Legg?Calv?Perthes disease
Learning objectives
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It is a self limiting disorder of the hip
produced by ischemia and varying
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degrees of necrosis of the femoral head.It is a self limiting disorder of the hip produced by ischemia and varying degrees of necrosis of the
femoral head.
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Epidemiology
? Incidence : 1:1000
? Usual age : 4-8 years
? Boys:girls ? 5:1
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? Higher incidence in Caucasian, Chinese,Japanese, Inuits, Northern Europe
Aetiology-
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Coagulation disorders.Arterial status of femoral head.
Abnormal venous drainage.
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Abnormal growth and development.
Trauma.
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Hyperactivity or attention deficit disorder.Genetic component.
Environmental influences.
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As a sequel to synovitis.
Pathogenesis
? Ischemia of femoral head
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Up to 4 months
1. Metaphyseal vessels
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2. Lateral epiphyseal
3. Scanty vessels in ligamentum
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teres4-7 years
1. Lateral epiphyseal vessels
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2. Metaphyseal supply DISSAPEAR
Susceptible to ischemia,
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as it depend entirely onlateral epiphyseal vessel.
7 years
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1. Vessels in ligamentum teres have
developed
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Pathology-Stage 1-IschaemiaPathological process 3-4
years1 ? ischemia and bone
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death? Al /part of bony nucleus
of femoral head is dead
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? Cartilaginous part ?
remains viable and
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thicker? Thickening and edema of
synovium and capsule
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Stage 2 ? revascularization and repairStage 3 ? Distortion and remodeling
? Repair process
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- Rapid and complete :
shape is restored
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- Tardy : bony collapseand growth distortion
Clinical feature
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Symptoms? Typically male ? 4-8 years
? Painless limping ? continues for weeks or
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recur intermittently
? Pain in groin, thigh and knee ? activity related,
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relieved by restSigns
? Hip pain with passive range of movement
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? Reduced range of movement (abduction &
internal rotation)
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? Hip flexion contracture? Leg length discrepancy
? Mild muscle wasting ? thigh, calf, buttock
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? Tredenlenburg test ; positive
Investigation
? X-ray of both hips (AP & Frog lateral view)
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? Bone scan? CT scan ? follow up
? Arthrography : to see congruity, head deformity
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and determine method of treatment
? Blood inflammatory marker
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- FBC- ESR
- CRP
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X-ray
? Widening of joint space
? Sclerosis
? Necrotic phase : increase density of ossific nucleus
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? Fragmentation : alternating patches of density andlucency
? Lateral uncovering of femoral head
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? Acetabular remodellingWaldenstr?m classification based on
radiographic changes
Stage 1 ( increased density)
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- ossific nucleus smaller and denser
- subchondral fracture
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- radiolucencies in the metaphysisCaffey's sign
Subchondral fracture
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in the anterolateral
aspect of the femoral
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capital epiphysisProduces crescentic
radiolucency
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Waldenstr?m classification based onradiographic changes
Stage 2 (fragmentation
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and revascularization)
- lucency in epiphysis
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- pil ars aredemarcated
- metaphyseal changes
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resolve
-acetabular contour
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changeStage 3 (healing or reossification stage)
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- new bone formation- homogenous epiphysis
Stage 4 (remodel ing)
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- femoral head is reossified and remodels- acetabular remodel ing
Classification
According to radiologic stage of
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disease ?
Waldenstr?m classification
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According to prognostic outcome ?? Herring lateral pil ar
According to defining outcome ?
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Stulberg classification
The Herring lateral pil ar classification?
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lateral pil ar not>50% of height of
<50% of height of
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affected
lateral pil ar preserved lateral pil ar preserved
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Herring stage A
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Good prognosisHerring stage B
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Herring stage CPrognostic features
? Child under 6 years ? excel ent
? Age on higher side
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? Progressive loss of hip motion more so abduction? Obese child
? Progressive uncovering of the epiphysis
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Differential diagnosis? Multiple epiphyseal dysplasia
? Spondyloepiphyseal dysplasia
? Mucopolysaccharidoses
? Hypothyroidism
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? Other Causes of Avascular Necrosis? Sickle cell disease
? Steroid medication
? Sequela of traumatic hip dislocation
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? Treatment of developmental dysplasia of the hip? Septic arthritis
D/D-Hypothyroidism
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D/D-Multiple Epiphyseal dysplasia
Management-Principles
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1. Prevent deformity to femoral head before
remodel ing phase
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2. Restore and maintain ROM3. Concept of containment
4. Relief of symptoms
Guidelines to treatment
? Decision are based on :
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? Stage of disease
? Prognostic x-ray classification
? Age and clinical feature particularly range of
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abduction and extensionGuidelines by Herring (1994)
? Child <6 years(age at onset)
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? Symptomatic treatment-Rest/Analgesics
? Operative intervention-no added benefit
Age at onset 6-8 years
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Group B
Group A
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Uncontained/RestrictedSymptomatic
abduction
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Contained
Medial capsular
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releaseSurgical
Symptomatic
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Petrie casting for 6
containment
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weeksAge at onset 8-11 years
? Surgical treatment gives better outcome
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? In very early stage when fragmentation yet to
appear,do perfusion MRI
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? If severe ischaemic changes-Surgicalcontainment
? Group B,B/C-Surgical containment
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Symptomatic? Pain control
? Hospitalization for bed rest and short period
traction
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? Gentle exercise to maintain movement
Containment
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Harrison and Menon stated ;`if the head is contained within the acetabular
cup, then like jelly poured into a mold the head
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should be the same as the cup when it is
al owed to come out after reconsitution `
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Containment ? non operative
Containment ? surgical
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? Done before irreversible deformation of femoralhead occurs (early in fragmentation stage )
Varus 20
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Femoral VDRO osteotomies
Surgical
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Derotation 20-30containment
Pelvic
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Salters
osteotomies
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osteotomiesFemoral varus derotational osteotomy
Pelvic osteotomy
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Summary