The Management Of Bilateral Proximal Femoral Phocomelia

By Gael R. Frank, M.D. Thelma Pedersen, R.P.T. Ray Buddin, C.P.O.

During the past four years, the Oklahoma City Clinic has grappled with the problem of treating a child with bilateral proximal femoral phocomelia and partial paraxial fibular hemimelia on the right. We would judge these anomalies to be also of the type D category of proximal femoral focal deficiency described by Aitken.* This report documents some of the difficulties encountered when we fitted this patient with prostheses.

* Management of the Juvenile Amputee, Prosthetic-Orthotic Education, Northwestern University Medical School, Chicago, 1965, p. 203.

The patient was born at the University of Oklahoma Hospital in 1950 and was followed in the Orthopaedic Outpatient Clinic until 1962, primarily for problems related to the fitting of shoes. At that time prosthetic fitting was initiated primarily to bring the boy's height up to that of his contemporaries (Fig. 1 ).

In 1958, X-rays showed bilateral absence of the femur, with partial absence of the fibula on the right (Fig. 2 ). X-rays taken in 1964 showed ossification of a bony center between the tibia and pelvis which probably represented a portion of the femur. That same year push-pull films showed instability of the articulation between the proximal tibia and what could be interpreted loosely as an acetabulum (Fig. 3 and Fig. 4 ).

We felt that this boy should be provided with especially made assistive devices which would incorporate elements of both prostheses and braces. His feet were placed in extreme plan-

tar flexion in a fabricated device which determined the level of the knee joint (Fig. 5 and Fig. 6 ). Our initial fitting of this boy in bilateral prostheses included plastic ischial weight-bearing sockets extended posteriorly to support the feet and with leather lacers anteriorly, outside knee hinges with optional locks, standard wood feet with toe joints, and ankles with rubber plantar flexion stops and dorsiflexor bumpers.

With these initial prostheses we encountered many problems in fitting and training, which are discussed below by the physical therapist and the prosthetist. These problems were eventually overcome. At the present time the boy is utilizing his prostheses satisfactorily and derives considerable psychological benefit from them, even though they do not add significantly to his function.

Physical Therapy Management

The patient was first seen in Amputee Clinic during February 1962, when he was 11 years of age. Up to that time, no bracing had been ordered. The feet were normal, despite the partial absence of the fibula on the right side, and he was able to ambulate. He was able to participate in sports, such as baseball and football, in the neighborhood with his friends, and also at recess time in school. At this time he first began to feel self-conscious about being so much shorter than other boys his age.

He was referred to physical therapy at this time for evaluation of muscle power and general strengthening exercises in anticipation of gait training as soon as the prostheses were fabricated.

The results of his muscle strength tests at that time were as follows:Table 1

The patient was taught a routine of exercises and encouraged to follow a regular daily exercise schedule at home. Upon receipt of his prostheses, he was admitted to the hospital for physical therapy training, and spent approximately 4 1/2 hours each day in the Physical Therapy Department. The pre-fitting exercise program was continued, and concentrated training in balance was added. All types of activities which would aid in the patient's use of his prostheses were encouraged. Initially he used a four-point gait with crutches, but as soon as he was able to handle himself well he was advanced to a two-point gait. His motivation was good and he progressed satisfactorily. He seemed to enjoy being taller. He was able to do most of the activities suggested (such as stair climbing and sports activities of a stationary nature, like throwing, catching, and batting a ball, also tossing a basketball into the basket). He was willing to try anything that would enable him to become more adept in the use of his prostheses. Shortly before his discharge from the hospital, the boy's parents were asked to review his exercise program so that they could help him at home. A new problem presented itself at this point, inasmuch as regular trousers could not be worn over the prostheses.

When the patient sat down, the feet made the diameter of the knee too great to be accommodated in regular trousers. Splitting the side seams and putting in a gusset was tried. This did not work out, however, as it made the bottom of the trousers too large, and the patient was self-conscious about walking around in culottes. We next tried splitting the trousers at the seam line in the knee area, putting in a gusset and closing it with a zipper. This did not affect the shape of the trousers, but when the patient was ready to sit down, opening the zippers provided room at the knees for the feet to come forward. When the patient was ready to resume his standing position, the zippers could be closed and the trouser modifications were not noticeable.

The patient continued to use his prostheses part time, but occasionally refused to wear them because they limited his activities. Over the next two months he progressed from crutches to canes and wore his prostheses to school regularly.

In the meantime the prostheses had again been modified in that they were lengthened to make the patient approximately 5 feet 4 inches tall. The prosthetist had also been able to offset the knee joints, thus decreasing the diameter of the prostheses in that area.

The patient has just received his new prostheses, and a regular schedule for gait training will be set up. He is now 16 years of age and has a higher level of motivation to wear the limbs than was the case when he was younger.

Problems in Prosthetic Fitting

The initial set of limbs was fabricated with ischial weight-bearing plastic sockets fashioned to quadrilateral shape and extended to include enclosure of the feet with anterior laced openings. Pelvic joints and a belt were used to increase stability. Hip sockets and French lock knee joints were prescribed. It was felt the fixed knees would be safer until the patient learned to ambulate and balance himself at this height.

The side strap joints posed an alignment problem, as the body surface of the sockets was narrow and irregular. It was difficult to position the socket within the joints in proper alignment and balance. In addition, the patient's feet were near normal size, and could not be cast in more than normal plantar flexion. This necessitated setting the socket as far posteriorly as balance- would permit to minimize anterior protrusion of his feet at the level of the prosthetic knee centers .

After several modifications in sockets and joint alignments, we finally settled on Bock positive-locking above-knee setups. These components provided a better means of achieving the desired alignments. The plastic sockets were then bonded onto the knees. The positive locking knees were eventually replaced with regular constant-friction knees, which are being used at the present time (Fig. 7 ). A new set of replacement sockets has recently been fabricated. We have modified the sockets by providing a wider flare in order to increase support at the ischium (Fig. 8 ). In the new sockets the alignment was corrected to eliminate the hyperextension at the knee noted in Fig. 7 .

By Gael R. Frank, M.D., Thelma Pedersen, R.P.T., and Ray Buddin, C.P.O. are associated with the Amputee Clinic University of Oklahoma Medical Center Oklahoma City, Oklahoma