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| Coxa vara | |
|---|---|
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| Left to right:coxa vara, normal femur,coxa valga | |
| Specialty | Medical genetics |
Coxa vara is adeformity of thehip, whereby the angle between the head and the shaft of thefemur is reduced to less than 120 degrees. This results in the leg being shortened and the development of alimp. It may becongenital and is commonly caused byinjury, such as afracture. It can also occur when thebone tissue in the neck of the femur is softer than normal, causing it to bend under the weight of the body. This may either be congenital or the result of abone disorder. The most common cause of coxa vara is either congenital ordevelopmental. Other common causes includemetabolic bone diseases (e.g.Paget's disease of bone),post-Perthes deformity,osteomyelitis, and post traumatic (due to improper healing of a fracture between thegreater andlesser trochanter).Shepherd's Crook deformity is a severe form of coxa vara where the proximal femur is severely deformed with a reduction in the neck shaft angle beyond 90 degrees. It is most commonly a sequela ofosteogenesis imperfecta, Paget's disease, osteomyelitis,tumour and tumour-like conditions (e.g.fibrous dysplasia).
Coxa vara can happen incleidocranial dysostosis.
In early skeletal development, a common physis serves the greater trochanter and the capital femoral epiphysis. This physis divides as growth continues in a balance that favors the capital epiphysis and creates a normal neck shaft angle (angle between the femoral shaft and the neck). The corresponding angle at maturity is 135 ± 7 degrees. Another angle used for the measurement of coxa vara is the cervicofemoral angle which is approximately 35 degrees at infancy and increases to 45 degrees after maturity.
Clinical feature: presents after the child has started walking but before six years of age. Usually associated with a painless hip due to mild abductor weakness and mild limb length discrepancy.
If there is a bilateral involvement the child might have a waddling gait or trendelenburg gait with an increased lumbar lordosis. The greater trochanter is usually prominent onpalpation and is more proximal. Restricted abduction and internal rotation.
X-ray: decreased neck shaft angle, increased cervicofemoral angle, vertical physis, shortened femoral neck decrease in femoral anteversion. HE angle (Hilgenriener epiphyseal angle- angle subtended between a horizontal line connecting thetriradiate cartilage and the epiphysis); normal angle is <30 degrees.
Treatment:HE angle of 45–60 degrees: observation and periodic follow up.
Indication for surgery: HE angle more than 60 degrees, progressive deformity, neck-shaft angle <90 degrees, development ofTrendelenburg gait
Surgery: subtrochanteric valgus osteotomy with adequate internal rotation of distal fragment to correctanteversion;common complication is recurrence. If HE angle is reduced to 38 degrees, less evidence of recurrence; post operative spica cast is used for a period of 6–8 weeks.
Coxa vara is also seen inNiemann–Pick disease.
Presence at birth is extremely rare and associated with other congenital anomalies such as proximal femoral focal deficiency, fibular hemimelia or anomalies in other part of the body such as cleidocranial dyastosis. The femoral deformity is present in the subtrochanteric area where the bone is bent. The cortices are thickened and may be associated with overlying skin dimples. External rotation of the femur withvalgus deformity of knee may be noted. This condition does not resolve and requires surgical management. Surgical management includes valgus osteotomy to improve hip biomechanics and length and rotational osteotomy to correct retroversion and lengthening.
