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Legg Calve Perthes disease
- 2. Definition Legg-Calvé-Perthes disease is an idiopathic condition in which the blood supply of the capital femoral epiphysis is disrupted Epiphyseal osteonecrosis and chondronecrosis with cessation of growth of the ossific nucleus
- 3. Natural Course Necrotic epiphyseal bone is resorbed and replaced by new bone Coxa plana and Coxa Magna Remodels to a variable degree of roundness until skeletal maturity
- 4. History Arthur Legg onset 5 - 8 years of age, h/o trauma, a painless limp, minimal or no spasm, shortening of the affected limb United States Jacques Calvé minimal atrophy of the leg and no palpable hip swelling France Georg Perthes a self-limiting, non inflammatory condition, affecting the capital femoral epiphysis with stages of degeneration and regeneration, leading to restoration of the bone nucleus Germany
- 5. Epidemiology 12% 4-8 years 1 in 10000 4 : 1 Incidence Common age group affected Bilateral Involvement
- 6. Etiology Type II collagen alpha 1 chain (COL2A1) mutation Collagenopathy lower stimulated TPA activity thus leading to hypofibrinolysis and venous occlusion of femoral head vessels Passive smoking Abnormal growth and development, IGF Susceptibility in a child Protein C or S deficiency Factor V Leiden mutation Hypofibrinolysis Coagulopathy Mechanical loading of the hip joint and increased subclinical trauma Hyperactivity vascular interruption secondary to trauma Trauma
- 7. Blood supply Branches of the medial and lateral circumflex femoral arteries ascend on the posterosuperior and postero inferior parts of the neck of the femur and ascend in synovial retinacula Perforate the bone distal to the head of the femur, anastomose with branches from the artery of the ligament of the head of the femur and with medullary branches located within the shaft of the femur The artery of the ligament of the head of the femur, branch of the obturator artery
- 9. Metaphyseal vessels which penetrate the growth disc Lateral epiphyseal vessels running in the retinacula Scanty vessels in the ligamentum teres Pathogenesis 7 4
- 10. Ischemia and bone death Revascularization and repair Distortion and remodeling Stages
- 11. Pathogenesis of Femoral Head Deformity Following Ischemic Necrosis NORMAL ISCHEMIC VASCULAR REPAIR
- 12. Clinical features Signs Limp Abduction and internal rotation Limb length discrepancy Trendelenberg test positive
- 13. • Combination of an antalgic gait and a Trendelenburg gait • In the stance phase of gait, the patient leans the body over the involved hip to decrease the force of the abductor muscles and the pressure within the hip joint. Perthes limp
- 14. Diagnosis X-ray MRI Ultrasonography & Arthrography Scintigraphy & CT Clinical Presentation Physical Examination
- 15. • shrunken appearance of the bony nucleus • corresponding increase in depth of the cartilage space • patchy changes of density - fragmentation
- 16. • Femoral head is markedly flattened • Femoral neck is short
- 18. MRI
- 19. • Non-contrast T1 weighted image • Contrast-enhanced subtraction image • Lacking contrast enhancement in most of the epiphysis
- 20. Scintigraphy
- 21. • Epiphysis has retained its height • Less than half the nucleus is sclerotic • only the anterior portion of the epiphysis is affected. • More of the anterior segment is involved and a central sequestrum is present • Half the nucleus is sclerotic • some collapse of the central portion • Whole epiphysis is sequestrated • Ossific nucleus is flat and dense • Metaphyseal resorption is marked • Most of the epiphysis is sequestrated • Most of the nucleus is involved, with sclerosis, fragmentation and collapse of the head • Metaphyseal resorption may be present Catterall Classification
- 22. Metaphyseal lesion Calcification in the cartilage lateral to the ossific nucleus Radiolucent area at the lateral edge of the bony epiphysis (Gage’s sign) Horizontal physeal line and Lateral subluxation Head at risk
- 23. Salter-Thompson Classification Group B More than half of femoral head involvement Group A Less than half of femoral head involvement Extent of subchondral fracture present in the AP and lateral views of the femoral head
- 24. Lateral pillar classification Density change minimal No loss of height occurs Lucency observed loss of height up to 50% epiphysis Lateral pillar very narrow band of ossification Height more than 50% of the original height LP collapses to less than half its original height The LP is frequently lower in height than the central pillar A B B/C c
- 25. Stulberg Classification GROUP Femoral head collapsed, Acetabulum not flattened Femoral head ovoid, Acetabulum matches head Femoral head flattened more than 1 cm on weight- bearing areas, Acetabulum also flattened Femoral head round, within 2 mm of circle Same circle both views Femoral head normal 1 2 3 4 5
- 26. • Transient synovitis • Tuberculosis of hip • Meyers dysplasia • Other causes of Avascular necrosis • Infectious or inflamatory Toxic synovitis, Septic arthritis, Juvenile arthritis Differential Diagnosis
- 27. Treatment Goals Restore and maintain hip mobility To reduce hip irritability To prevent femoral head deformity To regain a spherical femoral head
- 28. Management Symptomatic treatment • Pain control, Restore motion • Traction, gentle exercise • Sport and strenuous activities are avoided. Containment • Active steps to seat the femoral head congruently and as fully as possible in the acetabular socket • Hips widely abducted, in plaster or in a removable brace • Surgical
- 29. Guidelines Children <6 years • No specific form of treatment has much influence • Symptomatic treatment Activity modification Children 6–8 years LP A and B (or Catterall stage I and II) symptomatic treatment LP C (or Catterall stage III and IV) – abduction brace Bone age at or <6 years LP A and B (Catterall stage I and II) abduction brace or osteotomy LP C (Catterall stage III and IV) outcome probably unaffected Bone age over 6 years Children > 9 years Except in very mild cases (which is rare) operative containment is the treatment of choice
- 30. Symptomatic therapy • Bed rest (with or without traction) • local rest by non–weight bearing on the affected hip Longitudinal traction “slings and springs” abducting the affected leg and restoring range of motion to an irritable hip The Snyder sling
- 31. • Hips in 45 degrees of abduction and 5-10 degrees of internal rotation -knees slightly flexed • Walks by using crutches in front and back Broomstick plasters- Petrie casts
- 32. • Ambulatory abduction orthosis • Thigh cuffs - a metal frame- knees are held fixed in 10 degrees of flexion • Shoes – footplates maintains the hips in relative internal rotation Newington orthosis
- 33. • Bobechko • Ambulatory abduction orthosis with crutches • Two thigh cuffs - a triangular frame- horizontal bars - plates are attached • Hips in 45 degrees of abduction and in internal rotation Toronto orthosis
- 34. • Typically for postoperative purposes • Without crutches • No rotational control Atlanta Scottish Rite Orthosis
- 35. • Kneeling bar and chain • Altered crutch- abducted, internally rotated limb to clear the body when the patient walks Birmingham brace
- 36. • Femoral Osteotomy • Innominate Osteotomy • Chiari Osteotomy • Shelf Arthroplasty • Hip Joint Distraction Surgical Containment
- 37. Varus osteotomy • Femur is abducted in the socket • Femoral shaft remains in the neutral position
- 39. Wainwright’s shelf acetabuloplasty Chiari’s pelvic displacement osteotomy
- 42. Thank you