Proximal Femoral deficiency

Basically there is proximal femoral deficiency, the proximal femur is partially absent and the entire limb is shortened.

Frequency:

Incidence ranges from one case per fifty thousand to one case per two Lac populations.

Etiology:

·         No exact known cause

·         Injury to neural crest cells (embryonic cells of spinal cord and brain)

·         Defect in proliferation and maturation of chondrocytes in proximal growth plate due to anorexia, ischemia, irradiation, bacterial, viral infection, toxins, hormones, mechanical injury, thermal injury, thalidomide (anticancer drugs). No genetic etiology. There is associated high incidence of other anomalies like fibula deficiency, Valgus feet, cleft palate, and club foot.

AITKEN classification of proximal femoral deficiency:

AITKEN divided it into the following four categories.

Class A:

Femoral head is often absent

Class B:

Femoral head is absent + proximal femur

Class C:

Absent femoral head dysplastic acetabulum, tronchanter doesn’t develop.

Class D:

Severe shortened shaft, No acetabulum present

Proximal Femoral deficiency

Remember in the 1^st two categories ossification may occur

Diagnosis:

Radiographic studies

Management:

·         Prosthesis (head of femur)

• Treatment is delayed in child after 3 years.