Legg Calve Perthes Disease        Joseph Donnelly, M.D.        December 10, 2001
Overview♦ History♦ Epidemiology/ Etiology♦ Pathogenesis   – Radiographic stages♦ Presentation/ Exam♦ Imaging♦ Treatment
History♦ Late 19th century: “hip infections” that  resolved without surgery♦ First described in 1910♦ Early path studies: ...
Epidemiology♦ Disorder of the hip in young children♦ Usually ages 4-8yo♦ As early as 2yo, as late as teens♦ Boys:Girls= 4-...
Etiology♦ Unknown♦ Past theories: infection, inflammation,  trauma, congenital♦ Most current theories involve vascular  co...
Etiology: blood supply
Pathogenesis♦ Histologic changes described by 1913♦ Secondary ossification center= covered by  cartilage of 3 zones:  – Su...
Pathogenesis: cartilage zones
Pathogenesis♦ Epiphyseal cartilage in LCP disease:   – Superficial zone is normal but thickened   – Middle zone has 1)area...
Pathogenesis♦ Physeal plate: cleft formation, amorphis  debris, blood extravasation♦ Metaphyseal region: normal bone  sepa...
Radiographic Stages♦ Four Waldenstrom stages:   – 1) Initial stage   – 2) Fragmentation stage   – 3) Reossification stage ...
Initial Stage♦ Early radiographic signs:  – Failure of femoral ossific nucleus to grow  – Widening of medial joint space  ...
Initial Stage
Initial Stage
Fragmentation Stage♦ Bony epiphysis begins to fragment♦ Areas of increased lucency and density♦ Evidence of repair aspects...
Fragmentation Stage
Fragmentation Stage
Reossification Stage♦ Normal bone density returns♦ Alterations in shape of femoral head and  neck evident
Reossification Stage
Reossification Stage
Healed Stage♦ Left with residual deformity from disease  and repair process♦ Differs from AVN following Fx or  dislocation
Presentation♦ Often insidious onset of a limp♦ C/O pain in groin, thigh, knee♦ 17% relate trauma hx♦ Can have an acute onset
Physical Exam♦ Decreased ROM, especially abduction and  internal rotation♦ Trendelenburg test often positive♦ Adductor con...
Imaging♦ AP pelvis♦ Frog leg lateral♦ Key= view films  sequentially over  course of dz♦ Arthrography♦ MRI role undefined
Differential Diagnosis♦ Important to rule out infectious etiology  (septic arthritis, toxic synovitis)♦ Others:   –   Chon...
Radiographic Classifications♦ Describe extent of epiphyseal disease♦ Catterall classification= most commonly  used  – 4 gr...
Group I
Group II
Group III
Group IV
Lateral Pillar Classification♦ 3 groups:   – A) no lateral pillar     involvment   – B) >50% lat height     intact   – C) ...
Salter-Thompson Classification♦ Simplification of Catterall♦ Based on status of lateral margin of capital  femoral epiphys...
Prognosis♦ 60% of kids do well without tx♦ AGE is key prognostic factor:  – <6yo= good outcome regardless of tx  – 6-8yo= ...
Prognosis♦ Flat femoral head incongruent with  acetabulum= worst prognosis♦ Do not treat in reossification stage  (>15mos)
Non-operative Tx♦ Improve ROM 1st♦ Bracing:  – Removable abduction orthosis  – Pietrie casts  – Hips abducted and internal...
Bracing
Non-operative Tx♦ Check serial radiographs  – Q3-4 mos with ROM testing♦ Continue bracing until:  – Lateral column ossifie...
Operative Tx♦ If non-op tx cannot maintain containment♦ Surgically ideal pt:   – 6-9yo   – Catterral II-III   – Good ROM  ...
Surgical Tx♦ Surgical options:   – Excise lat extruding head portion to stop     hinging abduction   – Acetabular (innomin...
Varus Osteotomy
Late Effects of LCP♦ Coxa magna♦ Physeal arrest patterns♦ Irregular head formation♦ Osteochondritis dessicans
The End
Legg calve perthes disease donnely 2001 5afad2fc5e0b007027c03a29b821eb3c
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Legg calve perthes disease donnely 2001 5afad2fc5e0b007027c03a29b821eb3c

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  • -described 1910 independently by Legg, Calve, Perthes, and Waldenstrom -Legg= “obscure affectation of the hip”, thought secondary to pressure injury flattening the femoral head -Calve: same year, 10 cases non inflamm/self limiting disease due to delayed osteogenesis. Also reported coxa vara and increased femoral head size -Perthes- “arthritis deformans juveniles, possible inflammatory condition -Waldenstrom: thought it was TB
  • -incidence of positive family hx ranges from 1.6% to 20%, but no hard evidence of predisposition -is more common in certain geographic areas (urban&gt;rural)=nutritional?, later born children, strong association with ADHD (33%)
  • -Phemister- thought it was infectious but cx neg -Axhausen- thought bacillary embolism with weak infection which healed quickly -1975 Matsoukas showed association with prenatal rubella -1973 Sanches, infarcted animal femoral heads, unable to produce typical histologic picture of LCPdz with one infarction, could do it with a second. Supported by Inoue using human histologic material
  • -blood supply description (terminology)varies -3 main sources to the proximal femur: 1)extracapsular ring, 2)ascending cervical (retinacular branches) vessels 3)artery of the ligamentum teres -extracapsular ring: med and lat fem circumflex, gives rise to ascending cervical branches (extracapsular) which give of metaphyseal and epiphyseal branches -ant portion=mainly lat fem circ, post/lat/med=med fem circ -Chung found that greatest volume of flow= from lat ascending cervical (end of med fem circ)
  • -few human specimens have been studied, each showing only a stage of the dz and usually from sample of just one part of the involved head. Histologically not well illucidated
  • -Superficial zone=like adult articular cartilage -epiphyseal (middle) cartilage zone= becomes thinner as skeleton matures, epiphyseal bone enlarges -Deep thin zone= small clusters of cartilage cells that hypertrophy and degenerate -capillaries from below
  • -changes in zone 2 are abnormal, have different histochemical and US properties vs normal, also see small 2ndary ossification centers directly on the abnormal cartilage matrix -synovial fluid nourishes 2 superficial layers, continue to proliferate -deep layer affected by ischemic process
  • -a) superficial zone: area of disorganized cartilage -c) junction btwn normal and abnormal epiphyseal cartilage, note hypercellularity -d) extensive abnormal cartilage, bone forms directly on abnormal cartilage
  • -physeal plate: thinner than normal, irregular cell columns and cartilage masses -metaphysis: cartilage does not ossify, proliferates with bone, causes tongues of cartilage extending into metaphysis -skeletal surveys shows contour irregularities in 48% of normal contralateral capital epiphysis, suggesting it is a generalized disorder, more appropriately named a syndrome
  • -growth failure due to lack of blood supply -affected femoral ossific nucleus appears radiodense (relative osteopenia of surrounding bone vs. increased mass in that area?) -affected femoral head appears smaller vs. other side -wide med joint space due to: synovitis? Decreased head volume from necrosis and collapse? Due to increased blood flow to soft tissues (eg. Lig teres) causing lateral displacement? Most likely due to epiphyseal cartilage hypertrophy (x-ray phenomenon) -crescent sign= subchondral radiolucent zone, likely results from a subchondral stress fracture and the extent of this zone determines the extent of the necrotic fragment
  • -note smaller, denser ossific nucleus on L hip -crescent (fracture) sign -irregular physeal plate -blurry, radiolucent metaphysis
  • -same on frog
  • -increased radiodensity due to new bone forming on old bone
  • -fragmented epiphyseal bone, radiodense and lucent areas
  • -note shape deformity on R vs L
  • -AVN process after fx/dislocation does not undergo fragmentation
  • -must recognize thigh &amp; knee pain as possible hip pathology -pain usually mild and relieved by rest, often present late due to mild sx
  • -early decreased abduction due to synovitis/spasm, may become permanent after development of femoral head deformity -adduction contracture due to long standing spasm -atrophy due to disuse due to pain, shows long standing nature -short limb due to head collapse= poor prognosis
  • -frog leg= better for crescent sign -compare films with previous to determine change -arthrography can show status of cartilage not shown on x-ray, check ROM to r/o hinging abduction -hinging abduction due to large femoral head extruding laterally &amp; hinging over edge of acetabulum
  • -25% of anterocentral head involved -no sequestrum, no subchondral fx’s, normal metaphysis
  • -50% of anterolateral region involved -evidence of sequestrum/ subchondral (anterior) fx, med/lat pillars intact
  • -75% of head involved -large sequestrum, lat pillar (column) involved, sclerotic junction btwn normal/abnormal -subchondral fx line extends into post ½ of epiphysis
  • -whole head involved, widespread epiphyseal collapse -diffuse or central metaphyseal lesion -Posterior remodeling of ephiphysis -poor prognosis
  • -Catterral 1 and usually 2 do well without treatment
  • -to late to treat after 15mos
  • -abduction usually affected most of ROM -use PT to regain abduction (overcome spasm) and internal rotation -may require several weeks of abduction traction -don’t start bracing until abd/int rot restored to normal -arthrography pre-bracing to determine congruency throughout ROM -head collapse is independent of weight bearing, not necessarily NWB -hips braced in abd/ int rotation to transmit weight over wide area of acetabulum, prevents head collapse
  • -Study by Futami and Suzuki 1997, 6% of uninvolved contralat hips develop LCP when unbraced, 0 when braced -length of casting usually 6 weeks to start out
  • -coxa magna due to ossiffication of hypertrophied articular cartilage -OD=rare, occurs with the late onset of dz and with prolonged ineffectual repair stage
  • Legg calve perthes disease donnely 2001 5afad2fc5e0b007027c03a29b821eb3c

    1. 1. Legg Calve Perthes Disease Joseph Donnelly, M.D. December 10, 2001
    2. 2. Overview♦ History♦ Epidemiology/ Etiology♦ Pathogenesis – Radiographic stages♦ Presentation/ Exam♦ Imaging♦ Treatment
    3. 3. History♦ Late 19th century: “hip infections” that resolved without surgery♦ First described in 1910♦ Early path studies: cartilaginous islands in the epiphysis
    4. 4. Epidemiology♦ Disorder of the hip in young children♦ Usually ages 4-8yo♦ As early as 2yo, as late as teens♦ Boys:Girls= 4-5:1♦ Bilateral 10-12%♦ No evidence of inheritance
    5. 5. Etiology♦ Unknown♦ Past theories: infection, inflammation, trauma, congenital♦ Most current theories involve vascular compromise – Sanches 1973: “second infarction theory”
    6. 6. Etiology: blood supply
    7. 7. Pathogenesis♦ Histologic changes described by 1913♦ Secondary ossification center= covered by cartilage of 3 zones: – Superficial – Epiphyseal – Thin cartilage zone♦ Capillaries penetrate thin zone from below
    8. 8. Pathogenesis: cartilage zones
    9. 9. Pathogenesis♦ Epiphyseal cartilage in LCP disease: – Superficial zone is normal but thickened – Middle zone has 1)areas of extreme hypercellularity in clusters and 2)areas of loose fibrocartilaginous matrix♦ Superficial and middle layers nourished by synovial fluid♦ Deep layer relies on blood supply
    10. 10. Pathogenesis♦ Physeal plate: cleft formation, amorphis debris, blood extravasation♦ Metaphyseal region: normal bone separated by cartilaginous matrix♦ Epiphyseal changes can be seen also in greater trochanter, acetabulum
    11. 11. Radiographic Stages♦ Four Waldenstrom stages: – 1) Initial stage – 2) Fragmentation stage – 3) Reossification stage – 4) Healed stage
    12. 12. Initial Stage♦ Early radiographic signs: – Failure of femoral ossific nucleus to grow – Widening of medial joint space – “Crescent sign” – Irregular physeal plate – Blurry/ radiolucent metaphysis
    13. 13. Initial Stage
    14. 14. Initial Stage
    15. 15. Fragmentation Stage♦ Bony epiphysis begins to fragment♦ Areas of increased lucency and density♦ Evidence of repair aspects of disease
    16. 16. Fragmentation Stage
    17. 17. Fragmentation Stage
    18. 18. Reossification Stage♦ Normal bone density returns♦ Alterations in shape of femoral head and neck evident
    19. 19. Reossification Stage
    20. 20. Reossification Stage
    21. 21. Healed Stage♦ Left with residual deformity from disease and repair process♦ Differs from AVN following Fx or dislocation
    22. 22. Presentation♦ Often insidious onset of a limp♦ C/O pain in groin, thigh, knee♦ 17% relate trauma hx♦ Can have an acute onset
    23. 23. Physical Exam♦ Decreased ROM, especially abduction and internal rotation♦ Trendelenburg test often positive♦ Adductor contracture♦ Muscular atrophy of thigh/buttock/calf♦ Limb length discrepency
    24. 24. Imaging♦ AP pelvis♦ Frog leg lateral♦ Key= view films sequentially over course of dz♦ Arthrography♦ MRI role undefined
    25. 25. Differential Diagnosis♦ Important to rule out infectious etiology (septic arthritis, toxic synovitis)♦ Others: – Chondrolysis -Neoplasm – JRA -Sickle Cell – Osteomyelitis -Traumatic AVN – Lymphoma -Medication
    26. 26. Radiographic Classifications♦ Describe extent of epiphyseal disease♦ Catterall classification= most commonly used – 4 groups based on amount of femoral head involvement – Also presence of sequestrum, metaphyseal rxn, subchondral fx
    27. 27. Group I
    28. 28. Group II
    29. 29. Group III
    30. 30. Group IV
    31. 31. Lateral Pillar Classification♦ 3 groups: – A) no lateral pillar involvment – B) >50% lat height intact – C) <50% lat height intact
    32. 32. Salter-Thompson Classification♦ Simplification of Catterall♦ Based on status of lateral margin of capital femoral epiphysis♦ Group A (Catterall I & II equivalent)♦ Group B (Catterall III & IV equivalent)
    33. 33. Prognosis♦ 60% of kids do well without tx♦ AGE is key prognostic factor: – <6yo= good outcome regardless of tx – 6-8yo= not always good results with just containment – >9yo= containment option is questionable, poorer prognosis, significant residual defect
    34. 34. Prognosis♦ Flat femoral head incongruent with acetabulum= worst prognosis♦ Do not treat in reossification stage (>15mos)
    35. 35. Non-operative Tx♦ Improve ROM 1st♦ Bracing: – Removable abduction orthosis – Pietrie casts – Hips abducted and internally rotated♦ Wean from brace when improved x-ray healing signs
    36. 36. Bracing
    37. 37. Non-operative Tx♦ Check serial radiographs – Q3-4 mos with ROM testing♦ Continue bracing until: – Lateral column ossifies – Sclerotic areas in epiphysis gone♦ Cast/brace uninvolved side
    38. 38. Operative Tx♦ If non-op tx cannot maintain containment♦ Surgically ideal pt: – 6-9yo – Catterral II-III – Good ROM – <12mos sx – In collapsing phase
    39. 39. Surgical Tx♦ Surgical options: – Excise lat extruding head portion to stop hinging abduction – Acetabular (innominate) osteotomy to cover head – Varus femoral osteotomy – Arthrodesis
    40. 40. Varus Osteotomy
    41. 41. Late Effects of LCP♦ Coxa magna♦ Physeal arrest patterns♦ Irregular head formation♦ Osteochondritis dessicans
    42. 42. The End

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