The Child Amputee An Overview
George T. Aitken, M.D.*
It may seem unusual to some readers that in a monograph devoted to the problems of amputations and prostheses there is a special chapter on the juvenile amputee. Its inclusion is justified because children are not scaled down adults. The child is a growing biological organism, and all his organ systems reflect this growing state. This is in contradistinction to the static, decelerating, aging adult. Children are emotionally, socially, and functionally immature as opposed to the purportedly independent, mature, responsible adult. Further, depending upon their age, children are immature, in terms of the upper and lower extremity skills of manual function, standing, and walking. The child's skeletal immaturity influences one's decision regarding level of amputation, and his immaturity (particularly in hand skills) influences very definitely the training goals that are established for upper limb rehabilitation. For these and other evident reasons it is believed that there is adequate justification for dealing with the problems of the juvenile amputee separately, not only in the area of academic teaching but also in the practical clinical management of these problems.
In 1953 an article on the juvenile amputee discussing the differences between the child and the adult noted that the juvenile amputee had been somewhat neglected as an entity.4 The concept was set forth that certain congenital limb deficiencies were better treated by prosthetic fitting with or without surgical conversion than by traditional reconstructive orthopedic procedures, e.g., shoe lifts, braces, and a wide variety of reconstructive surgical procedures.
Immediately following World War 11 there was a renaissance in the development and manufacture of prosthetic components. This included the development of a wide selection of sizes of components. Previously unobtainable items became available in sizes and weights that were suitable for fitting very young patients. At the same time there were widespread innovations in fitting and alignment techniques, as well as in the use of new materials for fabrication of prostheses. These events contributed to the availability of prostheses appropriate to fit children at nearly any age.
In this discussion we will define the juvenile amputee as a skeletally immature individual who has either an acquired amputation or a congenital limb deficiency that seems best treated by application of a prosthesis with or without surgical conversion. The emphasis will be on the principles of management; prosthetic prescription writing, prosthetic components, fitting, fabrication and alignment techniques, and training modalities will not be covered.
Children with acquired amputations or terminal transverse limb deficiencies (homologues of acquired amputations) can be fitted with adult prostheses suitable for the level of amputation.11 It is necessary that size and weight considerations be given high priority. With the wide variety of commercially available child-sized components and the wide choice of socket materials, it is possible for a skillful prosthetist to fabricate a limb of suitable size and weight for a child of any age and with any level of amputation.
This is not to be interpreted as meaning that the prosthesis for the child should be identical to one for an adult with the same level of amputation. For example, a one year old above knee amputee would probably be fitted initially with a locked knee prosthesis, and the knee would not be unlocked until satisfactory standing and walking balance is accomplished -and then only on a trial and error basis. So also a one and one-half year old above elbow amputee would not be fitted with a cable controlled elbow joint as well as a cable controlled terminal device. The one and one-half year old child has not matured sufficiently to master the complexities of a dual control cable system in a functional manner.
Lower limb alignment techniques are nearly identical with those for the adult. Above knee and below knee adjustable legs are available in child sizes and should be used to promote optimal alignment.
In upper limb cases the standard checkout procedures are applicable to children, and cable efficiencies should be checked and adhered to in the fitting of children just as rigidly as they are in well organized adult amputation clinics.
The wide variety of abnormalities that fall into the intercalary and terminal longitudinal groups of limb deficiencies generally require custom fitting, fabrication, and alignment. In this group the prosthetic fitting is relied upon to overcome the often present malrotation, proximal joint instability, and muscular inadequacies of the stump.
AGE OF FITTING
Normal children undergo an orderly motor maturation during the first 12 to 14 months of their lives. They learn to roll over; then they learn to hold their heads erect; they then progress to independent sitting. Eventually they acquire the skill of assisted standing, from which they progress to independent standing. Finally they arrive at the ability to carry out independent alternating progression. In order to walk with a heel-strike, mid-stance, toe-off gait, it is necessary to be able to stand on one leg. Most children do not acquire this ability until they are between four and five years of age. A child with a comfortable stump who demonstrates the ability to stand assisted is ready for fitting, and if fitted with a limb of the proper prescription he can learn to walk as does a normal child his age. Training modalities and goals should be related to the child's age, and heel-strike, mid-stance, toe-off gait instruction should be deferred until the child is either past five years of age or can demonstrate unsupported one-legged standing. Articulation of the artificial knee or hip (in the case of hip disarticulation) should be deferred in young children until secure stiff-legged walking is accomplished.
Rehabilitation of the child with an upper limb loss is basically oriented toward providing him with prehension. The secondary goal is to provide wrist, elbow, and, if necessary, shoulder joints to permit the placement of the prehension device into a functional position in space. Sensory feedback and true cosmetic restoration are not within the current state of the art. In normal children grasp and release develop in an orderly manner. The ability of a child to place his hand in space is dependent upon a finely developed coordination of the shoulder, elbow, wrist, and hand musculature supervised by adequate stereoscopic vision. Grasp always precedes release. Until approximately nine months of age, children grasp primarily by bimanual palmar prehension. Following this, they develop thumb, index, middle, and ring finger grasp. At about one and one-half years they have thumb, index and middle finger pinch prehension. It is not until between 18 and 24 months that they can build a tower of more than four blocks. When they can do this, they have well controlled release with adequate stereoscopic vision to permit release to occur in the proper place in space. A measure of total arm coordination plus well coordinated release is possible when the child can throw overhanded. This is usually not until sometime between the ages of four and five years. If one relates this orderly development of grasp and release plus stereoscopic vision plus shoulder, elbow, and wrist control to the prosthetic components that are available and to a properly written prescription, upper limb amputees or their congenital homologues can be fitted at any age (Fig. 1A, Fig. 1B , Fig. 1C ).15
Ideally lower limb prosthetic rehabilitation training is supervised by a physical therapist who has had special educational advantages in this area. In dealing with the very young, it is essential to always be aware of how children walk at these early ages, and efforts to produce satisfactory station and gait should be directed toward the development of skills that are identical with those of their normal peers. Once the ability to accomplish heel strike, mid-stance, toe-off gait is acquired, every effort should be made to develop as smooth an alternating progression pattern as possible. The bilateral lower limb amputee at the below knee and long above knee levels should achieve independent walking without external support. Stair climbing and descending with the assistance of handrails should be mastered. At the bilateral high above knee level the use of a cane to improve time-distance factors and reduce fatigue is acceptable.
In the bilateral hip disarticulation, to acquire a satisfactory time-distance factor it is necessary to walk with crutches using a swing-to or swing-through gait pattern. Shuffling, unsupported walking for short distances on smooth, level surfaces can be accomplished by the bilateral hip disarticulation patient, but it is precarious and seldom functional.
Ideally upper limb prosthetic training is best carried out by an occupational therapist who has had some special education in the prosthetic field. In dealing with children it is essential that this therapist have a sound understanding of the development of upper limb skills in children as they relate to their chronological and mental ages. Goals should be realistic. The child with an upper limb amputation or its homologue should not be required to perform manual skills at a level above that of his normal peers. It is essential also that there be recognition of the fact that children are not independent individuals. They are members of a family group, and it is the family that will insist upon the prosthetic wear and practice that eventually leads to the development of functional skills that make the prosthesis not only acceptable but desirable and necessary to the patient. This concept must be recognized by all members of the amputee team. Unless the family can be convinced of the necessity and desirability of upper limb fitting, and unless they will cooperate in the area of insistence on wearing and functional practices, there will be a high rejection rate, particularly in the unimembral upper limb amputee group.
In the severely handicapped upper limb amputee (bilateral at any level above the very short below elbow), the problems are so complex that details of training techniques cannot be covered. They require ingenuity, patience, and often lengthy periods of training in order to obtain suitable restoration of upper limb function for basic activities of daily living.2,7,8 In bilateral amelia (bilateral shoulder disarticulation) the preservation and fostering of foot function is an essential part of a good post-prosthetic training regimen.18 Foot function is often a major adjunct to the marginal prosthetic function that is obtained at this level.
CONSIDERATIONS IN FITTING THE AMPUTATION STUMP
The concept of fitting an amputation stump with a prosthesis is traditional, ancient, and well accepted. The concept of applying a prosthesis to a congenitally deformed limb with or without surgical conversion is new. In intercalary and terminal longitudinal limb deficiencies many patients with the deformities do not obtain suitable cosmetic and functional results from traditional orthopedic reconstructive procedures. If this group is analyzed carefully, the biomechanical losses in the lower limb are inequality of leg length, malrotation, inadequacy of proximal musculature, and instability of proximal joints. In the upper limb the biomechanical losses are inequality of length, malrotation, inadequacy of proximal musculature, instability of proximal joints, and varying loss of prehension.
A prosthesis can immediately equalize leg or arm lengths. By suitable prosthetic alignment a major degree of malrotation can be corrected. With careful attention to fit, alignment, and design, mechanical stability can be introduced. Little can be done to improve inadequate musculature. In the upper limb, prehension losses can be at least partially compensated for by a suitable terminal device. There does, then, exist a justification for the consideration of prosthetic fitting in this group of patients.
If the presenting deformity is analyzed carefully, it can be considered a homologue of an amputation. In order to carry out this analysis, one must determine the most distal stable joint beyond which there is adequate tissue to function as a stump and then determine whether this is the homologue of a hip disarticulation, an above or below knee amputation, or an ankle disarticulation. In the upper limb it would be deter- mined whether it is a homologue of a shoulder disarticulation, above elbow amputation, elbow disarticulation, below elbow amputation, or wrist disarticulation (Fig. 2a , Fig. 2b ).
It then is necessary to determine whether surgical conversion to create a more suitable stump is desirable. There are two types of conversions: primary and secondary. Primary conversion includes those cases in which the life history of the deformity is so well known that one can, on the basis of previous experience, predict that conversion will be necessary. Secondary conversion includes those cases in which fitting around the presenting deformity is demonstrated to be unsatisfactory and the patient or the physician determines that conversion is desirable.
In the author's opinion there are no indications for primary conversion in congenital anomalies of the upper limb. In experience with 450 patients with upper limb involvement, secondary conversion to provide more suitable prosthetic fitting and function was necessary in 8 to 10 per cent of the cases.
In the lower limb anomalies there are limited indications for primary conversion (vide infra). In a series of 230 patients with lower limb anomalies, surgical conversion - either primary or secondary - was necessary in 52 to 55 per cent of the cases.
CLASSIFICATION OF CONGENITAL SKELETAL LIMB DEFICIENCIES
In order that orthopedic surgeons, radiologists, physiatrists, occupational and physical therapists, and prosthetists may speak intelligently to one another concerning limb deficiencies, it is desirable that there be an agreed upon, well defined classification of these conditions. For many years embryologists and comparative anatomists have utilized descriptive terms that never found their way into clinical usage. In 1961 Frantz and O'Rahilly11 introduced a classification for congenital skeletal limb deficiencies; this did not include twinnings, or reduplications. Subsequently Hall et al.12 developed their classification. These two classifications made it possible to codify 87 per cent of the presenting anomalies. With the hope of increasing the percentage of anomalies that could be coded, Burtch et al.6 developed still another classification. None of these classifications is used universally and at this time there is no agreed upon international classification.
Each clinic must choose the classification that it desires to use. Such a classification is essential for record-keeping and improved com munications between members of the medical and allied health team responsible for the care of these patients. In this article the Frantz-O'Rahilly classification has been used.
LOWER LIMB ANOMALIES
Those lower limb anomalies that fall into the classification of terminal transverse deficiencies are homologues of acquired amputations of the same level. They are easily identified as amputations, and prosthetic fitting and training are identical with those used with the homologous acquired amputation.
The terminal longitudinal and intercalary deficiencies are bizarre deformities that are not immediately apparent as homologues of amputations, and they are the ones in which it is necessary to identify the key joint and then to fit usually nonstandard prostheses in order to equalize leg lengths, correct malrotation, and increase stability. Surgical conversion is necessary in this group in a high percentage of the cases, probably because the lower limb anomalies become weight bearing stumps. Comfort dictates conversion in many instances.
Lower Limb Phocomelia
These patients present with a foot at the hip; usually there are no bony elements of the femur, tibia, or fibula. This deformity is a homologue of a hip disarticulation, and the patient may be fitted with a standard Canadian type of hip disarticulation prosthesis, with the prosthetic bucket fitted around the foot. Conversion in these cases by removal of the foot is not only not indicated - it is contraindicated. The foot gives an excellent weight-bearing surface. It provides sensory feedback concerning the position of the prosthesis and increases the stability of suspension of the hip disarticulation bucket (Fig. 3a ), Fig. 3b .
TERMINAL LONGITUDINAL AND INTERCALARY DEFICIENCIES
Excluding the terminal transverse deficiencies, the three most common terminal longitudinal and intercalary deficiencies seen in the lower limb, in order of frequency, are fibular hemimelia (congenital absence of the fibula), proximal femoral focal deficiency, and tibial hemimelia (congenital absence of the tibia).
Fibular hemimelia may involve complete or partial absence of the fibula. It may be unilateral or bilateral and is about equally distributed in males and females. Characteristically these patients present with a short tibial segment. There is an anterior bow of the tibia, usually at the junction of the distal and middle thirds. Generally there is a dimple in the skin over the tibia at the apex of the bow. If the lesion is intercalary, there is a five rayed foot; if it is a terminal longitudinal deficiency, there will be less than five rays. Often there are coalitions of the tarsals. The foot generally is in an equinovalgus position. The diagnosis generally can be made on inspection and is easily confirmed by suitable x-ray views (Fig. 4A ,B ,C ). The major biomechanical loss is inequality of leg lengths in the unilateral cases, and in the bilateral cases it is asymmetrical dwarfism. There have been many therapeutic approaches to this lesion.9,10,14 The inequality of leg lengths may be compensated for by shoe lifts with or without adjunctive braces, leg lengthening procedures, epiphysiodesis, or epiphyseal arrest of the contralateral leg (alone or in association with leg lengthening). Well documented reports of the results of these procedures indicate that the percentage of patients with equalization of leg lengths, good feet, and acceptable functional results is low.14
These patients may be treated by ankle disarticulation, closure with a Syme type of flap, and fitting with an end bearing Syme type of prosthesis with a patellar tendon bearing type of socket (Fig. 4D ,E ). This immediately equalizes leg lengths and in the bilateral case permits correction of the asymmetrical dwarfism. Since these are end bearing stumps, the bilateral cases may be so treated because the patient is able to ambulate in emergencies without his prostheses. There is also no major contraindication to this type of conversion in the bilateral case because these are at a below knee level. It is known from experience that even the elderly bilateral below knee amputee can function with a great degree of independence.
The life history of this lower limb deficiency is accurately enough delineated so that primary conversion may be carried out if the responsible physician concurs with this plan of treatment.
Occasionally fibular hemimelia is seen in conjunction with congenital shortening of the femur. This is a difficult entity to treat; the combination of the short femur and fibular hemimelia produces severe inequality of leg lengths as well as inequality of knee joint levels. Combinations of tibial and femoral lengthening, with or without contralateral epiphyseal arrests, do not seem to be an adequate solution. This combination of procedures is fraught with complications, and good functional results in the skeletally immature are infrequent. Ankle disarticulation and fitting with a Syme type of prosthesis will produce equalization of the total length of the legs, but it does not equalize knee joint levels. I do not have an ideal solution to this problem. My experience has been primarily with ankle disarticulation and fitting with a Syme type of prosthesis, but the cosmetic inadequacies of this result are recognized.
Proximal Femoral Focal Deficiency
None of the classifications previously mentioned includes incomplete absence of either the femur or the humerus. There are several abnormalities of the proximal end of the femur. The best known of these is infantile coxa vara. There is another group of deficiencies of the proximal end of the femur that is sometimes confused with coxa vara This group of proximal femoral deficiencies presents a characteristic picture.1 At birth the child with this lesion presents with a very short femoral segment, which is held in flexion, abduction, and external rotation. In the author's series 68 per cent have had associated fibular hemimelia on the same side. Generally the sole of the foot on the affected side is at about the level of the transverse axis of the knee joint on the unaffected side.
X-ray films of these patients reveal four subcategories (Fig. 5 ).
The inequality of leg length is primarily femoral, and at the present time there is no surgical mechanism known that will equalize the femoral lengths.
If one desires to treat such a patient prosthetically, the decision must be made as to whether he is to be treated as a below knee or an above knee patient. If he is to be treated as the homologue of a below knee amputee, the only method is Van Nes 180 degree rotational osteotomy of the tibia, utilizing the ankle joint as the knee joint and the "turned around" foot as the below knee stump.13,19 If properly selected this is applicable in unilateral cases.
If one elects to treat these patients as above knee amputees, there are several alternatives. A nonstandard above knee prosthesis can be fitted around the presenting short extremity, including the foot, and by proper prosthetic alignment the malrotation can be corrected. With the development of a good ischial seat the instability of the proximal joints can be partially overcome.
Ankle disarticulation with an end bearing Syme flap facilitates fitting as an above knee amputee, making the prosthesis less bulky and still retaining the advantages of a total end-bearing stump (Fig. 7 ).
Subsequent knee arthrodesis will correct some of the malrotation of the extremity, correct the hip flexion deformity, and produce a more stable stump. Arthrodesis of the knee must be deferred until it is certain that premature closure of the epiphysis secondary to the arthrodesis ,o will not produce too short an above knee stump.
A variety of operative procedures have been suggested to increase stability about the hip joint. Most of these have been moderately unsuccessful. If one elects to do reconstructive surgery about the hip in a patient with a proximal femoral focal deficiency, it is recommended that it be considered only in classes A and B, because it is only in these two classes that there is an adequate acetabulum with the head of the femur in the acetabulum. One hundred eighty degree valgus osteotomies, when successful, do increase stability in these hips and improve the character of the gait. It should be remembered that these children are already seriously handicapped, and iatrogenic disorders of the hip joint should be avoided.
The bilateral proximal femoral focal deficiency, in the author's opinion, should not be treated by the VanNes rotational osteotomy or bilateral ankle disarticulation. If these patients are so converted, they lose their inability to walk unaided without prostheses. This loss of functional mobility in their dwarfed stature is to be condemned. If they are subjected to bilateral ankle disarticulation, they are condemned to be bilateral above knee prosthesis wearers, and it is well established that the aging adult bilateral above knee amputee has a rapidly decreasing functional sphere.
Tibial hemimelia may involve complete or partial absence of the tibia.3 This is a severe congenital anomaly and is unusual in that it is seen in siblings. It can be complicated by reduplication of the number of rays in the foot. Patients with this condition frequently have central aphalangia of the hands ("lobster claw" hands; Fig. 8A , B ). In many cases there is a history of central aphalangia of the hands or feet in siblings or more distant blood relatives. In the author's series the incidence of dislocated hips in these children was 20 per cent. Parents of children with tibial hemimelia should be advised concerning the potential heritability of this condition.
Clinically these children present with a very short leg on the affected side. The proximal end of the fibula usually overrides the transverse axis of what should have been the knee joint. The fibula often is flat and bowed like a rib, and the foot usually is in severe varus with the plantar aspect facing the perineum. The diagnosis can be made by inspection and is readily confirmed by x-ray.
In these patients the biomechanical losses are inequality of leg lengths, malrotation, and instability of the proximal joint.
In complete tibial hemimelia there are two choices of treatment: (1) knee disarticulation and fitting with a knee disarticulation type of prosthesis, which will equalize leg lengths, correct the malrotation, and eradicate the instability of the knee joint; and (2) transplantation of the proximal end of the fibula beneath the distal end of the femur and subsequent ablation of the foot by ankle disarticulation and fashioning of a Syme type of flap, with prosthetic fitting as a below knee amputee.5,17 If this latter procedure is carried out and is successful, these patients will require knee joints and thigh corsets on their below knee prostheses because they never develop sufficient medial and lateral stability at the knee to permit fitting with a patellar tendon bearing prosthesis.
In partial tibial hemimelia one can effect synostosis of the fibula to the remnant of the tibia. When the synostosis is healed, this should be followed by ankle disarticulation and closure with a Syme flap; this permits fitting with a below knee type of prosthesis, which is advantageous. In these cases as well as in the fibular transplantation, medial and lateral instability at the knee joint requires prosthetic stability; this necessitates knee joints and thigh corsets on the below knee prostheses (Fig. 8C , D , E ).
Bilateral Lower Amelia
Bilateral lower amelia is a homologue of a bilateral hip disarticulation, and therefore falls in the terminal transverse group, which has been excluded from this discussion. Because this condition is present at birth and because for some period of time the patient does not have the ability to acquire crutch walking skills, it deserves special mention. At approximately four to six months of age these children should be fitted with a prosthetic bucket, which stabilizes the pelvis and facilitates development of sitting skills. Without the prosthetic bucket the child is unable to develop independent sitting. When independent sitting is well established and the child is approximately nine months of age, the prosthetic bucket may be applied to an Ontario lateral sway walker.16 This will permit limited ambulation on level surfaces. Between 18 and 24 months of age these children may be fitted with bilateral hip disarticulation prostheses, usually with nonarticulated knees, and be taught to walk with a swing-to or swing-through gait as does a paraplegic. The knees of these prostheses may be articulated whenever the child's motor skills indicate that he is capable of managing the articulated knees (Fig. 9a , Fig. 9b , Fig. 9c ).
UPPER LIMB ANOMALIES
Upper limb deficiencies of the terminal transverse type are evident and easily identified homologues of acquired amputations. The indications for fitting, types of prostheses, and training programs are identical with acquired amputations at the same level in children of the same age. This general statement excludes bilateral upper amelia (homologue of bilateral shoulder disarticulation).
In clinics treating patients with upper limb deficiencies there are a wide variety of intercalary and terminal longitudinal deficiencies that can be improved by prosthetic fitting. Some do not require prosthetic fitting with or without surgical conversion. The two major exceptions are terminal longitudinal or intercalary longitudinal radial hemimelia (radial clubbed hand) and central aphalangia (split ray or "lobster claw" hand). The management of these two upper limb deficiencies is probably best carried out according to traditional methods of treatment. Any discussion about the desirability of one treatment modality as opposed to another is not rightly a part of this discussion.
In upper limb deficiencies surgical conversion to produce a more suitable stump is never primarily indicated, and is secondarily indicated in not over 10 per cent of the cases.
Upper Limb Phocomelia
Patients with upper limb phocomelia present with a hand at the shoulder level. Usually there is no x-ray evidence of a humerus, a radius, or an ulna. The hand may have a variety of digits. Sometimes there are five with a definite thumb; more frequently there are less than five and usually in these cases the thumb ray is absent. Because there is a wide variation in force and excursion ranges, the prehension capabilities of phocomelic hands vary tremendously. In the unilateral cases they probably should not be fitted; the deformity should be accepted. The prehension capabilities of the hand are often adequate for assistive hand function. In the bilateral cases the strongest and most functional hand should be left free because it usually is capable of performing satisfactory feeding and writing function. The contralateral side sometimes can be fitted with a modified shoulder disarticulation prosthesis in order to assist in dress, undress, and toiletry. The phocomelic hand that is present sometimes has sufficient force and excursion to control the elbow locking mechanism (Fig. 10a , Fig. 10b ). With the advent of external power such an extremity may be a source of multiple controls for the external power.
Proximal and Distal Phocomelia
A child with proximal phocomelia presents with an absent humerus. He has a radius or ulna, carpus, and five or fewer digits. The distal phocomelic: patient presents with a humerus, no radius or ulna, and with a carpus and five or fewer digits. In unilateral cases a prosthesis is seldom indicated; usually, when tried, the prosthesis is rejected. In bilateral cases there occasionally is an indication for fitting the side that has the least prehension capability in order to provide an arm with sufficient length to permit improved dress, undress, and toiletry functions. These are difficult cases, and it has been the experience of the author that most of these patients reject the fitting of a prosthesis; they prefer assistive devices.
Ulnar hemimelia is one of the, more common upper limb deficiencies. It presents generally as a monodigital hand mounted on the distal end of a radius with absence of the ulna. Very frequently there is an antacubital pterygium. The condition varies in its severity but when present generally limits extension of the elbow to about 90 degrees. In severe cases the elbow is held at about 160 degrees of flexion, and excursion of not over 10 to 15 degrees is present.
Experience has demonstrated that soft tissue releases of the antecubital pterygium, whether by Z-plasty or some other mechanism, are not effective. The author has not been able to obtain extension beyond about 120 degrees even with anterior capsulotomies.
There are several fitting options. (1) If there is adequate strength in the single digit, an opponens post can be fashioned and limited prehension with retained sensation can be obtained. (2) If the patient has extension of the elbow to 90 degrees or greater, fitting as a below-elbow amputee can be accomplished. The forearm shell will equalize the lengths of the arms, and a voluntary opening terminal device will give improved prehension. The patient is able to operate this with a single control system. (3) In those cases in which the antecubital web prevents a functional range of elbow extension, the forearm can be flexed acutely against the humerus, and around this can be fashioned an elbow disarticulation socket. The flexed forearm produces a stump that has a sufficiently nonspherical configuration so that rotary stability is excellent. To this socket is attached an elbow disarticulation prosthesis. Forearm lift and terminal device control are effected by a single cable control. Generally there is sufficient power in the exposed single digit to control the elbow lock. Such a fitting produces very satisfactory elbow disarticulation prosthetic function (Fig. 11a , Fig. 11b , Fig. 11c ). (4) Elbow disarticulation amputation has been done in ulnar hemimelia when satisfactory range of flexion and extension could not be obtained. Following conversion the patient is fitted with standard dual control elbow disarticulation prostheses. If there is sufficient inequality in the humeral lengths, the amputation sometimes can be fitted as an above elbow rather than elbow disarticulation. When there is sufficient force and excursion in the monodigital hand to lock and unlock the elbow mechanism, acute flexion of the forearm on the humeral segment and fitting with a nonstandard elbow disarticulation prosthesis are preferable to surgical conversion to an elbow disarticulation.
Miscellaneous Upper Limb Anomalies
There are some upper limb anomalies that are difficult to classify but that may be treated prosthetically. These children may present with what appears to be total aphalangia (mitten hand). They generally have a radioulnar synostosis and may have limited extension at the elbow. Some have inadequacy of the shoulder musculature, often to the extent of complete absence of the deltoid. There arms are short and there is no pinch prehension available. X-ray evaluation of these hands reveals inadequate skeletal components to develop prehension by hand reconstructive procedures. Prostheses can be helpful in these cases in that the length of the extremities can be equalized. Voluntary opening prehension devices will produce satisfactory pinch prehension, and if there is an adequate range of extension and flexion at the elbow such patients can operate successfully with a single control system.
Bilateral Upper Amelia
Bilateral upper amelia is the most severe upper limb deficiency. To date the prosthetic fitting accomplishments have been less than ideal. The currently adopted treatment plan is initiated after the patient has developed independent sitting balance. He then is fitted with bilateral plastic laminate shoulder caps, which develop prosthetic tolerance. The caps increase the weight about the shoulders and are a kind of resistive exercise for torso musculature and improved sitting stability.
When tolerance to wearing the caps is accomplished, one side is fitted with a nonstandard (usually skeletonized) prosthesis. This consists of a manually adjustable friction joint at the shoulder, a manual friction elbow joint, and a passively operated, voluntary opening terminal device of appropriate size. This allows parental prepositioning of this upper limb prosthesis in a hand-to-mouth position and placement of objects in the terminal device to gratify sucking instincts. This also may create an awareness of the potentials of the prosthetic device. Between 12 and 18 months of age a cable can be introduced attached to either a perineal strap, a waistband, or the contralateral shoulder cap, thus producing terminal device opening. The shoulder and elbow remain manually con trolled friction types. At 30 to 36 months in alert patients one can introduce an elbow locking mechanism.
Externally powered programed feeding arms have been applied in this group, and their effectiveness in feeding has been demonstrated. Adaptability to the activities of daily living such as dress, undress, and toiletry have as yet not been fully developed in patients fitted with externally powered prostheses (Fig. 12 ).
These patients are fitted initially unilaterally. It is not until at least six or seven years of age that one should even consider the possibility of bilateral fitting.
Foot function is essential to the activities of these children. In general the bilateral upper limb amelic patient does not have to be instructed in foot prehension function. It is only necessary to arrange socks and shoes so that the child can easily free his feet for prehension activities. This is facilitated by quick-release closures for shoes or the wearing of the loafer type of shoe. Split socks (a Japanese glove type of arrangement to allow movement of the great toe) are helpful. Loose ungartered stockings are removed easily by these patients with minimal instruction.
ndence in these children in eating can be obtained with unilateral body powered or externally powered prostheses. Independence as regards dress, undress, and toiletry can be obtained by a combination of prosthetic fitting, foot function, and adaptive clothing, plus a highly motivated patient exposed to excellent training facilities.2,7,8,18
Quadrimembral amelia is a very rare congenital limb deficiency. Whether this rarity represents true infrequency of the abnormality or whether it represents a low survival rate of children with the anomaly is not known. The rehabilitation potential is exceedingly limited. In the absence of all limbs the child has no way of sitting because he cannot stabilize his pelvis and buttocks so that his abdominal muscles and spine extensors can hold his torso erect. The pelvis can be stabilized by fitting such a patient with a plastic laminate bucket that is molded snugly about the buttocks and is indented over the iliac crests. This will permit the development of the ability to sit and hold the head erect. Once this sitting ability has been developed, the pelvic bucket can be mounted on an Ontario sway walker and limited ambulation on a smooth surface can be obtained.
When independence in sitting has been established, the upper limb problem can be approached as outlined for the treatment of the bilateral upper limb amelic patient (Fig. 13 ).
Since these children have lost both arms and legs, and since it is estimated that each leg represents 18 per cent and each arm 9 per cent of the body surface, these patients have lost 54 per cent of their total body surface. Therefore, their ability to lose heat by surface convection is so seriously reduced that even minimal activities produce profuse sweating, often a temperature rise of a degree or more, and easy fatigability. For this reason functional activities, either walking or upper limb function, must of necessity be markedly limited. The rehabilitation goals in these patients must be evaluated realistically and established by the amputation team, and must be clearly explained to the parents.
External power for upper limb function is nearly a necessity in these severely handicapped children. If such is not commercially available, these patients should receive the benefit of research development items whenever possible.
*Lecturer in Orthopedic Surgery, Northwestern University Medical School, Chicago. Medical Co-director, Area Child Amputee Program. Chief, Department of Orthopedic Surgery, St. Mary's Hospital, Grand Rapids, Michigan.
- Aitken, G. T.: Proximal femoral focal deficiency. In Swinyard, C. W. (Editor): Limb Development and Deformity: Problems of Evaluation and Rehabilitation. Springfield, Illinois, Charles C Thomas, 1969.
- Aitken, G. T.: Management of severe bilateral upper limb deficiencies. Clin. Orthop., 37:53-60, 1964.
- Aitken, G. T.: Tibial hemimelia. In Aitken, G. T. (Editor): Selected Lower-Limb Anomalies-. Surgical and Prosthetics Management. Washington, D.C., National Academy of Sciences, 1971.
- Aitken, G. T., and Frantz, C. H.: The juvenile amputee. J. Bone joint Surg., 35A: 659664,1953.
- Brown, F. W.: Construction of a knee joint in congenital total absence of the tibia (paraxial hemimelia, tibia): a preliminary report. J. Bone joint Surg., 47A:695-704,1965.
- Burtch, R. L.: Nomenclature for congenital skeletal limb deficiencies: a revision of the Frantz and O'Rahilly classification. Artif. Limbs, 10:24-35, 1966.
- Carroll, L. J.: Prosthetic training techniques for bilateral upper amelia. Part 1. Amer. J. Occupat. Ther., 18:97-101, 1964.
- Carroll, L. J.: Prosthetic training techniques for bilateral upper amelia, Part IL Amer. J. Occupat. Ther., 18:144-146, 1964.
- Coventry, M. B., and Johnson, E. W.: Congenital absence of the fibula. J. Bone joint Surg., 34A:941-955, 1952.
- Farmer, A. W., and Laurin, C. A.: Congenital absence of the fibula. J. Bone joint Surg., 42A:1-12, 1960.
- Frantz, C. H., and O'Rahilly, R.: Congenital skeletal limb deficiencies. J. Bone joint Surg., 43A: 1202-1224, 196 1.
- Hall, C. B., Brooks, M., and Dennis, J.: Congenital skeletal deficiencies of the extremities: classification and fundamentals of treatment. J.A.M.A., 181:590-599,
- Hall, J. E., and Bochmann, D.: The surgical and prosthetic management of proximal femoral focal deficiency. In Aitken, G. T. (Editor): Proximal Femoral Focal Deficiency: A Congenital Anomaly. Washington, D.C., National Academy of Sciences, 1969.
- Kruger, L. M., and Talbott, R. D.: Amputation and prosthesis as definitive treatment in congenital absence of the fibula. J. Bone joint Surg., 43A:625-642, 196 1.
- MacDonell, J. A.: Age of fitting upper extremity prostheses in children. J. Bone joint Surg., 40A:655-662, 1958.
- McLaurin, C. A.: Swivel walker. In Annual Report, Prosthetic Research & Training Unit, Ontario Crippled Children's Centre, Toronto, 1965.
- Putti, V.: The treatment of congenital absence of the tibia and fibula. Int. Abstr. Surg., 50:42, 1930 (abstracted from Chit. Org. Mov., 7:513, 1929).
- Schmid, H.: Foot skills in children with severe upper limb deficiencies. Amer. J. Occupat. Ther., 25:159-163, 1971.
- VanNes, C. P.: Rotation-plasty for congenital defects of the femur. J. Bone joint Surg., 32B:12-16, 1950.
- Westin, G. W., and Gunderson, F. 0.: Proximal femoral focal deficiency: a review of treatment experiences. In Aitken, G. T. (Editor): Proximal Femoral Focal Deficiency: A Congenital Anomaly. Washington, D.C., National Academy of Sciences, 1969.
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Publications by George Thomson Aitken, MD
Not only was George Aitken, MD, a remarkable clinician and administrator, he was also an eloquent contributor to the professional literature. His printed legacy includes significant journal articles, monographs and book chapters, as indicated by the following complete bibliography.
- Aitken, G. T.: Review of Care of Thrombocytopenic Purpura Treated by Spleenectomy J Mich State Med Soc 37:628-629, 1938.
- Aitken, G. T., F. Mitts and C. Lamboley: Method of Recording Scoliosis. Physiother Rev 18:252-254, 1938.
- Aitken, G. T.: Anatomical and Mechanical Considerations of the Knee Joint. Physiother Rev 20:201-206, 1940.
- Aitken, G. T.: Early Treatment of Compound Fractures. J Mich State Med Soc 42: 353365,1943.
- Aitken, G. T.: Lessons Learned from a Review of Hand Cases Evacuated from the South Pacific. Instructional Course Lectures, American Academy of Orthopedic Surgeons 1: 202208,1944.
- Aitken, G. T., and C. H. Frantz: The Juvenile Amputee. J Bone Joint Surg 35-A: 659664,1953.
- Aitken, G. T., and C. H. Frantz: Prostheses for the Juvenile Amputee. Am J Dis Child 89:137-143, 1955.
- Aitken, G. T., and C. H. Frantz: Congenital Amputation of the Forearm. Ann Surg 141: 519-522,1955.
- Aitken, G. T.: Amputations, Prostheses and Braces: The Lower Extremity Juvenile Amputee. Instructional Course Lectures, American Academy of Orthopedic Surgeons 14: 329335,1957.
- Frantz, C. H., and G. T. Aitken: The Juvenile Amputee. J Mich State Med Soc 57: 233241,1958.
- Frantz, C. H., and G. T. Aitken: Management of the Juvenile Amputee. Clin Orthop 14:3049, 1959.
- Aitken, G. T.: Amputation as a Treatment for Certain Lower Extremity Congenital Abnormalities. J Bone Joint Surg 41 -A: 1267-1285, 1959.
- Aitken, G. T., and C. H. Frantz: Management of the Child Amputee. Instructional Course Lectures, American Academy of Orthopedic Surgeons 17:246-295, 1960.
- Aitken, G. T.: Hazards to Health: Etiology of Traumatic Amputations in Children. N Engl J Med 265:133-134, 1961.
- Aitken, G. T.: Current Concepts in the Management of the Juvenile Amputee. Orthot Prosthet Appliance J 16, 1962.
- Russell, H. E., and G. T. Aitken: Congenital Absence of the Sacrum and Lumbar Vertebrae with Prosthetic Management. J Bone Joint Surg 45-A:501-508, 1963.
- Aitken, G. T.: Surgical Amputation in Children. J Bone Joint Surg 45-A: 1735-1741, 1963.
- Aitken, G. T.: Management of Severe Bilateral Upper Limb Deficiencies. Clin Orthop 37:5360, 1964.
- Aitken, G. T.: Whither Prosthetics and Orthotics? Artif Limbs 9:1-3, 1965.
- Frantz, C. H., and G. T. Aitken: Complete Absence of the Lumbar Spine and Sacrum. J Bone Joint Surg 49-A: 1531-1540, 1967.
- Aitken, G. T.: Osseous Overgrowth in Amputations in Children, in Limb Development and Deformity: Problems of Evaluation and Rehabilitation, C.A. Swinyard, Ed. Springfield: Charles C Thomas, 1969.
- Aitken, G.T.: Proximal Femoral Focal Deficiency, in Limb Development and Deformity: Problems of Evaluation and Rehabilitation, C. A. Swinyard, Ed. Springfield: Charles C Thomas, 1969.
- Aitken, G. T.: Proximal Femoral Focal Deficiency-Definition, Classification and Management. Washington: National Academy of Sciences, 1969.
- Aitken, G. T., Ed: Proximal Femoral Focal Deficiency: A Congenital Anomaly. Washington: National Academy of Sciences, 1969.
- Aitken, G. T.: Occasion Instant; Decision Difficult. Presidential Address, American Academy of Orthopedic Surgeons. J Bone Joint Surg 53-A:791-795, 197 1.
- Aitken, G. T., Ed: Selected Lower-Limb Anomalies: Surgical and Prosthetic Management. Washington: National Academy of Sciences, 1971.
- Aitken, G. T.: Tibial Hemimelia. Washington: National Academy of Sciences, 1971. 28. Aitken, G. T.: The Child Amputee: An Overview. Orthop Clin North Am 3:447-472, 1972.
- Aitken, G.T., Ed: The Child with an Acquired Amputation, Washington: National Academy of Sciences, 1972.
- Aitken, G. T.: Proximal Femoral Focal Deficiency and Coxa Vara with Congenital Short Femur. The CIBA Collection of Medical Illustrations: Musculoskeletal System. To be published in 1984.