The Child with Terminal Transverse Partial Hemimelia: A Review of the Literature on Prosthetic Management
Barbara L. Sypniewski *
Introduction
This independent-study honors project dealt with congenital skeletal limb deficiencies. This paper discusses and
reviews the literature concerning the prosthetic management of the individual
with unilateral terminal transverse partial hemimelia of the upper extremity.
Specific topics considered are: a general description of the entity, including
etiology and incidence; psychological factors affecting the limb-deficient child
and his parents; normal and abnormal biomechanics of the upper extremity;
components of the prosthesis (terminal devices, wrist units, elbow hinges,
cuffs, harnessing, and sockets); prosthetic prescription and fitting; the trend
toward early fitting; preprosthetic therapy; and prosthetic training. One
section discusses the information elicited from a survey conducted by letters
and questionnaires that were sent to the 28 clinics participating in the Child
Prosthetics Research Program, conducted under the auspices of the Subcommittee
on Child Prosthetics Problems of the Committee on Prosthetics Research and
Development to ascertain the age of the congenitally skeletally limb-deficient
child at the time of his initial fitting for a prosthesis. An analysis of the
data from the 12 clinics replying is presented, along with
the developmental criteria for fitting.
The scope of this paper is limited to the
unilateral upper-extremity, below-elbow congenital amputee. Bilateral amputees,
cineplasty, surgical conversion, or externally powered prostheses are not
considered. The literature review was limited by time to the books and journals
published in 1960 or later, with selected earlier articles. Articles published
before 1960, as well as those not available at the Albany Medical College
Library or through the inter-library loan system, are listed in the
"Bibliography." Both reference lists were compiled from Index Medicus;
Amputees, Amputations, and Artificial Limbs (published by the Committee on
Pros-thetic-Orthotic Education of the National Academy of Sciences-National
Research Council, Washington, D.C.); and the bibliographies of articles I
reviewed.
Terminal transverse hemimelia indicates
congenital absence of the entire distal part of the limb below the elbow. The
term is part of the modified Frantz-O'Rahilly classification
nomenclature. Hemimelia is the absence of a large part of a limb, from the Greek
melos meaning limb and hemi, half. Partial hemimelia
indicates that less than half the limb is missing. The defect we are considering
is transverse rather than longitudinal, presenting a short or very short stump
similar to that of an acquired below-elbow
amputation.
The etiology of skeletal limb
deficiencies is largely unknown, except for the well-documented teratogenic
effects of thalidomide. The thalidomide tragedy has led to an increased interest
in, and awareness of, what can be done for the congenital amputee.
The list of proposed etiological factors
includes environmental conditions such as drugs, maternal health and nutrition,
genetic factors or predisposition, and chromosomal aberrations. Most congenital defects have their origin during the first eight weeks of
embryonic life.
Glessner indicates that there
are two distinct groups of congenital absence of limbs: (1) spontaneous
intrauterine amputation after limb formation, caused by focal deficiencies, and
(2) limb-bud arrests or agenesis of the terminal part of the limb. Amniotic
bands wrapped tightly around part of an extremity may lead to necrosis and
eventual intrauterine amputation. Terminal deficiencies due to
limb-bud arrests are by far the most common type of congenital absence. The terms congenital amputation and congenital
skeletal limb deficiency are used interchangeably in the
literature.
Terminal transverse partial hemimelia is
the most common type of congenital limb deficiency. There is unexplained
preponderance of left-sided absence (2 or 3 to 1), and females are involved more
frequently than males. Studies by Bergholtz, Davies, Friz, and
Clippinger, Munson and Dolan, and Gehant
failed to show the greater incidence in females exhibited in Kay and
Fishman's report.
The measures of prosthetic management in
habilitation of a congenital amputee are somewhat different than those employed
in the rehabilitation of an "acquired" amputee. The child must learn functional
skills that he never possessed, rather than relearning substitute functional
activities. The fact that the juvenile amputee is neither skeletally nor
emotionally mature is an important consideration in the prosthetic management.
The growth and development of the limb-deficient child is essentially the same
as that of the normal child; the environmental stimuli to motor development are
not decreased significantly by unilateral deficiency. Ideally, prosthetic
management should extend from birth through vocational training.
Function of the upper extremity is
extremely complex and relatively independent of the contralateral extremity.
With unilateral absence, there is an increased use of the remaining extremity,
since the ability of a prosthesis to compensate for the loss of an arm is
significantly less than is possible in the lower extremities. Below-elbow
amputees are least in need of externally powered prostheses. They can effectively use body power to activate the prosthesis and receive
the benefits of sensory feedback through the socket and harness. The prosthesis
should be considered as an assistive device in bimanual activity. Because
absence of one extremity can be easily compensated for, getting the unilateral
amputee to use his prosthesis presents a great challenge. Fitting and training
should be started as early as possible, before these compensations can
develop.
It is generally believed that a team
approach is most successful in the management of the limb-deficient child. The
foremost members are the mother, who spends the most time with her child and
influences him the most, and the child. Other possible members
of this interdisciplinary team are the physician, orthopedist, prosthetist,
occupational therapist, physical therapist, psychologist, social worker, and
biomedical engineer. Each child presents unique problems to be met. Epps and
Brennecke outlined a sequence of treatment that includes referral,
history and medical examination, intake evaluation, preprosthetic physical and
occupational therapy, prescription, fabrication, thorough check-out by
the team, training, and regular recheck every
three or four months.
Factors influencing the cost of the
prosthesis are: age at initial fitting, regular maintenance, frequency of
harness adjustment, wearing pattern, operating skill, acceptance, and components
prescribed.Average service for a prosthesis ranges from two to
three years, but a child fitted during infancy may require three to five
prostheses before school age. The additional cost of early fitting
is compensated for over the years, especially in regard to the
benefits of skill and acceptance.
Psychological Aspects
The importance of parental attitudes
towards the child, his disability, and the idea of a prosthesis, and their
effect on the eventual acceptance or rejection of a prosthesis, has been
emphasized throughout the literature. There is no direct correlation between the
degree of the child's deficiency and the mother's perception of the child's
abnormality, her feelings toward him and the way she handles him. The way
in which parents deal with the birth of a limb-deficient child depends to a
great degree on how they have coped with previous crises. Replacement of a
missing extremity with a well-functioning artificial one is valuable only if the
parents can accept the idea of a prosthesis. Often, children have rejected
prostheses because the parents, consciously or unconsciously, could not accept
the fact that it was necessary.
The way in which the parents are informed
of the child's deficiency may influence their later reactions. If he desires to
do so, the father should be allowed to inform the mother, in the presence of a
physician. Mothers can be profoundly influenced by the reactions of
the delivery-room staff. The training of the limb-deficient child
can best begin by providing the parents with a detailed, factual, realistic, and
sympathetic appraisal of their baby and his prospects for future educational,
vocational, and social rehabilitation. Unrealistic
claims that modern prosthetics and engineering can provide artificial devices as
natural-looking and as efficient as the human hand can seriously hinder the
habilitation program. The first few hours after the birth of the child are
crucial; it is during this period that parents form attitudes and defenses that
can have tremendously far-reaching effects.
With the birth of a deformed child, the
parents suffer a severe psychological shock, for which they are totally
unprepared. Certain emotions have been commonly expressed by parents of
congenital amputees: guilt, hopelessness, death wishes, fear, anger, rejection,
despair, shame, repulsion, grief, shock, hostility, and abandonment. The need for prompt, professional assistance is crucial.
Parents are extremely sensitive to the reactions and attitudes of others, and
they need help to know that they and their child are accepted. In addition to
individual counseling by a psychologist, social worker, or other qualified
persons, group sessions have been established. Parents
benefit from the opportunity to verbalize their feelings and receive support and
help in handling their emotions and in developing constructive attitudes.
Wallace noted the impact of these group-therapy sessions on the
fathers, citing fewer absences, less hesitation about expressing their feelings,
and awareness that their attitudes affect the child's adjustment and help to
mold his self-image.
If, instead of realistic acceptance,
strong defense mechanisms are built up by the parents during this early period,
they will not be able to communicate with their child when he becomes aware of
and questions his deficiency. One indication of the mother's acceptance of the
child is the way she handles the baby. Some important factors to look for in
observing parental behavior are: avoidance of direct contact with the baby,
ritualistic organization and emphasis on cleanliness, barriers to communication,
aggression toward professionals, and subconscious refusal
to accept the existence of the child's abnormality. The mother will eventually become the
child's best therapist, and the early months must provide a basis for her later
role. Parents must be aware of the importance of their love in the future
rehabilitation of their child. Hall and Mongeau and others
advocate that children become an integral part of the family immediately.
Mongeau found that children taken home directly from the hospital after birth
have shown greater capacity for adaptation than those who were
institutionalized. A strong family basis can be of great help to the child when
he may later face repeated hospitalizations for prosthetic training or other
reasons. According to Gesell and Amatruda, a child's basic behavior
traits are fairly well established by the time he is a year old. Some of these
traits are hereditary and some are absorbed from the attitudes of the
family.
Crisis intervention, as described by
Brooks and others, is the awareness of impending crises in the development
of the limb-deficient child and the intervention by qualified professional
personnel to aid in making those transitory periods as easy as possible. One
such crisis is that of homecoming. The curiosity and concern of relatives and
friends must be faced. The effect of the birth of a limb-deficient child
naturally has a great impact on his siblings. They too must be
aided in adjusting to this stress situation. Other potential crisis periods are
prosthetic fitting, entering school, and adolescence. During the child's period of growth and
development, he has the same needs for independence and self-sufficiency that
normal children have. Dependence and overprotection must be avoided. Discipline
must be consistent and realistic, neither extremely permissive nor extremely
restrictive. The profound effects of the parents on the child cannot be
overemphasized.
The manner and degree to which
the child is influenced by his deficiency is
determined before he reaches conscious awareness of his condition. If he has
been provided with a sense of security, acceptance, and love, he will have a
strong basis from which he can develop a positive self-image and achieve
independence. The limb-deficient child faces the same problems and sequence in
emotional and social development as normal children, but each crisis is likely
to be of greater intensity and magnitude. The child who has
received encouragement and support from his family will expect the same type of
relationship from outsiders and will approach social contacts spontaneously,
rather than attempting to avoid them. The child will attain a balance between
the dominance of his parents' influence and the satisfaction he gains from his
independence. He should be encouraged to enter into social
relationships with a minimum of special attention.
Taylor has discussed at
length the psychological needs of handicapped children. In addition to the
fundamental needs of love and acceptance, she cites the needs for adventure and
exploration, rebellion to release pent-up frustration, limitation of freedom,
friends and social experience, privacy, achievement as a basis of self-esteem,
and the need for awareness of the child as a person. These needs are the same as
those operating in all nonhandi-capped individuals.
Gouin-Decarie recognized that
a pertinent problem in studying the psychology of a limb-deficient child relates
to his conception of space, which is closely associated with the formation of
the body image. She found that these children made use of a visual, rather than
a tactile, image in recognizing familiar objects. Several authors have discussed
the concept of body image, or schema, in child amputees.
All have indicated the absence of marked distortion of body image in most of
these individuals. Alteration of body image is, however, a significant problem
in noncongenital amputees. Centers and Centers analyzed the results
of a draw-a-person test administered to
congenital amputees. The majority of amputees represented themselves
realistically, either leaving out the missing limb or including the prosthesis.
They concluded that, while body images differed in a matter-of-fact way, they
did not differ markedly in signs of greater conflict, anxiety, or defensiveness.
The study did not support the authors' hypothesis that amputee children will
have more conflict and defensiveness about their bodies than will nonamputee
children.
The body image is critical in relation to
the acceptance or rejection of a prosthesis. Congenital amputees experience the
same processes in the formation of body image as normal children. The earlier
the child is trained to wear a prosthesis, the easier it will become a part of
his body image. One factor in the ready incorporation of the
prosthesis is that modern prostheses are functionally adequate for many of the
activities engaged in by young children. A prosthetic device is
never really useful until it is integrated into the body schema. Acceptance and
rejection of the prosthesis is more extensively considered in the section on
early fitting.
The question of the possibility of the
phenomenon of phantom sensation in congenital amputees is an interesting one. A
discussion of the theories concerning the cause of this phenomenon is beyond the
scope of this paper. Hoover, Lambert, and Simmel believe that neither phantom-limb sensation nor pain exists in this group of
individuals. Lambert bases his belief on the principle that nerve endings going
to the distal limb have never developed. Simmel attributes the impossibility of
phantom sensation to the fact that the absent part has never been represented in
the body schema. In their census of the juvenile-amputee population, Kay and
Fishman reported three instances of phantoms in congenital amputees,
but these could not be substantiated by further interrogation. Weinstein and
Sersen reported phantoms in 5 out of 30 children with congenital
deficiencies. If the presence of a phantom reflects the
"need" of the child to experience a missing part, it should have functional
properties. The phantoms reported in this study were usually shrunken,
telescoped parts with gaps and missing appendages.
Certain other psychological aspects can
best be discussed as they relate to the chronological age groups of the
congenital amputee. The significant divisions are: preschool, entry into school,
latency, and adolescence.
In the preschool category, a period of
negativism and resistance occurs around two years of age. This is a normal
reaction; the child is trying to establish his personality and achieve a little
independence. This period of negativism often conflicts with
prosthetic-training procedures, especially terminal-device
activation.
Entry into school is an important
milestone for any child. He moves from the security of his home environment into
a competitive social society. The limb-deficient child needs a reliable basis
for dealing with this new group of people. This is provided by his parents and
family during the early childhood years. In his group experience, the child will
test and validate ways of dealing with people outside his family .
Adjustment is facilitated if the teacher and class are prepared and informed
in advance. Healthy curiosity is the most frequent reaction of classmates, and a
factual explanation of the prosthesis and its use should lead to acceptance by
the classmates and increased self-confidence of the limb-deficient child. Wilson
expresses the belief that it is preferable for the
limb-deficient child to attend regular school. Unnecessary special consideration
should be avoided. The handicapped child may experience feelings of social
devaluation, which any member of a minority group feels. Centers
and Centers discuss the results of a social-discrimination
questionnaire. The hypothesis that peer-group children express more covert
rejecting attitudes toward amputees than toward nonamputee children was
supported. They attribute this finding to the fact that one of the
most significant variables operating in social interaction is personal
appearance. Centers and Centers conducted their study almost ten years ago. It
would be interesting to retest this hypothesis in light of recent social trends
toward greater acceptance of minority groups and increased emphasis on
individual merit as opposed to sterotyped generalizations.
The preadolescent latency period is
relatively calm, with no major crisis periods. The normal child experiences many
conflicts during adolescence, many of which are associated with appearance.
These conflicts are all compounded in the limb-deficient child. During this
period, a cosmetic hand is often prescribed for the adolescent amputee to
replace the functional hook for social occasions. Vocational guidance becomes
increasingly important during this period of adolescence.
Normal and Abnormal
Biomechanics
The arm enables the hand to be placed in
position for skilled functional activities. The most commonly recognized forms
of prehension include tip, palmar, three-jawed-chuck, lateral, hook grasp,
cylindrical grasp, and spherical grasp. Palmar prehension employing opposition
of the thumb predominates in picking up objects and holding them for use. Long
tendons with muscles at a distance permit the great variety of motion
characteristic of the human hand. In addition to skill, the hand frequently
functions in support postures. Sensation is another major function of the hand.
The hand is richly supplied with sensory-nerve endings mediating touch,
temperature, pain, and position. Large areas of the cerebral cortex represent
the complex sensory and motor function of the hand. Boivin advocates
investigation into the prehension patterns and sequences commonly used in
activities of daily living. Stabilization of the wrist in various positions aids
prehension. For example, the wrist assumes an angle of 145° when very strong
prehension is required. Finley, Wirta, and Cody
studied the synergic action of muscles of the
upper extremity resulting in a better understanding of the relationship between
central and peripheral control of movement. The three major components of the
response phenomenon that they noted were: cognitive, ballistic-type physical
displacement, and apparent sensing to compare, confirm, or adjust to assure
successful accomplishment of the desired act. The information regarding time
sequences is useful as reference material in studying
pathomechanics.
Finger and hand movement, wrist flexion
and extension, and varying degrees of pronation and supination are lacking in
the congenital below-elbow amputee. Prosthetic replacement of the wrist and hand
is poor, only crude prehension and positioning are possible, and there is no
substitution for the lack of sensory feedback. Maximum utilization of the
residual biomechanics is essential in prosthetic replacement. The
biggest challenge is to design an upper-extremity prosthesis that (1) can be
powered by and controlled with little effort, (2) can perform through the almost
spherical range of a normal arm, (3) has a terminal device that can achieve
prehension, (4) will respond to sensation, and (5) is cosmetically acceptable. Upper-extremity prosthetics are significantly deficient in all of
these areas. Because of the fixed prehension pattern of the terminal device and
the fixed wrist, nearly all fine orientation movements must be made at levels
higher than the forearm by compensatory motions of the elbow, hand, and shoulder
. Prosthetic controls permit only the simplest motions decomposed
into their basic elements and executed slowly, in series, one at a
time.
Stoner notes that no
prosthesis accomplishes any of the wrist-flexion movements. The reasons for this
neglect of wrist replacement are: (1) usually no controls from the harness are
available to furnish the power, (2) wrist motions are used in fine movement of
the hand and are not essential to bring the hand into the major spheres of
action about the body, and (3) loss of wrist flexion can be compensated
for grossly by other arm motions. Preposition flexion devices are available and
are useful for activity close to the body.
Pronation and supination are functions of
forearm length. Wrist joints allow passive positioning for the most advantageous
angle of terminal-device operation. With shorter forearm stumps, the mechanical
advantage of flexion is decreased, in addition to the loss of pronation and
supination.
Joint motions in congenital amputees are
often bizarre. Kruger and Breyan report that, in an X-ray
evaluation of 16 extremities with terminal transverse partial hemimelia, 13
showed dislocation of the head of the radius. Of these, 77% showed dislocation
before prescription of the initial prosthesis. It is therefore concluded that
the phenomenon is inherent in the disability itself. The dislocation is
asymptomatic. The authors offer two possible explanations for the phenomenon:
deficiency of the ligamentous structures, or unopposed action of the biceps
brach-ialis muscle. They consider the latter explanation the more likely. In
short stumps, the pronator teres muscle is absent, and the biceps in flexing and
supinating meets no opposition, thereby dislocating the radial head.
Harnessing
Harnessing techniques for upper-extremity
prostheses must be based on bio-mechanical analyses of the remaining movements.
Successful use of the prosthesis requires a harness that allows the most
efficient use of those movements that are available. The socket limits some of
the residual motion of the stump itself, and the harness limits the motion of
the sound extremity to some extent. The harness should distribute the weight of
the prosthesis evenly over a wide area and be functional in as many positions of
normal use as possible. It should transmit power with a minimum of interference
and be operable by relatively inconspicuous body motions. Power is provided by
the stump itself (elbow flexion) or by the relative motion between two body parts
(glenohumeral flexion and/or scapular abduction). Control-cable systems transmit
this power from the amputee's body to the prosthesis. The suspension system may
use a figure-of-eight, figure-of-nine, or shoulder-saddle chest-strap type of
harness. The most common suspension is a figure-of-eight harness with a
Northwestern ring-type cross. The Northwestern ring allows
adjustment of individual harness straps. The figure-of-nine harness is often
used for power transmission with Munster-type sockets, which do not require a
great deal of additional suspension. The chest strap is useful in spreading the
load in heavy work and maintaining the prosthesis in the proper
position in the presence of baby fat. The harness provides some degree of
feedback from the environment. O'Shea has described a
shoulder-saddle chest-strap harness with the primary advantage of increased
comfort. Hile described the adaptation and reinforcement of a
brassiere to replace the chest-strap harness when breast development
occurred.
Requirements for suspension and
harnessing vary from individual to individual, and skillful use of the available
power sources is essential to good prosthetic use. Rapid rate of growth and
limited power are critical factors in designing harnesses for congenital
amputees. Frequent adjustment by the prosthetist assures optimum
harness and prosthetic function.
Components of the Prosthesis
Terminal Devices
Two major considerations in the design of
a prosthesis for a child are the continual neuromuscular and skeletal changes
due to growth and the child's limited sources for power and control. Linear
growth is more rapid than circumferential growth. The prosthesis can be
fabricated to allow for later adjustments for growth, thus extending the
functional life of the device. The components must be sturdy enough to withstand
vigorous use, yet must be light enough to be controlled by the
child. Some of the problems involved in the prosthetic replacement of human body
parts are control, feedback, reliability, size, and appearance.
Upper-extremity prostheses for children are essentially scaled-down models
of adult types. However, Hall and Wilson note that
recent advances in children's prosthetics include improved design and function
of terminal devices, lightweight plastic sockets and shells, and more efficient
harnessing methods. There are a large number of mechanical components available
that can be combined to best meet the needs of the individual child. Split
mechanical hooks stress the restoration of function at the expense of abnormal
appearance, while artificial hands with cosmetic gloves attempt to combine
modest levels of function with near-normal static appearance. Both hooks and
artificial hands should be given the same care as the normal hand; since
sensation is absent, they are more prone to damage.
There are two mechanisms of
terminal-device operation: voluntary opening and voluntary closing. In the
voluntary-opening type, tension on the control cable opens against a variable
spring force, while in the voluntary-closing type, control-cable tension closes
against the spring force. Hooks and hands are available with either mechanism.
Voluntary opening is the simplest form of prehension mechanism: the prehension
force is provided by special heavy rubber bands. Among the disadvantages of this
type are the inability to handle delicate or heavy objects, and the fact that
this mechanism is opposite to the prehension of the normal hand. An advantage of
the voluntary-closing terminal device is that it more accurately simulates
normal prehension, and pressure can more easily be graded to the object to be
grasped. Formerly, manually controlled locks were employed, but now automatic
locking is available. The fact that, to release the lock, the cable pull must be
greater than the pull that closes the terminal device may be a
disadvantage. Neither mechanism has been proved
superior in a wide range of activities, but research to
improve both types for juvenile amputees is continuing.
Ritter and Sammons have
elaborated on the advantages of voluntary-closing devices for children's
prostheses. The fact that normal prehension is simulated is especially relevant
in bilateral grasping. Performing different hand patterns simultaneously, as is
necessary with voluntary-opening devices, is particularly difficult for the
preschool child to learn, since he is still developing refinement of prehension.
A description of the Army Prosthetics Research Laboratories (APRL)
voluntary-closing hand, which provides palmar prehension of the three-jaw-chuck
type, has been presented by Stoner. Teska and Swinyard have described a test to evaluate its functional capacity, versatility, and
durability. Research is also being conducted concerning the Robins-Aid
voluntary-opening hand. The concept of cosmesis, or the
appearance of the prosthesis, is difficult to define, but is very important. It
is a very individualized concept, having varying importance for different
people. Function, cosmesis, and acceptance are almost inextricably allied. The area of compromise between function and cosmesis is a delicate and crucial one. Those professionals vitally concerned with function must be
careful not to look down on the parents who may seem to be overly concerned with
cosmesis. Several new plastics have been reported that, while not
identical to the color and texture of the human skin, do convey an idea of
softness and warmth. These new terminal-device designs represent an attempt to
combine improved function with an aesthetically satisfactory appearance, but
without trying to imitate representationally the characteristics of the missing
part.
It was formerly common practice to
provide the congenital amputee with a plastic mitt or wafer as the initial
terminal device. Dean, Lineberger, and Watkins and Ford
have presented arguments supporting this practice. Among the major
reasons given are: cosmetic appeal, flexibility, support without slipping in
creeping, avoidance of injury to the child himself or others during play, and
other factors supporting early fitting in general.
The infant passive hook is now considered
the better choice as an initial terminal device. Some of the reasons for its
preferred function are listed by Blakeslee: (1) it provides for gross palmar
prehension and body-support activities with skill equal to the mitt, (2) it
allows the infant to hook over objects for support in pulling to a standing
position, (3) it provides a holder for small objects that are placed in it, (4)
it helps the infant to develop bilateral prehensile awareness, being recognized
as a device to hold objects, and (5) parents who were willing to accept a
prosthesis for their child readily accepted the passive hook. Shaperman
reported the results of an evaluation of the passive mitt and the passive
hook with similar results. She also noted improved skill and increased speed of
learning when the control cable was added to the passive hook. Initially, the
hook presented a slightly greater safety hazard, but the injuries that did occur
were minor. Shaperman noted that the hook was one ounce heavier than the mitt,
but it appeared to be well within the limits of the infant's ability to lift and
manipulate it easily.
Hooks are available in a variety of
sizes, shapes, and weights. The Dorrance 12P or 10P hook are commonly provided
for the unilateral juvenile amputee. They are canted and plastic-covered.
Proponents of prescribing hooks cite the advantages of greater prehensile
function, with greater visibility and facility available. Numerous authors
have expressed a preference for the use of
the hook rather than the hand. Edelstein maintains that the cosmetic appeal of a
skillfully used hook is greater than that of a cadaverous-looking glove. The
idea that the hook can only be accepted as a tool, and that therefore it is hard
to see the need for a more cosmetic socket, has been expressed by Boivin
. Research toward improved hook design and
function is being carried out. The literature reveals progress reports in the
development of the Sumida hook, the Northwestern University
Center control hook, the Steeper split hook no. 65, and
other more recent advances in prosthetics. Carroll conducted a study to
analyze the prehension force needed by child amputees. The test items were
related to function and varied with the age of the child. Most items tested
static prehension only; the individual could either hold the object, or it
slipped out of the hook because of insufficient prehension force. Dynamic
prehension, or the child's ability to control the prehension force, was tested
by the ability to hold a paper cup with water in it. The results of this study
showed that more children were fitted adequately in regard to the size of the
terminal device than in relation to the prehension force. None of the children
were found to be wearing an excessive number of rubber bands. With the exception
of the toddler group, the prehension force was found to be inadequate for
performance of one or more of the test items. One result of this study was a set
of suggested pinch forces for below-elbow amputees:
Age (years)
|
Pounds of
force
|
2-4 |
2.25
|
3-9 |
3.5
|
5-9 |
4
|
8-17 |
5
|
15-20 |
6
|
Greater consideration needs to be given
to the adequacy of prehension forces for the functional activities of congenital
amputees.
Cosmetic hands are often prescribed when
the juvenile amputee reaches adolescence. Interlocking wrist-unit mechanisms are
available that permit the use of a hook for functional activities and a more
cosmetic hand for social occasions. These hands usually provide a modified
three-jaw-chuck prehension between movable index and middle fingers and a thumb
that can lock in position. Hands available for children include the
Dorrance no. 2 hand and the APRL-Sierra child-size no. 1 hand. One disadvantage that must be considered is the greater weight of the hand as compared to the hook. The APRL-Sierra no. 1 hand weighs
170 grams, while the Dorrance 10x hook weighs 60 grams. This is
especially important, considering that this additional weight has the mechanical
advantage of a long forearm lever and the congenital amputee does not possess a
great deal of muscle power.
The APRL-Sierra no. 1 hand was developed
to meet the need for a functional and cosmetically acceptable hand for juvenile
amputees. It is a voluntary-opening mechanism with a hand shell of cast
aluminum, articulated index and middle fingers, a two-position thumb, and
nonarticulated but flexible ring and little fingers. In this field
study, only 7 of 77 children rejected the hand completely. The remaining
participants fell into four groups: those that used the hand exclusively, those
that used the hand predominantly, those that used both equally, and those that
used the hook predominantly. The authors suggest that the age of the child is a
major factor regarding hook or hand preference. Younger children may experience
difficulty with hand weight and opening forces, may be more careless in their
use of the hand, and may be less subject to social pressures toward cosme-sis.
Sex appeared to be an even greater consideration than age. Girls of all ages
appear to be potentially the best candidates for the Sierra-APRL no. 1 hand,
while younger boys would seem least likely to accept the device. Fishman and Kay
performed a study to delineate the relative usefulness of the hook
and the hand. The results were at variance with previous clinical impressions,
which indicate that a hand is a significantly less functional terminal device
than a hook. In an extensive evaluation of the Dorrance no. 2 hand in 72
bimanual activities, Gorton found that no definite trends emerged to
indicate that the hook was measurably more functional than the hand or that the hand was significantly more
functional. The test employed by Fish-man and Kay analyzed general and specific
patterns of grasp by means of functional activities. The rating scale for
performance of activities was somewhat subjective, but the detailed analysis of
the results was excellent. From this study, the authors concluded that: (1) the
APRL-Sierra no. 1 hand was heavier and, in most cases, more difficult to operate
than the previously used hook, but these were not serious drawbacks for the
majority of subjects; and (2) the hand provided somewhat less pinch force than
most of the hooks and a less precise grasp. While the majority of children
reported that they could perform more activities better with the hook, they also
were able to specify a number of activities that were performed better with the
hand, such as picking up a pencil, grasping paper, and holding silverware for
eating.
Constant research and re-evaluation of
prostheses is essential. Boivin has written an
excellent article criticizing present artificial-hand design. He maintains that
an inherent belief exists that the refinement of the normal hand cannot
presently be reproduced, leading to the assumption that it can never be
reproduced. He cites the apparent lack of coordination and integration in
biomedical engineering research, and proposes that a reason for this is that the
goal is providing normal hand function, but that this is being attempted without
sufficient consideration for the actual anatomical and physiological functions
of the hand according to the kinesiological data presently available. One
example is the fact that artificial hands flex only at the metacarpophalangeal
joint, while the flexor digitorum profundus, the most active finger flexor,
flexes at the interphalangeal joints as well. Boivin presents two suggestions
for modification of artificial-hand design: first, that the normal transverse
arch be reproduced in artificial hands, adding to cosmesis and function; and
second, that artificial hands be made smaller and covered with a soft
subcutaneous tissue-like material under the glove. Besides
improved cosmesis, this would improve grasp by allowing better molding of the
fingers over the object to be grasped. This second approach is presently being
used by the Otto Bock Orthopedic Industry, Incorporated, in their new modular
arm. The catalogues illustrate an above-elbow arm, but it is quite possible to
employ this system for below-elbow amputees by fabricating the socket, attaching
the proper length tube and the terminal device. This "System Arm" can be used
for every level of upper-extremity amputation except wrist disarticulation and
extremely long below-elbow amputations. Child-size systems are available. (This
information was received from personal communication with Otto Bock Orthopedic
Industry, Incorporated.)
Wrist Units, Elbow Hinges, and
Sockets
Wrist units perform the dual function of
attaching the terminal device to the prosthetic forearm and providing
terminal-device rotation for manual preposi-tioning. There are manual-friction,
manual-lock, and active-rotation units. Manual-friction is the most commonly
used type. A rubber washer and a metal washer are compressed as the terminal
device is screwed into place. Behavior of the unit is unpredictable because of
the uneven compression and the easy accumulation of dirt, but it has the
advantages of simplicity and easy maintenance. Manual-lock units allow rotation
and locking of the terminal device by separate steps through the use of
cylindrical inserts that have index teeth around their circumference. The inserts are threaded to fit the terminal-devicestud. Active-rotation devices use stump rotation to produce rotation of the terminal device and are
able to amplify residual stump rotation. Wrist-flexion units that provide partial
replacement for lost palmar and dorsal flexion of the wrist are available. By
adding the extra degree of freedom, they can minimize the need for compensatory
motions at higher levels. These units are presently only suitable for light duty. Clarke, Kral, and Shaperman
evaluated wrist-flexion units for children. The advantages of the
addition of a wrist-flexion unit to an upper-extremity prosthesis include: (1) the ability to bring the arms close to the body for self-care activities,(2) the ability to bring the arms
together in the midline for bimanual activities, and(3) less need for body exertion and
bending to accomplish these activities. The authors found that one angle of
flexion or flexion and radial deviation is sufficient for all activities. Wrist
flexion of 25° or less is comfortable and useful, and there is no advantage
above 25°. They advocate that the conventional wrist unit be laminated into the
forearm unit in a flexed position, after careful evaluation to determine the
most advantageous angle. This overcomes the disadvantages of wrist-flexion units
for children, such as added weight of the terminal device, an additional
component to preposition, and mechanical unreliability. It would seem that the
need for dorsiflexion at the wrist for functional activities should be further
evaluated, since this study only considered variable degrees of palmar
flexion.
Flexion of below-elbow prostheses is
provided by hinges of various types; the main classes are "rigid," "semirigid,"
and "flexible." They can be made of metal, leather, or metal cable. Some elbow
hinges are polycentric and have a step-up ratio to provide a greater range of
motion for a short below-elbow amputation. This is useful if adequate power is
available, since flexion strength is lost through this mechanism. When both
power and range are insufficient, it is possible to utilize the stump power to
activate a locking hinge. Flexion of the forearm is then provided by humeral
flexion.
Most below-elbow prostheses require an
upper-arm cuff made of leather to help to stabilize the connection between the
amputee and the prosthesis necessary to adequate control. The most
common types are the very light triceps pad and the open cuff. These would be the most
useful for congenital amputees; the heavy-duty closed cuff would not usually be
necessary.
The socket is the foundation of all
upper-extremity prostheses. The standard socket designs are used for juvenile
amputees, but they may fit poorly because of the large amount of soft tissues in
the child and the lack of well-developed bony prominences. It is through the
socket that power and control are transmitted from the stump to the prosthesis
and some degree of feedback is received. Double-wall construction allows a
stump-fitted inner wall with an outer wall designed for structural uniformity
and cosmesis. Retention of pronation and supination in short and very short
below-elbow amputees is usually not a consideration, since pronation and
supination are factors of forearm length. Another important matter is stability
in flexion. In short and very short stumps, a single-axis hinge helps to provide
this stability.
Among the types of sockets available are
single-socket, split-socket, preflexed socket, and Munster-socket designs.
Single sockets are often lacking in the necessary flexion stability for
congenital amputees. Because of limited range of motion, a short or very short
stump may require a split socket with a step-up hinge. One degree of stump
movement gives 2° or 3° of prosthesis movement, thereby increasing the range of
motion, but two or three times normal force is needed to accomplish this.
VanDer-werker and Rosenberger described the mechanism and
installation of a flexor assist for use with the step-up split socket. Pellicore
noted the unfavorable cosmesis of the split socket, which was later
largely replaced by the use of a preflexed forearm. This improved the cosmesis
somewhat and increased the functional forearm power, but the range of motion was
limited to 100°-110° instead of the normal 135°.
A great deal of the recent literature is
devoted to a description and discussion of the Munster-type socket. The
technique, involving intimate encapsulation of the stump, was developed by Dr.
O. Hepp and Dr. G. G. Kuhn of Munster, Germany, and introduced into the United
States in 1958. Short below-elbow stumps present a small attachment area, poor
leverage, and a decreased useful range of motion. Some of the characteristics of
the Munster technique that help to overcome these deficiencies are: (1) the
elbow is set in a preflexed position yielding the most useful range of motion,
usually about 35 deg., (2) a channel is provided at the antecubital space for
the biceps tendon to avoid interference between the socket and biceps tendon
during flexion, and (3) the posterior aspect of the socket is fitted high around
the olecranon and the epicondyles, taking advantage of these bony prominences to
provide attachment and stability to the socket. These
characteristics eliminate the need for split sockets with step-up hinges, giving
improved prosthetic control and feedback, and often eliminate the need for a
harness for suspension purposes. Younger congenital amputees may require more
harnessing to maintain the prosthesis in place.
Epps and Hile described the
fabrication techniques and evaluated the Munster prosthesis. Among the
favorable points they found were: simplified harnessing, light weight, no
perspiration problem, and excellent stability under axial-load testing. They
also noted the elbow hyperextension characteristic of the individual with
terminal transverse partial hemimelia. They concluded that the Munster-type
prosthesis is the fitting choice for the child with a unilateral short or very
short below-elbow amputation. In their investigation of the applicability of
Munster-type fittings, Fishman and Kay found that all of the
subjects were definitely in favor of this type of prosthesis. The decrease in
flexion range had no appreciable effect on prosthetic function for unilateral
amputees. (Some modifications, such as lowering the anterior trim line and
provision of a wrist-flexion device, may be necessary for the bilateral amputee.) Among the advantages
cited are the facts that the stump does not slip out while performing overhead
activities, and that less energy is required in operation of the prosthesis.
They suggest that this type of fitting is functionally advantageous for amputees
with very short to medium below-elbow stumps. Two factors limit the
applicability of this technique for stumps of longer lengths: (1) the pronation
and supination in these stumps cannot be harnessed with a Mun-ster prosthesis,
and (2) the proximal socket opening at a sharp angle to the shaft presents
increasing difficulty in donning and doffing the prosthesis as stump length
increases.
Gazeley, Ey, and Sampson
reviewed four cases of fitting children with Munster sockets and concluded
that the technique is not satisfactory for bilateral amputees, because of the
limited flexion. Except for that, they were very pleased with its use. Gorton, Kay and Fishman, and Pellicore have all
cited the usefulness of the Munster-type prostheses in fitting short and very
short below-elbow stumps. Gorton found the positive factors to be: increased
stability and socket retention, socket comfort with minimal stump motion within
the socket, harness comfort with the elimination of the triceps pad and front
support strap, and improved cosme-sis due to the minimization of the harnessing
system. The negative features listed were: decreased range of motion (limited to
about 70°), limited elbow flexion, and harness discomfort due to the control
strap riding low across the back. The other authors discovered similar findings.
With the limited range of motion, it is necessary to make this the most
functional range. Partial flexion is necessary to keep the prosthesis on the
stump. Complete extension is not as essential to functional activity as an
adequate flexion range.
The use of sockets that do not completely
enclose the stump is more extensive in Germany than in the United States. With
this type of prosthetic fitting, the end of the stump remains free for
gripping and touching. According to Fletcher
and an article in the British Medical Journal, in
congenital limb deficiency the end of the limb has a tactile sensation
equivalent to that of a normal fingertip, even when the distal two-thirds of the
forearm is missing. He attributes the prosthetic rejection by many children to
the fact that standard prostheses rob them of this important sense of touch. He
feels that fitting such an individual with an artificial limb is, in effect,
performing a physiological amputation. Kuhn and Jentschura,
Marquardt, and Rudel have described an open-end socket that enables
the patient to use the sensory surface of his stump as well as the terminal
device. The socket is provided with a friction joint on the dorsum of the
prosthesis so that the terminal device can be bent away from the end of the
stump. The economic advantage of an increased "life span" of the prosthesis, as
well as the functional advantages of the open socket, have been presented by
Jaramillo and Lehneis. The preservation of tactile sensation is an
important consideration in upper-extremity prosthetic design. Increased research
on open sockets is indicated, since they seem to provide a critical advantage
over the standard prostheses, especially for the bilateral amputee.
Prosthetic Prescription and
Fitting
The prescription of a prosthesis for a
congenital amputee, as for any amputee, is best achieved by a team approach. The
child's functional needs and developmental status must be ascertained in order
to provide the optimum combination of components. Actual fabrication is followed
by a final check-out of the compatibility of the amputee and the
prosthesis.
The physician, prosthetist, and physical
and occupational therapists are the main members of the prosthetic-clinic team. The physician, in writing the prescription, must combine his
knowledge of the individual with the results of evaluations performed by other
members of the team. The prosthetist advises about possible solutions to the case, measures the
patient, fabricates the prosthesis and harness, and evaluates the functional
results of fitting. The physical and occupational therapists evaluate motor
development, range of motion, and muscle strength, advise the physician and
pros-thetist of available body power for control, suggest possible solutions to
fitting problems, and perform the final checkout evaluation.
As a functional replacement for the
missing limb, the prosthesis must be a simple, lightweight device that will
enable the child to perform certain tasks, but not necessarily all tasks. Stamp,
Mahon, and Morgan found that, with the unilateral below-elbow
amputee, the use of a prosthesis improves the function of the opposite, normal
extremity. The combination of a normal extremity and a prosthesis is much more
functionally efficient than is the combination of a normal extremity and a
stump.
The functional needs of the child must be
determined in order to provide a prosthesis that will fill these needs.
Self-care needs are an important part of the functional evaluation. Observing
the compensatory patterns that the child has naturally developed for holding or
reaching yield an indication of his specific functional needs. One approach to
functional evaluation has been to observe which parts are missing and to
formulate a prescription on the theory that these are the parts that need to be
replaced prosthetically. This theory assumes that, once these are provided, the
child will meet all of his activity needs. It is important that the total effect
of the prosthesis is a significant gain in function. The advantages and
disadvantages for each individual must be carefully considered.
It is necessary in the early examination to determine the developmental status of the child. This evaluation
bears a significant relationship to the timing and type of prosthetic fitting.
In much of the literature, the achievement of a secure sitting balance is designated as an
important criterion to upper-extremity prosthetic fitting. (The criteria for
fitting are discussed more completely in the section on the trend toward early
fitting.) An important part of the evaluation is the observation of the infant's
prehension patterns. The infant's ability to control and relate his various arm,
hand, and body movements predicts his pattern of prosthesis operation and use
. The development of compensatory prehension patterns is one of the positive
indications for fitting the child with a cable-operated hook. The child's
interest, attention span, and coordination must also be determined. All of this
information aids in prescribing a prosthesis and planning a training
program.
In addition to this evaluation of
neuromuscular development, the therapist must also determine muscle strength and
range of motion. The prosthetist needs to know which structures are present and
which are absent, and what sources of power are available. Muscle defects may
accompany skeletal defects, as pectoral agenesis occasionally accompanies
below-elbow deficiency. Some of the abnormalities of neuromuscular-system
function to notice are: involuntary motion, deviations in the speed of motion,
resistance to passive movement, atrophy, fatigue, and static or dynamic postural
deviations. Functional muscle testing as described by Daniels,
Williams, and Worthingham provides valuable information.
Range-of-motion tests are useful in noting any contractures or other factors
limiting the range and in determining the scapular movement available to operate
the devices prescribed. Sequential testing and accurate recording are necessary
in functional, motor-developmental, muscle-strength, and range-of-motion
evaluations.
Exact body measurements, both
longitudinal and circumferential, are often made by the prosthetist at the time
of fitting. In the unilateral amputee, the epicondyle-to-thumb length is
important as a sizing reference for the total
length of the finished prosthesis.
The choice of the components for the
prosthesis is based on a thorough knowledge of the functional needs and the
potentials of the individual. It was formerly accepted practice to prescribe a
passive mitt, but this practice has been replaced by the use of a passive,
plastic-covered hook. The hook gives the child the opportunity to incorporate
the concept of a prehensile device from the start. The manual-friction wrist
unit is often useful for congenital amputees. At first it can be positioned by
the parents, and later by the child himself. Sockets that permit rotation are
not usually indicated in short below-elbow stumps, since residual pronation and
supination is minimal. The Munster-type socket, or modifications of it, as well
as conventional below-elbow double-walled laminated sockets, seem to be
successful in fitting the individual with terminal transverse partial hemimelia.
Harnessing and suspension are highly individualized and can make the difference
between successful and unsuccessful prosthetic prescription. Some of the
greatest problems in prescribing and fitting the congenital amputee arise from
his rapid, uneven rate of growth, the presence of baby fat, the lack of
well-defined bony prominences, and the almost constant mobility of all young
children. It must be emphasized that good prescription of prosthetic components
must be based on a thorough knowledge of the individual. The prosthesis should
allow him to function at his highest level in his environment. For the
congenital amputee, this may mean providing him with the opportunity to assume a
normal pattern of development of bimanual activity. In unilateral amputees, the
prosthesis functions as a helper, not as the dominant hand.
Fabrication and interim fittings are
performed by the prosthetist. After careful initial measurements, a plaster cast
of the stump is made. This is used to make a mold of the stump. A full
description of the techniques for fabricating the prosthesis is beyond the scope of this paper;
however, a step-by-step account of fabrication is given in the Manual of
Upper Extremity Prosthetics. There is no universally acceptable
check-out procedure for the child amputee. The standardized adult forms are not
useful, because child prosthetics is a relatively new field in which
improvements in techniques are constantly being made. Additional
contraindications to a standardized form are the varied ages and developmental
levels of the children, philosophies of case management and prescription which
may vary from clinic to clinic, and the fact that so many modifications of the
prostheses for congenital amputees are needed. The standard check-out forms must
be adapted if they are to be used for child amputees. The clinic team must
evaluate the fit and function. The pros-thetist's primary interest is the
mechanical aspects, the therapist's is the child's functional benefit. The
physician must coordinate the efforts of all of the paramedical personnel.
Blakeslee has presented some of the important considerations regarding
check-out for the juvenile amputee.
Prosthesis fit
- Is the prosthesis cosmetically
acceptable? Is it well made, and does the workmanship follow all of the
specifications of the prescription?
- Is the prosthesis of the proper
length, and is the socket fit satisfactory? Do bony prominences have sufficient
space? Do the component controls appear to be within reach of the
amputee?
- In the upper-extremity
prosthesis, is the harness adjusted properly and is it comfortable?
- When the prosthesis is
removed, are there any excessive pressure points in the socket area?
Functional
considerations All components must be checked to make
certain they are in good working order, and must be adjusted for efficient
operation by the child and/or adult. Some of the primary functional
considerations are:
- Is the prosthesis properly
aligned?
- If it is an upper-extremity
device, is the control system appropriate for this child? Will he be able to
control the arm and operate the controls in the desired range of motion? Is the
terminal device in good condition and does it operate smoothly? Does the harness
appear to be correctly positioned and in balance?
- Can the prosthesis be applied with
ease? Is the amputee comfortable in the standing, sitting, and walking positions
and while performing functional activities?
These check-out procedures emphasize the
points to consider in preprosthetic evaluations, prescription of components, and
fabrication. The prosthesis must be made to fit the needs of the child; the
child should not be expected to adapt to the prosthesis.
The Trend Toward Early Fitting
A great deal has been written concerning
the advantages of early fitting, and a variety of developmental criteria for
fitting have been described. This section deals with the advantages of early
prosthetic fitting for the upper-extremity juvenile amputee, a brief discussion
of normal motor development, and a discussion of fitting at various ages. The
age levels can be roughly grouped as follows: before school age, nine to twelve
months, six to eight months, four to six months, and three months or younger.
This grouping is the distribution that occurred naturally in the literature. The
concept of prosthetic acceptance or rejection is also discussed in this
section.
The philosophy of early fitting is the
dominant theme of much of the literature. The difference exists in the
definition of the term early. Before this concept was accepted,
prescription of an artificial limb was not advised until the patient reached the
middle or late teens, in order to avoid the expense of purchasing a
device that soon would be outgrown. More recently, the child was fitted just
prior to school age, but still after the child had become
oriented to one-handed function. Frantz has presented a brief
history of the management of the juvenile amputee during the past twenty
years.
Mongeau and others recommend
that the habilitation of congenitally deformed children be initiated at an
early age. Many other authors have proposed
reasons for early fitting. Friedmann lists the following advantages:
(1) to stimulate bilateral function, (2) to help the child and parents to accept
the prosthesis for function or cosmesis, (3) to incorporate the prosthesis into
the child's body image, (4) to improve balance, (5) to get the child accustomed
to the normal length of the limb, (6) to prevent scoliosis and other skeletal
abnormalities due to asymmetry, (7) to make the child aware of prehensile
function, and (8) to promote eye/hand control. In addition to the advantage of
greater acceptance, Blakeslee cites the fact that early fitting leads
to a more normal development of the residual parts and diminishes atrophy caused
by disuse and hypogenesis. The prosthesis encourages physical activity, which
increases growth and strength. The avoidance of substitute patterns of grasp,
such as holding objects in the axilla or elbow-bend and working in an awkward or
energy-consuming position, was noted as an advantage by Blakeslee, Brooks and others, Gillis, and Klopsteg, and Wilson
et al.. More of the movement patterns of the upper extremity are
acquired than in the lower extremity, thus increasing the importance of early
fitting. Gillis maintains that the movement patterns necessary to control the
prosthesis are most perfectly developed at the same time as those for the
natural limb. The possibilities of atrophy through disuse and the development of
contractures are greater with later prosthetic fitting. As the
result of a study conducted at the Rehabilitation Institute of Montreal, Gingras
and others found that in a majority of cases there was hypotrophy of
the deficient limb. They found an average difference of one centimeter between
the lengths of the humeri. The hypotrophy was attributed to disuse because it
had been observed that patients who had early prosthetic training were enabled
to put their muscles to greater use and therefore they showed less limb-length
inequality. An additional advantage of early fitting mentioned by Edelstein is that it aids the
limb-deficient child in crawling. Children learn to use the upper-extremity
prosthesis as well as, if not better than, adults. The advantages of
skill in prosthetic use resulting from early fitting have been cited by Brooks
and others, Dean, and Mayo. Some of the
favorable results of early prosthetic fitting for the unilateral below-elbow
amputee presented by Brooks and Shaperman include: (1) full-time wearing of
the prosthesis, (2) skillful operation of the prosthesis, (3) natural and
spontaneous patterns using the prosthesis and including it in normal activities,
(4) good habits of prosthesis maintenance, and (5) good acceptance of the
prosthesis by the child, family, and community.
In reviewing the literature, the author
noted that earlier fitting was advocated more often for children with bilateral
and multiple limb deficiencies than for those with unilateral deficiencies. One
possible explanation for this may be the comparatively greater need for sensory
input for development and function by the former group. The supposition of
earlier fitting was substantiated in a census study by Kay and Fishman.
They suggested that this may be related to the greater need by multiple
limb-deficient individuals for prosthetic assistance.
The developmental norms of Gesell and
Amatruda form the basis of much developmental evaluation. They are
especially relevant to the unilateral congenital amputee. For instance, he may
first be aware of his missing limb at about three months of age, when he
attempts two-handed grasp. Vitali cautions that a limb-deficient
child should not be expected to achieve standards of developmental performance
before others in his age group.
In an analysis of data collected over a
two-year period ending on June 30, 1967, Davies, Friz, and Clippinger
noted that a relatively high percentage (32%) of congenital amputees were
not fitted until after their eleventh birthday. Since the current philosophy is
to fit congenital amputees at a very early age, it would be
interesting to know the reason for this delay. The authors could not determine
whether the fault lay with the amputee clinics or with parents who were
reluctant to take their children to clinics or ignorant of the prosthetic
opportunities available to them.
In discussing the advantages of early
fitting, there is variability in the definition of early. Brooks and
Shaperman, Kay and Fishman, and Watkins and Ford support the idea of fitting the unilateral below-elbow amputee before school
age, at the latest. Of those authors advocating fitting when sitting balance has
been achieved, some are referring to independent sitting without support (about
ten months of age) and others to sitting with support (about six months). In
either case, this leaves the upper extremities free in a functional position.
The group of proponents includes Aitken, Brooks and others,
Caine and Reeder, Catto and MacNaughtan, Jansen, Shaperman, and Wilson. Several authors indicate a preference for
fitting at six to eight months of age. Among these are Blakeslee, Gillis, Hall, Kempner, Lineberger, and
Vitali. Lineberger and Gillis have cited the benefit of having a
prosthesis to aid in crawling and pulling to a standing position.
Encouraging bilateral movement patterns
and establishing familiarity with and tolerance for the limb are advantages of
prosthetic fitting between four and six months of age. This is considered the
best age for fitting by Edelstein, MacNaughtan, Martin, and Mayo. Lambert and others maintain
that the congenital amputee should be fitted with a prosthesis as soon as he
needs it. For the unilateral upper-extremity amputee, this may be as early as
three months. According to Gingras and others, fitting this early is
based not only on considerations of function, but also on the idea of helping the child incorporate the
presence of an artificial arm into his body image and to accept it better.
Tolerance and adaptation to the prosthesis as well as aid in developing sitting
balance has been stressed by Nichols and others. Prosthetic acceptance or rejection is a
very complex concept. It is an accepted psychological principle that an
individual is better able to achieve adequate adjustment to a total loss of
function than to a partial one, yet prosthetic devices restore partial function.
The relationship of the amputee to his prosthesis is that of man to machine. It
is an intimate and long-term contact between a human being and a mechanical
device. The gadget tolerance of the individual is of great importance,
especially as the child grows older and develops greater skill in using the
prosthesis. Both the visual consideration of cosmesis and the auditory factors
of a mechanical device, such as the sound of a terminal device closing on an
object, play major roles in the formation of the individual's attitude toward
his prosthesis. If the prosthesis is regarded as a tool that makes him less
different and gives him a better opportunity for integration into his peer
group, then the child is more likely to wear and use his prosthesis. If he
believes that the prosthesis accentuates the difference between himself and
others, it is likely that he will reject it. Throughout the literature, it has been
emphasized that children usually accept a prosthesis without too much difficulty
. It helps if the individual can gain immediate
satisfaction from its use, rather than feeling that it is a deterrent to his
activity. A child can be helped to appreciate the usefulness of the prosthesis
by providing him with toys and chores that require two hands. Both a full-time
wearing pattern and the ability to talk freely and openly about the prosthesis
are good indicators of acceptance.
Several authors have emphasized the
positive relationship between early fitting and good prosthetic acceptance. A
patient most easily accepts a prosthesis if
he obtains it before becoming accustomed to one-handed activity.
Kempner, Mongeau and others, and Wilson believe that early fittings lead to complete patient and family acceptance.
In evaluations by Brooks and Shaperman, children with short below-elbow
stumps fitted before two years of age received the best scores for "acceptance."
Gingras and others found that rejection is a common occurrence if
prosthetic fitting takes place after adolescence, while Blakeslee found
excellent acceptance and utilization if the child was fitted before four years
of age, and increased rejection after that age.
Congenital amputees experience the same
structuring process in regard to body image as do normal children. If a child is
presented with a prosthesis during the critical stage when his body image is
forming,, he will incorporate the limb into his pattern of activity and
self-image. Centers and Centers note that modern
prostheses are functionally adequate for many of the activities engaged in by
children. This may be a factor in the incorporation of the prostheses
into their body images. Personality factors are directly related to acceptance
of a prosthesis.
In the case of the congenital amputee,
his parents' attitudes affect his personality and his acceptance or rejection of
a prosthesis. Parental influence cannot be overemphasized. It is within the
family structure that all of the child's attitudes are developed. A clear view
of parental influence is presented by Brooks and Shaperman in their
discussion of a group of children who had rejected their prostheses. The group
was characterized by a lack of parental support and guidance in the child's
general behavior. There was a great deal of emphasis on the child's
accomplishments without the prosthesis. These parents expected less of their
children than their potential, openly expressed dislike for the appearance of
the prosthesis, and had a limited ability to communicate feelings and problems. One
review indicated that the better-educated middle-class families are
most likely to help their children accept prosthetic appliances.
All of these considerations regarding
acceptance and rejection are interrelated.
Questionnaire Survey Concerning Age at
Initial Fitting
The questionnaire survey sought to
document a trend toward earlier initial fitting of upper-extremity prostheses in
the congenital amputee. As the most frequently occurring limb deficiency,
unilateral terminal transverse partial hemimelia was selected as the focus of
consideration. An extensive review of the literature had seemed to indicate a
trend toward earlier fitting. While children were formerly fitted just prior to
school age or even during the middle or late teens, the achievement of
independent sitting balance is now a widely accepted criterion for prosthetic
prescription. According to Gesell and Amatruda's studies of motor development, the norm for the achievement of this maturational level is nine
months (36 weeks).
It was the belief of the author that (1)
even earlier fittings are being performed in significant numbers, (2) a passive
hook is most frequently prescribed, and (3) the development of the Münster-type
socket has played a role in the trend toward earlier fitting.
Questionnaires were mailed to the 28
clinics participating in the Child Prosthetics Research Program, a cooperative
endeavor conducted under the auspices of the Subcommittee on Child Prosthetics
Problems of the Committee on Prosthetics Research and Development. The
information requested was of three types: age at time of initial fitting, type
of socket and terminal device most frequently prescribed, and basic
developmental levels considered essential for fitting the prosthesis.
The sample consisted of 40 new patients
with upper-extremity terminal transverse partial hemimelia who were initially
fitted between March 1, 1969, and approximately March 1, 1971. The frequency of
fittings is indicated in Table 1.
One clinic whose data arrived too late to
be included in the chart reported fitting more than 200 cases. A relatively
small number (between 15 and 20) were fitted between the ages of 6 and 9 months,
and a much larger number (50 or 60) were fitted after the age of 12 months. Two
other clinics indicated that the information needed to complete the
questionnaire was not readily available. (One of these stated that all of their
children were fitted after the age of 12 months.) In requesting the data, no upper limit
was set on the last interval (later than 12 months). For this reason, no
statistical analysis of the central tendency (mean or median) was possible. The
return on this survey was 43%, the low response level being partly attributable
to the fact that no date was designated for the return of the
questionnaire.
The frequency distribution indicated that
65% of the children were fitted under one year of age. Using nine months as the
age for reaching the developmental level of independent sitting, the data
indicates that 37.5% were fitted before that age. It is also interesting to note
that 20% of the sample was fitted before six months and 7.5% before three
months. This information indicates a trend toward fitting earlier than the
widely accepted criterion of independent sitting balance. The very important
concept of parental attitudes and other intangible factors were not considered,
nor was the age when the child was first seen at the clinic taken into
account in this study. If it were, perhaps
an even stronger trend toward earlier fitting would be noticed.
Regarding the type of terminal device,
seven clinics prescribed a Dorrance 10P or 12P passive hook most frequently. One
fitted a nonfunctioning hand (mitten) initially and changed to a hook at about
two years of age. The other clinic listed both the passive hook and the passive
hand in their response. Five of the clinics prescribed a conventional
double-walled plastic-laminate socket most frequently, and four clinics most
often prescribed a Münster or modified Münster socket.
An interesting outcome of this survey was
the compilation of the developmental criteria for fitting employed by the
various clinics. In the following chart, the list of criteria is paired with the
developmental norms described by Gesell and Amatruda.
Developmental
Criteria
|
G and A Norms
(mo)
|
Beginning to prop on
elbows
|
3
|
Readiness for bimanual
activity
|
4
|
Head control
|
5
|
Object transfer
|
7
|
Beginning sitting
|
8
|
Independent sitting
balance
|
9
|
Controlled voluntary grasp and
release
|
9-12 |
One clinic responded that they did not
adhere to any developmental criteria, but felt that as soon as the child was
three or four months old, a prosthesis could be fabricated with adequate socket
fit. It was their belief that the earlier the socket was fitted, the
better.
The data collected on this sample did not
establish a relationship between the development of the Münster-type socket and
the trend toward earlier fitting.
It is hoped that persons responsible for
prescribing prostheses might consider the criteria proposed by other clinics for
fitting of prostheses for congenital upper-limb amputees. The advantages that
prompted the change from pre-school-age fitting to fitting at the developmental
level of independent sitting continue to exert an influence toward still earlier
fitting. The greatest advantage claimed is that of acceptance of the prosthesis.
Logically, if the artificial limb is provided before a one-handed activity
pattern is developed, changes for acceptance are increased. It would further
seem logical that, when the capacity for two-handed grasp in the midline
develops (at approximately four months), a prosthetic limb should be there to
oppose the normal limb. The proximal stability necessary for control is
developed previously in the on-elbows position. Many factors interact to affect
the age of initial fitting. The age at which the limb-deficient child is
referred to the clinic is certainly a significant one. Parental attitudes are
closely associated with this consideration. The development of prosthetic parts
specifically designed for children is important, as is the increase in knowledge
in the entire field of prosthetic management of the juvenile amputee.
Dissemination of this knowledge to the related health fields, especially to
those individuals in contact with the mother of the newborn child with limb
deficiencies, may promote earlier referral to the appropriate prosthetic
team.
It is believed that the trend toward
earlier fitting is advantageous. A difference in the practice of various clinics
has been noted. A polarity exists with a tendency for some clinics to fit
predominantly at a very early age range and others only later. Three of the
clinics indicated fitting only after 12 months. It would be useful for all the
clinics that participate in the management of congenital amputees to carefully
evaluate their criteria for prosthetic fitting and training.
Preprosthetic Therapy
Preprosthetic care should begin as early
as possible. Hall believes that physical and occupational therapy
should be started as soon as the child begins to take part in his environment. A
highly individualized treatment program to correct the deficiencies in range of
motion, posture, and muscle strength is an important goal of preprosthetic
therapy. The evaluations described earlier as prerequisites
to prescription are also a part of the preprosthetic therapy program. Jaramillo
and Lehneis suggest that the child's poor attention span or
negativism may be due to the lack of preprosthetic training by means of a good
exercise program, rather than to poor family cooperation.
Several authors have emphasized the
important role the mother plays as the therapist. She can
be the best therapist for her child, since she spends more time with him than
anyone else. She must understand the purposes of the therapy program and carry
out the program at home. A good home program will facilitate prosthetic
training. A well-informed mother can help to prevent contractures and postural
deviations and to correct existing problems. It is a significant psychological
asset for the mother to be an active member of the prosthetic team. An
additional consideration that is the mother's responsibility in the early stages
of habilitation is stump hygiene. The stump should be washed, rinsed, and dried
thoroughly and inspected daily for any minor irritation or abrasion. The
limb-deficient child perspires more than normal because of reduced body area.
He should be dressed in light, unrestrictive clothing for cooling and to
allow freedom of movement.
Limitations of range of motion do not
occur as often in the upper extremity, and when they do occur, they do not as
markedly affect its use.The best treatment is prevention. This
can be accomplished by instructing the parents in positioning and active
exercises to prevent contractures and build strength and endurance. Extreme
caution should be used in stretching any joints in the congenital limb-deficient
child. The elbow is especially vulnerable, and passive stretching is
contraindicated. (The tendency for radial-head dislocation has already been
discussed.) The best techniques for increasing range of motion are those that
achieve relaxation of the shortened group by heavy resistance to the
antagonist muscle group. The PNF techniques of
repeated contractions, slow reversal, slow reversal-hold, rhythmic
stabilization, hold-relax, or slow reversal-hold-relax, as described by Knott
and Voss would be appropriate. Since the young child is more flexible in his
muscular structure, it is easier to reverse the adaptive shortening of the
muscles than it would be in adults. Blakeslee also notes the use of passive
stretching, casts, and braces for the correction of flexion
contractures.
The delay in the early neuromuscular
development of children with congenital skeletal limb deficiencies has been
noted by Blakeslee, Hall , Jaramillo and Lehneis , and Steele. The child may be delayed in the development of head and neck
control, rolling over, creeping, and sitting. He may need assistance in
achieving developmental tasks. For example, if the child lacks head and neck
stability, placing a small pillow under his chest allows development of the
trunk and neck extensors. During this early period, assistance may be needed to
help strengthen the neck and trunk flexors, extensors, and rotators. Later, it
may be necessary to stimulate bimanual activity, especially gross grasp, by
providing large objects for the child to hold. The upper-extremity amputee may
need help in pulling to a standing position so that he can adequately develop
his lower-extremity musculature.
Essential muscle groups are exercised to
maintain mobility and increase strength. Specific muscle groups must be
strengthened in order to provide sufficient power to operate the prosthesis.
Bates and Honet and Montero advocate the use of isometric
exercises for this purpose. Exercises for neck and back extensors, flexors, and
rotators are best accomplished through play activity. Catto and MacNaughtan
suggest using mirrors to stimulate the desired movement. The sound
side should be included in the exercise program. Emphasis on strengthening the
shoulder-girdle musculature for elevation, depression, scapular abduction and adduction, and general
chest expansion (respiratory exercises) is important, since these muscles are
needed to operate the prosthesis. For the below-elbow
amputee, strengthening elbow flexion and extension and any available pronation
and supination is of prime importance. Blakeslee has emphasized the
importance of general conditioning. Limb-deficient children tend to have a low
energy output. This was observed even in below-elbow amputees who were otherwise
normal in appearance and physiognomy. Greater than average endurance and output
are required to operate a prosthesis. He also mentions that individual and group
sports and other group activities have been successful in increasing energy
output and improving general physical condition. Swimming has been found
particularly advantageous.
A preprosthetic therapy program provides
a good foundation for later training of the child in the use of the
prosthesis.
Prosthetic Training
Prosthetic training begins when the
congenital amputee receives his prosthesis and continues periodically through
vocational training. The initial training and orientation with a passive
terminal device is essentially the same as that with an active terminal device,
so both are considered together in this section.
Training is one of the most difficult and
important phases in the management of the congenital amputee. It is essential
that the child is enabled to handle his environment rather than adapting the
environment to his needs. Training a congenital amputee is very different than
training a traumatic juvenile or an adult amputee who once had a functional
extremity. The functional level of a normal child of the same age should be the
basis of achievement goals. The program progresses naturally from
gross bimanual grasp to skilled functional activity. Factors affecting training
are the child's neuromuscular development, attention span, functional requirements, and
parental cooperation.
The parents play an important role in the
training of the juvenile amputee. The care and function of the prosthesis must
be carefully explained to the parents, and they must be very aware of what it
can and cannot do. The importance of the parents in prosthetic training has been
emphasized by many authors. Unless contraindicated by medical or
other reasons, full-time wearing of the prosthesis from the first application
should be the aim. According to Blakeslee, one advantage to achieving
a full-time wearing pattern as early as possible is the avoidance of the habit
of removing the prosthesis for little or no reason. Later in childhood, the
wearing pattern will be interrupted for repairs and refitting, so a stable
pattern is desirable. Infants accept prosthesis-wearing easily, unless there is
discomfort or the parents do not allow the prosthesis to be worn all day.
Mac-Naughtan, Shaperman, Steele, and
Watkins and Ford advise a gradual increase in tolerance leading to
full-time wear except for sleeping, bathing, and rough contact sports. This
seems to be a more logical approach than to expect immediate full-time wearing
after the child has become accustomed to complete freedom of movement. During
the period when the child has a passive prosthesis, he should be encouraged to
use it as a "helper" in bimanual grasp, crawling, and pulling to a standing
position. Toys are an excellent medium for encouraging bimanual activity. The
infant amputee who receives his prosthesis during the first year of life shows
remarkably early proficiency in gross arm movements; he develops habits of
including the arm as a total unit rather than any specific part of the arm such
as the hook, tip, or elbow. An awareness of the hook's holding
function should be developed as early as possible.
In response to the questionnaire survey
conducted by the author, the University of California at Los Angeles included
a discussion of the criteria for the
addition of a cable. Some of the factors proposed as prerequisites for
terminal-device activation are: the readiness for bimanual activity, a
reasonable attention span (approximately five minutes), the ability to follow
two-step directions, tolerance of handling by the therapist, the presence of
sufficient neuromuscular development to operate the cable, a full-time
prosthesis-wearing pattern, and an awareness of the hook's holding function. At
UCLA, the cable is usually added at a developmental age of two or two and
one-half years.
Like the variations observed in the age
of choice for initial fitting, similar variability occurs in the age at which
the terminal device is activated. The usual age seems to be about two years.
Mac-Naughtan has expressed the opinion that training should be
conducted at the 14-to-20-month age. Depending on the ability of the child and
the nature of his deformity, active control can be accomplished at 16 to 24
months, according to Hall and Kempner. Edelstein cites 18 months, and Lambert cites 18 to 24 months for the
below-elbow amputee. By the age of 21 to 24 months, the child has developed a
two-handed functional pattern, and he shows signs of a need to develop a pinch
grasp as opposed to purely palmar prehension. By two years of age,
according to Blakeslee, the child is ready for effective terminal-device
activation, although this is typically a period of profound negativism. Dean
and Mayo suggest that a single control cable be
activated at 24 to 30 months, while Gingras and others believe that,
if active prosthesis training is begun by age two or three years, control can be
achieved by four years of age.
A study by Trefler reveals
the drawbacks of normally fitting around two years of age. Some of these
considerations are that the child is ready for bilateral grasp before that age;
he may be difficult to work with at the "terrible twos" stage of hyperactivity
and negativism, and he may have already developed compensatory
patterns, which are more easily prevented than broken. The advantages of
terminal device activation at 15 months of age with a goal of spontaneous
terminal-device use are: (1) the child is easy to work with for short periods of
time (he has an attention span of one to two minutes), (2) when the cable system
is applied to the child's prosthesis, it often helps to eliminate the problem of
excessive external rotation of the socket, and (3) the availability of active
grasp can enhance the activity pattern of an intelligent child. No disadvantages
of early terminal-device activation were discovered. The cable did not restrict
the child's movement during play at all.
Wendt and Shaperman conducted an interesting study to determine whether an infant amputee with
unilateral below-elbow deficiency who was fitted initially with a prosthesis
that included a cable would achieve purposeful control of the terminal device as
part of his normal developmental progression without formal training. The
results indicated that only a minority of the patients (approximately 25%) did
achieve spontaneously the degree of skill usually acquired after formal
training. Some patients partially learned skills, and others remained unaware of
the function of the hook. It is possible that some children were negatively
conditioned by the experience of trying to operate the terminal device and
finding that they were unable to do so because of a lack of skill and guidance
and concluding that the hook was a poor and unreliable tool. An alternative
method of case management that has been suggested is to add the cable when
manual hook-opening appears and then to allow natural development of
terminal-device control. If the skill does not develop spontaneously, the
therapist should intervene with the training program. This emphasizes manual
hook-opening as a relevant step toward the eventual development of active
opening. It was found that children who do learn terminal-device operation
without training develop good skill and use
patterns. If they are going to do so independently, they give evidence of this
well before two years of age and achieve a well-established pattern by that
time. It seems that, if a child is ready to develop the skill for
terminal-device operation naturally, he should be allowed to do so.
Prosthetic training once the control
cable has been added is composed of two parts: training in the control of the
terminal device and later functional training in activities of daily living. The
child's ability to operate a hook relates primarily to his maturity.
Because of the child's short attention span, brief, frequent training
sessions are desirable. Patients may sometimes be required to enter the hospital
for the initial training sessions and occasional retraining later. At home, the
mother can encourage these brief, frequent practice sessions. The child can best
learn the correct control operations and realize the potentials of this
prosthesis through play. There is a tendency for the child to continue to use
his prosthesis as a passive device even after active control has been added. Early training before the control cable
is added should establish the concept of the prehensile function of the
prosthesis. Manual hook-opening, at first by the parent and later by the child,
and placing toys into the hook, should be encouraged. Flexion of the humerus
opens the terminal device. The child must be helped to achieve the awareness of
the relationship of these two incidents. The concept of stabilizing the sound
shoulder in order to operate the terminal device is a difficult one to grasp.
Having the child reach toward the terminal device with his sound arm may be
helpful, or the therapist may need to stabilize the harness. The technique of
immobilization seems to be mastered abruptly and inexplicably, but it may
take a great deal of time. The important objective is to get the child to open
the hook, no matter how awkwardly it is accomplished. It may be necessary to cut
down on the number of rubber bands on the hook to enable the child to open it;
at this point in training, a large prehension force is not needed. The therapist
can help hy offering objects to the child and placing them so that the hook will
open when he reaches for them. One of the most difficult things for a child to
learn is to pick up objects from a horizontal surface.
The sequence of learning grasp and
release with the prosthesis has been described by Blakeslee, Richardson and
Lund, Shaperman, and Wendt and Shaperman.
Although there may be variations in the pattern among individuals, it is
agreed that a pattern does exist for learning terminal-device operation. A brief
summary of the patterns observed by the above authors is presented
here.
Children learn first to actively maintain
the hook in an open position and then to initiate hook-opening actively. Early
opening is often accomplished by abducting and internally rotating the arm
rather than by flexing the shoulder. This closely resembles grasp by the normal
infant. The child finds it easier to open the terminal device with the elbow
extended than in any other position. There is a tendency for the child to place
objects into the hook with the sound hand. The ability to actively close the
hook around an object develops before active release. At first, release of
objects is accomplished by pulling them out of the hook with the sound hand. The
child seems to be unaware that he can open and close the hook for release and
that this requires the same motions that were used for grasp. It takes a long
time and a great deal of practice for a child to become proficient in the use of
the prosthesis. He must learn how far to open the hook to accommodate objects of
different sizes and shapes, to position the hook accurately, and to properly
time the release of an object. The child must also learn to extend the
prosthetic arm and still maintain grasp on an object by releasing his sound
shoulder so it no longer acts as the reaction point for control. The younger
child cannot be expected to learn these more complex skills.
Training hints have been offered by many
therapists. The most frequent suggestion is the use of toys that require
bimanual activity. A lengthy list of toys suitable to each
age group and each desired activity can be compiled. It was also mentioned that
feeding time has been found to be one of the most successful training periods.
Drill activities cannot be neglected, but relating them to functional play
activities as soon as possible is desirable. The Limb-Deficient Child
contains an excellent and extensive section on prosthetic
training.
Three prime functions that require
prosthetic training are feeding, toilet care, and dressing. Other functional
patterns that add to patient independence and satisfaction are: playground,
household, and schoolroom activities, sports, musical instruments, card playing,
and any other activities commensurate with the child's age. Special assistive
devices are available commercially or can be fabricated when necessary.
Vocational training and preparation is a major consideration as the child
grows older. For the unilateral amputee, the prosthesis is a helping or
assisting device, and the sound arm is the dominant one in all activities. The
part of functional training described in this paper is donning and removing the
prosthesis. It is not practical to expect the very young amputee to be able to
put on his prosthesis independently from the beginning. This is in contrast to
the training procedure in adults, which would begin with this skill. Application
is accomplished in the same manner as putting on a coat. The
socket is grasped with the sound arm and the stump is slipped under the
inverted-Y strap. If the prosthesis is raised above the head so that the harness
hangs down, the sound arm can reach back through the axilla loop, and the
harness then can be properly placed. To remove the prosthesis, the child raises
both arms over his head and grasps the socket with his sound arm. He can
withdraw the stump while pulling up on the socket and then remove the axilla loop. Although a stump sock is
usually worn to absorb perspiration, prevent suction, and allow greater comfort
in the socket, it is a matter of individual preference. Some children with
below-elbow deficiencies prefer not to wear a stump sock. It is
recommended that a T-shirt be worn under the harness to decrease local pressure
and irritation, especially in the axilla, and to absorb perspiration.
Successful training will permit the child
to function freely and independently in his environment. Additional training may
be required when the needs of the individual change.
Follow-up studies of juvenile amputees
after long-range treatment from infancy to adulthood have been conducted by
Davies, Friz, and Clippinger, Hamilton, and Lambert,
Hamilton, and Pellicore. All three indicate the excellent results of
long-term prosthetic management as indicated by good social adjustment,
excellent prosthetic utilization, high employment rates, and high levels of
educational achievement. Increases in these favorable results can be expected as
children with congenital limb deformities are referred to prosthetic centers for
treatment earlier and earlier.
Conclusion
This paper has discussed the prosthetic
management of the congenital amputee with upper-extremity terminal transverse
partial hemimelia. Psychological aspects, components of the prosthesis,
prescription and fitting, the trend toward early fitting, preprosthetic therapy,
and prosthetic training have been considered. A review of the literature and a
questionnaire survey were completed. Several questions are raised and areas for
further research are suggested as a result of this study.
Research concerning the etiology of
congenital limb deficiencies is indicated, including the unexplained phenomenon
that the highest incidence of these deficiencies involve terminal transverse
partial hemimelia of the left upper extremity in
females.
Information regarding phantom sensation
in the congenital amputee is lacking. Study in this area might help to explain
the phenomena of phantom pain and sensation in traumatic amputees.
Reports regarding peer attitudes toward
juvenile amputees show some disagreement. Some authors maintain that the
attitude exhibited is one of healthy curiosity easily satisfied by an
explanation, while a study by Centers and Centers showed more covert rejecting
attitudes toward this group of individuals. It would be interesting to retest
this hypothesis of social discrimination in the light of recent changes in
attitudes toward many minority groups, since this study was conducted nearly ten
years ago.
A great deal of research is indicated and
is being conducted in the area of prosthetic design. The results of
biomechanical and kinesiological studies must be incorporated in the design of
components. Analysis of the forces used in prehension and the most frequent
types of prehension employed would be beneficial in improving terminal-device
design. Further evaluation of the hooks and hands presently available and the
voluntary-opening and voluntary-closing mechanisms are needed to determine which
is most efficient and to delineate areas for further research. Some work has
been done regarding optimum wrist-flexion (palmar) angles for functional
activity close to the body. However, no consideration has been made as to the
need for dorsiflexion, which is used very frequently in functional activity of
the normal hand. The field of plastics offer a great source for improvements in
fabrication of prostheses. Durable hooks with improved cosmesis may be a
possibility with the new plastic materials available, as it has already aided in
light weight and durable socket design and fabrication. The open-ended sockets
that permit the use of the sensation at the tip of the stump seem to be an
excellent development, especially for the bilateral amputee. Investigation into the
advisability of increased use in the United States is indicated.
Some disagreement exists concerning the
development of prosthetic tolerance by the juvenile upper-extremity amputee. It
is not, however, a significant controversy, since the goal of full-time wear is
agreed upon, with differing opinions only concerning the rate at which this goal
is reached.
The results of the questionnaire survey
indicate a trend toward earlier prosthetic fitting of the congenital amputee.
Among the most interesting and valuable of all the information received was the
developmental criteria for fitting. This information should be made available to
the clinics participating in the Child Prosthetics Research Program, thereby
enabling each of them to re-evaluate their criteria in light of this newly
accumulated knowledge. Perhaps this can be accomplished through the
Inter-Clinic Information Bulletin.The survey conducted did not consider
the important factors of parental attitudes and age at time of referral to the
prosthetic center. Any future study should incorporate these factors. Another
study might better be able to establish or negate a relationship between the
development of the Münster-type socket and the trend toward early
fitting.
Additional information concerning
activation of the terminal device is needed. The proposal by Wendt and Shaperman
of allowing natural development of the terminal device control once manual
opening occurs, then intervening with formal training if control is not
established by two years of age, merits consideration.
Prosthetics for congenital amputees is a
relatively new area, largely developed since the thalidomide tragedy of a few
years ago. It has many areas requiring further research, such as the need for
lightweight prostheses that can be operated with the available muscle power and
the constant consideration of rapid growth. Research in this specific field of
prosthetics for congenital amputees will contribute to and continue to benefit from
the ongoing research in prosthetics in general. The goal of this research is
improved functional ability for individuals with congenital skeletal limb
deficiencies of varying degrees of severity and for all amputees.
Addendum
Three additional responses from the
questionnaire survey were received after the statistical analysis had been
completed and the article had been prepared. These brought the total return to
53.5%. A summary of the information received is presented here.
The results were generally similar to
those previously reported, with a number of individuals fitted at each interval
except the first (less than three months).
The developmental criteria presented
were: bilateral gross grasp, beginning to sit, independent sitting, and (not
previously mentioned) initiation of hand-eye coordination, as with holding a
bottle, blocks, and general grasp for objects.
Two of the clinics indicated that they
usually fit a first prosthesis at six months of age if the developmental level
allows it. Those fitted later in the statistics returned were not referred to
the clinics until after that age.
Acknowledgments
I would like to thank Miss Dorothy Page,
my advisor, for her help and guidance during this project. I would especially
like to thank Miss Mildred C. Ey, O.T.R., Director of Occupational Therapy at
Sunnyview Rehabilitation Center Hospital; and Mr. Klaus H. Lohman, C.P., of
LaTorre Orthopedics Laboratory. I also extend my appreciation to Dr. Sidney
Fishman, Mr. Hector W. Kay, the A. J. Hosmer Corporation, the Dorrance Company,
the Otto Bock Company, and the clinics answering the questionnaire.
References:
- Aitken, George T., Management of severe bilateral upper limb deficiencies, Clin. Orthop. no. 37:53-60, 1964.
- Amputations and substitutes for limbs, Brit. Med. J. 2:195-196, Apr. 22, 1967.
- Bates, Marion D., and Joseph C. Honet, Isometric exercises for the upper-extremity stump, Journal of the American Physical Therapy Association 44:1093-1094, Dec. 1964.
- Bergholtz, Susan G., Patient Census at Child Amputee Clinics—1969, Prosthetics and Orthotics, New York University Post-Gradu-ate Medical School, June 1970.
- Blakeslee, Berton (ed.), The Limb-Deficient Child, University of California Press, Berkeley and Los Angeles, 1963.
- Boivin, G., Nothing like the human hand, Inter-Clinic Inform. Bull. 7:4:17-19, 22, 1968.
- Brooks, Milo B., and Julie Shaperman, Infant prosthetic fitting: a study of the results, Amer. J. Occup. Ther. 19:6:329-334, Nov.-Dec. 1965.
- Brooks, Milo B., Lila L. Beal, H. Lorraine Ogg, and Berton Blakeslee, The child with deformed or missing limbs: his problems and prostheses, Amer. J. Nurs. 62:11:88-92, Nov. 1962.
- Brooks, Milo B., Yoshio Setoguchi, Joan Thue, Lila L. Beal, and Doris Tom, Crisis intervention, Inter-Clinic Inform. Bull. 4:11:7-15, 1965.
- Burtch, Robert L., A study of congenital skeletal limb deficiencies, Inter-Clinic Inform. Bull. 2:7:1-6, 1963.
- ---------, The classification of congenital skeletal limb deficiencies: a preliminary report, Inter-Clinic Inform. Bull. 3:1:4-9, 1963.
- Caine, Donald, and A. J. Reeder, The problem of the congenital amputee, Med. J. Aust. 50: 1:301-305, Mar. 2, 1963.
- Campbell, Harry E., and Julie Shaperman, Prosthesis costs for the unilateral below-elbow child amputee, Rehab. Lit. 26:305-307, Oct. 1965.
- Carroll, Leila, Sizing and prehension forces of Dorrance voluntary opening devices, Inter-Clinic Inform. Bull. 2:9:7-10, 1963.
- Catto, A. M., and A. MacNaughtan, Physiotherapy and occupational therapy in the management of the upper-limb amputee, Physiotherapy 52:186-188, June 1966.
- Centers, Louise, and Richard Centers, A comparison of the body images of amputee and non-amputee children as revealed in figure drawings, J. Project. Techn. 27:158-165, June 1963.
- ---------, Peer group attitudes toward the amputee child, J. Soc. Psychol. 61:127-132, Oct. 1963.
- Child Amputee Prosthetics Project, Cosmesis: can it be defined?, Inter-Clinic Inform. Bull. 5:10:4-9, 1966.
- Clarke, Susan, Carole Kral, and Julie Shaperman, Built-in wrist flexion for children's prostheses, Inter-Clinic Inform. Bull. 9:5:1-7, 1970.
- Cohen, Pauline C, Impact of the handicapped child on the family, Social Casework 43:137-142, Mar. 1962.
- Contini, Renato, Engineering in medicine, Bull. Pros. Res. 10-8:4-19, Fall 1967.
- Daniels, Lucille, Marian Williams, and Catherine Worthingham, Muscle Testing: Techniques of Manual Examination, 2nd ed., W. B. Saunders, Philadelphia, 1956.
- Davies, Elizabeth J., Barbara R. Friz, and Frank W. Clippinger, Jr., Children with amputations, Inter-Clinic Inform. Bull. 9:3:6-19, 1969.
- ---------, Amputees and their prostheses, Artif.Limbs 14:2:19-48, Autumn 1970.
- Dean, Carleton, Prosthetic Devices for Children with Emphasis on Fitting Upper Extremity Amputees, Michigan Crippled Children Commission, Lansing, Mich., ca. 1957.
- Edelstein, Joan E., News notes, Inter-Clinic Inform. Bull. 9:4:15-16, 1970.
- Epps, Charles H., Jr., Upper extremity limb deficiency with concomitant infantile structural scoliosis, Inter-Clinic Inform. Bull. 5:2:1-9, 1965.
- Epps, Charles J., Jr„ and Frances E. Bren-necke, Juvenile amputee program, Med. Ann. D. C. 31:295-297, May 1962.
- Epps, Charles H., Jr., and John H. Hile, Experience with the Muenster-type below-elbow prosthesis: a preliminary report, Inter-Clinic Inform. Bull. 7:10:1-6, 1968.
- Finley, F. Ray, Roy W. Wirta, and Kevin A. Cody, Muscle synergies in motor performance, Arch. Phys. Med. Rehabil. 49:655-660, Nov. 1968.
- Fishman, Sidney, Amputation, in Psychological Practices with the Physically Disabled, ed. James F. Garrett and Edna S. Levine, Columbia University Press, New York, 1962.
- Fishman, Sidney, and Hector W. Kay, Acceptability of a functional-cosmetic artificial hand for young children, part I, Artif. Limbs 8:1:28-43, Spring 1964.
- ---------, The Münster-type below-elbow socket, an evaluation, Artif. Limbs 8:2:4-14, Autumn 1964.
- ---------, Acceptability of a functional-cosmetic artificial hand for young children, part u, Artif. Limbs 8:2:15-27, Autumn 1964.
- Fletcher, Ian, Artificial limbs, Physiotherapy 52:182-186, June 1966.
- ---------, Malformations of the upper limb, Proc. Roy. Soc. Med. 62:1:55-56, Jan. 1969.
- Frantz, Charles H., An evolution in the care of the child amputee, Artif. Limbs 10:1:1-4, Spring 1966.
- Frantz, Charles H., and Ronan O'Rahilly, Congenital skeletal limb deficiencies, J. Bone Joint Surg. (Amer.) 43-A:8:1202-1224, Dec. 1961.
- Friedmann, Liesl, Special equipment and aids for the young bilateral upper-extremity amputee, Artif. Limbs 9:2:26-33, Autumn 1965.
- Friedmann, Lawrence W., Rehabilitation of amputees, in Rehabilitation and Medicine— 1968, ed. Sidney Licht, Waverly Press, Baltimore, 1968.
- Gazeley, William E., Mildred C. Ey, and William Sampson, Follow-up experiences with Muenster prostheses, Inter-Clinic Inform. Bull. 7:10:7-11, 1968.
- Gehant, Barbara A., Patient Census at Child Amputee Clinics—1968, Prosthetics and Orthotics, New York University Post-Graduate Medical School, Oct. 1969.
- Gesell, Arnold, and Catherine S. Amatruda, Developmental Diagnosis: Normal and Abnormal Child Development, 2nd ed. rev., Harper and Row, New York, 1947.
- Gillis, Leon, Thalidomide babies: management of limb defects, Brit. Med. J. 2:5305:647-651, Sept. 8, 1962.
- Gingras, G., and C. Corriveau, Modern amputations and prosthetics, Appl. Ther. 9:537, June 1967.
- Gingras, G., M. Mongeau, P. Moreault, M. Dupuis, B. Hebert, and C. Corriveau, Congenital anomalies of the limbs: part I, medical aspects, Canad. Med. Assoc. J. 91:2:67-73, July 11, 1964.
- ---------, Congenital anomalies of the limbs: part u, psychological and educational aspects, Canad. Med. Assoc. J. 91:3:115-119, July 18, 1964.
- Glessner, James R., Jr., Spontaneous intrauterine amputation, J. Bone Joint Surg. (Amer.) 45-A:2:351-355, Mar. 1963.
- Goldner, J. Leonard, Observations and findings concerning upper-extremity prosthesis wearers, Inter-Clinic Inform. Bull. 3:8:1-4, 1964.
- Gorton, Ann, Field study of the Muenster-type below-elbow prosthesis, Inter-Clinic Inform. Bull. 6:8:8-10, 1967.
- ---------, Dorrance model 2 hand field study, Inter-Clinic Inform. Bull. 6:8:11-13, 1967.
- Gouin-Decarie, Therese, The mental and emotional development of the thalidomide children and the psychological reactions of the mothers: a follow-up study, Inter-Clinic Inform. Bull. 7:4:1-6, 1968.
- Hall, Cameron B., Recent concepts in the treatment of the limb-deficient child, Artif. Limbs 10:1:36-51, Spring 1966.
- Hamilton, Robert C, A vocational evaluation of juvenile amputees who have attained the age of twenty-one years: a preliminary report, Inter-Clinic Inform. Bull. 3:7:8-9, 1964.
- Hebert, Bernard, the psychological implications of traumatic amputation in children, Inter-Clinic Inform. Bull. 7:4:7-10, 21, 1968.
- Hile, John, Below-elbow harness without axillary loop, Inter-Clinic Inform. Bull. 6:5:7-8, 1967.
- Hoover, Roy M., Problems and complications of amputees, Clin. Orthop. no. 37:47-52, Nov.-Dec. 1964.
- Janelle, Claire, The role of the social service worker in the rehabilitation of the juvenile amputee, Inter-Clinic Inform. Bull. 7:4:20-21, 1968.
- Jansen, Knud, Amputation: principles and methods, Bull. Pros. Res. 10-4:5-41, Fall 1965.
- Jaramillo, Selene, and Hans R. Lehneis, A therapeutic program for children with limb deformities—preservation of rudimentary appendices and prosthetic design, Inter-Clinic Inform. Bull. 9:4:1-7, 1970.
- Jentschura, G., B. Marquardt, and E. M. Ru-del, Inter-Clinic Inform. Bull. 4:9:11-14, 1965. (Reprinted from Behandlung und Vorsorgung bei Fehlbildungen und Amputationen der oberen Extremitdt, Georg Thieme Verlag, Stuttgart, 1963.)
- Kay, Hector W., and Sidney Fishman, 1018 Children with Skeletal Limb Deficiencies, Prosthetics and Orthotics, New York University Post-Graduate Medical School, Mar. 1967.
- Kay, Hector W., Kevin A. Cody, George Hart-mann, and Dominick E. Casella, The Münster-type below-elbow socket, a fabrication technique, Artif. Limbs 9:2:4-25, Autumn 1965.
- Kempner, Shirlee, Recent articles of interest, Inter-Clinic Inform. Bull. 5:2:19-20, 1965. (Abstract, Recent concepts in the treatment of the limb-deficient child, Cameron B. Hall, Manitoba Med. Rev. 44:552-557, 1964.
- Klopsteg, Paul E., Philip D. Wilson, et al., Human Limbs and Their Substitutes, McGraw-Hill, New York, 1954.
- Knapp, Miland E., Upper-extremity amputations: surgical considerations, Postgrad. Med. 45:2:237-240, Feb. 1969.
- Kruger, Leon M., and Nicholas R. Breyan, A study of radial-head dislocation in children with transverse partial hemimelia of the upper limb, Inter-Clinic Inform.Bull.10:1:1-4, 1970.
- Kuhn, Gotz Gerd, Treatment of the child with severe limb deficiencies, Inter-Clinic Inform. Bull 10:3-S:l-26, 1970.
- Kyllonen, Ronald R., Body image and reaction to amputations, Conn. Med. 28:19-23, Jan. 1964.
- Lambert, Claude N., The juvenile amputee, Illinois Med. J. 123:514-517, May 1963.
- Lambert, Claude N., Robert C. Hamilton, and Raymond J. Pellicore, The juvenile amputee program: its social and economic value: a follow-up study after the age of twenty-one, J. Bone Joint Surg. (Amer.) 51-A:6:1135-1138, Sept. 1969.
- Lineberger, Mildred I., Habilitation of child amputees, Journal of the American Physical Therapy Association 42:6:397-401, June 1962.
- McCollough, Newton C., Interpersonal problems of the handicapped child, Inter-Clinic Inform. Bull. 4:11:1-4, 16, 1965.
- McGraw, Myrtle B., Neuromuscular Maturation of the Human Infant, Columbia University Press, New York, 1943.
- McLaurin, Colin A., and Fred Sammons, Independent-control harnessing in upper-extremity prosthetics, Artif. Limbs 7:1:11-16, Spring 1963.
- MacNaughtan, A., The role of the occupational therapist in the training of the child arm amputee, Physiotherapy 52:201-203, June 1966.
- McWilliam, R., and S. R. Montgomery, Artificial arms—are they practical?, Med. Biol. Illus. 19:4:200-201, 1969.
- Martin, J. K., Congenital malformations associated with thalidomide and their management, Amer. Heart J. 67:284-285, Feb. 1964.
- Mayo, Eileen J., Upper extremity prostheses for children, Canad. Nurse 58:145-148, Feb. 1962.
- Mitchell, C. Leslie, Amputation and prosthesis: past research and future needs, Clin. Orthop. no. 37:110-112, Nov.-Dec. 1964.
- Mongeau, M., G. Gingras, E. D. Sherman, B. Hebert, J. Hutchison, and C. Corriveau, Medical and psychosocial aspects of the habilitation of thalidomide children, Canad. Med. Assoc. J. 95:390-395, Aug. 27, 1966.
- Montero, Jose C, Rehabilitation of the amputee, Mod. Treatm. 5:5:1047-1056, Sept. 1968.
- Munson, Nancy K., and Clyde M. E. Dolan, Patient Census at Child Amputee Clinics— 1967, Prosthetics and Orthotics, New York University Post-Graduate Medical School, May 1968.
- Murphy, Eugene F., The challenge of replacing human parts and functions, Bull. Pros. Res. 10-3:4-19, Spring 1965.
- Nichols, P. J. R, E. E. Rogers, M. S. Clark, and W. G. Stamp, The acceptance and rejection of prostheses by children with multiple congenital limb deformities, Artif. Limbs 12:1:13, Spring 1968.
- O'Shea, Barbara, A chest strap harness for the below-elbow child amputee, Inter-Clinic Inform. Bull. 6:7:1-4, 18, 1967.
- Peizer, Edward, Veterans Administration Prosthetics Center research, Bull. Pros. Res. 10-6:257-260, Fall 1966.
- ---------, Veterans Administration Prosthetics Center research, Bull. Pros. Res. 10-10:270, Fall 1968.
- Pellicore, Raymond J., Experiences with the Hepp-Kuhn below-elbow prosthesis: a preliminary report, Inter-Clinic Inform. Bull. 3:11: 1-8, 1964.
- Richardson, Geraldine, and Aida Lund, Upper extremity prosthetic training for the young amputee, Amer. J. Occup. Ther. 13:2:57-63, Mar.-Apr. 1959.
- Ritter, Diane, and Fred Sammons, An interesting terminal device modification, Inter-Clinic Inform. Bull. 4:9:7-10,19, 1965.
- Santschi, William R., (Ed.), Manual of Upper Extremity Prosthetics, 2nd ed. rev., University of California, Los Angeles, 1958.
- Shaperman, Julie Werner, Orientation to prosthesis use for the child amputee, Amer. J. Occup. Ther. 14:1:17-23,26, 1960.
- ---------, Learning techniques applied to prehension, Amer. J. Occup. Ther. 14:70-74, Mar.-Apr. 1960.
- ---------, A comparison of two infant terminal devices, Inter-Clinic Inform. Bull. 3:7:1-6, 1964.
- Simmel, Marianne L., The absence of phantoms for congenitally missing limbs, Amer. J. Psychol. 74:467-470, Sept. 1961.
- Sokolow, Jack, Management of the amputee in practice, Med. Clin. N. Amer. 53:3:659-664, May 1969.
- Spring, John M., and Charles H. Epps, Jr., The juvenile amputee: some observations and considerations, Clin. Pediat. 7:76-79, Feb. 1968.
- Stamp, Warren G., Sharon Mahon, and Harry C. Morgan, Problems of management of the child with multiple amputations, Arch. Phys. Med. Rehabil. 46:354-368, May 1965.
- Stanek, William F., Orthopedic service at children's hospital: the amputee center, Rocky Mountain Med. J. 63:54, Oct. 1966.
- Staros, Anthony, and Edward Peizer, Veterans Administration Prosthetics Center research report, Bull. Pros. Res. 10-12:331-333, Fall 1969.
- Steele, Shirley, Children with amputations, Nurs. Forum 7:411-423, 1968.
- Stoner, Emery K., Functional evaluation of the upper extremity, in Handbook of Physical Medicine and Rehabilitation, ed. Frank H. Krusen, Frederick J. Kottke, and Paul M. Ellwood, Jr., W. B. Saunders, Philadelphia, 1965.
- Street, Dana M., and Frank Cunningham, Congenital anomalies caused by intra.-uterine bands, Clin. Orthop. no. 37:82-97, Nov.-Dec. 1964.
- Swanson, Alfred B., Phocomelia and congenital limb malformations: reconstruction and prosthetic replacement, Amer. J. Surg. 109:294-299, Mar. 1965.
- Swinyard, Chester A., Kay Perfect, George G. Deaver, and Leon Greenspan, Counseling parents of children with congenital deformities of the limbs, Inter-Clinic Inform. Bull. 3:6:1-4, 1964.
- Taylor, Craig L., The biomechanics of control in upper-extremity prostheses, in Selected Articles from Artificial Limbs, Robert E. Krieger Publishing Co., Huntington, N.Y., 1970.
- Taylor, Isabelle Wagner, Psychological needs of the handicapped child, Inter-Clinic Inform. Bull. 9:8:9-17, 1970.
- Teska, Ann, and Chester A. Swinyard, Evaluation of a standardized test for child's APRL-Sierra no. 1 hand, Amer. J. Occup. Ther. 15: 17-18, Jan.-Feb. 1961.
- Trefler, Elaine, Terminal device activation for infant amputees, Inter-Clinic Inform. Bull. 9:9: 11,14, 1970.
- VanDerwerker, Earl E., Jr., and Josef Rosen-berger, A simple flexor assist for below-elbow prostheses, Inter-Clinic Inform. Bull. 3:1:1-3, 1963.
- Veterans Administration Prosthetics Center, Semiannual report, Bull. Pros. Res. 10-3:135-136, Spring 1965.
- ---------, Semiannual report, Bull. Pros. Res. 10-4:157-159, Fall 1965.
- Vitali, Miroslaw, Management of congenital deformities, including thalidomide children, in Great Britain, Inter-Clinic Inform. Bull. 2:7:7-12, 1963.
- Wallace, Maxine T., Group therapy for parents of congenital amputees, Inter-Clinic Inform. Bull. 5:2:10-14, 1965.
- Watkins, Arthur L., and Dorothy E. Ford, Rehabilitation after amputation of an upper extremity: a ten year study, Arch. Phys. Med. Rehabil. 43:293-296, June 1962.
- Weinstein, Sidney, and Eugene A. Sersen, Phantoms in cases of congenital absence of limbs, Neurology 11:905-911, Oct. 1961.
- Wendt, Jeannine D., and Julie Shaperman, A study of development of prehension patterns: the infant with a cable-controlled hook, Amer. J. Occup. Ther. 24:393-402, Sept. 1970.
- Wilson, A. Bennett, Jr., Limb prosthetics— 1967, Artif. Limbs 11:1:1-46, Spring 1965.
- ---------, Limb prosthetics —1970, Artif. Limbs 14:1:1-52, Spring 1970.
- ---------, The prosthetics and orthotics program, Artif. Limbs 14:2:1-18, Autumn 1970.
- For an Additional Bibliography, please refer to the PDF at the top of this page.
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