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Legg calve-perthes disease
- 1. Legg-Calve-Perthes disease - Dr.Rajeev kumar Junior resident UPUMS,Saifai
- 2. Definition • A selflimiting 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 diseaseaffects 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 • Mutationin 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 • •Infantsand 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)
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- 8. Caffey’s hypothesis • Avascularnecrosis 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 • Malesex • Age 4to 8 yrs • Socioeconomic deprivation • Hypercoagulable states Weak • Urban population • Hip jt effusion • Passive smoking • Skeletal dysplasias • Cong. Anomalies
- 10. Clinical presentation • Age-4to 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 • Examinationmay 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 • Bilateralhip 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 • Septicarthritis • 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 ofthe 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 Perthesdisease (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 • Group1- 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
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- 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 GroupA - 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 atthe 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 inPerthes 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 MRIat 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.
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- 31. Treatment • Treatment ofPerthes 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 treatmentprotocol 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 • Containmentof 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 • Surgicalcontainment 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 varusosteotomy and fixation
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- 42. Salvage treatment • Salvageis 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 • Deformityof 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 isrelated 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.
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