Perthes Disease
Definition   It is a self limiting disorder of the hip    produced by ischemia and varying    degrees of necrosis of the ...
Synonyms   Coxa Plana   Pseudocoxalgia (Calve)   Arthritis deformans juvenilis    (Perthes)   Osteochondroses of the h...
   Arthur Legg of    the United States
   Jacques Calve of    France
   Georg Perthes of    Germany
Historical aspect   Parker started the use of broomstick    cast in 1929.   Eyre-Brook introduced traction in bed    for...
Blood supply to femoral head   Retinacular arteries   Metaphyseal    arteries   Artery of the round    ligament
Blood supply to femoral head   Infants    1.   Metaphyseal arteries .    2.   Lat epiphyseal arteries    3.   Lig teres –...
Blood supply to femoral head   4 yrs to 7 years    1. Epiphyseal plate forms a barrier to       metaphyseal vessels.   P...
Blood supply to femoral head   Adolescent    After skeletal maturity metaphyseal    vessels again come into picture
Incidence   Male : Female = 4-5:1   2.5:1 in India   Age of onset earlier in females.   Age –            Range – 2-13 ...
Etiology   Etiology not known.   Coagulation disorders.   Altered arterial status of femoral head.   Abnormal venous d...
Coagulation disorders   Protein C or S deficiency   Thrombophilia   Hypofibrinolysis
Altered arterial status   Angiographic studies have shown obstruction of    superior capsular arteries and decreased flow...
Abnormal venous drainage    Increased venous pressure in the      femoral neck    Congestion in the metaphysis    Venou...
Abnormal growth and            development   A delay in Bone age of 1.5 to 2    years   Low birth weight   Low levels o...
Trauma.   In the developing femur (4 – 7    yrs),the major lateral epiphyseal    vessels must course through a    narrow ...
Hyperactivity or attention    deficit disorder
Genetic component   Familial association.   X-Linked recessive inheritance.
Environmental influences   Low socioeconomic status.
Sequel to synovitis   Synovitis of the hip occurs early in    Perthes disease.   Increased pressure in synovitis may    ...
Pathogenesis    Waldenstrom staged the     pathological process of the disease     as2.   Initial or ischaemic stage3.   ...
Pathogenesis   Ischaemic stage      - Necrosis      - Crushing of trabaculae.      - degeneration of basal layer of      ...
Ischaemic stage
Pathogenesis cont…   Resorption stage     - Invasion of vascular connective tissue.     - Resorption of dead bone by     ...
Resorption stage
Pathogenesis cont…   Reparative stage     - pathological fracture.     - creeping substitution and       apposition of vi...
Reparative stage
Remodelling stage     (replacement by biologically             plastic bone)      If treated           If untreated   Fem...
Clinical Features   Painless limp leads to painful limp   Pain in the groin,anterior hip     or greater trochanter   Re...
   Decreased range of motion especially    abduction and internal rotation.   Atrophy of thigh muscles.   Shortening
Investigation   X-Ray –AP & Frog leg Lat view    (Lowenstein view)   USG   Arthrography   Bone Scan   MRI
X-Ray   Ossific nucleus    smaller
X-Ray   Cresent Sign or    Salters sign or    Caffey’s sign
X-Ray   Increased Radio    opacity of femoral    head due to    collapse, new bone    formation and    calcification of  ...
X-Ray   Fragmentation of    epiphysis
X-Ray   Metaphyseal    widening and cystic    changes in femoral    neck
X-Ray   Lateral extrusion of    femoral head.   Hinged abduction.
X-Ray   Sagging rope sign    in adults with    history of perthes
Ultrasound   Synovial effusion   Cartilage hypertrophy in early stages
Arthrography   Shows configuration of the femoral    head and its relation with the    acetabulum.   Containment   Cong...
Bone Scan   Diagnosis possible months before    signs appear on X-Ray.   Avascular areas show cold spots.
Bone Scan   Convay et al    classification   Stage 1 is total    lack of uptake
Bone Scan( stage 2)   Revascularisation of a    lateral column   Failure to    revascularise at lat    column is a grave...
Bone Scan( stage 3)   Gradual filling of    anterolateral part
Bone Scan( stage 4)   Return to normal
MRI   Accurate in early diagnosis.   Shows    congruity,containment,synovial    hypertrophy well.
Classification   Waldenstroms classification.   Catterall classification   Salter classification   Herrings lateral pi...
Waldenstroms classification (Pathological classification)
Catterall classification          (based on x ray AP and Lat view).   I – only anterior portion of epiphysis         affe...
Salter Classification   Type A = I & II Catterall   Type B = III & IV Catterall.
Herring Lat Pillar   Group-A no involvement of the lateral    pillar, with no density changes and no loss    of height of...
Prognostic Factors1.   Age at diagnosis        <6 yrs     – good         6 – 9 yrs – fair        >9 yrs     - poor5.   Ext...
Catterall “head at risk”             signs   Clinical   Radiographic
    Clinical2.   Progressive loss of hip motion more     so abduction.4.   Obese child
     Radiographic3.    Gage sign5.    Calcification lateral to epiphysis7.    Diffuse metaphyseal rarefaction9.    Latera...
Physeal disruption
Metaphyseal rarefaction
Femoral head extrusion
Gage’s sign   Rarefaction in the    lateral part of the    epiphysis and    subjacent    metaphysis.
Classification of results   Uniplanar methods     - CE angle of Weiberg.     - Salters extrusion Index.     - Salters ext...
CE angle of Weiberg   Indicator of acetabular depth   It is the angle formed by a    perpendicular line through    the m...
Salters extrusion Index   If AB is more    than 20% of    CD it    indicates a    poor    prognosis
Salters extrusion angle   Normal is 50    degrees or    more
Epiphyseal index & quotient   Epiphyseal index = greatest height of    the epiphysis divided by its width.   Epiphyseal ...
Mose Classification   Good < 1 mm   Fair < 2 mm   Poor > 2 mm
Stulberg classificaton   Class I – Shape of the femoral head was              basically normal.   Class II – Loss of hea...
Stulberg classificaton   Class I & II – Spherically congruent.   Class III & IV – Congruous Incongruity                 ...
Stulberg classificaton   Class I    Shape of    the    femoral    head is    basically    normal.
Stulberg classificaton   Class II    Loss of    head    height    but    within 2    mm
Stulberg classificaton   Class III    Deviates    more    than 2    mm
Stulberg classificaton   Class IV    Head    Flattene    d
Stulberg classificaton   Class V    Collapse of    femoral    head,    Acetabular    contour    does not    change
Differential diagnosis   Tuberculosis of the hip   AVN due to leukemia, lymphoma,    gauchers disease,    hemoglobinopat...
Treatment   Objectives      - To produce a normal femoral        head and neck      - To produce a normal acetabulum     ...
Treatment   Treatment efforts are directed    towards      - Restoration and maintenance of        full mobility of the h...
Treatment   Caterall group 1 and    group 2 ( < 7 years)                             No   Herring group 1 &        Treat...
TreatmentTreatment is divided into 3 phases   Initial Phase – restore & maintain    mobility   Active Phase – Containmen...
Treatment ( Initial Phase )   Physiotherapy – active and passive                     range of motion                     ...
Treatment ( Active Phase )   Consists of containment of the    femoral head within the acetabulum.    This can be achieve...
Treatment (Orthosis)   Non Ambulatory weight releiving    1. Abduction broomstick plaster cast    2. Hip pica cast   Amb...
    Ambulatory unilateral    1. Tachdjian trilateral socket orthosis
Treatment (Orthosis)   Atlanta Scotish Rite    Brace
Treatment (Orthosis)   Atlanta Scotish Rite    Brace
Treatment (Orthosis)   Newington orthosis
Treatment (Orthosis)   Birmingham brace
Treatment (Orthosis)   Toronto Brace
Treatment (Orthosis)   Tachdjian trilateral    socket orthosis
Treatment (Orthosis)   Orthotic treatment is discontinued when the    disease enters the reparative phase and    healing ...
    The radiographic evidence of healing are    1. Appearance of irregular ossification in the       femoral head.    2 ....
Treatment ( Surgical)   Femoral varus osteotomy.   Inominate osteotomy.   Combined femoral and inominate    osteotomy ...
Femoral varus osteotomy
Femoral varus osteotomy
Femoral varus osteotomy
Femoral varus osteotomy
Femoral varus osteotomy
Recent Advances   Anticoagulant   Ibadronate
Thank You
Legg – calve – perthes disease (2)
Legg – calve – perthes disease (2)
Legg – calve – perthes disease (2)
Legg – calve – perthes disease (2)
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Legg – calve – perthes disease (2)

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Legg – calve – perthes disease (2)

  1. 1. Perthes Disease
  2. 2. Definition It is a self limiting disorder of the hip produced by ischemia and varying degrees of necrosis of the femoral head.
  3. 3. Synonyms Coxa Plana Pseudocoxalgia (Calve) Arthritis deformans juvenilis (Perthes) Osteochondroses of the hip.
  4. 4.  Arthur Legg of the United States
  5. 5.  Jacques Calve of France
  6. 6.  Georg Perthes of Germany
  7. 7. Historical aspect Parker started the use of broomstick cast in 1929. Eyre-Brook introduced traction in bed for 18-24 months.
  8. 8. Blood supply to femoral head Retinacular arteries Metaphyseal arteries Artery of the round ligament
  9. 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. 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. 11. Blood supply to femoral head Adolescent After skeletal maturity metaphyseal vessels again come into picture
  12. 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. 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. 14. Coagulation disorders Protein C or S deficiency Thrombophilia Hypofibrinolysis
  15. 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. 16. Abnormal venous drainage Increased venous pressure in the femoral neck Congestion in the metaphysis Venous outflow exits more distally in the diaphysis.
  17. 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. 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. 19. Hyperactivity or attention deficit disorder
  20. 20. Genetic component Familial association. X-Linked recessive inheritance.
  21. 21. Environmental influences Low socioeconomic status.
  22. 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. 23. Pathogenesis Waldenstrom staged the pathological process of the disease as2. Initial or ischaemic stage3. Resorption or fragmentation stage4. Reparative stage5. Remodelling stage
  24. 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. 25. Ischaemic stage
  26. 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. 27. Resorption stage
  28. 28. Pathogenesis cont… Reparative stage - pathological fracture. - creeping substitution and apposition of viable bone in dead trabaculae.
  29. 29. Reparative stage
  30. 30. Remodelling stage (replacement by biologically plastic bone) If treated If untreated Femoral head is  Subluxation and congrous deformity
  31. 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. 32.  Decreased range of motion especially abduction and internal rotation. Atrophy of thigh muscles. Shortening
  33. 33. Investigation X-Ray –AP & Frog leg Lat view (Lowenstein view) USG Arthrography Bone Scan MRI
  34. 34. X-Ray Ossific nucleus smaller
  35. 35. X-Ray Cresent Sign or Salters sign or Caffey’s sign
  36. 36. X-Ray Increased Radio opacity of femoral head due to collapse, new bone formation and calcification of dead marrow.
  37. 37. X-Ray Fragmentation of epiphysis
  38. 38. X-Ray Metaphyseal widening and cystic changes in femoral neck
  39. 39. X-Ray Lateral extrusion of femoral head. Hinged abduction.
  40. 40. X-Ray Sagging rope sign in adults with history of perthes
  41. 41. Ultrasound Synovial effusion Cartilage hypertrophy in early stages
  42. 42. Arthrography Shows configuration of the femoral head and its relation with the acetabulum. Containment Congruity Not routinely used .
  43. 43. Bone Scan Diagnosis possible months before signs appear on X-Ray. Avascular areas show cold spots.
  44. 44. Bone Scan Convay et al classification Stage 1 is total lack of uptake
  45. 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. 46. Bone Scan( stage 3) Gradual filling of anterolateral part
  47. 47. Bone Scan( stage 4) Return to normal
  48. 48. MRI Accurate in early diagnosis. Shows congruity,containment,synovial hypertrophy well.
  49. 49. Classification Waldenstroms classification. Catterall classification Salter classification Herrings lateral pillar classification.
  50. 50. Waldenstroms classification (Pathological classification)
  51. 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. 52. Salter Classification Type A = I & II Catterall Type B = III & IV Catterall.
  53. 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. 54. Prognostic Factors1. Age at diagnosis <6 yrs – good 6 – 9 yrs – fair >9 yrs - poor5. Extent of involvement6. Sex7. Catterall “head at risk” signs
  55. 55. Catterall “head at risk” signs Clinical Radiographic
  56. 56.  Clinical2. Progressive loss of hip motion more so abduction.4. Obese child
  57. 57.  Radiographic3. Gage sign5. Calcification lateral to epiphysis7. Diffuse metaphyseal rarefaction9. Lateral extrusion of femoral head11. Growth disturbance of physis
  58. 58. Physeal disruption
  59. 59. Metaphyseal rarefaction
  60. 60. Femoral head extrusion
  61. 61. Gage’s sign Rarefaction in the lateral part of the epiphysis and subjacent metaphysis.
  62. 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. 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. 64. Salters extrusion Index If AB is more than 20% of CD it indicates a poor prognosis
  65. 65. Salters extrusion angle Normal is 50 degrees or more
  66. 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 <0.4 = poor
  67. 67. Mose Classification Good < 1 mm Fair < 2 mm Poor > 2 mm
  68. 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 <1cm. Acetabulum contour matches the head contour Class V – Collapse of femoral head, Acetabular
  69. 69. Stulberg classificaton Class I & II – Spherically congruent. Class III & IV – Congruous Incongruity OR Aspherical congruity. Class V – Incongruous incongruity OR Aspherically incongruent.
  70. 70. Stulberg classificaton Class I Shape of the femoral head is basically normal.
  71. 71. Stulberg classificaton Class II Loss of head height but within 2 mm
  72. 72. Stulberg classificaton Class III Deviates more than 2 mm
  73. 73. Stulberg classificaton Class IV Head Flattene d
  74. 74. Stulberg classificaton Class V Collapse of femoral head, Acetabular contour does not change
  75. 75. Differential diagnosis Tuberculosis of the hip AVN due to leukemia, lymphoma, gauchers disease, hemoglobinopathies etc Meyers dysplasia AVN following dislocation. Transient synovitis
  76. 76. Treatment Objectives - To produce a normal femoral head and neck - To produce a normal acetabulum - A congruous hip which is fully mobile - To prevent degenerative arthritis of the hip later in life
  77. 77. Treatment Treatment efforts are directed towards - Restoration and maintenance of full mobility of the hip - Containment of the femoral head. - Resumption of weight bearing and full activity as soon as possible
  78. 78. Treatment Caterall group 1 and group 2 ( < 7 years) No Herring group 1 & Treatment group 2 (< 6 years)
  79. 79. TreatmentTreatment is divided into 3 phases Initial Phase – restore & maintain mobility Active Phase – Containment and maintainance of full mobility. Reconstructive phase – correct residual deformities.
  80. 80. Treatment ( Initial Phase ) Physiotherapy – active and passive range of motion exercises to restore motion Traction – B/L skin traction and gradually abducting over 1-2 weeks till full abduction is regained.
  81. 81. Treatment ( Active Phase ) Consists of containment of the femoral head within the acetabulum. This can be achieved by orthosis or by surgery
  82. 82. Treatment (Orthosis) Non Ambulatory weight releiving 1. Abduction broomstick plaster cast 2. Hip pica cast Ambulatory Both limbs included 1. Petrie Abduction cast 2. Toronto orthosis 3. Newington orthosis 4. Birmingham brace 5. Atlanta Scotish Rite Brace
  83. 83.  Ambulatory unilateral 1. Tachdjian trilateral socket orthosis
  84. 84. Treatment (Orthosis) Atlanta Scotish Rite Brace
  85. 85. Treatment (Orthosis) Atlanta Scotish Rite Brace
  86. 86. Treatment (Orthosis) Newington orthosis
  87. 87. Treatment (Orthosis) Birmingham brace
  88. 88. Treatment (Orthosis) Toronto Brace
  89. 89. Treatment (Orthosis) Tachdjian trilateral socket orthosis
  90. 90. Treatment (Orthosis) Orthotic treatment is discontinued when the disease enters the reparative phase and healing is established.
  91. 91.  The radiographic evidence of healing are 1. Appearance of irregular ossification in the femoral head. 2 . Increased density of femoral head should disappear. 3 . Medial segment of femoral head should increase in size and height. 4 . Metaphyseal rarefaction involving the lateral cortex of the metaphysis should ossify. 5 . There should be intact lateral column.
  92. 92. Treatment ( Surgical) Femoral varus osteotomy. Inominate osteotomy. Combined femoral and inominate osteotomy Valgus osteotomy Shelf arthroplasty
  93. 93. Femoral varus osteotomy
  94. 94. Femoral varus osteotomy
  95. 95. Femoral varus osteotomy
  96. 96. Femoral varus osteotomy
  97. 97. Femoral varus osteotomy
  98. 98. Recent Advances Anticoagulant Ibadronate
  99. 99. Thank You
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