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Chapter 36A - Atlas of Limb Prosthetics: Surgical, Prosthetic, and Rehabilitation Principles

Special Considerations: The Multiple-Limb-Deficient Child

Ernst Marquardt, M.D. 

Prior to the 1950s adult prosthetics in Germany ranged from good to excellent in both the quality of the prosthesis and medical care. However, for children, each prosthetist had to build on his own personal experiences. There was no systematic treatment of the limb-deficient child, nor were there organized limb deficiency clinics or child amputee clinics. There was no special education or organized interchange of ideas, practices, and experiences among workers with child amputees. No center was able to claim the presence of a complete clinic team, such as that started in Grand Rapids, Michigan, or other similar teams in the United States. There were no special devices or equipment for children's prosthetics. Patients with congenital limb deficiencies were treated on an individual basis in the regular orthopaedic hospitals. In some of these hospitals, such as in Berlin, Hannover, Heidelberg, Miinster, and Volmarstein, experience in certain limb deficiencies existed in the orthopaedic and prosthetic services. However, they did not work together as a full team, and each center developed its own ideas without exchange of ideas with others in the same field.

The philosophy of upper-limb devices centered on the development of technical aids for limb-deficient persons. Soft leather sockets for transradial amputees had adaptors so that spoons, forks, pencils, or other items could be attached directly to the prosthesis.In 1917, Biesalski demonstrated a 9-year-old boy with new bilateral terminal devices. On one side, the boy had the "Finger-Klaue" and, on the other side, the "Spann-Klaue," both developed by Fischer in Berlin. This boy was able to demonstrate that both of these were as practical as the hook; however, these terminal devices were not widely used and were soon forgotten.

It was widely assumed that children with transverse deficiencies of the upper limbs or children with traumatic amputations should be fitted with prostheses only after they had completed their growth. Because of this philosophy, it is understandable that the Krukenberg procedure had greater acceptance in Germany than in the United States. With this procedure, the bilateral amputee was able to achieve independence in many of the activities of daily living. For the very high upper-limb amputee, the Heidelberg pneumatic prosthesis gave additional independence for some individuals but did not have a wide sphere of influence.

Lower-limb prostheses for children were even more provincial and personalized. They were generally fitted with the most simple walking devices that could be constructed. Many of these were stirrups fixed by splints or leather sockets that functioned as extensions.

THE BEGINNING OF MODERN PROSTHETICS

The 1950s proved to be an exciting and revolutionary time for children's prosthetics in Germany. Three factors were central to the changes that occurred in the field. The first of these was the visit of the German Study Group to the United States in 1952 and the outpouring of new ideas resulting from this stimulation. The second was the international cooperation that developed with Kessler of New Jersey, who provided advice and stimulation to his German colleagues in the development of comprehensive clinic programs. The third event was the thalidomide catastrophe.

In 1952, the German orthopaedic surgeon Hepp and a group of experts in prosthetics came to the United States to review the progress and problems encountered in the prosthetic centers in that country. In the following years, Hepp and his coworker Kuhn developed special casting techniques for the upper limb and the fabrication of a new plastic socket. This is now well known as the Minister socket (Fig 36A-1.). They introduced the active hook, which proved to be the most universal and most functional of all terminal devices. Their work in research and development was made easier by the German federal government providing money for prosthetic centers, first in Kiel and later in Miinster. However, despite these advances, there were still no special units for children in all of Germany.

The second of these major factors was my visit to the Kessler Institute for Rehabilitation in New Jersey and the development of a close friendship with Kessler. I also made contact with numerous other prosthetic clinic teams in the United States, and a generous exchange of information resulted. While observing Kessler's patients with kineplasty and body-powered as well as pneumatic prostheses, I learned of enormous possibilities inherent in the body's own compensatory functions and of the limited value of artificial limbs for congenitally armless persons. By watching the children with and without their prostheses, my colleagues and I realized the need to improve all of the body's own compensatory functions as the child develops activities of daily living. Following these experiences, we developed the Pat-A-Cake prosthesis for armless babies, which allowed us to give them simple grasp at an early age. When these were fitted early, combined with prosthetic training that emphasized motivation and play, better results than had previously been thought possible were obtained.

During the years 1958 to 1962, there was an enormous increase in newborn children with multiple, symmetrical, longitudinal limb deficiencies. In the upper limbs, there was a predominant reduction on the radial side of the hand and forearm; this was combined with lower-limb deficiencies in either the tibia, femur, or both.[†In November 1978, 5,294 upper limbs of 2,647 thalidomide victims were reviewed and registered by the "Stiftung Hilfswerk fur behinderte Kinder" Bonn-Bad Godesberg.] These limb deficiencies were often associated with abnormalities of the spine and potentially with every other body system. These abnormalities were later shown to be associated with maternal ingestion of thalidomide early in pregnancy.

These types of longitudinal deficiencies were not new and had been reported since antiquity. What was new was the enormous number that seemed to be almost an epidemic of multiple-limb-deficient children. Prior to the thalidomide episode, between 1953 and 1958, 37 children were seen in the orthopaedic hospital at the University of Heidelberg. However, from 1959 through 1962, 216 children had multiple-limb deficiencies. In the following 2 years, after the withdrawal of thalidomide from the market, only 10 children were seen with this type of limb deficiency. In view of these numbers it is understandable that a major investment in time, research, and ingenuity was directed to the multiple-limb-deficient child.

PHILOSOPHY OF TREATMENT-1960s

The simple fitting of a prosthesis or multiple prostheses for these children is not adequate. As we struggled to make them as independent as possible, a whole philosophy of care developed in our center.

The treatment of a limb-deficient child is centered in the family, not in the hospital. The mother is the child's best therapist under the supervision of the clinic team-physician, occupational therapist, and physical therapist (Fig 36A-2.). All instruction should be channeled through her, the father, or both. Whenever possible, the training, whether the child uses the prosthesis or not, should be conducted in the home.

It is important that all possible sensory contact with the feet be stimulated. The infant should be permitted to see his feet uncovered and encouraged to play with them (Fig 36A-3.). If there are small digits or hands off the shoulder they should be trained to appreciate touch and grasp. Even if they seem functionless, eventually they may have important prosthesis control functions, which will need the most acute sensory input. If there is hip dysplasia, the Pavlik harness or similar treatment is recommended, since the feet will be allowed full freedom. If there are no arms, the head must be protected from bruising as the child starts to walk. A helmet or a ring of sponge rubber or other material can provide such protection until stability of walking has been achieved (Fig 36A-4.).

If the child can eat and play, even deformed hands are better than a prosthesis. However, if one side is functionally behind the other and cannot be used in bimanual activity, a prosthesis is necessary. The infant at 8 months of age can be successfully fitted with a passive prosthesis allowing gross grasp. At this age, it is expected the infant will incorporate the prosthesis into its body image. If it allows for increased function and activity, the prosthesis should be accepted without difficulty.

The provision of an actively powered prosthesis starts about the beginning of the second year of life. Shoulder movements or, if present, small digits emanating directly from the shoulders can be used to control externally powered prostheses in the sixties, powered by carbon dioxide; in the eighties and nineties by batteries; in the seventies, by both in hybrid systems (Fig 36A-5.). The active prosthesis for children over the age of 4 years has an active grasping function with active wrist rotation and an active elbow joint with automatic locking. Training of the child with the prosthesis is adapted for the stage of development present at that time. The actual technical provision of the prostheses and exercise within a therapy setting are insufficient. If these prostheses are to become functional, they must be supplemented cleverly by individual pedagogic and psychological guidance of the children and their parents, which is provided by the prosthetic team. Nevertheless, the success of bilateral arm amputees fitted with pneumatic prostheses cannot be reached by amelic and pho-comelic children.

On the other hand, 65 of 67 children fitted with different types of pneumatically powered prostheses have ultimately rejected them, some after years of wearing and with excellent prosthetic accomplishment. Only 2 of these 67 continued to wear externally powered arm prostheses (Fig 36A-6.).

The Heidelberg philosophy may then be summarized as follows:

1. For the upper-limb amelic child, the feet and not the arm prostheses will provide maximum function. For the phocomelic child, all digits, both upper and lower, should be used to compensate for loss of normal function.
2. In general, conventional body-powered prostheses for amelic and phocomelic children provide so little function that they are likely to be rejected, particularly by those who are able to use their feet as hands. By the same token, externally powered prostheses are too heavy and complicated. They provide too little effective function for the patients need, and this, coupled with high cost and discomfort of wearing, causes rejection.
3. Prostheses for amelic and phocomelic children are still in the experimental state. The major problem is in positioning the terminal device in space and not so much the terminal device itself. Simpson has developed a brilliant technical solution to this problem, but the weight and complexity of the mechanism have precluded its general acceptance.
4. When amelic or phocomelic individuals request an arm prosthesis at or after the age of puberty, this request is considered and discussed with the patient, along with a discussion of all pros and cons. When a decision is made to prescribe prostheses for these individuals, it is our policy to prescribe one functional externally powered prosthesis for the dominant side and a cosmetic arm with the power pack in it for the nondom-inant side. Both are fixed on a Simpson frame. This is at best a compromise and provides the best possible appearance, the lightest weight, and some function (Fig 36A-6.). Practically all of these individuals continue to use the feet as hands.

Of major importance is the recognition of the fact that the prosthesis should never impede the child's function. When it is recognized that wearing a prosthetic device diminishes important functions, the prosthesis should be removed in favor of such function as was present without it.

In 1962 and 1963, the Federal Republic of Germany opened special units for "Dysmeliekinder" in eight other centers, in addition to those clinics that already existed in Miinster and Heidelberg. Programs for research and development of prosthetic devices as well as the testing of prostheses and technical aides for limb-deficient children were developed. Annual workshops and meetings were organized, and in 1964, I made my first statement about the development of compensatory functions of the clubhand by the patient and warned against early operative intervention. In addition to developing new body-powered and externally powered prostheses for the upper limbs, a new electrically driven vehicle was developed in Miinster by Kuhn. My Horowitz lecture in 1968, "The Total Treatment of the Limb Deficient Child," and the publication in 1974 of "10 Jahre Entwicklung und Erprobung von Hilfen und Hilfsmitteln fur behinderte Kinder" concisely spelled out the development of prosthetics and technical aids and training for limb-deficient children, which I believe should be integrated into the total philosophy of care. In the years following the thalidomide episode, in which some 2,500 children were victimized in the Federal Republic of Germany, such a philosophy was developed at Heidelberg and in the other centers. This was espoused and financed by the government and especially by the Social Security System for the benefit of these victims. On the basis of evaluation of 2,000 of these 2,500 children, my conclusions on the treatment and rehabilitation program have continued to develop and, in instances, to change. The care of these patients continues. Secondary damage such as arthropathy or osteoarthritis to the hip joints, knee joints, etc., as well as to the spine, are increasing problems for future years.

SPECIAL SURGICAL PROCEDURE FOR THE MULTIPLE-LIMB-DEFICIENT CHILD

A number of surgical procedures have been used to advantage in children with multimembral deficiencies and might have application only occasionally in selected cases of single-limb deficiency. Because of the degree of functional disability in patients with multiple deficiencies, procedures that are designed to provide even limited improvement in function are more frequently indicated. Several procedures that have been used to treat bony overgrowth, enhance function of transhum-eral prosthetic use, and provide surgically assisted prehension in a forearm stump as well as considerations in the positioning of residual prehensile members are reviewed in some detail.

Stump Capping

Contrary to a generally held opinion of a few years ago, bony overgrowth has, in fact, been observed in transverse diaphyseal deficiencies. This overgrowth and its concomitant stump attenuation is particularly troublesome when it occurs in a weight-bearing segment such as the tibia and is, of course, most disabling when the opposite limb is also the site of a deficiency. Bony overgrowth of a transhumeral deficiency may be equally disabling. It can eventuate in catastrophe should it occur in a bilateral short transhumeral stump and necessitate revision to a higher level such that the prosthesis would be of the shoulder disarticulation type rather than the transhumeral type.

The conventional practice in treating bony overgrowth has, in the past, taken the form of either resection of the overlying bursa and shortening of the bony stump or, in some cases, a formal reamputation at a higher level. In 1972, Swanson reported the development of a silicone rubber implant for capping a transtib-ial amputation stump. He also used this silicone cap for revising amputation stumps in which bony overgrowth had occurred. Buchtiarow attempted stump capping by using a bone and cartilage transplant for transfemo-ral stumps. Stimulated by these experiences, I have developed a technique for capping stumps with osseous overgrowth that uses autogenous cartilage-bone transplants. The goal is conversion of a transhumeral or transtibial amputation into a stump resembling that seen in a disarticulation (Fig 36A-7.).

In the case of a transtibial stump, the easiest available transplant is the head of the fibula and, in the case of a humeral stump, the spina iliaca posterior, but in the quadrimembral-deficient child, various reconstructive procedures on other parts may make a transplant source readily available . When a proximal femoral focal deficiency (PFFD), for instance, is present, the cartilaginous and bony cap from the femur may be used. In the same condition, when knee arthrodesis is planned, the articular surface of the distal portion of the femur may be salvaged for transplant.

The operation may be used in cases of overgrowth in congenital deficiencies of the humerus or in treatment of the condition in a transfemoral or transtibial amputation. In the humerus it seems preferable to use a cartilagebone transplant without a growth plate, unless there is good-quality soft tissue overlying the end of the stump or the patient has bilateral short transhumeral stumps.

In the weight-bearing bones, on the other hand, it is desirable to procure a transplant with an epiphyseal plate to obtain additional length. In those cases where amputation is carried out for trauma, an epiphysis from the amputated limb may be used. When the procedure is done for overgrowth of the humerus, an effort is also made to attain optimal growth. In most instances weight-bearing training will stimulate continued growth in the proximal humeral growth plate. If the condition of distal skin permits, transplantation of a growth plate here may be carried out. For cases with severe scars at the end of the humeral stump(s), the wide excision of the scars followed by the stump-capping procedure and by a musculocutaneous latissimus dorsi island flap with its intact neurovascular supply is recommended; the latter is contraindicated in persons who need crutches or canes.

Surgical Procedure

The incision is planned to avoid scarring in the skin of the end-bearing area. A medial or lateral longitudinal incision is made, starting a few centimeters proximal to the end of the stump (Fig 36A-8.,B). The bursal sac is opened, and the bony overgrowth is transected at its entry into the bursa and removed. Two or three periosteal and muscular flaps are then developed on the sides of the diaphysis and are reflected proximally. The di-aphysis of the bone is then split longitudinally for a distance of at least 3 to 4 cm proximally from its tip (Fig 36A-8.,C). The split ends are gently spread apart, with care taken to prevent fracturing. The cartilage and bone transplant is prepared with two grooves fashioned on either side of the bony portion to accept each of the arms of the split long bone (Fig 36A-8.,D). Fixation of the transplant may be accomplished with two crossed Kirschner wires or with a centrally placed long intramedullary screw. The defect between the split ends of the long bone is packed with additional autogenous cancellous bone (Fig 36A-9.). The periosteal-muscular flaps are then reattached to the transplant with sutures passed through small drill holes in the graft. The wound is repaired so as to avoid skin tension, and in lower-limb stumps, a rigid plaster dressing is used. In transhumeral stumps a soft compression dressing is preferred.

Postoperative Care

It is important to recognize that the success of this procedure depends not only on the surgical technique but also on the postoperative physical therapy training program, as well as on acceptance and use of a carefully designed prosthesis. These children should be maintained on a daily physical therapy program throughout growth. Just as the normal individual or the athlete requires constant conditioning to maintain maximum muscle strength and joint motion, so the limb-deficient child requires an ongoing training program.

Approximately 3 months after the stump-capping surgery, the conditioning, which is called for emphasis "end-bearing training," is commenced. The therapist teaches the patient to apply weight to the reconstructed stump. Initially, 2 or 3 kg of pressure is used and gradually increased until the patient is able to take at least 50% of his body weight directly over the stump end. During this training an effort should be made to apply pressure to different surface areas of the stump so that the loading will ultimately be distributed over the entire stump end. This training is conducted in coordination with the other daily exercises necessary for rehabilitation. End-bearing training should be repeated at intervals throughout the day.

Immediately after the end-bearing training session the therapist should also show the patient and the parent the technique of stretching the skin distally over the stump to prevent contracture or tightening of the skin over the reconstructed end. During this period of time the healing and conditioning of the stump should be evaluated and the decision made as to the time for prosthetic prescription and training. In the weight-bearing limbs the prosthesis should be designed to use the end-bearing capacity of the stump to ensure continued hypertrophy and tolerance.

Case Report

The child in Fig 36A-10 (a-c)., Fig 36A-10 (d-f)., and Fig 36A-10 (g-j). was born on July 12, 1965, the first child of healthy parents with two normal siblings. There was no history of congenital limb deficiency in the family, nor were there other congenital anomalies. In the early pregnancy, however, the mother had had considerable illness and taken numerous medications. At the time of birth it was noted that the child had transverse deficiencies of all four limbs, with the forearm deficient in the upper third bilaterally and the leg similarly deficient in the upper third bilaterally, as well as aplasia of the fibula. Additionally the child had micrognathia and dysplasia of the tongue. She was first seen in my outpatient clinic on May 2, 1966, and in August her first stubby prostheses were fitted to her lower limbs. In January 1967, she was fitted with cable-controlled transradial prostheses and transtibial prostheses with a thigh corset, but no knee joints were prescribed. In 1968, she was fitted with a transtibial prosthesis for the left leg with supracondylar wedge suspension. On the right side, because of the extreme short transtibial stump, she was fitted with a bent-knee prosthesis, with the knee flexed at 90 degrees, and she walked independently with these prostheses. In June 1969, she received two new transradial prostheses with Miinster sockets and Dorrance hooks, as well as two new lower-limb prostheses. By December 1970, osseous overgrowth of the left transtibial stump was noted, and by October 1972, this had increased even though she had received treatment of skin traction and "extension therapy" of the skin-manual stretching by the mother. New sockets were prescribed. In October 1975, there was increased spiking at the distal end of the tibia, which was now being handled with liners in the plastic sockets of her prosthesis. By June 1976, she had a painful bursa about the area of spiking at the distal end of the tibia of the left transtibial stump, and there was a varus deviation of the tibia despite the fact that the fibula was absent. In July 1976, a stump-capping procedure of the left transtibial stump was carried out with a homologous cartilage bone transplant to the cap supplemented by autogenous cancellous bone to fill the gap between the two branches of the split tibia. In December 1976, the same procedure was carried out on the right tibia. Three months after surgery, end-bearing training was instituted, and the left side did well; however, the right showed reduction of the transplant and increased pain. It required revision in March 1978. The left transtibial stump remained healthy. Presently, this patient is wearing a transfemoral knee prosthesis on the right side with 90-degree knee flexion and a transtibial prosthesis on the left for 12 to 13 hours a day. She wore her forearm prostheses only part-time; nonetheless, she is completely independent in all activities of daily living.

Angulation Osteotomy of the Humerus

An amputee with a long transhumeral deficiency can use a body-powered prosthesis quite effectively. With the conventional fitting, however, the important and useful function of active pronation and supination of the forearm is absent. This may be provided by passive rotation of the terminal device at the wrist, but must be preset by the patient. This is particularly difficult for the bilateral transhumeral amputee to accomplish. In addition, mediolateral positioning of the forearm segment and terminal device through humeral rotation is usually deficient in strength and range. Passive rotation through a turntable elbow unit or the use of an externally powered rotation unit has never been completely satisfactory.

Although most amputees with long or middle-length stumps have essentially normal shoulder function and strength, full abduction and, particularly, full shoulder rotation cannot be transmitted through the stump-socket interface to the terminal device with the conventional transhumeral fitting.

In an effort to provide the transhumeral amputee with a simpler and more effective prosthetic device that might help to provide these additional functions, I have developed a technique of angulation osteotomy of the humeral stump. Following this osteotomy of the distal portion of the humerus, an open-socket prosthesis suspended only by two straps may be used. In the long transhumeral amputee the dorsal end of the stump may be left exposed for tactile sensation. With this fitting, the artificial elbow joint is set somewhat proximal to the distal end. External locking hinges are used. In the middle-length stump the distal angulated humeral segment is maintained by one or two straps. This strap suspension is easy for the bilateral amputee to apply himself. More important, however, is that it frees the shoulder for full abduction and elevation, as well as allows full internal and external rotation to be transmitted to the prosthesis. The flanges necessary for stability in the conventional prosthesis are eliminated, and rotational stability and motion are provided by the angulated distal end of the stump in the socket. When the forearm is in a moderately extended position, the humeral rotation can also transmit a useful amount of pronation and supination at the terminal device.

The angulation osteotomy should be used primarily for the bilateral long transhumeral amputee as well as for the elbow disarticulation stump with hypoplastic humeral condyles. In the case of middle-length stumps, however, the shortening of the humerus conditioned by the length of the angulated humeral segment should be measured before surgery by help of a radiographic sketch. The angulation osteotomy is contraindicated if the stumps would be unable to touch each other in front of the chest after surgery or additional stump lengthening would be indicated.

Surgical Procedure

For planning of angulation osteotomy, the length of the remaining humeral segment should be carefully measured. The planned length of the distal osteotomy fragment will depend on the volume of the stump. In a long slender stump, as little as 3 cm for the angulated distal fragment may be acceptable. To accomplish this, the osteotomy must be started 4 cm above the distal end of the stump. In a large, flabby stump, however, it is necessary to have at least 5 cm of bone in the angulated segment to provide adequate suspension for the socket. An anterior angulation of the distal end in the sagittal plane of from 70 to 90 degrees in the neutral-zero method of measurement is planned.

The incision should be planned to extend no further distally than the lower level of the osteotomy site. It is important to prevent any damage to the blood supply of the distal fragment. No stripping of the periosteum of this distal segment is carried out. An incision 5 to 7 cm long is made from the distal level of the planned osteotomy site proximally. The periosteum is incised longitudinally directly over the area from which the wedge of bone is to be removed, usually anteriorly. The periosteum is carefully dissected from the area of wedge removal and further periosteal dissection carried only proximally to avoid denuding the distal fragment. Approximately three quarters of the thickness of the diameter of the bone is then removed in the shape of a trapezoid wedge. In the child, this can be most easily done with a bone-cutting forceps or rongeur. Proximally and distally the open cortex may be notched with a fine rongeur so that when the angulation osteotomy is closed, the notches will interdigitate to provide some stability and good bone apposition (Fig 36A-11.).

The posterior cortex, supported by its intact periosteum, is now bent into the planned angulation. In the younger child this produces a fairly stable "greenstick" fracture but maintains stability. The osteotomy is then fixed with a single Kirschner wire, which passes obliquely across the angulated bone at an angle of 45 degrees from the distal fragment through the proximal fragment and penetrates both cortices of each fragment. If, during the attempt to bend the posterior cortex, fracturing occurs and inherent stability is lost, a second Kirschner wire passed parallel to the first but crossing the osteotomy should provide adequate fixation (Fig 36A-12.). If there is any separation at the osteotomy site, the defect may be packed with cancellous bone from the removed wedge. The periosteum is then repaired. Suction drainage is instituted, and the wound is closed in layers. A compression dressing is applied, but no plaster of paris cast is necessary. Suction tubes may be removed in 48 hours.

Patients with a shorter transhumeral stump may be treated by exposing the bone on its posterior side to carry out the posterior angulation osteotomy.

Postoperative Care

At 6 to 8 weeks after angulation osteotomy the radiographs should demonstrate secure union. The Kirschner wires may be removed at this time. As with the stump-capping procedure, end-bearing training as well as gentle traction on the osteotomy site with the parent's finger should be instituted as soon as bony healing is secure. This is intended to condition and prepare the stump for prosthetic use and over the long term to stimulate growth of bone in both size and length. The fitting with the new prosthesis may proceed at approximately 6 to 8 weeks after the operation (Fig 36A-13.). In a young child it may be expected that the given angle will diminish approximately 1.3 to 1.5 degrees for every month of growth, but quicker after end-bearing training only and slower after daily traction exercises corresponding to the Bavarian "Fingerhakelu."

The Krukenberg Procedure

In 1917, Hermann Krukenberg developed an operative procedure to convert a long transradial amputation stump into a pincer-like grasping organ with tactile sensation. This operation has not received wide acceptance in the United States, probably due to cosmetic objections, but in Germany and elsewhere it is quite widely used.

It has been generally accepted that the prime indication for the Krukenberg procedure is the bilateral transradial amputee who is blind and absolutely requires tactile sensation for independence. Children with mul-timembral deficiencies, however, who have bilateral long or medium-length transradial stumps should also be considered for this procedure. Swanson reports on excellent results in children and extends the indication "for patients living in areas where prosthetic services are unavailable. . . .The advantages of readily available prehension with sensation are significant, especially in dressing, bathing, eating and toilet activities."

Surgical Procedure

In his original technique, Krukenberg used a simple U-shaped incision and bisected the forearm with either one or two V-shaped flaps, based proximally, to cover the proximal portion of the cleft of the split forearm.A large free-skin graft was necessary to cover the grasping surface of the ulnar half of the forearm. To diminish bulk, some of the muscles of the distal part of the forearm were excised. Bauer, in 1949, described a similar incision but employed radical excision of all distal muscles except the brachioradialis, pronator teres, and supinator to allow primary skin closure without grafting. Kreuz, during and after World War II, performed approximately 700 Krukenberg operations. He did not excise muscle in the belief that a better blood supply was thus preserved and a better grasping surface and proprioception created. Large skin grafts were, however, required on the grasping surface of the ulna.

In an attempt to combine the advantages of the Kreuz and Bauer modifications and avoid the necessity of excising muscles for closure, I use, like Tubiana, an incision with two L-shaped flaps rather than the simple bisecting U-shaped incision (Fig 36A-14.). In closure, the volar flap is advanced or rotated over the grasping surface of the ulna and the dorsal flap over that of the radius so that the opposing tactile surfaces will be covered with skin having normal tactile sensation. Skin grafts are applied to the outer surfaces of the digits where sensation is less important. Interdigitating V-flaps, based proximally, are used to cover the proximal web of the cleft. It is important that the surgeon review the anatomy of the cutaneous nerves of the forearm (Fig 36A-15.) so that these are not transected in the incisions used for creating the flaps.

After developing the skin and fascial flaps, the forearm muscles are carefully dissected into radial and ulnar groups. The interosseous membrane is divided throughout its length along its ulnar periosteal attachment to avoid damage to the interosseous vessels and nerves (Fig 36A-16., A).

The musculature that will motor the forceps action of the reconstructed limb must be carefully dissected and preserved. The supinator will become the major adductor. The pronator teres muscle is part abductor and part adductor. The brachioradialis will open the digits. After division of the muscle groups, gentle separation of the radius and ulna is carried out to create an angle of 25 to 30 degrees. The distal fibers of the supinator must be carefully observed during this part of the procedure since overstretching or tearing of its muscle fibers must be avoided.

If the radius is approximately 22 cm in length-an average normal for a 12- to 14-year-old-the length of the opening V of the digits will be approximately 12 cm and the opening span between the tips approximately 8 cm. Shorter stumps may have less opening, but useful function and power can be expected. In the very short stump, proximal transposition of the radial insertion of the pronator teres may be advisable to gain additional opening space and span.

When the dissection is completed and the radius and ulna spread to a maximum without damaging muscle, closure is carried out. The two L-shaped flaps are rotated so as to cover the grasping surfaces and sutured to the skin over the tips of each digit. The V-flaps are sutured in an interdigitating fashion to cover the cleft between the separated radius and ulna. Skin defects will be present on the outer side of each forearm branch. These are covered with splint-thickness or free full-thickness skin grafts taken from the groin. Suction drainage is placed between the V-flaps in the cleft. Closure is completed with care to avoid undue tension (Fig 36A-16.,B and C).

Following closure, the two branches are separated to a little less than maximum opening position and sterile dressings applied with a wedge of soft material to maintain the open position (Fig 36A-16.,D).

Postoperative Care

The suction drainage tubes are removed 2 days after the operation without disturbing the other dressing. All wounds are dressed at a minimum of 10 to 14 days aftersurgery. It is advisable to thoroughly wet the dressings down so that they can be removed easily without damaging the skin grafts.

If healing is satisfactory, physical and occupational therapy may be started. The voluntary actions to be exploited are pronation and supination. Pronation has been converted into an opening motion as the pronator teres, acting with the biceps and with the brachioradia-lis, abducts the radius away from the ulna, which is stabilized by the triceps. Supination has been converted to a closing action since the supinator adducts the radius against the ulna. During the training period the patient must be taught to properly use and balance these muscles to gain this function.

Between training sessions the patient should be provided with a well-shaped wedge to maintain maximum opening and prevent contracture. For some period of each day, however, the wedge is removed and a circumferential bandage applied to maintain the fully closed position. In this way maximum mobility of the joint is maintained and the contractures prevented.

As the patient gains the primary function and strength in the forceps action of the stump, additional devices such as wedges or clip-ons are fabricated to hold particular instruments for writing or eating. These assistive devices increase the versatility of the stump and allow the patient to perform specially needed tasks more easily.

In addition to these special devices, experience has shown that those patients who have good vision want, in addition to their Krukenberg stump, prostheses that will provide some function but are primarily desired for aesthetic reasons. These patients refer to the prostheses as their "Sunday hands." For this purpose a conventional body-powered prosthesis in which the socket is modified to accept the Krukenberg stump is usually prescribed. A powered electric system is also possible in which the microswitches are placed so as to activate terminal device opening by supination and closing by pronation (Fig 36A-17.).

Case Report

The child in Fig 36A-18. was born Dec 20, 1963, of healthy parents with two normal siblings. There was no history of drug ingestion, but there had been a threatened abortion in the third month. At birth it was recognized that the child had bilateral total carpal transverse deficiencies of both upper limbs. The child was first seen in March 1965, at which time open-end prostheses were prescribed. He was admitted for training in the use of the prostheses but preferred the tactile contact with the stumps, especially on the right side. In 1970, 10X Dorrance hooks were substituted for his earlier terminal devices and, on June 15, 1978, a Krukenberg operation of the left upper limb was carried out. The decision to operate on the left side rather than the right was based on a subluxation of the proximal end of the radius on the right side and a tendency to dysplasia in this limb. Postoperatively he became an excellent Krukenberg user and at first, used the right open-end prosthesis in association with his "Krukenberg." Then he rejected every prosthesis, even the myoelectric system. After high school, he studied law and is now a judge. He is completely independent in activities of daily living and in his profession and uses the Krukenberg limb in cooperation with the right forearm stump.

Surgical Considerations in the Radial Clubhand

The implication of radial clubhand as a component of multiple deficiencies is quite different from when it occurs as an isolated anomaly. In the child with multi-membral anomalies in which longitudinal deficiency of the radius (radial clubhand) is present, consideration of the contraindications to surgery deserve more emphasis than do the indications. The position of uncorrected deformity in the bilateral case may have very significant functional advantages, which should not be sacrificed in attempts at cosmesis.

It has already been emphasized that in the infant and small child the shortened arm must be left exposed and not covered or concealed by clothing, as parents often are wont to do, so that the child will develop maximum function of the fingers, elbow, and shoulder. During the day, the hands and fingers should be free for play. In addition, corrections with traction, shown and controlled by the physical therapist, should be done by the mother or father every day several times to minimize the contracture. Splints to maintain the corrected position are used, if at all, only at night. Most important is the physical therapy program to emphasize maximum mobility of all joints.

In watching and assessing the developing function in these children the careful observer will note important compensatory functions that depend on the radial positioning of the hand. Many of these patients have limited elbow flexion, and radial abduction of the wrist is an important substitution to bring the hand to the mouth or face. Radial abduction combined with volar flexion is often used for grasping. Hypermobility of the wrist and metacarpals substitutes for lost pronation and supination (Fig 36A-19.). and greatly enhances dexterity. In the radial clubhand the fifth finger is much more often used for fine pinch than is the index finger, when there is a hypoplastic or absent thumb, and may be more suitable for pollicization than the index in terms of useful function.

The range of motion of finger joints, particularly the metacarpophalangeal joints, is important in assessing function. In many of these children the power of the hook created by radial deviation of the hand is the only power grasping action. They will develop fine grasp between the fingers, especially on the ulnar side.

These children must be carefully evaluated by both the surgeon and therapist before surgical treatment is planned to be sure that no important functions will be lost by centralization of the hand (Fig 36A-20.). The contraindications should be recognized. The most important is limited elbow flexion, which, with centralization of the hand, will prevent the individual from using it for any activity approaching the neck, throat, face, or mouth. It should be recognized that the radially deviated hand, often in combination with volar flexion, provides for lifting capacity with strength not available with a weakened finger grasp. This function may be necessary in using a rail for stair climbing. When good function of the digits and of the elbow joint is present, it may indicate early surgical correction, but this should be carried out only by a hand surgeon well experienced in congenital malformations. Buck-Gramcko recommends centralization, or better, his radicalization procedure after an optimal conservative correction within the first year of life, followed by pollicization of the index. In the quadrimembral-deficient child, however, I still prefer, as do Witt et al., to do these surgical procedures only after the completion of growth provided that, in spite of conscientious examination and observation, there are no major contraindications (Fig 36A-21.).

Surgical Procedure (Centralization of the Hand)

Where indicated, surgical correction of the radially deviated hand for centralization is carried out in two stages. The first, only for cases without the possibility of passive correction, is primarily a procedure in the form of a Z-plasty of the skin on the radial side of the wrist with soft-tissue release. After developing the skin flaps, the fascial contractures are released and tendons lengthened as necessary. This may be adequate to allow correction of the radial deviation, but in severe deformity it may be necessary to open the radial side of the wrist joint capsule. In this process care must be taken in the volar dissection to identify and protect the radially displaced median nerve. As the hand is deviated in an ulnar direction, care must be exercised to avoid stretching the nerve, which may, in fact, limit the extent of correction at this first stage. With the desired correction attained, the flaps of the Z-plasty are reversed and the wound closed.

The hand is positioned to avoid tension on skin sutures, and the correction is maintained in a plaster of paris dressing. Postoperative therapy emphasizes function and motion of the fingers. In some instances, especially in patients with acutely angulated radial abduction who require the function of the clubhand position, this procedure is adequate to obtain enough correction to increase the range of movement without losing this functional need. In other instances, this procedure is a preliminary release of the shortened radial structures to permit bony centralization of the hand on the ulna with a minimum of shortening of the bony elements.

The second stage is accomplished after the conclusion of growth and after thorough healing of the first stage through an S-shaped dorsal incision beginning on the ulnar side of the distal forearm, curving around the styloid of the ulna, transversely crossing the proximal wrist, and then turning distally to the base of the second metacarpal. The transverse portion of the incision is superficial, and the large dorsal veins and cutaneous nerves, as well as the extensor tendons, remain intact and protected. These structures are then elevated from the underlying ulna and dorsal carpal ligaments as an intact soft-tissue bridge beneath which the procedure is completed.

The dorsal capsule is then elevated from the ulna and dissected distally to expose the carpus. The carpal bones are seldom normal. For the most part the carpal bones on the radial side of the wrist are extremely hypoplastic or absent. Frequently there are various degrees of synostosis of the carpal bones. The region of the lunate bone in the synostosis is excavated to form a bed for reception of the shortened ulna. The ulna is shortened sufficiently to obtain full correction of the clubhand. If the lunate and triquetral bones are separate, I propose excavating both of them while carefully protecting their distal joints to prevent displacing them. These two bones together have a larger base and with it a better resistance against recurrent deformity than does the lunate bone alone. The excellent mobility of the joints distal to these bones with regard to dorsi-flexion and volar flexion will be preserved, and we should save mobility and function for the multimem-bral-deficient person as much as possible without increasing the risk of recurrent deformity. If there is insufficient shortening to permit centralization without excessive soft-tissue tension or if the lunate bone is too dysplastic, the lunate may be excised completely and the bed for the reception of the ulna excavated in the capitate bone or in the distal portion of the synostotic carpal block. Other hand surgical techniques for correction of the radial clubhand have been described by Blauth and Schneider-Sickert and by Buck-Gramcko,as well as by Lamb et al. in "Suggested Readings."

As in the soft-tissue release, reduction of the hand around the shortened ulna should be carried out gently and gradually and the tension on nerves and vessels carefully observed. With correction obtained and centralization acceptable, fixation is accomplished by two crossed Kirschner wires. In addition, the tendons of the radial flexor and extensors of the wrist will be transposed to a dorsal-ulnar position, and the tendon of the ulnar extensor muscle of the wrist will be shortened.

Before the wound is closed, the tourniquet is released and circulation in hand and fingers observed. If circulation is satisfactory and bleeding controlled, repair of the wrist joint capsule is carried out. Suction drainage tubes are inserted and the wound closed.

Immobilization is applied with sterile dressings and plaster of paris extending from above the elbow to the proximal interphalangeal joints of the fingers. The metacarpophalangeal joints are maintained in slight flexion.

Postoperative Care

Suction drainage is removed at 48 hours. Plaster is changed and sutures removed at about 14 days postoperatively. Immobilization, however, after this procedure should be maintained a minimum of 3 months and, on occasion, up to 6 months to ensure stability. If immobilization is extended beyond 3 months, all finger joints should be freed, and physical therapy for finger motion should be instituted. When bony fusion of the ulna to the carpus is complete, the Kirschner wires may be removed and a full physical therapy program begun. Night splints maintaining full correction of the hand are employed for at least a year postoperatively.

Procedure With a Hypoplastic Radius (After the Growth Period)

For those patients in whom the radius is hypoplastic rather than absent and radial deviation of the hand is present, no attempt should be made to perform arthrodesis to the wrist. The procedure should be a release of the contracted structures on the radial side of the wrist, followed by elevation of a capsular flap and shortening by removal of a segment of the ulna while preserving approximately 2 cm of its distal end. The ulna should be shortened to a level of about 1 mm proximal to the distal end of the radius. A wedge of bone with its base ulnarward and dorsalward is removed from the distal carpal row. When the wedge defect is closed, centralization and correction of the deformity is obtained (Fig 36A-22.). As with the other procedures, care must be taken to not place the neurovascular structures under tension. Circulation must be checked prior to internal fixation with crossed

Kirschner wires. In this procedure an additional technical detail should be noted. In reducing the shortened ulna, the distal fragment should be externally rotated slightly so that the line of pull of the extensor carpi ulnaris within its groove is partly changed to that of ulnar abductor. This tendon should be shortened proximal to the ulnar styloid process under sufficient tension so that it prevents passive radial deviation of the wrist. Immobilization and aftercare are similar to the procedure previously described. Nowadays the lengthening of the radius, instead of the described shortening of the ulna plus wedge resection, would be possible. The correction with shortening of the bones, however, is less risky with regard to function of the fingers. Too much tension on the tendons may have fateful consequences for the future function of the hand, even if blood supply and sensation are intact. This is true during the whole period of lengthening and underlines the need for careful observation and daily medical examinations.

CASE REPORTS

Retention of Feet When Hands Are Missing

Fig 36A-23 (a-b).,Fig 36A-23 (c-g)., and Fig 36A-23 (h-k). show the firstborn of a 39-year-old mother. The child was born on June 15, 1971, by cae-sarean section with total bilateral transverse deficiencies of the forearms, complete bilateral longitudinal deficiencies of the fibula, and fifth metatarsophalangeal deficiencies also present. There were numerous lesser deformities, including some hypoplasia of the right femur and, in particular, bowing of the tibias bilaterally. She was first seen in the outpatient clinic in October 1972 at the age of 16 months. Orthoprostheses were prescribed for the lower limbs, a thigh corset and knee joint on the right side, and more of an orthopaedic shoe on the left side. No upper-limb prostheses were prescribed. By December of the same year, the child was beginning to take her first steps in a walker. She was not seen again until April 1975, when it was recognized that she had a small rudimentary ulnar fragment present on the right. At this time upper-limb prostheses were prescribed. In October 1975, surgical intervention in the form of osteotomy of the tibia and fixation of a digital transplant from the great toe of this foot to the left ulnar fragment to create a better transradial stump was carried out. New orthoprostheses were prescribed. Bilateral transradial cable-controlled prostheses were prescribed. Her parents would only consider functional hands and rejected the use of a hook. Between 1975 and 1977, the child was not seen. During this period she rejected her prostheses and resumed ambulation on her own feet but with gradually increasing deformities so that when she was again seen in October 1977, she was independent in the activities of daily living and used her forearm stumps but not her prostheses. She did use her upper-limb prostheses when she went to a restaurant. At this time Syme ankle disarticulations for the lower limbs were considered, but after consultation with the parents, the decision was made against this, and only further correction of the tibial deformity was carried out. In February 1977, new lower-limb orthoprostheses were prescribed (Fig 36A-23 (a-b)., Fig 36A-23 (c-g)., and Fig 36A-23 (h-k).). The patient continues to use her forearm stumps for all activities. In 1987, the bowing of the right tibia had increased enormously so that alignment and maintenance of appearance of this orthoprosthesis were difficult; she asked for amputation, and we constructed an end-bearing modified Pirogoff stump, followed by an optimal prosthetic fitting (published with different figures in Seminars in Orthopaedics, Vol. 5, pp. 44-45, 1990). She has friends and is a pleased and happy person, completely independent in the activities of daily living as well as fully ambulatory with her lower-limb prostheses; she finished high school with excellent results and began her years of university study in 1990.

Reconstruction and Prosthetic Fittings for Multiple-Limb Deficiency

The child in Fig 36A-24 (a-d). and Fig 36A-24 (e-h). was born Sept 17, 1968, to healthy parents with a 3-year-old normal sibling. There was, however, a significant family history in that he was born with a twin who had limb deficiencies as well (Fig 36A-25 (a-d). and Fig 36A-25 (e-i).). There was an additional history of the father's sister having given birth to twins who died at birth with unknown limb deficiencies. There was no history of unusual medication during the pregnancy.

At the time of birth, the infant was noted to have bilateral upper-limb deficiencies consisting of total longitudinal deficiency of the ulna, partial carpal deficiency, and a total metacarpophalangeal deficiency of digits 2 through 5. There was additionally hypoplasia of both the radius and humerus, as well as a flexion deformity at the radiohumeral joint. The child had bilateral longitudinal deficiencies of the lower limbs consisting of a total tibia, a partial tarsal, and total metatarsophalangeal deficiencies of digits 1 through 3. The combination of total ulna and tibia longitudinal deficiencies is extraordinary. There was also a left coxa vara with bowing and hypoplasia of the femur. The child's twin was born with bilateral lower-limb deficiencies (Fig 36A-25 (a-d). and Fig 36A-25 (e-i).).

The patient was first seen in April 1972, at which time training in the activities of daily living was immediately instituted. Bilateral pneumatic upper limbs were prescribed for the child and reconstruction of the knees and feet undertaken. In August 1972, he was fitted with his first lower-limb prostheses. The initial fitting was temporary plaster of paris sockets with solid-ankle, cushion-heel (SACH) feet. These were replaced by orthoprostheses with stiff knees and SACH feet (Fig 36A-24 (a-d).,D). In December 1972, the child was ambulatory. Meanwhile, his training with the upper-limb prostheses was quite satisfactory, and he was able to feed himself as well as play by using the artificial limbs. Further efforts at reconstruction concluded in June 1977, with arthrodesis of both knees, partial-foot amputation, and stabilization of the astragalus to the fibula bilaterally (Fig 36A-24 (e-h).,E and F). Knee disarticulation was contraindicated because of the hypoplasia of both femora and also to preserve his independence. Use of transfemoral prostheses would have required the help of others to don them. Following this surgery, the child was fitted with new orthoprostheses. The prescription included plastic transfemoral sockets with windows and Velcro suspension, knee joints with Swiss locks, and articulated feet. Again, he became ambulatory and independent in donning and doffing his four prostheses. New limbs were prescribed in February 1979. The patient is fully ambulatory, wears his lower limbs full-time, and is able to play soccer while wearing them, but at home he likes to walk on his stumps. He used his upper limbs approximately 8 hours a day for years, attended regular school and high school, and is independent in eating and drinking, writing, and other activities of daily living. He is now a philosophy student, good humored, and gifted. His upper-limb prostheses, however, got troublesome to him; he felt more comfortable without and rejected them completely without any diminution in activities of daily living or in his psychological stability. For many years, however, his upper-limb prostheses had been of importance for him as well as for his parents.

Hip Reconstruction for Proximal Femoral Focal Deficiency With Multimembral Deficiency

The child in Fig 36A-26 (a-d)., Fig 36A-26e., Fig 36A-26 (f-i)., and Fig 36A-26j. was born on June 9, 1962, after a full-term pregnancy; the father and mother are both entirely normal, as were three older siblings. There was no family history of limb deficiency or other congenital deformity. There was, however, a history of the mother having ingested thalidomide in early pregnancy. The child was born with bilateral pho-comelic upper limbs consisting of hypoplastic rudimentary humeral segments synostosed with the ulna, a longitudinal deficiency of the radius, and total metacarpophalangeal 1 and 2 deficiency, with hypoplasia of the remaining digits. The lower limbs showed a left partial longitudinal deficiency of the femur (intermediate-the equivalent of an Aitken class A PFFD). On the right side there was coxa vara with bowing. Other congenital anomalies included a mild scoliosis, strabismus, and a pyloric stenosis corrected by surgery on the second day of life. The child was first seen in September 1962, at which time a physical therapy program was instituted as well as extension splinting of the lower limbs. In August 1964, a valgus osteotomy of the right femur was carried out, and in September 1964, she was supplied with an extension orthosis for the left leg. In 1967, the child was admitted to the hospital for self-care training, especially in the technique of using the feet as well as the vestigial hands for activities of daily living. A full physical therapy program to maintain functional mobility of the spine was likewise undertaken. At this time a left upper-limb prosthesis was prescribed. This was a ball bearing-supported elongation of the left upper limb with a pneumatic hook and a pneumatic wrist rotation unit operated by the left phocomelic hand (see Fig 36A-5.). By the following year the child had rejected the pneumatic prosthesis. In 1968, she was enrolled in a special preschool for the physically handicapped. In 1969, she was supplied with an orthoprosthesis for the left lower limb with a stiff knee and SACH foot (see Fig 36A-4.). In 1974, the left subtrochanteric pseudoarthrosis was resected and the first stage of correction of the severe varus deformity undertaken, as well as correction of the severe hip flexion deformity. In 1975, the second stage for correction of the varus deformity was carried out, and 6 weeks later the patient was fitted with a new orthoprosthesis. In 1976, the hardware was removed and a Chiari osteotomy carried out to provide increased coverage of the femoral head. At this time the trochanter was also transplanted distally. After healing of this surgery a new orthoprosthesis was prescribed with a left thigh corset, free knee joint, and SACH foot. The child became fully and independently ambulatory, used her toes as well as her vestigial hands for self-care, and attended school in her own village. Years later, she is a young woman, completely independent in activities of daily living, and has her own apartment. She is working full-time in an office on behalf of disabled children and is happy and feeling fine except that she now observes a flexion contracture of the left hip and low back pain. For the present, physical therapy is planned on an inpatient basis in a rehabilitation hospital and should continue at yearly intervals to preserve mobility and independence and to postpone total hip replacement.

Reconstructive Surgery in Upper and Lower Limbs Prior to Prosthetic Fitting

The child in Fig 36A-27 (a-d). and Fig 36A-27 (e-h). was born of healthy parents on May 15, 1961. The history, however, reveals that at approximately 6 weeks after her last menstrual period, the mother of the patient ingested thalidomide for a period of 3 days for surgery. The pregnancy was uneventful with a normal birth. The child had the following limb deficiencies: upper limbs showed bilateral hypoplasia of the radius with partial carpal absence and, on the right, partial absence of metacarpophalangeal 1, whereas on the left, metacarpophalangeal 1 was totally absent. In the lower limbs there was bilateral total longitudinal deficiency of the tibia, with coxa vara of the femur on the right and a longitudinal deficiency of the proximal portion of the femur on the left (Aitken class A) and subtrochanteric pseudarthrosis.

The patient was first seen in 1964, prior to which he had been fitted with stubby prostheses at the University of Tubingen. Shortly thereafter, centralization of the fibula under the femur and of astralgus under the fibula was carried out at Tubingen for stiffness on the right. In 1967, orthoprostheses as well as a night splint were provided, and he began a physical therapy and occupational therapy program. In 1968, the left index finger was pollicized. In 1969, arthrodesis of the right knee was carried out, and in 1970, new orthoprostheses were prescribed for the lower limbs. In 1971, polliciza-tion of the right index finger was carried out in Heidelberg, and in 1974, the left subtrochanteric pseudarthrosis was resected and the varus deformity corrected, subsequent to which new prostheses were prescribed. In 1978, because of increasing flexion adduction deformity in the left knee, arthrodesis of this knee was carried out. New orthoprostheses were prescribed. In addition to all of his physical problems, this child's schooling had been much delayed by these multiple surgical procedures. Nonetheless, as indicated in Fig 36A-27 (a-d). and Fig 36A-27 (e-h)., he is independently ambulatory, and his pollicized index fingers function quite well as thumbs. Parallel to his increased walking and grasping abilities, his cognitive skills improved, and he is now in a full-time job as an office worker; he is independent in activities of daily living, drives his own car, and is accepted and accepts himself as he is. This patient demonstrates the importance of reconstructive surgery in both the upper and lower limb prior to prosthetic fitting, but he also demonstrates the need for a better consideration of the child's psychological development if orthopaedic surgery and hospitalization are indicated. In following years we established the rooming-in system for preschool children with their mothers in the hospital, extended the hospital school, improved cooperation with the parents, developed the efficiency of the outpatient clinic, and reduced the period of hospitalization (average period in the dysmelia department: 1970, 43.4 days of hospitalization; 1980, 22.6 days of hospitalization; 1985, 14.5 days of hospitalization; and 1988, the last year of my full professional life, it had been 13.8 days).

Hip Disarticulation Prostheses for Bilateral Lower-Limb Deficiency

This child was born Nov 4, 1962 (Fig 36A-28 (a-c). and Fig 36A-28 (d-h).). There was no family history of limb deficiencies, and his two siblings were normal. There was no history of ingestion of any medication during the pregnancy.

The child was born with bilateral transverse lower-limb deficiencies, deficiency of the upper third of the thigh, and a transverse deficiency of the forearm, proximal third (with a very short ulna segment remaining). He had other congenital anomalies.

The child was first evaluated in November 1972, at which time he was using primarily a wheelchair but was able to get around on the floor by using his hands. He was also noted to have a scoliosis. Bilateral lower-limb prostheses were prescribed with automatic knee locks in the stance phase and automatic combined hip and knee flexion for sitting. A left transhumeral prosthesis was constructed with a crutch attached to the functional hand for ambulation. These prostheses were first applied July 1973, and after 4 weeks of training he was able to ambulate on a level surface and, with considerable effort, even manage stairs. He was also able to sit and stand independently. Stairs were particularly important because without this ability, he would not be permitted to attend high school at home but would have to attend a special school. In 1974, his upper-limb prosthesis was changed to a cable-controlled Hosmer outside-locking elbow with a Dorrance 10X hook interchangeable with an Otto Bock hand. He was additionally supplied with a cosmetic forearm prosthesis for swimming. His lower-limb socket had to be changed in 1976, again in 1978, and most recently in 1979 to extend the socket somewhat proximally to act as a support for his progressive scoliosis. It was believed that because of his problems in ambulation with bilateral hip disarticulation-type prostheses, Harrington instrumentation should not be considered.

CONCLUSION

An effort has been made to document a philosophy for management of the multimembral-deficient child. Particular emphasis is placed on using all function in tactile areas, including vestigial digits on deficient upper limbs, in the early life of these children. Substitution patterns are discussed, especially the use of the foot for prehension by the armless child. Reconstructive procedures that have special benefits for the multimembral-deficient child as opposed to the unimembral-deficient child are discussed. Prosthetic solutions are presented for both upperand lower-limb deficiencies. Stress is placed on the limitation of functional value and particularly on the excess of the weight of currently available externally powered upper-limb devices. Finally, case studies are used to illustrate the complexity of the problems involved with the quadrimembral-defi-cient child. It is important to recognize that in no way could such a chapter as this cover all combinations of deficiencies and that this is an effort to present the problems in general with some particular solutions.

SUGGESTED READINGS

Boos O: Die Versorgung von Ohnhandern. Stuttgart, West Germany, FK Schattauer Verlag, 1960.

Gocht H: Atiologie, Pathogenese und Therapie der Deformi-tiiten im allgemeinen, in Hoffa A (ed): Orthopddische Chirurgie. Stuttgart, Germany Ferdinand Enke Verlag, 1925.

Hepp O: Information on Measures for Habilitation for Children With Dysmelia. Heidelberg, Deutsche Vereiningung fur die Rehabilitation Behinderter, 1963.

Jones D, Barnes J, Lloyd-Roberts GC: Congenital aplasia and dysplasia of the tibia with intact fibula: Classification and management. J Bone joint Surg [Br] 1978; 60:30-39.

Kallio KE: Recent advances in Krukenberg's operation. Acta Chir Scand 1948; 97:165.

Kelikian H: Congenital Deformities of the Hand and Forearm. Philadelphia, WB Saunders Co, 1974, pp 780-824.

Kessler HH: Cineplasty. Springfield, 111, Charles C Thomas Publishers, 1947.

Kuhn GG: Neue technische Hilfen fur schwer Korperbe-hinderte kinder, in Baumgartner R (ed): Amputation und Prothesenversorgung beim Kind. Stuttgart, West Germany, Ferdinand Enke Verlag, 1977.

Lamb DW: Radial club hand. J Bone Joint Surg [Am] 1977; 59:1-13.

Manske PR, et al: Centralization of the radial clubhand: An ulnar surgical approach. J Hand Surg 1981; 6:423-433.

Marquardt E, Neff G: The angulation osteotomy of above-elbow stumps. Clin Orthop 1974; 104:232-238.

Martini AK: Klumphandkorrektiir nach Wachstumsabschliits. Handchirurgie 1980; 12:229-233.

Nathan PA, Nguyen BT: The Krukenberg operation; a modified technique avoiding skin grafts. J Hand Surg 1977; 2:127-130.

Sauerbruch F: Die willkiirlich bewegbare kiinstliche Hand, Eine Anleitung fur Chirurgen and Techniker mit anato-mischen Beitnigen von G. Ruge and W. Felix unter Mitwirkung von A. Stadler, Berlin, 1916, Julius Springer Verlag, 1916.

Thomsen W: Diskussionsbeitrag zum Thema Krukenberg-Plastik. Verh Dtsch Orthop Ges 1949; 36:60-61.

Willert HG, Henkel HL: Klinik und Pathologie der Dysme-lie. Die Fehlbildungen an den oberen Extremitaten bei der Thalidomidembryopathie, in Experimentelle Medizin, Pathologie und Klinik, vol 26. Berlin, Springer-Verlag, 1969.

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Chapter 36A - Atlas of Limb Prosthetics: Surgical, Prosthetic, and Rehabilitation Principles

O&P Library > Atlas of Limb Prosthetics > Chapter 36A