Conservative Management of Bilateral Proximal Femoral Focal Deficiency
T. Hussain, F.R.C.S. A. Emmerson
On several occasions bilateral proximal femoral focal deficiency (PFFD) combined with bilateral paraxial fibular hemimelia has been reported and discussed in the Inter-Clinic Information Bulletin. It has also been considered extensively in other publications5.
Kritter and Gillespie4 summarized the features of the syndrome and expressed the opinion that most authors were in agreement concerning the basic philosophy for the treatment of patients with this condition, especially if the limbs were approximately equal in length and the patient could ambulate on his own feet. The treatment of choice, which was perhaps first advocated strongly by Aitken1, involves conservative prosthetics treatment without surgery. Where such conditions obtain the patient may need prostheses only to bring his height up to that of his peers. Aitken stressed the value of giving the child the option of either walking on his own feet within the confines of his home or elsewhere, or walking on prostheses.
Some patients, however, may not be able to ambulate independently without the use of prostheses or external aids, and in some cases the associated deformities are so severe that prosthetics fitting is extremely difficult or impossible and surgery has to be considered to facilitate the fitting. Hall3 reported one case for whom nonprosthetics ambulation was impossible and the malformed feet made prosthetics fitting difficult. Following bilateral Syme's amputations prosthetics fitting was simpler and more cosmetic while function without prostheses was said to be unaffected. Presumably the child could walk on her Syme's stumps. In contrast to this approach Coventry2 advised a tibial osteotomy to correct the equinus deformity and did not advise amputation.
Despite the general consensus, therefore, it appears that some difference of opinion on treatment procedures still exists. Hence we considered ourselves fortunate to have had an opportunity to study two boys with PFFD, one of whom had had amputations while the other had had no surgical treatment but had been fitted with prostheses at an early age.
Case A was initially fitted at another centre where, at the age of one year and nine months, Syme's amputations were carried out bilaterally ( ). It was felt that this procedure would facilitate the subsequent fitting of prostheses. This boy was referred to us for limb fitting at the age of two years. He was first fitted with rocker-bottom pylons with lap-type hip joints and pelvic bands. Later on these pylons were replaced by a pair of non-articulated limbs. Finally he was fitted with articulated limbs with semiautomatic knee controls, and feel with mobile ankles.
Case B has attended our clinic from early childhood and as he developed he was able to stand on his own feet and get about with considerable agility. Hence it was decided not to operate, but to start his treatment with the fitting of rocker pylons. Plastic sockets with panel openings to allow entry of the feet were manufactured.
The child learned to stand and walk satisfactorily in these devices and they were gradually lengthened. At a later stage the appliances, which initially had no joints, were replaced by prostheses with knee and ankle joints. Pelvic bands were attached to the lateral walls of the sockets and this addition gave a more positive control over the prostheses, and semiautomatic knee locks contributed to the boy's stability. Recently the sockets were modified to allow the feet to rest in a position of equinus in order to improve the cosmetic effect ( ). This modification has had no adverse effect on gait, and the child has not complained of any discomfort.
It was noted that the requirements of balancing produced an increase in lumbar lordosis in both children. This increase was more marked in Case A, and a plastic-laminate support was made to encase the pelvis and spine up to the angle of the scapula.
We were able to watch and compare the progress of two boys of the same age, one who had been amputated and one who had not received surgical treatment of any kind but had merely been fitted with prostheses.
Case A.-Initially this boy had some difficulty in maintaining his balance in both the rocker pylons and the artificial limbs. Without his prostheses he could not move rapidly and he could not balance on his stumps without support from his hands.
He has had to attend a special school for the handicapped and has proved to be a problem to his teachers. This personality problem may be attributable in some measure to the fact that there was a degree of parental rejection as the boy grew up.
Case B.-This boy acquired early balance and mobility, no doubt due to proprioceptive feedback from his intact feet. He attends a normal school and has an average I.Q. When at home he discards his prostheses and moves around the house on his bare feet ( ).
Recent modifications to the prostheses have improved their cosmetic appearance ( ). As growth proceeds we will no doubt have to consider modifications to deal with the increasing length of the feet. It is to be hoped that surgery will not be required. It is our impression that the retention of the feet was of considerable advantage to this child in that he could balance and walk better at an early age, and he still prefers to run around the house without the burden of his prostheses, when he returns home from school.
Limb Fitting Centre Victoria Infirmary Glasgow, Scotland
1. Aitken, G. T., Proximal femoral focal deficiency-Definition, classification, and management, Proximal Femoral Focal Deficiency: A Congenital Anomaly. National Academy of Sciences, Washington, D.C., pp. 1-22, 1969.
2. Coventry, M. B., and E. W. Johnson, Congenital absence of the fibula. J. Bone and Joint Surg.,34A:941-955, 1952.
3. Hall, J. E., and D. Bochmann, The surgical and prosthetic management of proximal femoral focal deficiency, Proximal Femoral Focal Deficiency: A Congenital Anomaly, National Academy of Sciences, Washington, D.C., pp. 77-99, 1969.
4. Kritter, A. E., and T. Gillespie, Bilateral proximal femoral focal deficiency and bilateral paraxial fibular hemimelia. Inter-Clin. Inform. Bull., 11:12:1-8, September 1972.
5. National Academy of Sciences, Proximal Femoral Focal Deficiency: A Congenital Anomaly, George T. Aitken, ed. A Symposium held by the Subcommittee on Child Prosthetics Problems, Washington, D.C., 1969,111 pp.