Follow-Up Studies Of Children With Severe Lower-Extremity Limb Deficiencies
Helmut John, O. M.
The large number of dysmelias encountered in West Germany, especially in the years from 1959 to 1961, presented us with many difficult technical problems. In the following studies we will present material on some of the children treated, in order to illustrate procedures that should be stressed in the future, as well as those that should be avoided.
This boy was born in 1956 with several skeletal dysmelias of the trunk and the left lower extremity. He had a scoliosis with some malformed vertebrae, a malformed left pelvic bone which lacked the os pubis and tuber os ischii, a high luxated hip and dysmelia of the left femur, a luxation of the severely malformed tibia, absence of the fibula, and a malformed and dislocated foot. He also lacked one kidney. When first seen, the boy was one year old (Fig. 1 ). Conservative treatment of the hip and the scoliosis was initially planned. We were unsuccessful in relocating the hip because of the bad pelvic situation. A suggested heart disease and the absence of the kidney prevented us from operating on him.
The First Prosthesis
The boy received his first prosthesis (Fig. 2 ,Fig. 3 ) when he was three and a half years old, at which time he moved about by hopping on one leg or by crawling. Making the cast presented difficulty at the outset: because of the hip luxation we should have used the ischial tuberosity for support, but there was none. We had to make use of the whole thigh and as much as possible of the lower parts of the gluteus maximus for support. We had to make a groove in the perineum to accommodate a bony edge. The calf and foot had to be brought into the line of support. Bony spurs were marked, and this was all we could do. Practice had to determine whether or not the boy would walk. In this case elastic plaster of paris bandage proved to be very effective for making the wrap cast. The positive mold was elongated and then tested by means of a check-socket. Since the entrance proved to be smaller than the diameter of the knee, we had to open up the socket and apply a lacer. Furthermore, it was not possible to use a mechanical knee joint. This situation was not satisfactory, as it caused the boy to develop a gait pattern which could not be easily changed later on.
Epoxy resin Araldit (Ciba) and promoter HY 956 were used. We made a fill consisting of eight layers of perlon tricot and glass reinforcement at the thigh and at the transition from the shank to the foot. In the area of the lacer we used a more flexible resin mixture. On the right and left of the "knee," two light metal bars were incorporated to give more support. A SACH foot was applied. When the boy started taking his first steps, it proved necessary to give him a pelvic belt with a mechanical hip joint.
After a comparatively short time, the child was able to walk alone without any support. Nobody would imagine that the slight limping which was evident hid such a severe deformity. There were no sore parts, even in the areas around the proximal brim of the socket. This absence of irritation surprised us. The socket was perforated in the lower parts to allow for the evaporation of perspiration. The child always wore a perlon tricot stump sock. In 1961 a new prosthesis was built and an arch support was provided for the other foot.
After six years, when it had become clear that no heart disease was present, the boy was amputated through the knee, since there was no function in the calf or the foot (Fig. 4 ). We could now fit an above-knee prosthesis, using a total contact suction socket, a mechanical knee joint with friction, and a SACH foot. At this time the small pelvic belt was not considered adequate, and we made a broad belt of polyethylene with a strong mechanical hip joint and a mechanical joint in the rear (Fig. 5 ). The patient walked without any auxiliary support, his gait was quite good, and he quickly adapted to the knee joint. Since he experienced some inconvenience in school from the broad pelvic belt, we tried to get along with only a pelvic belt made of cotton webbing. We had to change the line of support in his new prosthesis and tried to give support near the center of gravity. This attempt was successful (Fig. 6 ).
This girl, H.H., was born in 1953 with hip luxations on both sides, a dysplasia of the right thigh, absence of the calf, and dysplasia and malformation of the foot (Fig. 7 ). The awkward position of the right foot and the luxation of the left hip were treated first. The treatment of the foot was only partly successful, but later on its clubfoot attitude proved to be advantageous in that the mechanical knee joint could be placed in its proper position without being disturbed by the foot.
The relocation of the hip by conservative means was unsuccessful, so it was operated upon, with a good result. The child wore braces for the shortened (right) leg and as support for the operated (left) leg. The construction on the left side consisted of a long leg brace with ischial tuberosity support, a movable knee joint and ankle, and a hip joint with a brake. The foot rests in a leather sandal worn inside the shoe. On the right side a modified Thomas splint without a hip or knee joint was provided. The two braces were connected, and the pelvis was fixed by the pelvic support after Gocht. The fitting of the right thigh was not very satisfactory, and it would have been better if the child had been given a prosthesis on the right side with knee joint, foot, and movable hip joint. This would have provided better function and cosmesis than the brace construction (Fig. 8 and 9 ).
In 1959, when H.H. was six years of age, she was given her first prosthesis. The leather thigh cuff with Hessingshoe was connected to the bars so that the length of the bars between the hip joint and the knee joint could be increased to accommodate growth. A SACH foot was used (Fig. 10 ). This prosthesis provided good function, but was heavy. Moreover, it was difficult to bend the medial bar around the malformed foot. Frequent repairs were necessary. The child's foot could have been amputated, but we used it to control the prosthesis and as an end to the thigh that was less sensitive than an amputation stump would have been.
In September 1962 the child was fitted with a new prosthesis, as she had outgrown the old one. We decided not to use leather and bars again, in spite of the possibility of adjustment to growth. A plastic socket with an anterior opening for the foot was made (Fig. 11 , Fig. 12 and Fig. 13 ). A check-socket was first built to guarantee good fitting. The result was so satisfactory that a lacer was unnecessary. The mechanical hip joint could be eliminated and was replaced by a textile pelvic belt. The availability of the SACH foot and the knee joint for children solved one part of our problem.
This girl, M.P., was born with a peromelia on the left side and on the right side a dysplasia of the thigh and the tibia, an absent fibula, an angular akylosis in the hip and knee, and a poorly located foot. While the left peromelic stump was in good condition, the right leg was shorter than the left, because of the 75-degree ankylosis. M.P. had an S-scoliosis. The scoliosis was treated first, and the development of the lower extremities was watched. When M.P. was two years of age, the knee joint should have been corrected and a prosthesis subsequently provided. She was actually operated on at the age of four years (Fig. 14 ). A bony wedge was taken out of the knee, and the end of the peromelic stump was corrected (Fig. 15 ). Following this surgery, she was given her first prostheses.
On the left side the child was fitted with a conventional above-knee leg. On the right side the foot had to be accommodated in the construction. It was put into an equinus position, with the toes bending downward, and with a metal ring protecting the foot. The fastenings and rubber kickstraps used can be seen in Fig. 16 Fig. 17 .
At first M.P. had a bad lumbar lordosis. She was insecure and had to use crutches. But after five weeks of training with the prostheses and physical exercise she could stand alone, the lordosis had improved, and she walked with hand assistance. She was proud to be as tall as other children and liked the prostheses. She went home and now she walks without any support. The knee movement is too abrupt, and we want to try the U.S. hydraulic regulator (U.S. Manufacturing Company Hydra-Knee) for the movement.
It is very difficult to build good prostheses for short to medium length ectromelias (Fig. 18 ). The skeleton of the pelvis is changed, the ischial tuberosities are located more medially, and the hip joints are nonexistent. Sometimes one has the impression that the bones have been poured into the body chaotically. In the beginning prostheses were built in the conservative manner. They were not satisfactory, their use was energy-wasting, and it was nearly impossible to provide sitting.
In order to remedy this problem, we attempted to bring a certain amount of automatic movement into the gait. We fabricated a pelvic socket with a resin saddle and an adjustable hip joint in front, in the manner of the Canadian hip joint. When the pelvic socket is lifted, the leg moves forward. In order to obtain this effect, we constructed the joints so that they could be moved against side stops by tilting the trunk (Fig. 19 and 20 ).
But we failed. What had happened? After a lengthy experience, during which we thought that the child was causing most of the troubles, we built an automatic gait machine of this type for ourselves and tried to use it (Fig. 19 and 20 ). We soon found out that we had set the child an almost impossible task. It was not easy to tilt the pelvic socket, and it was nearly impossible to push ourselves forward over the leg in front with a stick or crutch. Further, the SACH foot was not at all suitable for these experiments. Feet with movable ankle joints were far superior, as they are pressed to the floor at heel contact.
We therefore reproduced a construction invented in Ontario (Ontario Crippled Children's Centre Swivel Walker), in which a pendulum action of the body is translated into forward progression, with better results (Fig. 21 ). A construction developed at the University of Heidelberg was also superior, but our whole experience with automatic gait devices was so poor that we felt they should be reserved for genuine amelias only. For these cases the Ontario and Heidelberg devices are doubtless effective.
Using the experiences we had gathered with the walking machine, we again built conservative braces or prostheses for our patient and were surprised by the results. The child ambulated immediately and was not at all insecure. She walked over thresholds nearly 2 inches high and up an inclined plane. Thus, a project that had extended over many months was successfully completed.
The principles involved in our fitting, which we recommend, are:
The feet should have joints.
The calfs are made of hard foam, and are connected with the ankle by quick-hardening resin. After good alignment has been achieved and no further changes are needed, the foam is laminated and then removed to make the prostheses lighter.
The knee joints consist of two brace joints with covered brakes made of stainless spring steel.
The leather cuff which incorporates the seat for the ischial tuberosity has to be manufactured carefully. The plaster of paris casting work is done with elastic bandages. The position of the legs in the braces and the medially located tubera, which typically have bony edges, have to be taken into account. To make an individual seat we use latex milk with sawdust. The mixture is placed on the positive mold, and after approximately six hours at 100 to 140 F, the seat has become an elastic sitting accommodation, exactly according to the model (Fig. 22 ). Over this seat the leather cuff is attached. The metal cuff is riveted to the leather. In order to avoid trouble in the perineum, a piece of soft leather can be placed over the two seats before dressing.
The cuffs to extend the legs are important. A leather strap attached to the calf provides a close connection between the limb and the brace. This connection helps stabilize the body, offers some assistance in bringing the leg forward, and provides positional feedback. The functions of the connecting straps become evident if the extension is disconnected (Fig. 23 ).
To find the mechanical center of motion of the hip joint, we use metal plates with a number of holes, and by trial and error locate the best situation for sitting, standing, and walking. The hip joints should move easily, and we therefore used our joint with oblique ballbearings for the first time. This proved to be highly satisfactory.
The pelvic support is made of two halves of polyethylene connected in the rear with a plate of desmophen-desmodur.
In the beginning, an elastic gluteal strap should be used to provide security; later it may be dropped.
It is important to find a power source to bring the prostheses forward. We have used nylon tapes which start in the rear at the pelvic support, cross in the back before they reach the shoulder, and pull on a short lever below the hip joint or run into the calf. A piece of light metal is bent to conform to the shoulder and padded with rubber. The leg is pulled forward when the shoulder is lifted upward and backward (Fig. 24 , Fig. 25 ).
In ambulation we first start with the so-called rollator (Fig. 26 and 27 ); later crutches are used. The prostheses should not be worn all day long. For relaxation the children are given a tricycle which is driven by the arms. This device provides physical exercise with comfort and less energy consumption (Fig. 26 and 27 ).
At birth, the prospects for these children looked nearly hopeless, but a few years later they were walking around. The success achieved with these difficult cases gives us the courage to go on and to try to solve even bigger problems.
Helmut John, O. M. is associated with Annastift Hannover-Kleefeld, Western Germany (Medical Director: Professor G. Hauberg, M.D.)