Prosthetic Fitting of A Child With Marked Bilateral Proximal Femoral Deficiencies

William F. Donaldson. M.D. H. Andrew Wissinger, M.D.

The increased emphasis being placed upon the care of children with congenital defects, particularly those whose treatment falls within the province of the orthopedist, has challenged us to develop better and better reconstructive and rehabilitation programs. The response to this stimulation has been particularly apparent in the area of congenital limb deficiencies. As a result, vast changes in the philosophy of the management of these patients have occurred, involving earlier fitting, better prosthetic fabrication, a specific prosthetic prescription tailored to the individual needs of an amputee rather than just a conventional prosthesis, and intensive training by qualified personnel.

The application of this philosophy to the serious individual problems encountered is not only a refreshing experience but an exceptionally rewarding one. At the Amputee Clinic, Home for Crippled Children, we believe that, dollar for dollar, more is accomplished in the rehabilitation of this group of patients than in any other we know. This aggressive and inquiring clinical and scientific approach is a model for the care of other birth defects.

In the care of these patients, the old admonition, "First do no harm," must still continue to be our guide.

The following case report well illustrates the adaptability of present-day prosthetic techniques to a child born with multiple congenital limb deficiencies.

Case Report

G.F., white, male, age seven years one month, was first seen at the Children's Hospital, Pittsburgh, on August 25, 1961, at the age of one year seven months. He was a first child and the product of an uncomplicated pregnancy. The mother was not a diabetic and had had no known illnesses during her pregnancy. In addition, she had not taken any significant medications during the period of gestation.

At birth, congenital abnormalities of all four extremities were apparent (FIg. 1, 2, and 3 ). A detailed description of these defects follows:

The right upper extremity presented a transverse amputation of the forearm in its upper third. The rudimentary radius (Fig. 4 ) was fused to the distal end of the humerus. Clinically, a vestigial thumb was present.

The left upper extremity showed a longitudinal central ray defect, with absence of index and long rays and their corresponding metacarpals (Fig. 5 ).

The left lower extremity had an absent fibula, with a suggestion of a calcaneotalar coalition and an absent ray (Fig. 6 and 7 ). Roentgenographic examination revealed that the femur was absent except for its epiphysis and adjacent metaphysis. The acetabulum was hypoplastic and exhibited marked dysplasia (Fig. 8 and 9 ).

The right lower extremity had a similar foot deformity, with a more definite calcaneotalar coalition and fewer ossification centers for the cuneiforms (Fig. 6 and 7 ). The fibula was absent, and the proximal half of the femur was deficient on this side. The acetabular development, while not normal, was better than on the opposite side.

The patient walked at 11 months of age. His gait was characterized by waddling and circumduction. His trunk "hung" on his lower extremities as he stood and walked.

This child's obviously excellent mentality and consistent attempts to ambulate encouraged us to try to aid him mechanically.

Various possible surgical reconstructive procedures were considered--e.g., an attempt to stabilize the proximal ends of the short femora in the acetabula. Most of these ideas were discarded, as it was felt that they would either entail the sacrifice of some of the child's independent function or were inadvisable because of potential interference with his growth. The only surgical procedures that have been performed on his lower extremities up to the present time are the excision of the rudimentary fibrous fibular bands and realignment of his foot (astragalus) beneath the tibia. The results of this surgery have been quite satisfactory and have made his prosthetic fitting easier.

Following this surgery, the child was provided with braces to stabilize his deficient extremities (Fig. 10, 11, and 12 ). Although his ambulation was somewhat improved with these braces, they were obviously not a satisfactory answer cosmetically or, indeed, functionally.

Therefore, it was decided to construct extremities of a prosthetic type in order to better meet this boy's needs. In the prescription of these prostheses, certain factors were felt to be necessary. They should provide stability yet be capable of independent motion. They should be of such length that the boy's height would approximate that of his classmates.

Fig. 13, 14, and 15 show the types of prostheses that were constructed. Fig. Fig. 16, 17 and 18 show the prostheses in place.

The right prosthesis, which is of plastic, has an ischial weight-bearing socket and a SACH foot. It is constructed so that a portion of the boy's weight can be borne on the heel of his right foot. An opening in the distal anterior portion of the socket permits easy application of the prosthesis. This opening is then closed by using cohesive straps, thus providing stability for the tibia and the foot.

The left prosthesis is of quadrilateral socket construction, with the inner wall again shaped to accommodate the heel and permit some weight bearing. Again, an opening in the anterior distal portion of the socket provides easy application. A knee joint, as well as a SACH foot, has been installed in this prosthesis, as can be seen in Fig. 13, 15 and 16, . The height of the prosthesis from the floor to ischial seat is 24 inches.

Seeing this child walk about on the level without cane or crutches is one of the most rewarding experiences we of the Amputee Clinic have had. He can go up and down steps using a railing. He can fall down and get up again without external assistance.

This case is reported in the hope that our experience in fitting prostheses to a patient with marked bilateral proximal femoral deficiencies, although highly individualized, may nevertheless be of help to others facing similar problems.

William F. Donaldson. M.D. and H. Andrew Wissinger, M.D. are associated with the Amputee Clinic Home for Vrippled Children Pittsburgh, Pennsylvania