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Incidence- rare- 1/ 25000 live births, M=F, R=L, Bilat in 30%
- an autosomal dominant inheritance pattern has been described
- may be more common in negroes
Aetiology- Histological abnormalities have been found in Bx of the femoral necks- Increase in width of the growth plate
- absenceof normal orderly progression of cartilage columns (similar to appearance of Blounts Dx)
- current theory - deformity due to a primary ossification defect in the inferior femoral neck. Physiologic shearing stresses in weight bearing fatigue the abnormal bone Resulting in progressive deformity
Clinically- Most present with a progressive gait deformity bw walking age and ~ 6 yo pain is rare
- unilateral dx- present with gait 2o to abductor weakness and a LLD
- bilateral dx - present with waddling gait similar to that of DDH
- Examination
- prominent and elevated greater trochanter
- +ve TrendelenbergLLD - usually mild, av 2.5 cm
- FFD with ROM - esp abduction and IR
- more vertical position of the physeal plate- defined by the angle bw Hilgenreiners line and the line through the epiphyseal plate- normally 25o or less
- Fairbanks triangle - a triangular metaphyseal fragment in the inferior femoral neck surrounded by an inverted radiolucent Y ( represents a zone of abnormal osification with an interposed triangular segment of dystrophic bone)
- in normal anteversion of the prox femur ( may become retroverted) coxa breva
- rarely mild acetebular dysplasia
Category:
Orthopedics Notes
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