A girl was born in 1984 with congenital anomalies in all 4 limbs. These included bilateral proximal femoral focal deficiency (PFFD), an absent right fibula, abnormal right foot and ankle, short left fibula, and rudimentary forearms with only single digits below the humerus bilaterally. The etiology remains unknown. Cerebral function was normal. Her father had spina bifida. At the age of 1 year, she underwent a Symes amputation of the right foot and was fitted with a below-knee orthosis, with which she began to walk. During the following year, radiographs suggested a pseudarthrosis between the right femoral head and the upper femoral shaft (Figure 1). Both acetabulae were developing well, with femoral heads enlocated. This was in keeping with an Aitken type-A or type-B PFFD of the right hip (Aitken 1969). The left proximal femur, although not normal, appeared in solid continuity with the femoral head. Figure 1. Both hips at 1 year of age. Exploration and bone grafting of the right hip was carried out at the age of 2 years through a modified Smith-Peterson approach. At operation, there appeared to be no pseudarthrosis between the head and neck, only cartilaginous tissue at this level with a marked flexion deformity of the femur. Corticocancellous graft strips from the iliac crest were taken, laid upon the neck, and sutured in place, with the hope of encouraging ossification. In the months following this, she began to walk again without pain or dysfunction relating to the hip joint. At the age of 5, the patient underwent a Grice procedure to arthrodese her left subtalar joint. Simultaneous examination under anesthesia of both hips was performed: there was found to be no contact between the head and upper femoral shaft on the right side. Therefore, shortly after this, the right hip was re-explored, using the previous approach. There was only fibrous tissue between the head and upper femur. It was felt that it would be impossible to perform a femoral neck reconstruction, so King’s procedure was carried out (King 1966, 1969). The upper end of the femur was sharpened and driven into a hole in the remnant of the femoral neck. The periosteal sleeve removed from the proximal femur was wrapped around this, and the construct was stabilized with a Kirschner wire (Figure 2). A full hip spica was applied. Figure 2. King’s procedure: immediately after surgery. The spica was left for 3 months, after which the Kirschner wire was removed and the hip was left free. Radiographs taken at this stage showed good bone formation around the proximal femur (Figure 3). 1 year after this, the patient fell and suffered a fracture across the neck of the right femur; this was treated in a hip spica and healed without incident. She continued to walk and run with the use of an orthosis. Figure 3. Right hip 3 months postoperatively. Further surgical procedures included a valgus osteotomy to the left femoral neck when 8 years old for a coxa vara of 90° causing impingement, a left foot tibialis anterior transfer for a varus hindfoot at age 10, and talonavicular and calcaneocuboid arthrodeses of this foot at age 22 (effectively making a triple arthrodesis, given her previous Grice subtalar arthrodesis). She has had no surgery to her upper limbs and functions well with these. At 24 years of age, the patient is currently able to walk 5 km with only mild-to-moderate pain in her hips. Stairs present no difficulty, and she does not use any walking aid. Running is possible for short distances, but co-ordination is difficult for this. She is gainfully employed in journalism. Current radiographs show a solid femoral neck with an enlocated hip joint (Figure 4). She has not had any surgery to the acetabulum of this hip: the radiograph shows evidence of heterotopic ossification at the superolateral margin of the hip joint. Figure 4. Hip 19 years postoperatively.
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Jun 1, 2013
Li Kun Guan + 2
Open Access