legg calve perthes disease-umy

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2. Disorder of the hip in young children Usually ages 4-8yrs As early as 2yrs, as late as teens Boys:Girls= 4-5:1 Bilateral 10-12% No evidence of inheritance 3. Unknown Past theories: infection, inflammation, trauma, congenital Most current theories involve vascular compromise Sanches 1973: second infarction theorysometimes called as coronary artery disease of hip 4. Infants 1. Metaphyseal arteries . 2. Lat epiphyseal arteries 3. Lig teres insignificant4 mts 4 years 1. Lat epiphyseal 2. Metaphyseal art. decrease in number (due to appearance of growth plate). 5. 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. 6. Susceptible child : delayed bone age -- Trauma -- Hereditary factors : controversial(HLA-A antigens in lymphocytes) -- Coagulopathy : protein c& s -- Hyperactivity -- Passive smoking -- Synovitis FACTORS UNLIKELY TO BE ETIOLOGY-- Endocrinopathy -- Urban envt. -- 7. Histologic changes described by 1913 Secondary ossification center= covered by cartilage of 3 zones: Superficial Epiphyseal Thin cartilage zoneCapillaries penetrate thin zone from below 8. 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 matrixSuperficial and middle layers nourished by synovial fluid Deep layer relies on blood supply 9. Physeal plate: cleft formation, amorphis debris(Bone dust), blood extravasation Metaphyseal region: normal bone separated by cartilaginous matrix Epiphyseal changes can be seen also in greater trochanter, acetabulum 10. Often insidious onset of a limp,excaberated by activity. C/O pain in groin, thigh, knee Few relate trauma hx Can have an acute onset 11. Decreased ROM, especially abduction and internal rotation: initially due to muscle spasm Abductor limp Trendelenburg test often positive Muscular atrophy of thigh/buttock/calf Limb length discrepency 12. Coxa magna Premature physeal growth arrest Central-short neck,trochanteric overgrowth Lateral-externally tilted head trochantric overgrowth acetublar deformity Irregular head Osteochondoirtis dessicans 13. AP pelvis Frog leg lateral Key= view films sequentially over course of dz Arthrography MRI role undefined 14. WALDENSTROM Modified Elizabethtown Classification Catterall classification Salter-Thompson Classification Lateral Pillar Classification 15. Four Waldenstrom stages: 1) 2) 3) 4)Initial stage Fragmentation stage Reossification stage Healed stage 16. Stage of Avascular Necrosis IschemiaA part ( anterior) or whole of capital femoral epiphysis is necrosed. On X-ray The ossific nucleus looks smaller Classically of Perthes, looks dense The articular cartilage remains viable & becomes thicker than normal increased joint space. 17. Stage of REVASCULARIZATION / FRAGMENTATION Ingrowths of highly vascular & cellular connective tissue.Necrotic trabecular debris is resorbed & replaced by vascular fibrous tissue the alternating areas of sclerosis and fibrosis appear on X- ray as fragmentation of epiphysis.New immature bone laid on intact necrosed trabeculae by creeping substitution further increases the density of ossific nucleus on X-ray. 18. Stage of REVASCULARIZATION / FRAGMENTATION (contd.) The femoral head may extrude from acetabulum at this stage. 19. Stage of Ossification / Healing New bone starts forming and epiphyseal density increases in the lucent portions of the femoral head. 20. Remodeling / Residual stage This is the stage of remodeling and there is no additional change in the density of the femoral head. Depending on the severity of the disease the residual shape of the head may be spherical or distorted. 21. I Sclerotic A: no loss of height B: loss of height II Fragmentation A: early B:late III Healing A: peripheral B:>1/3epiphysis IV Healed Stages220 days240 days255 days 22. Stage Ia - the initial stage of the disease, characterized by sclerosis of the epiphysis without any loss of epiphyseal height . Stage Ib - epiphysis is sclerotic and there is loss of height of the epiphysis. In this stage the epiphysis is still in a single piece and no fragmentation is visible in either anteroposterior or lateral views . 23. Stage Iia- epiphysis has just begun to fragment; one or two vertical fissures in the epiphysis are seen in either view . In stage IIb - fragmentation of the epiphysis is advanced, but there is no evidence of new bone formation lateral to the fragmented epiphysis . 24. In stage IIIa - evidence of new bone formation at the periphery of the necrotic fr agment; the new bone is not of normal texture and covers less than one-third the circumference of the epiphysis . In stage IIIb - new bone is of normal texture and covers more than one-third the circumference of the epiphysis . In stage IV the healing is complete and there is no radiologically identifiable avascular bone 25. extent of epiphyseal involvement and percentage of collapse as seen in x-ray (both AP and Lateral view) most commonly used 4 groups based on amount of femoral head involvement Also presence of sequestrum, metaphyseal rxn, subchondral fx 26. 3 groups: A) no lateral pillar involvment B) >50% lat height intact C) 25 = good, 20-25= fair, < 20 = poor 40. Surgical ContainmentFemoral VDRO osteotomiesVarus 20 Derotation 20-30PelvicShelf Redirectional Displacement 41. Contd. varus osteotomy : INDICATIONS- patients with a spherical femoral head, little or no acetabular dysplasia (center-edge angle of at least 15 to 20 degrees),lateral overloading, and a valgus neck-shaft angle of more than 135 degrees. DISADVANTAGES-varus angulation that may not correct with growth (especially in an older child), further shortening of an already shortened extremity, the possibility of a gluteus lurch produced by decreasing the length of the lever arm of the gluteal musculature, the possibility of nonunion of the osteotomy, requirement of a second operation to remove the internal fixation 42. ADVANTAGE-Anterolateral coverage of the femoral head, lengthening of the extremity (possibly shortened by the avascular process), and avoidance of a second operation for plate removal. DISADVANTAGES-1)inability sometimes to obtain proper containment of the femoral head, especially in older children; 2)an increase in acetabular and hip joint pressure that may cause further avascular changes in the femoral head; 3)an increase in leg length on the operated side compared with the normal side that may cause a relative adduction of the hip and uncover the femoral head. Eg.-Salters ostoeotomy 43. Aims of treatment Relieve pain Correct Trendelenburg gait Minimize the risk of development of degenerative arthritis 44. Valgus osteotomyJoint distractionSurgical dislocation and osteochondroplastyCheilectomy(Osteochondroplasty)Arthrodesis 45. Greater trochanter advancementLengthening of the femoral neckImproving acetabular coverage of the femoral head by periacetabular osteotomy 46. Valgus extension osteotomy indication -hinge abduction of hip Cheilectomy indication malformed femoral head with lateral protuberance Coxa plana Chiari osteotomy indication malformed femoral head with lateral subluxation Trochanteric advancement indication premature capital femoral physeal arrest Greater trochanteric epiphysiodesis indication premature capital femoral physeal arrest Shelf augmentation procedure indication coxa magna coxa magna & lack of acetabular coverage 47. Patients presenting at 8+yrs Have a worse prognosis Severe femoral head deformity more likely Deformity at maturity predicts outcome Particularly if Catterall III or IV Or Herring C (B/C) Girls have a poorer prognosis 48. In some patients collapse was more pronounced in the middle pillar rather than the lateral. Neither the Catterall grouping nor the Herring grading correlated with the final outcome Osteoporosis premature fusion of: the triradiate cartilage, trochanteric growth plate and the capital femoral growth plate. 49. The outcome of the disease in adolescents is poor. Many of the patients with the destructive pattern required salvage surgery to relieve pain. It is likely that patients with the other patterns of the disease will develop degenerative changes in due course. 50. Thank you for attention !UMY