1. Legg Calve Perthes Disease Dr nitin kaushik Junior resident ESIC PGIMSR PAREL

2. HISTORY Jacques CalveFrance 1875 -1954 Arthur LeggUSA 1874 - 1939 George Perthes Germany 1869 - 1927 3. DEFINITION A self limiting non-inflammatory condition, affectingthe capital femoral epiphysis with stages ofdegeneration and regeneration, leading to restorationof the bone nucleus. Osteonecrosis of the proximal femoral epiphysis in agrowing child caused by poorly understood nongenetic factors 4. Epidemiology Usually ages 4 8 yearsAs early as 2 years, as late as teens Boys:Girls 4 : 1 Bilateral10 - 12% No evidence of inheritance ?? 10% 5. EtiologyUnknownPast theories: -infection, inflammation, -trauma, -hereditary, environmental, -predisposed child, attention deficit disorder.Most current theories involve vascular compromise -Coagulation abnormalities -Sanches : second infarction theory 6. blood supply 7. Pathogenesis Histological 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. Pathogenesis: cartilage zones 9. Pathological stages Stage 1, ischaemia:-dead trabecular bone-collapsed trabecular bone-thickened articular cartilage-physeal disruption-cartilage extending from the physis into themetaphysis 10. Pathological stages Stage 2, resorption, fragmentation, re-vascularisation, and repair:-invasion of vascular granulation tissue-new bone forming on dead trabeculae-woven new bone formation Stage 3, re-ossification and resolution: -new bone, woven and lamellar Stage 4, re-modelling: -return to normal architecture 11. CYCLE OF EVENTS 12. Presentation Often insidious onset of a limp Pain in groin, thigh, knee 17% relate trauma history Can have an acute onset 13. Physical Exam Decreased ROM, especially abduction and internalrotation Trendelenburg test often positive Adductor contracture (due to long standing spasm) Muscular atrophy of thigh/buttock/calf Limb length discrepency (due to head collapse) 14. Differential Diagnosis CAUSES OF AVASCULAR NECROSIS- sickle cell disease- thalassemia- other hemoglobinopathies- steroid medication- after traumatic hip disloction EPIPHYSEAL DYSLASIAS- multiple epiphyseal dysplasia- spondyloepiphyseal dysplasia 15. Differential Diagnosis EPIPHYSEAL DYSLASIAS ctd - mucopolysaccharidosis - hypothyroidism OTHER SYNDROMES - osteochondromatosis - metachondromatosis - schwartz-jampel syndrome - trichorhinophalangeal syndrome. 16. Imaging AP pelvis Frog leg lateral Key = view filmssequentially over the course of disease 17. CLASSIFICATION The classifications for LCPD can be divided into- the one that defines the stage of the disease and- the ones used to prognosticate outcome.Waldenstroms radiographic classification defines 4 stages of LCPD during the active phase of the disease 18. CLASSIFICATION Three radiographic classification systems, namely - the Catterall, - Salter-Thompson, and - lateral pillar,have been developed as prognosticators of outcome that are to be applied at the stage of fragmentation. 19. Radiographic Stages Four Waldenstrom stages: 1) Initial stage 2) Fragmentation stage 3) Reossification stage 4) Healed stage 20. Initial Stage Early radiographic signs: Failure of femoral ossific nucleus to grow "Waldenstroms sign" (increased joint space and apparent mild pseudosubluxation) a subchondral fracture may be seen in the early stages in the infracted area (crescent, Salters or Caffreys sign). Irregular physeal plate Blurry/ radiolucent metaphysis 21. Initial Stage 22. Fragmentation Stage Bony epiphysis begins to fragment Areas of increased lucency and density osteolysis of the superolateral portion of the femoralhead (Gage sign on x-ray) Evidence of repair aspects of disease 23. Fragmentation Stage 24. Fragmentation Stage 25. GAGES SIGN 26. Reossification Stage Normal bone density returns Alterations in shape of femoral head and neck evident 27. Reossification Stage 28. Healed Stage Left with residual deformity from disease and repairprocess Differs from AVN following Fx or dislocation 29. Radiographic Classifications Describe extent of epiphyseal disease Catterall classification= most commonly used 30. Catterall classification 31. Catterall classification 32. Catterall classification 33. Catterall classification 34. Salter-Thompson Classification Simplification ofCatterall Based on status of lateralmargin of capital femoralepiphysis Group A (Catterall I & IIequivalent) Group B (Catterall III &IV equivalent) 35. Lateral Pillar Classification (Herring Classification) 3 groups: A) no lateral pillarinvolvment B) >50% lat heightintact C) 12 yrs 61. TREATMENT EARLY IN THE COURSEOF THE DISEASE Children less than 5 years of age at the onset of the disease:treatment is seldom needed regardless of severity of involvement of the femoral head. (However, if femoral head extrusion occurs treatment will be needed) 62. TREATMENT EARLY IN THE COURSEOF THE DISEASEChildren 5 years or older but less than 8 years of age:a. Early containment is indicated if it is possible todetermine that more than half the femoral epiphysis isnecrotic.b. Early determination cannot be made in most patients.These patients should be monitored with periodic(4-monthly) radiographs to detect early extrusion ofthe femoral head. 63. TREATMENT EARLY IN THE COURSEOF THE DISEASEc. Containment treatment should be considered as soonas extrusion of the femoral head is detected.d. Extrusion is determined by a break in the Shentonline.e. No containment is needed in this age group whenextrusion does not occur 64. TREATMENT EARLY IN THE COURSEOF THE DISEASEChildren >8 years but < 12 years of age:a. Should be treated by containment as soon as thedisease is diagnosed regardless of the extent ofnecrosis. Containment should be initiated before thefragmentation stage and before extrusion wheneverpossibleb. Alternative methods should be considered when thepatient presents in the late stage of fragmentation 65. TREATMENT EARLY IN THE COURSEOF THE DISEASE Children 12 years of age or older at the onset of thedisease:Containment should NOT be considered in these adolescents as it does not work.Treatment considerations should be similar to treatment of adults with osteonecrosis 66. TREATMENT LATE IN THE COURSEOF THE DISEASE The goal of treatment of Legg-Calve - Perthes late inthe course of the disease is to attempt to minimize theextent of deformation of the femoral head that hasalready developed. The treatment in the late fragmentation stage may beremedial or salvage depending on - the deformity of the femoral head or - the presence of hinge abduction 67. TREATMENT LATE IN THE COURSEOF THE DISEASE In children who have hinge abduction the goal oftreatment is -to correct hinge abduction and -facilitate some remodeling of the femoral head. Containment may be considered if the femoral headcan be contained without hinge abduction. If hinge abduction is present. A valgus femoralosteotomy is a reliable choice to improve motion andreduce pain. 68. TREATMENT AFTER HEALING OFTHE DISEASE The goals of treatment of adolescents or young adultswith healed Legg-Calve - Perthes disease anddeformity of the femoral head is to - improve function, - relive pain, and - delay the onset of secondary degenerative arthritis The treatment approach depends on the specific cause of pain, dysfunction, or deformity 69. TREATMENT AFTER HEALING OFTHE DISEASE If the femoral head is spherical or ovoid and there iscoxa brevis with a Trendelenburg gait, considertrochanteric advancement with or withoutlengthening the femoral neck If there is pain on account of femoro-acetabularimpingement consider repairing the labral pathologyand/or correcting impingement 70. COXA BREVISARTRIOTROCHANTRIC DISTANCE Normal Values (age 513) Females 16 3.6mm Males 23 4. mm 71. TREATMENT AFTER HEALING OFTHE DISEASE A deficient acetabular roof may require- labral support or- pelvic osteotomy with or without realignment of theproximal femur Symptoms caused by osteochondritis dessicans can sometimes be relieved by removing the loose fragment 72. TREATMENT AFTER HEALING OFTHE DISEASE The role of reshaping a grossly deformed femoral head is uncertain although in a few selected cases of moderate deformity, it may be of benefit-Femoral head reduction osteotomy done for coxa megna. 73. F.H.R.O FOR COXA MEGNA 74. TREATMENT AFTER HEALING OFTHE DISEASE When the articular surface is severely damaged salvage procedures such a total hip replacement should be considered 75. ROLE OF CORE DECOMPRESSION May be a useful adjunct to current methods oftreatment. Younger children with early stage disease may benefitfrom multiple small drillings, Largecore decompression, debridement, and graftingfor Perthes disease in older children with larger lesionsand more advanced stages of disease. 76. CORE DECOMPRESSION