Treatment of the Patient with Bilateral Proximal Femoral Focal Deficiency
DONALD J. HAYHURST, C.P.O. ARTHUR ECKHARDT, M.D.
The long-term treatment plan for the patient with bilateral proximal femoral focal deficiency centers around whether or not to ablate the feet at the time prosthetic fitting is contemplated. As a bilateral PFFD child gets older he develops an increasing height discrepancy between himself and his peers. This difference often becomes a physical and social problem as school age nears. At this time the child usually should be fitted with some type of device to compensate for his short stature. The device is generally cumbersome, is relatively heavy, and requires a great expenditure of energy for ambulation. These patients also have abnormal bone structure and weak musculature about their hips, and both of these factors add to their instability when wearing prostheses.
There are three factors favoring ablation of the feet: I) badly deformed feet that do not serve well in ambulation, 2) a significant leg-length discrepancy, 3) the more cosmetic prosthetic fitting possible with ablation at the anklejoint. A factor which our clinic feels is very important against foot ablation is the patient's ability to get about when not wearing prostheses.
Currently at our facility we are treating three bilateral PFFD patients. Each is a product of a normal pregnancy', except for the first three months of gestation when the mothers took a medication unknown to them or us. Of these three children we are treating, one was placed in elevated shoes at the age of 3 and 1/2. The first prosthetic fitting for all three was at 5 years of age.
Case Report 1
S.F. was born April 29, 1965, with scoliosis, right femoral phocomelia complete, and left proximal femoral focal deficiency. This girl was first seen in our clinic at age 3 and 1/2 ( Figure 1 , Figure 2 , and Figure 3 ). She was noted to have a right 44-degree dorsal scoliosis, which was the main concern at the time. She was placed in a shoe lift of 6.3 cm (2 and 1/2 in.) to accommodate her deformity ( Figure 4 ). She ultimately received a Harrington instrumentation and spinal fusion to stabilile the scoliosis. She continued with the right shoe lift and ambulated with forearm crutches.
In February 1973 bilateral PFFD prostheses were fabricated. Because of the external rotation of the left femur and the rigidity of her ankles, the patient's feet were externally rotated and placed in a plantigrade position in her prostheses.
In December 1975, S.F. received her second pair of prostheses ( Figure 5 and Figure 6 ), which she is currently wearing eight hours a day. She can ambulate short distances without external support, but for the most part she uses crutches.
Case Report 2
D.M. was born June 28, 1968, with complete absence of the right femur and fibula, left proximal femoral focal deficiency, left dysplastic acetabulum, and bilateral talipes equinovarus.
This girl was first seen in our clinic at 6 months of age ( Figure 7 and Figure 8 ). She was followed for about four years without treatment until she became a free ambulator. One month short of her fifth birthday she received bilateral PFFD prostheses to raise her to peer height ( Figure 9 , Figure 10 , and Figure 11 ).
At the peak of her wearing time in June 1974, she was wearing the prostheses for a total of eight hours while at school. D.M. liked being at the height of her peers, but the energy consumption was too much for her. It was especially difficult for her to ambulate on shag carpeting.
On her last clinic appointment in February 1976, she was using a roller walker and had a good gait: but she seldom wore her prostheses because of the energy-use factor.
Case Report 3
F.S. was born January 3, 1970, with bilateral proximal femoral focal deficiency and hypoplasia of the pelvis.
This young man was first seen by us at 2 months of age ( Figure 12-A , Figure 12-B , and Figure 13 ). At this time he was placed in a Denis-Browne Splint with hips in 20 degrees of abduction and feet in neutral position. He was followed for the next three years without any other form of treatment.
At age 3 and 1/2 he was getting around quite well, but when he was in a hurry he would get around on all fours. At this point bilateral 11.5-cm (4 and 1/2-in.) heel and sole lifts were applied to his shoes. With these lifts he could ambulate quite well but could not move fast enough to suit him, so he wore them short periods of time.
F.S. started school in shoe lifts and got along fine, being at the height of peers. Then he outgrew his shoes and started having behavioral problems because of his short stature. In April of this year bilateral PFFD prostheses were fabricated without knee joints and with the patient's feet held in equinus ( Figure 14 , Figure 15 , and Figure 16 ). Raising F.S. back to the height of his peers seemed to solve his behavioral problems and made him quite happy.
He ambulates with crutches and wears his prostheses while at school and an hour or two at home in the evening. During the summer he wore his prostheses an hour or two during the day.
If the feet of the bilateral PFFD patient are not badly deformed, if the hips are relatively stable, and if there is no significant leg-length discrepancy, our clinic feels the feet should remain. Leaving the feet allows the patient to ambulate without relying on prostheses. The child is able to get up during the night, go to the bathroom, and walk around the house easily and rapidly without donning his or her prostheses. Also, an important consideration is the psychological advantage felt by the patient. We feel these reasons outweigh ablations for prosthetic cosmesis.