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Perthes Perthes Disease Disease

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  • 1. Perthes Disease
  • 2. Definition It is a self limiting disorder of the hip produced by ischemia and varying degrees of necrosis of the femoral head.
  • 3. Synonyms Coxa Plana Pseudocoxalgia (Calve) Arthritis deformans juvenilis (Perthes) Osteochondroses of the hip.
  • 4. Arthur Legg of the United States
  • 5. Jacques Calve of France
  • 6. Georg Perthes of Germany
  • 7. Historical aspect Parker started the use of broomstick cast in 1929. Eyre-Brook introduced traction in bed for 18-24 months.
  • 8. Blood supply to femoral head Retinacular arteries Metaphyseal arteries Artery of the round ligament
  • 9. Blood supply to femoral head Infants 1. Metaphyseal arteries . 2. Lat epiphyseal arteries 3. Lig teres insignificant 4 mts 4 years 1. Lat epiphyseal 2. Metaphyseal art. decrease in number (due to appearance of growth plate).
  • 10. Blood supply to femoral head 4 yrs to 7 years 1. Epiphyseal plate forms a barrier to metaphyseal vessels. Pre-adolescent 1. After 7 yrs arteries of lig teres become more prominent and anastomose with the lateral epiphyseal vessels.
  • 11. Blood supply to femoral head Adolescent After skeletal maturity metaphyseal vessels again come into picture
  • 12. Incidence Male : Female = 4-5:1 2.5:1 in India Age of onset earlier in females. Age Range 2-13 years. Most common 4-8 years. Average 6 years. Bilateral in 10-12 % Incidence more in Caucasians as compared to Negroid, mongoloid.
  • 13. Etiology Etiology not known. Coagulation disorders. Altered arterial status of femoral head. Abnormal venous drainage. Abnormal growth and development. Trauma. Hyperactivity or attention deficit disorder. Genetic component. Environmental influences. As a sequelae to synovitis.
  • 14. Coagulation disorders Protein C or S deficiency Thrombophilia Hypofibrinolysis
  • 15. Altered arterial status Angiographic studies have shown obstruction of superior capsular arteries and decreased flow in medial circumflex femoral arteries . The intracapsular ring has been found to be incomplete.
  • 16. Abnormal venous drainage Increased venous pressure in the femoral neck Congestion in the metaphysis Venous outflow exits more distally in the diaphysis.
  • 17. Abnormal growth and development A delay in Bone age of 1.5 to 2 years Low birth weight Low levels of somatomedin C
  • 18. Trauma. In the developing femur (4 7 yrs),the major lateral epiphyseal vessels must course through a narrow passage ,which could make it susceptible to trauma.
  • 19. Hyperactivity or attention deficit disorder
  • 20. Genetic component Familial association. X-Linked recessive inheritance.
  • 21. Environmental influences Low socioeconomic status.
  • 22. Sequel to synovitis Synovitis of the hip occurs early in Perthes disease. Increased pressure in synovitis may cause a tamponade effect on the vasculature
  • 23. Pathogenesis Waldenstrom staged the pathological process of the disease as2. Initial or ischaemic stage3. Resorption or fragmentation stage4. Reparative stage5. Remodelling stage
  • 24. Pathogenesis Ischaemic stage - Necrosis - Crushing of trabaculae. - degeneration of basal layer of articular cartilage - Thickening of peripheral cartilagenous cap. - Shape of head maintained.
  • 25. Ischaemic stage
  • 26. Pathogenesis cont Resorption stage - Invasion of vascular connective tissue. - Resorption of dead bone by Osteoclasts. - loss of epiphyseal height due to 1) Collapse of bony trabaculae. 2) Resorption of dead bone
  • 27. Resorption stage
  • 28. Pathogenesis cont Reparative stage - pathological fracture. - creeping substitution and apposition of viable bone in dead trabaculae.
  • 29. Reparative stage
  • 30. Remodelling stage (replacement by biologically plastic bone) If treated If untreated Femoral head is Subluxation and congrous deformity
  • 31. Clinical Features Painless limp leads to painful limp Pain in the groin,anterior hip or greater trochanter Referred pain to the knee Combination of antalgic & trendelenburg gait.
  • 32. Decreased range of motion especially abduction and internal rotation. Atrophy of thigh muscles. Shortening
  • 33. Investigation X-Ray AP & Frog leg Lat view (Lowenstein view) USG Arthrography Bone Scan MRI
  • 34. X-Ray Ossific nucleus smaller
  • 35. X-Ray Cresent Sign or Salters sign or Caffeys sign
  • 36. X-Ray Increased Radio opacity of femoral head due to collapse, new bone formation and calcification of dead marrow.
  • 37. X-Ray Fragmentation of epiphysis
  • 38. X-Ray Metaphyseal widening and cystic changes in femoral neck
  • 39. X-Ray Lateral extrusion of femoral head. Hinged abduction.
  • 40. X-Ray Sagging rope sign in adults with history of perthes
  • 41. Ultrasound Synovial effusion Cartilage hypertrophy in early stages
  • 42. Arthrography Shows configuration of the femoral head and its relation with the acetabulum. Containment Congruity Not routinely used .
  • 43. Bone Scan Diagnosis possible months before signs appear on X-Ray. Avascular areas show cold spots.
  • 44. Bone Scan Convay et al classification Stage 1 is total lack of uptake
  • 45. Bone Scan( stage 2) Revascularisation of a lateral column Failure to revascularise at lat column is a grave sign Also called scintigraphic head at risk sign Precedes radiographic head at risk sign by 2-3 mths
  • 46. Bone Scan( stage 3) Gradual filling of anterolateral part
  • 47. Bone Scan( stage 4) Return to normal
  • 48. MRI Accurate in early diagnosis. Shows congruity,containment,synovial hypertrophy well.
  • 49. Classification Waldenstroms classification. Catterall classification Salter classification Herrings lateral pillar classification.
  • 50. Waldenstroms classification (Pathological classification)
  • 51. Catterall classification (based on x ray AP and Lat view). I only anterior portion of epiphysis affected. II anterior segment involved central sequestrum present III most of epiphysis sequestered with unaffected portions located medial and lateral to central segment IV all of epiphysis sequestered.
  • 52. Salter Classification Type A = I & II Catterall Type B = III & IV Catterall.
  • 53. Herring Lat Pillar Group-A no involvement of the lateral pillar, with no density changes and no loss of height of the lateral pillar Group-B hips have lucency in the lateral pillar and may have some loss of height , but not exceeding 50% of the original height. Group-C hips are those with more lucency in the lateral pillar and >50% loss of height
  • 54. Prognostic Factors1. Age at diagnosis 9 yrs - poor5. Extent of involvement6. Sex7. Catterall head at risk signs
  • 55. Catterall head at risk signs Clinical Radiographic
  • 56. Clinical2. Progressive loss of hip motion more so abduction.4. Obese child
  • 57. Radiographic3. Gage sign5. Calcification lateral to epiphysis7. Diffuse metaphyseal rarefaction9. Lateral extrusion of femoral head11. Growth disturbance of physis
  • 58. Physeal disruption
  • 59. Metaphyseal rarefaction
  • 60. Femoral head extrusion
  • 61. Gages sign Rarefaction in the lateral part of the epiphysis and subjacent metaphysis.
  • 62. Classification of results Uniplanar methods - CE angle of Weiberg. - Salters extrusion Index. - Salters extrusion angle. - Epiphyseal index. - Epiphyseal quotient. Biplanar methods - Mose classification. - Stulberg classification.
  • 63. CE angle of Weiberg Indicator of acetabular depth It is the angle formed by a perpendicular line through the midpoint of the femoral head and a line from the femoral head center to the upper outer acetabular margin. Normal = 20 to 40 degrees Angle >25 = good, 20-25= fair, < 20 = poor
  • 64. Salters extrusion Index If AB is more than 20% of CD it indicates a poor prognosis
  • 65. Salters extrusion angle Normal is 50 degrees or more
  • 66. Epiphyseal index & quotient Epiphyseal index = greatest height of the epiphysis divided by its width. Epiphyseal quotient = Epiphyseal index of involved hip divided by the index for uninvolved hip. >0.6 = good 0.4-0.6 = fair 2 mm
  • 68. Stulberg classificaton Class I Shape of the femoral head was basically normal. Class II Loss of head height but within 2 mm to a concentric circle on AP and frog leg X-Ray Class III Deviates more than 2 mm and acetabulum contour matches the head contour Class IV Head Flattened, Flattened area