Perthes disease Dr. D. N. Bid lecture 2013


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Perthes disease Dr. D. N. Bid lecture 2013

  1. 1. Perthes Disease & Physiotherapy Management Dr. D. N. Bid [PT] Senior Lecturer Sarvajanik College of Physiotherapy, Surat.
  3. 3. DEFINITION Legg-Calvé-Perthes disease (LCPD) is the name given to idiopathic osteonecrosis of the capital femoral epiphysis in a child.
  4. 4. Normal joint Increased Joint space Smaller head Denser head
  5. 5. Epidemiology  Prevalence: Transient synovitis SCFE 8.5% Infection Perthes' disease Other 5.3% 2.8% 6.0% 77.4%
  6. 6. Epidemiology  Race: Caucasians are affected more frequently than persons of other races.  Sex: Males are affected 4-5 times more often than females.  Age: LCPD most commonly is seen in persons aged 3-12 years, with a median age of 7 years.
  8. 8. Causes  Exact cause unknown.  Proposed theories.  Inherited protein C and/or S deficiency.  Venous thrombosis  Arterial occlusion  Raised intra osseous pressure
  9. 9. Causes  Proposed theories.     Excessive femoral anteversion. Synovitis. Generalized skeletal disorder. Arterial anomalies.
  10. 10. Causes
  11. 11. Pathophysiology  The capital femoral epiphysis always is involved. In 1520% of patients with LCPD, involvement is bilateral.  The blood supply to the capital femoral epiphysis is interrupted.  Bone infarction occurs, especially in the subchondral cortical bone, while articular cartilage continues to grow. (Articular cartilage grows because its nutrients come from the synovial fluid.)  Revascularization occurs, and new bone ossification starts.
  12. 12. Pathophysiology  At this point, a percentage of patients develop LCPD, while other patients have normal bone growth and development.  LCPD is present when a subchondral fracture occurs. This is usually the result of normal physical activity, not direct trauma to the area  Changes to the epiphyseal growth plate occur secondary to the subchondral fracture.
  13. 13. Pathogenesis          Avascular necrosis Temporary cessation of growth Revascularization from periphery Resumption of ossification and trauma Pathological fracture Resorption of underlying bone Replacement of biologically plastic bone Sublaxation Deformity
  14. 14. Clinical History: Symptoms usually have been present for weeks.  Hip or groin pain, which may be referred to the thigh  Mild or intermittent pain in anterior thigh or knee  Limp  Usually no history of trauma
  15. 15. Clinical Physical:  Painful gait  Decreased range of motion (ROM), particularly with internal rotation and abduction  Atrophy of thigh muscles secondary to disuse  Muscle spasm  Leg length inequality due to collapse
  16. 16. Clinical  Short stature: Children with LCPD often have delayed bone age.  Roll test  With patient lying in the supine position, the examiner rolls the hip of the affected extremity into external and internal rotation.  This test should invoke guarding or spasm, especially with internal rotation.
  17. 17. Differential Diagnosis  Listed are some other disorders that should be included in the differential diagnosis for LCPD:  Septic arthritis-This is an infection in the joint  Sickle cell-Osteonecrosis of the hip can be a result of this disease  Spondyloepiphyseal Dysplasia Tarda-This disease typically affects the spine and the larger more proximal joints  Gaucher’s Disease- This is a genetic disorder that often times includes bone pathology  Transient Synovitis-This is an acute inflammatory process and is the most common cause of hip pain in childhood
  18. 18. Workup Lab Studies:  CBC  Erythrocyte sedimentation rate - May be elevated if infection present
  19. 19. Workup Imaging Studies:  Plain x-rays of the hip are extremely useful in establishing the diagnosis.  Frog leg views of the affected hip are very helpful.  Plain radiographs have a sensitivity of 97% and a specificity of 78% in the detection of LCPD  Multiple radiographic classification systems exist, based on the extent of abnormality of the capital femoral epiphysis.  Waldenstrom, Catterall, Salter and Thompson, and Herring are the 4 most common classification systems.  No agreement has been reached as to the best classification system.
  20. 20. Radiographic stages  Five radiographic stages can be seen by plain x-ray. In sequence, they are as follows:
  21. 21. Radiographic stages 1. Cessation of growth at the capital femoral epiphysis; smaller femoral head epiphysis and widening of articular space on affected side.
  22. 22. Radiographic stages 2. Subchondral fracture; linear radiolucency within the femoral head epiphysis
  23. 23. Radiographic stages 3. Resorption of bone
  24. 24. Radiographic stages 4. Re-ossification of new bone
  25. 25. Radiographic stages 5. Healed stage
  26. 26. Catterall classification  Catterall Group I: Involvement only of the anterior epiphysis (therefore seen only on the frog lateral film)  Catterall Group II: Central segment fragmentation and collapse. However the lateral rim is intact and thus protects the central involved area.  Catterall Group III: The lateral head is also involved or fragmented and only the medial portion is spared. The loss of lateral support worsens the prognosis.  Catterall Group IV: The entire head is involved.  Catterall's classification has a significant inter and intra observer error.
  27. 27. Catterall:  I – anterior epiphysis  II – up to 50% with collapse  III – only small posterior portion not involved  IV – whole head involvement
  28. 28. Catterall I:
  29. 29. Catterall II:
  30. 30. Catterall III:
  31. 31. Catterall IV:
  32. 32. Catterall classification  Groups I and II had a good prognosis (in 90%) and required no intervention.  Groups III and IV had a poor prognosis (in 90 %) and required treatment.  The classification is applied to the frog lateral and AP film during the fragmentation phase
  33. 33. Salter and Thompson Classification  Salter and Thompson recognized that Catterall's first two groups and second two groups were distinct and therefore proposed a two part classification.  Salter & Thompson Group A: Less than 1/2 head involved.  Salter & Thompson Group B: More than 1/2 head involved.  Again the main difference between these two groups is the integrity of the lateral pillar.
  34. 34. (Herring) Lateral Pillar Classification  Lateral Pillar Group A: There is no loss in height of the lateral 1/3 of the head and minimal density change. Fragmentation occurs in the central segment of the head.  Lateral Pillar Group B: There is lucency and loss of height in the lateral pillar but not more that 50% of the original (contralateral) pillar height. there may be some lateral extrusion of the head.  Lateral Pillar Group C: There is greater than 50% loss in the height of the lateral pillar. The lateral pillar is lower than the central segment early on.
  35. 35. Herring A:
  36. 36. Herring B: Herring C: And now there is “B/C Border” since the ability to differentiate B / C proved elusive
  37. 37. Unilateral Perthes with entire head involvement and fragmentation. The reossification phase has not yet begun.
  38. 38. Unilateral Perthes disease with widening of the medial joint space, blurring of the physis, increased density of the head and lucency between the medial and central 1/3's of the head corresponding to early fragmentation phase.
  39. 39. Unilateral Perthes in the reossification phase with a visible subchondral line similar to Waldenstrom's sign. However Waldenstrom's subchondral fracture is seen very early in the disease process, before fragmentation. In this case the lateral pillar has maintained some integrity.
  40. 40. “Head at risk signs” 1. Gage's sign. a V shaped lucency in the lateral epiphysis. 2. lateral calcification (lateral to the epiphysis) (implies loss of lateral support) 3. lateral subluxation of the head.(implies loss of lateral support) 4. A horizontal growth plate.(implies a growth arrest phenomenon and deformity)
  41. 41. Workup  Technetium 99 bone scan - Helpful in delineating the extent of avascular changes before they are evident on plain radiographs.  The sensitivity of radionuclide scanning in the diagnosis of LPD is 98%, and the specificity is 95%.  Dynamic arthrography - Assesses sphericity of the head of the femur.
  42. 42. Ultrasonography in transient synovitis and early Perthes' disease  Ultrasonography may provide significant diagnostic clues to differentiate early Perthes' from transient synovitis. T Futami, Y Kasahara, S Suzuki, S Ushikubo, and T Tsuchiya Journal of Bone and Joint Surgery - British Volume, Vol 73-B, Issue 4, 635639
  43. 43. CT Scan  Staging determined by using plain radiographic findings is upgraded in 30% of patients.  Not as sensitive as nuclear medicine or MRI.  CT may be used for follow-up imaging in patients with LPD.
  44. 44. MRI  It allows more precise localization of involvement than conventional radiography.  MRI is preferred for evaluating the position, form, and size of the femoral head and surrounding soft tissues.  MRI is as sensitive as isotopic bone scanning.
  45. 45. Outcome variables        Age Extent of involvement Duration Remodeling potential Premature physeal closure Type of treatment Stage of disease at treatment.
  46. 46. Treatment  Goals of treatment  Achieve and maintain ROM  Relieve weight bearing  Containment of the femoral epiphysis within the confines of the acetabulum  Traction
  47. 47. Rational behind "containment"  Containment of the head within the acetabulum is reported to encourage spherical remodelling during the reossification and subsequent phases.  However if there is total head involvement and the lateral pillar collapses then the effect of containment is probably less.  Therefore it seems that the extent of involvement of the head is the critical factor and containment simply optimizes the situation.
  48. 48. Medications  Medical treatment does not stop or reverse the bony changes.  Appropriate analgesic medication should be given.  Nonsteroidal anti-inflammatory drugs      Ibuprofen Adult dose: 200-400 mg PO q4-6h; not to exceed 3.2 g/d. Pediatric dose: 6 months to 12 years: 20-40 mg/kg/d PO divided tid or qid; start at lower end of dosing range and titrate upward; not to exceed 2.4 g/d >12 years: Administer as in adults.
  49. 49. Non surgical containment
  50. 50. Scottish Rite abduction brace
  51. 51. LCPD – Petrie broomstick POP  Petrie-1971
  52. 52. LCPD - Braces
  53. 53. LCPD – Atlanta “Scottish Rite”
  54. 54. LCPD – Unilateral BraceBirmingham
  55. 55. LCPD – Bilateral Brace
  56. 56. LCPD – Bicycle Containment
  57. 57. LCPD – Bike and Brace
  58. 58. Japanese modification of Petrie abduction cast
  59. 59. Surgical containment
  60. 60. Greater trochanteric overgrowth  The trochanteric overgrowth can be dramatic on radiographs but several studies have shown that a Trendelenberg gait does not always occur.  If it does occur, and is significant, then trochanteric advancement may improve the gait.  An alternative is to perform a trochanteric arrest at an earlier date but this assumes that the first statement will not apply to the particular child.
  61. 61. RECONSTRUCTIVE SURGERY  INDICATIONS:  Hinge abduction.  Malformed femoral head in late group III or residual group IV.  Coxa magna.  A large malformed femoral head with lateral sublaxation.  Capital femoral physeal arrest. valgus subtrochanteric osteotomy. Garceau’s cheilectomy. shelf augmentation Chiari’s pelvic osteotomy. trochanteric advancement or arrest.
  62. 62. SUMMARY  For patients less that 6 years old the prognosis is good for the majority.  If they are stiff or painful they respond to bed rest, traction and pain relieving antiinflammatory medication.  There is no evidence that abduction splints or surgical intervention is warranted in the majority of these younger patients.
  63. 63. SUMMARY  For patients between 6 and 8 years but with a bone age less than 6 and an intact lateral pillar (Herring A and B) the prognosis is similar to that for the first group and observation is as good as surgical intervention for the majority.  If they have bone ages greater than 6 years and Herring lateral pillar classification B then "containment" of the head within the acetabulum seems to be warranted.  This may be done by abduction bracing, femoral varus osteotomy or a pelvic osteotomy.
  64. 64. SUMMARY  If they are between 6 and 8 and are in lateral pillar group C then the result of intervention are equivocal.  Children presenting with Perthes disease at age 9 or older often have lateral pillar B or C and a poor prognosis.  The trend is towards early containment of these hips although stiffness can be a problem following early pelvic (Salter's) osteotomy.
  65. 65. Follow-up  Initially, close follow-up is required to determine the extent of necrosis.  Once the healing phase has been entered, follow-up can be every 6 months.  Long-term follow-up is necessary to determine the final outcome.
  66. 66. Prognosis  The younger the age of onset of LCPD, the better the prognosis.  Children older than 10 years have a very high risk of developing osteoarthritis.  Most patients have a favorable outcome.  Prognosis is proportional to the degree of radiologic involvement.
  67. 67. Outcome Measures  The Lower Extremity Functional Scale is one that measures how this disease is affecting the child in a functional way. Since this questionnaire does ask about certain activities the child may not be allowed to perform (i.e. running, hopping, etc), it may not be the outcome measure of choice.  The Harris Hip score is another questionnaire that has more to do with a lower level of functional activities such as walking, stair climbing, donning/doffing shoes, sitting, etc.  Questionnaires that test the patient on a functional level are useful to provide a baseline and monitor functional progress in the patient’s activities.
  68. 68. Examination  1. The limp: The limp is usually antalgic.[4] It is possible that the child has a Trendelenburg gait (a positive Trendelenburg test on the affected side) which is marked by a pelvic drop on the unloaded side during single stance. [9] The child can also have a Duchenne gait, which is marked by a trunk lean toward the stance limb with the pelvis level or elevated on the unloaded side. [9]
  69. 69.  2. Range of motion: [4] The restriction of hip motion is variable in the early stages of the disease; Many patients, may only have a minimal loss of motion at the extremes of internal rotation and abduction. At this stage there usually is no flexion contracture. Loss of hip ROM in patients with early Perthes’ disease without intra-articular incongruity is due to pain and muscle spasm. [10] This is why, if the child is examined for instance after a night of bed rest, the range will be much better then later in the day. Further into the disease process; Children with mild disease may maintain a minimal loss of motion at the extremes only and thereafter regain full mobility. Those with more severe disease will progressively lose motion, in particular abduction and internal rotation. Late cases may have adduction contractures and very limited rotation, but the range of flexion and extension is only seldom compromised.
  70. 70.  3. Pain: [4] Pain occurs during the acute disease.[1] The pain may be located in the groin, anterior hip area, or around the greater trochanter. Referral of pain to the knee is common.  4. Atrophy: [4] In most cases there is atrophy of the gluteus, quadriceps[11] and hamstring muscles, depending upon the severity and duration of the disorder.
  71. 71. PHYSIOTHERAPY MANAGEMENT  There is no consensus concerning the possible benefits of physiotherapy in LCP disease, or in which phase of the development of the health problem it should be used.  Some studies mention physiotherapy as a preand/or postoperative intervention, while others consider it a form of conservative treatment associated with other treatments, such as skeletal traction, orthesis, and plaster cast.
  72. 72.  In studies comparing different treatments[12], physiotherapy was applied in children with a mild course of the disease.  The characteristics of the patients were:  Children with less than 50% femoral head necrosis (Catterall groups 1 or 2) [12]  Children with more than 50% femoral head necrosis, under six years, whose femoral head cover is good (>80%)[12]  Herring type A or B[13]  Salter Thompson type A[13]
  73. 73.  For patients with a mild course, physiotherapy can produce improvement in articular range of motion, muscular strength and articular dysfunction.  The physiotherapeutic treatment included:     Passive mobilisations for musculature stretching of the involved hip. Straight leg raise exercises, to strengthen the musculature of the hip involved for the flexion, extension, abduction, and adduction of muscles of the hip. They started with isometric exercises and after eight session, isometric exercises. A balance training initially on stable terrain, and later on unstable terrain.
  74. 74.  For children over 6 years at diagnosis with more than 50% of femoral head necrosis, proximal femoral varus osteomy gave a significantly better outcome than orthosis and physiotherapy[12].
  75. 75. Goals of PT Rx  The main goals of conservative management of LCPD are to promote and optimize range of motion, strength, and joint preservation to minimize impairments and maximize function.  Containment of the femoral head in the acetabulum is the most important focus during each stage of LCPD.
  76. 76.  Relieving Pain • Hot Pack for pain management with stretching2 • Cryotherapy for 20min at a time2
  77. 77.  Increasing Strength • Hip abductors • Hip flexors • Hip extensors • Hip internal & external rotators • Gluteus medius **Avoid single limb stance activities due to increased force through hip joint during severe involvement stage
  78. 78.  Improving Range of Motion **ROM is typically lost in this population due to muscle guarding secondary to pain from incongruent joint surfaces • Static Stretching of LE musculature • Dynamic ROM and AAROM for muscle guarding due to pain and if unable to achieve through static stretching • AROM & AAROM following passive stretching to maintain newly gained ROM • Stretching should include the following muscle groups: hip internal and external rotators, hip abductors, hip extensors, and any other LE motion that is significantly limited.
  79. 79.  Functional Activities • Single leg dynamic function activities such as side steps and step ups, according to WB status and involvement phase  Home Exercise/Modality Use • Cryotherapy for 20min at a time may be used to decrease pain and inflammation • The HEP should be based on orthopedist recommendations due to the staging of the disease, age of onset of the disease, radiographic data, and patient presentation
  80. 80.  Post-Surgical Physical Therapy - There is no evidence on the best post-op physical therapy interventions following femoral osteotomy in children with LCPD • The child will most likely be non-weight bearing approximately 6-8 weeks3 • During the non-weight bearing phase AROM of the joints above and below the surgical site should be done to prevent stiffness and undue weakness3 • Once the cast is removed, AAROM, AROM, and resistive exercises should be done to restore strength lost from immobility3 • Static stretches held for 30 sec+ and contract-relax stretches should be done every day followed by AROM exercises to restore and maintain full ROM
  81. 81. References   Conservative Management of Legg-CalvePerthes Disease-pdf  Post-Operative Management of Legg-CalvePerthes Disease-pdf  Evidence based guideline for LCPD from NGC