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Paediatrics FCRS > Legg Calve Perthes disease > Flashcards

Legg Calve Perthes disease Flashcards

1
Q

What is Perthes disease?

A
  • Idiopathic AVASCULAR NECROSIS Of the PROXIMAL FEMORAL EPIPHYSIS IN CHILDREN
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2
Q

What is the epidemiology of perthes?

A
  • 1:6000 in scotland
  • 4-8 years most common
  • male: female ratio 5:1
  • more common in urban populations vs rural
  • bilateral in 12% pts- but NEVER at the same STAGE

risk factors

  • FHx
  • Low birth weight
  • abnormal birth presentation
  • delayed skeletal maurity and activity ++ ADHD
  • children exposed to second hand smoke
  • asian, inuit, central european decent
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3
Q

What conditions has Perthes been associated with?

A
  • ADHD- 33% cases
  • Dlayed bone age- 89%
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4
Q

What is the percentage of bilateral cases?

A
  • 12-15% but not at same stage of disease
  • *** think Multiple epiphyseal dysplasia MED- skeletal survey
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5
Q

Can you describe the pathology? Who described it?

A
  • WALDENSTROM
  • INTIATION - infarction-> smaller sclerotic epiphysis with medial joint space widening
  • FRAGMENTATION- head appears to fragment- result of revascularisation and bone resorption-> collapse and increase in density- lateral pillar classification based on this stage
  • REOSSIFICATION-ossific nucleus undergoes reossification as new bone appears as necrotic bone is resorbed- may last 18months
  • REMODELLING/HEALING- femoral head remodels-> Coxa Magna ( larger head), coxa planar ( flattened) coxa Breva( short neck), until skeletal maturity
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6
Q

Who described the lateral pillar classification? When it is determined?

A
  • HERRING!!
  • During the BEGINNING OF THE FRAGMENTATION STAGE by waldenstrom
  • usually at 6 months after onset of symptoms
  • has best interobserver agreement
  • designed to give prognostic information
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7
Q

What is the herring classification based on?

A
  • Height of the LATERAL pillar of the CAPITAL FEMORAL EPIPHYSIS on AP IMAGING OF THE PELVIS
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8
Q

Can you describe the herring/ lateral pillar classification

A
  • Group A- lateral pillar full height
  • Group B- maintains >50% height
  • Border B/C- lateral pillar narrowed 2-3mm or poorly ossified with approx 50% height
    • recently added to increase consistency and prognosis of classifciation
  • Grade C- <50% height of lateral pillar maintained
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9
Q

How does the grade determine prognosis ?

A
  • Group A- uniform good outcome
  • Group B- POOR OUTCOME w BONE AGE >6 yrs
  • Group C- POOR Outcome in all patients
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10
Q

What does a pt with perches present with?

A

Symptoms

  • Insidious onset
  • may cause PAINLESS LIMP
  • INTERMITTENT knee pain, hip, groin or thigh pain
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11
Q

What do you find on physical exam?

A
  • Hip stiffness with loss of INTERNAL ROTATION and ABDUCTION
  • TRENDELENBERG GAIT- head collapse-> decreased tension of abductors
  • ANTALGIC GAIT
  • LLD- LATE finding
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12
Q

What are the signs on radiograph?

A
  • AP and frog lateral
  • MEDIAL JOINT SPACE WIDENING - EARLY
  • IRREGULARITY of femoral head ossification
  • CRESENT SIGN- subchondral fracture!!
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13
Q

is bone scan useful?

A
  • Yes
  • can confirm suspected case of LCP
  • decrease uptake(cold lesion) can predict changes on radiograph
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14
Q

What happens in the histology?

A
  • Femoral epiphysis and physis exhibit areas of disorganised cartilage with areas of hypercelluarity and fibrillation
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15
Q

What is your differential dx ?

A
  • Multiple epiphyseal dysplasia
  • spondyloepiphyseal dysplasia
  • Sickle cell disease
  • Gaucher’s disease
  • Hypothyroidism
  • Meyers dysplasia
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16
Q

what are the tx aims? why?

A
  • Keep the femoral head contained
  • Maintain good motion
    • containment limits deformity and minimises loss of sphericity of the femoral head
    • Reduces subsequent degenerative changes
17
Q

what is tx for lateral pillar A or B age <8 ?

A
  • observe alone activity restriction and protected wb
  • during earlier stages until reossification is complete bracing and casting controversial - aim to keep hip mobile
  • all pts require clinical and radiographic follow up until completion of disease process
  • All children <8 yrs in pillar A/B did well REGARDLESS of tx
18
Q

what are good outcomes associated with ?

A
  • Good outcomes correlate with spherical head
    • 60% don’t require operation
    • good outcomes with lateral pillar A and caterall group 1
    • all patients will need clinical ad radiological follow up until completion of disease process
19
Q

What are the indications of surgery?

A
  • Lateral pillar B , B/C with children >8yrs ( bone age 6 years)
  • Lateral pillar C with child <8yrs
20
Q

What would surgery involve?

A
  • Femoral osteotomy
    • ​proximal varus osteotomy- to provide containment
  • pelvic osteotomy- salter, triple inominate, dega or pemberton, later Chiari/Shelf for salvage
21
Q

What are the outcomes of perthes?

A
  • Herring lateral pillar types A/B in age <8 did well regardless of tx
  • Types B / BC over 8 years had imporved outcomes with surgery
  • Type C had poor outcomes regardless of tx
22
Q

What is the prognosis of Perthes?

A
  • Important prognostic variables
    • age of patient at presentation- Bone age
    • Sphericity of femoral head & congruency at skeletal maturity ( Stulberg classification)
  • _Prognosis worse with _
    • age bone age >6 at presentation
    • female sex
    • decreased hip ROM- Abduction
  • prognosis improved with
    • Age bone age <6 at presentation
23
Q

What is the natural hx of Perthes?

A
  • Pt do well untiil 5th/6th decade of life in which degenerative changes of hip become present
  • approx 1/2 of pts develop premature OA 2ary to aspherical femoral head
24
Q

What did Cattrell describe?

A
  • A classification system based on degree of head involvement
  • Group 1- involvement of anterior epiphysis only to Group 4 - total head involvement. added at risk signs to indicate a more severe disease course
  • The 5 at risk signs which indicate a more severe disease course including
  • Gage Sign
    • radiolucency on hte shape of a V in the lateral portion of the epiphysis
  • Calcification lateral to epiphysis
  • Lateral subluxation of femoral head
  • Horizontal physis
  • Metaphyseal cysts

Paediatrics FCRS (27 decks)

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