographic findings is upgraded in 30% of patients.
• Not as sensitive as nuclear medicine or MRI.
• CT may be used for follow-up imaging in patients with LPD.
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MRI• It allows more precise
localization of involvement than conventional radiography.
• MRI is preferred for evaluating the position, form, and size of the femoral head and surrounding soft tissues.
• MRI is as sensitive as isotopic bone scanning.
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Outcome variables
• Age
• Extent of involvement
• Duration
• Remodeling potential
• Premature physeal closure
• Type of treatment
• Stage of disease at treatment.
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Treatment Options
Overall goal of treatment1.Reduce hip irritability and pain2.Restore/maintain hip mobility3.Prevent femoral head from extruding or
collapsing “CONTAINMENT”4.Regain spherical shape of femoral head
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Below 6 years and Herring A/B• Mainstay of treatment would be to OBSERVE
with 6-12 month reassessment.• Patients in this age group need bed rest and
anti inflammatory medication at most. NO evidence that abduction splints or surgery beneficial
• Prognosis is good for the majority
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Non Surgical treatment1.NSAIDS2.Traction3.Casts and braces (Scottish Rite Orthosis)
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Above 6 and Herring class B• Containment of the head within the
acetabulum is warranted
This is achieved by;• Abduction bracing• Femoral varus osteotomy• Pelvic ostotomy
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Age between 6-8 and Herring class C• Results of intervention have been equivocal.
• Above 9 years1.Often have Herring class B or C2.Prognosis is poor3.Early containment is key, by pelvic
osteotomy and internal fixation
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Osteotomies
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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.
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• 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.
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• 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.
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Follow-up• Initially, close follow-up is required to
determine the extent of necrosis.• Once the healing phase has been entered,
follow-up can be every 6 months.• Long-term follow-up is necessary to
determine the final outcome.
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Complications
Femoral ▫Shortening▫stiffness▫Malrotation▫Limp▫Positive
trendelenburg
Pelvic▫Lenghtening▫Stiffness▫Chondrolysis▫Failure of
containment
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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.