You are on page 1of 118

Introduction to the Child Amputee

John R. Fisk, M.D.

This Atlas is about prosthetics, but the needs of the limb-deficient child are not merely those of replacement
parts. Even in the discussion of surgical principles, there is a different set of rules to follow when working
with children. The disabled child missing all or part of one or more limbs is a growing, developing, and
learning individual. Equally important, the child has parents and siblings who are growing and learning
along with them. Thus, when we work with the limb-deficient child, we have many different considerations
beyond those involved with treating adults. The chapters that follow address many of these unique
concerns.

The child is not just a small adult. In addition to the obvious differences brought about by growth, children
react differently to disabilities than do adults. There are two major categories of pediatric patients with limb
deficiencies. Those who were born with them, i.e., congenital deficiencies, and those who acquired them
after birth, i.e., amputations. In the first instance, the child has no sense of loss and nothing new to get
adjusted to. Anything of a prosthetic nature is an aid, not a replacement, and if it is not truly an aid, the child
will reject it. Those who lose a limb due to trauma or disease, unless their amputation occurred when they
were very young, will have a profound sense of loss and undergo a period of readjustment. How well they
manage this change greatly affects their acceptance of replacement prosthetic limbs.

The motivations of the two groups are different. In the first instance, congenitally limb-deficient children will
try to do whatever other kids do. We find that their only limitations are those placed upon them by adults.
Left to their own devices, they are very adaptable. Acquired amputees, on the other hand, want to be as
they were prior to the amputation. They will mourn the loss of their limb; they will be angry and resentful.
Their motivation will be greatly influenced by their ability to resolve this inner turmoil.

The two groups of children have parents and relatives involved in their care. Whether of congenital or
acquired etiology, their child's limb deficiency is a great source of guilt for them. They will proceed through
the universal grief response. We are greatly indebted to Kubler-Ross for her work in helping to clarify the
stages of the grief process. Initially there will be shock, and then denial and anger. There will be a great
sense of disappointment because of lost dreams and expectations not able to be met by their disabled
child. With resolution and acceptance, new, more realistic expectations will be set so that in the end, if all
goes well, the child will receive the support and nurturing that are doubly necessary to assist growth with
the disability. Health care professionals involved with a family working through these stages must learn to
recognize the changes as they occur and be prepared to alter their approach accordingly.

Rev. Harold Wilke, born completely without arms and well known for his work with the disabled, once said
that the most important action that his parents took while he was growing up was to decide to have another
child. That gesture showed him that they loved him for who he was sufficiently to risk having another child.
There are many of our patients' families who never reach this point of adjustment; they never completely
resolve their grief. The pediatric limb deficiency clinic team therefore not only has the patient to be
concerned with, but also the parents and relatives.

As these children grow, they proceed through the same stages of development as anyone else. In infancy,
the accomplishment of normal motor milestones should be facilitated. A passive arm may offer balance or a
prop for sitting and crawling. A prosthetic leg becomes necessary when it is time to pull to stand. Develop-
mentally oriented physical and occupational therapists are an invaluable part of the clinic team.

There is little sound evidence to explain why some children adapt to a prosthesis quite readily and others
reject anything that is placed on them. There is, however, a difference in acceptance of upper vs. lower
prosthetic limbs. Legs are required for mobility, and as soon as the child appreciates this, the prosthesis
rarely comes off. Upper-limb prostheses, on the other hand, have varying degrees of acceptance.

The upper-limb prosthesis is not able to replace a missing part to the same degree that a prosthetic leg can.
At best it is a tool, and if it doesn't enhance function, it will be rejected.

There have been several studies of rejection of upper-limb prostheses by children. Few sound
conclusions have been reached. Clearly, cosmesis and function are two major concerns. A third factor,
acceptance by the parents of the child's disability, may be more important.
The fact that a cosmetic hand is easier for a parent to accept may have a far greater impact on whether or
not it gets worn than what the child thinks of it. Also, prosthetic wearing practices by the child are used as a
behavior to influence their relationship with their parents.

When pediatric amputees reach adolescence, they undergo the same intellectual and emotional changes
that other adolescents face. The limb deficiency makes this adjustment that much more difficult. Frequently
a limb is rejected as the child is confronted with a new group of peers. They don't want to be "different.'' As,
it is hoped, acceptance into the group occurs, the limb goes back on to aid with function. This concern can
pertain to lower-limb prostheses but applies much more so to upper-limb prostheses.

Lower-limb prostheses may be worn because they are needed for ambulation, but the wearer may go to
extremes to hide them. They may avoid swimming and clothing that fails to mask the limb loss such as
shorts or dresses. These actions reflect the acceptance by the child of his own body image to a greater or
lesser degree. Upper-limb prostheses tend to be rejected outright when there is frustration with self.

In considering all of these growth and adjustment issues, there is little question that the child is not just a
small adult. The clinic team is necessary to bring together professionals with expertise in addressing these
many adjustment issues.

In 1954, the need for an organized approach to the management of juvenile amputees across the country
was discussed at a meeting in Grand Rapids, Michigan. Subsequently, Gerald F.S. Strong, Chairman of the
Prosthetics Research Board, appointed an interim committee of ten members to pursue the issue. Dr.
Charles H. Frantz chaired the first meeting at UCLA in 1956. The group officially became the subcommittee
on Child Prosthetic Programs within the National Academy of Sciences Prosthetics Research and
Development Committee in 1959. The goal of the subcommittee was to raise the standards of prosthetic
care for children in the United States. Prior to this time prosthetic components were oftentimes not available
in pediatric sizes. Prescriptions were withheld until the child started school and was therefore deemed to
need a limb.

To begin the dissemination of information and the establishment of clinic criteria, four major symposia were
sponsored by the subcommittee to reflect the state-of-clinic expertise during the 1960s. By 1970, the
subcommittee was charged to enlarge its sphere of activity to include children's orthoses and mobility aids.
Under the guidance of Hector Kay, Assistant Executive Director of the Committee for Prosthetic Research
and Development, the annual conferences were expanded to include cooperating clinic chiefs and their
team members. The Association of Children's Prosthetic/Orthotic Clinics has held an annual interdisciplinary
conference since 1972. It is now the primary forum for the exchange of information on the limb-deficient
child in North America. Members include not only individuals but also the clinic teams as a unit. This
recognizes the importance of the team approach. As stated earlier, the limb-deficient child has multiple
needs that can only be addressed by the team.

Children have many unique prosthetic needs. As their minds and bodies are growing, so are their residual
limbs. Frequent socket revisions or replacements are necessary to accommodate this growth. Lambert has
reported that children followed at the University of Illinois required a new lower-limb prosthesis annually up
to the age of 5 years, biannually from 5 to 12 years, and then one every 3 or 4 years until 21 years of age.
The need for frequent and regular checkups by the clinic team is obvious.

The durability of the young healthy tissue on the residual limb of a child is quite different from that of the
dysvascular adult amputee. Consequently, alterations in fit are much better tolerated. Nevertheless, the
frequent changes necessary present an economic concern. To lengthen the useful life of a prosthesis,
materials that are easily modified and lengthened should be used. In addition, it should be noted that
durability of children's prostheses is more important than cosmesis. In that regard, soft covers, while
cosmetically desirable, are easily destroyed by the abuse children give them. Above all, prostheses must
facilitate function. Those that the child must be careful of should be avoided.

Healing in a child is much different than in the adult. Skin is much more elastic and will better tolerate
stretching to cover the end of the residual limb. Skin grafts will frequently mature sufficiently to tolerate
direct weight bearing as well as the shear forces experienced with socket wearing.

The skeletally immature child relies on growth of the residual limb to maintain sufficient length for good
biomechanical strength later on. This is generally not a concern with the congenital amputee. As a rule, the
relative length discrepancy experienced in a congenital limb deficiency is maintained. One must, however,
not leave this to chance. When planning the proper time for surgical intervention on a proximal femoral focal
deficiency (PFFD), for instance, proper use of serial scan-o-grams is necessary. With the advent of newer
techniques of limb lengthening and deformity corrections, proper documentation of growth potential is
increasingly important.

Residual-limb length is of vital concern for the acquired amputee. The concept of preserving as much length
as possible should be considered, especially by the use of disarticulation rather than transosseous ablation.
For example, since 70% of the growth of the femur comes from the distal femoral physis, a long trans-
femoral amputation in a 2-year-old becomes very short by the time that child becomes an adult. A knee
disarticulation will avoid this problem. If a knee disarticulation is done too close to the time of physeal
closure, on the other hand, the relative retardation of physeal growth on that side may not be sufficient to
avoid an overly long, uncosmetic thigh. The solution to this is a distal femoral epiphysiodesis. This provides
end-bearing ambulation with shorter length that will allow trans-femoral knee components, with resultant
good sitting and standing cosmesis.

There is another reason to perform joint disarticulations rather than diaphyseal transections in children
whenever possible. This is because the major complication of amputation surgery in children is bony
overgrowth. It does not occur with disarticulations but frequently follows metaphysealor diaphyseal-level
amputations. The incidence of this complication is variously reported in the range of 10% to 30%.
Histologically, this is appositional bone growth of the remaining diaphysis. It is clearly not growth from the
remaining proximal physis. Various techniques of handling the bone and periosteum during amputation
have failed to decrease the incidence of this complication. Silastic caps or plugs have been tried, but the
results are disappointing. Marquardt in Germany suggests transplanting a cartilaginous apophysis from
the ilium or preserving an epiphysis from the amputated portion of the limb. Usually there is bursal
formation over the end of the bone that can become exquisitely tender. Occasionally, skin breakdown
occurs, and the bone may penetrate. Socket modification can delay revision, but once the residual limb
becomes pencil shaped, revision is necessary. This is often required more than once until skeletal growth
ceases. The residual limb does not need to be appreciably shortened overall since the appositional growth
effectively adds length to the bone.

There is one area where the option of preserving length at all costs must be carefully exercised. This is the
post-traumatic partial-foot amputation. For example, forefoot amputation due to lawn mower injuries
frequently leaves an infected residuum with plantar scarring. While it is advantageous to be able to walk
barefoot without a prosthesis, making a functional partial-foot prosthesis, especially for a less-than-optimal
partial-foot amputation, is technically challenging. Concerns related to cosmesis, comfort, and function are
very difficult to satisfy. Frequently a Syme ankle disarticulation fitted with a prosthesis is the best option. It is
very cosmetically pleasing in a child, where malleolar size is not a problem. Nevertheless, after investing a
lot of time and emotional effort in preserving length at all costs, the family is often unwilling to consider a
revision as an alternative to a very clumsy partial-foot prosthesis. This problem should therefore be carefully
considered in the initial treatment of each partial-foot amputation.

In the past, age appropriateness for prosthetic prescription was related to the purported ideal age at which a
child could use an upperor lower-limb prosthesis. Now it pertains to appropriate ages for specific terminal
devices and feet. There has also been an effort to develop criteria for the prescription of costly myoelectric
limbs for very young children. Some clinics are claiming functional capabilities occurring earlier than we
know them to happen in sound limbs. What is needed are controlled studies to evaluate the functional
appropriateness of prescription ages. A recent collaborative study by a member of Shriners Hospital Clinics
has demonstrated equal acceptability by the very young of body-powered or myoelectric-powered cosmetic
hands.

Lower-limb components are being proposed for the pediatric population based on successes with adults. It
is often asked whether dynamic-response feet for children should be prescribed. Due to the small body
mass of the child and the frequent need for new limbs due to growth, their efficacy needs to be
demonstrated.

In the chapters that follow, a number of topics unique to the limb-deficient child will be addressed, including
acquired amputations (Chapter 32). The epidemiology of injuries causing traumatic amputations must be
studied to learn how to provide a safer environment for our young. Lawn mowers, farm instruments, and
recreational vehicles are all hazardous to the inexperienced.

The International Standards Organization (ISO) has recently adopted a definitive classification system for
congenital limb deficiencies (Chapter 33). No longer is it necessary to learn a series of ancient language
roots to describe our patients. This new system, utilizing just four words-longitudinal, transverse, partial, and
total-has been accepted by the International Society for Prosthetics and Orthotics and the Association of
Children's Prosthetic/Orthotic Clinics. It will allow for a more concise data base and communication of
statistics on an international basis.

Other areas of interest are the surgical and pros-thetic/orthotic management of upper-limb deficiencies
(Chapter 34A and Chapter 34B). Reluctance has been expressed in the past for doing revision surgery on
upper-limb amputees. It is time for this issue to be reconsidered. Prosthetic prescription, i.e., body vs.
myoelectric power and tool vs. cosmetic hand terminal devices, should be addressed. There is good
evidence that very young children are able to use myoelectric limbs. Is it necessary, advantageous, and
cost-effective? More studies are needed to answer all of these questions. The developmental approach to
upper-limb prosthetic training is discussed in detail (see Chapter 34D). As children grow and develop,
especially in their early years, their motor skill capabilities change rapidly. Prosthetic components and
training must change along with them.

The two primary considerations to be addressed in the area of lower-limb deficiencies are the unique nature
of some of the reconstructive surgical procedures for congenital deformities and the fact that prosthetic
design must take into consideration the factors of growth and durability (Chapter 34A and Chapter 34B).

To conclude the discussion of the child amputee with a chapter entitled Special Considerations is most
appropriate (Chapter 36). The needs of the limb-deficient child are indeed special as this introductory
chapter has sought to point out. The sections on the multiple amputee, tumor salvage procedures, and
recreational concerns address the new frontiers being developed in the field of juvenile amputee
management.

Acquired Amputations in Children


Robert E. Tooms, M.D.

Annual surveys of specialized child amputee clinics in the United States have repeatedly shown that
approximately 60% of childhood amputations are congenital in origin and 40% are acquired. In contrast,
a survey of prosthetic facilities has revealed that significantly more children with acquired amputations
receive prosthetic services than do those with congenital limb deficiencies. This discrepancy suggests
that children with the more complex congenital limb deficiencies are referred to specialized child amputee
clinics, whereas most acquired childhood amputations are managed in less specialized settings. In either
case, the number of children with acquired amputations is relatively small but represents a significant
segment of the pediatric population with major orthopaedic problems.

Acquired amputations are secondary to either trauma or disease, with trauma causing roughly twice as
many limb losses as disease. Although there are obviously many traumatic incidents that may result in
childhood amputations, power tools and machinery are the worst offenders, followed closely by vehicular
accidents, gunshot wounds and explosions, and railroad accidents. In the older child, vehicular accidents,
gunshot wounds, and power tool injuries are the most frequent causes of limb loss. In the 1- to 4-year-old
group, power tools such as lawn mowers and household accidents account for most amputations (Fig 32-
1.).
Fig 32-1. Etiology of acquired amputations due to trauma. (From Juvenile
Amputee Course Manual. Evanston, III, Northwestern University Medical School
Prosthetic-Orthotic Program, 1983. Used by permission.)

Of the disease processes necessitating amputation in children, malignant tumors are responsible for more
than half, the highest incidence occurring in the 12- to 21-year-old group. Vascular malformations,
neurogenic disorders, and a wide variety of miscellaneous disorders are responsible for the remainder of
amputations due to disease (Fig 32-2.).
Fig 32-2. Etiology of acquired amputations due to
disease. (From Juvenile Amputee Course Manual.
Evanston, III, Northwestern University Medical School
Prosthetic-Orthotic Program, 1983. Used by
permission.)

In over 90% of acquired amputations a single limb is involved, and it is a lower limb that is involved in 60%
of the cases. Males outnumber females in incidence of acquired limb loss in a ratio of 3:2, most probably
because males tend to engage in more hazardous activities.

SURGICAL PRINCIPLES

The well-established surgical principles for amputation surgery in the adult are just as applicable to
amputations performed in children. The cardinal dictum in children is to conserve all limb length possible,
consistent with appropriate treatment for the condition that requires the amputation. Trauma is the
proximate cause of most acquired amputations in children. In attempting to conserve length in the severely
traumatized limb, adequate tissue vascularity of the growing child may allow the surgeon to use surgical
techniques that are not successful in the adult. Skin grafts, firm traction, and wound closure under tension
may be judiciously used in the child to conserve limb length without compromising wound healing or
subsequent prosthetic use. Split-thickness skin grafts, even over large areas of the stump, may tolerate
prosthetic use quite well in the child. The increased elasticity of the child's skin coupled with an excellent
blood supply allows the surgeon to apply somewhat heavier skin traction to open amputations in the child
than would be safely tolerated in the adult. For the same reasons, open wounds may successfully be closed
under slightly more tension in the child than would be permissible in similar adult patients. In each instance,
however, good surgical judgment must be used since even the tissue tolerance of the child has its
limitations.

A second surgical dictum is, whenever possible, to perform a disarticulation rather than a transdiaphyseal
amputation in a growing child. Disarticulation preserves the epiphyseal growth plate and thereby ensures
longitudinal growth of the bone. Loss of stump length due to epiphyseal loss is most readily apparent in
trans-femoral amputations in young children. In amputations at this level, the distal femoral epiphysis, which
accounts for approximately 70% of the longitudinal growth of the femur, is sacrificed. When a midthigh
amputation is performed in a young child, the resultant stump present at 16 years of age will be quite short
and will be a considerably less than optimal skeletal lever for prosthetic use (Fig 32-3.). Disarticulation also
precludes the development of terminal or appositional overgrowth of new bone at the transected end of a
long bone-the most common complication of amputation surgery in the growing child. The prominent
condyles or malleoli resulting from disarticulation usually undergo atrophy with further growth of the child,
thereby eliminating the cosmetic objection to this type of surgery when it is performed in the adult.

Fig 32-3. A, a radiograph of the left lower limb illustrates tibial deficiency with severe soft-tissue
webbing in the popliteal area. B, this limb deficiency was treated by amputation through the distal
third of the femur. C, at skeletal maturity there is significant shortening of the femur on the
amputated side due to a loss of the distal femoral epiphysis, with a resultant very short amputation
stump. (From Tooms RE: The amputee, in Lovell WW, Winter RB (eds): Pediatric Orthopedics, vol 2.
Philadelphia, JB Lippincott, 1978, pp 999-1054. Used by permission.)

COMPLICATIONS

Terminal overgrowth is the most common complication of amputation surgery in the skeletally immature
individual. This is an appositional overgrowth of new bone at the transected end of a long bone. It is in no
way related to epiphyseal plate growth, and previous attempts to prevent this problem by epiphysiodesis
have not been successful. Terminal overgrowth occurs most often in the humerus, fibula, tibia, and femur,
in that order. In this condition, the appositional growth of new bone may exceed the growth of the
overlying soft tissues to such an extent that the bone end actually penetrates the skin (Fig 32-4.). Many
surgical techniques have been devised to prevent terminal overgrowth from developing. These include
using intramedullary implants of silicone rubber or porous polyethylene to cap the resected bone end
and prevent terminal overgrowth. This approach may eventually prove to be effective. However, the best
treatment method remains stump revision with appropriate resection of the bony overgrowth. This has
been necessary in 8% to 12% of several reported large series of acquired amputations in children. Once
surgery becomes necessary to correct the problem, recurrences are common and may necessitate
repeated stump revision at 2- to 3-year intervals until skeletal maturity.
Fig 32-4. Terminal overgrowth of the humerus in post-
traumatic trans-humeral amputation. Bony overgrowth
has actually penetrated soft tissues at end of
amputation stump. (From Tooms RE: The amputee, in
Lovell WW, and Winter RB (eds): Pediatric Orthopedics,
vol 2. Philadelphia, JB Lippin-cott, 1978, pp 999-1054.
Used by permission.)

Adventitious bursae frequently develop in the soft tissues overlying an area of terminal overgrowth.
Conservative treatment of such symptomatic bursae by aspiration, corticosteroid injection, and stump
wrapping is seldom more than temporarily effective. Bursae that form over bony prominences subjected to
recurrent pressure from a prosthetic socket are effectively managed by appropriate socket modifications.
Permanent relief from those symptomatic bursae overlying an area of terminal overgrowth usually requires
surgical excision of the bursa combined with appropriate resection of the underlying bone (Fig 32-5.).
Fig 32-5. A large bursa developed over the end of the humerus in an acquired
transhumeral amputation. Surgical excision of the bursa as well as underlying
bony overgrowth is necessary to eradicate the problem. (From Tooms RE: The
amputee, in Lovell WW, Winter RB (eds): Pediatric Orthopedics, vol 2.
Philadelphia, JB Lippincott, 1978, pp 999-1054. Used by permission.)

Bone spurs often form at the periphery of transected bone ends as a response to periosteal stimulation at
the time of surgery. Such bone spurs rarely necessitate stump revision and should be easily distinguished
from terminal overgrowth.

Extensive stump scarring from trauma, previous surgery, or skin grafting is usually well tolerated by the child
amputee. Stump revision is seldom necessitated by scarring alone, but may require prosthetic modification
to disperse weight-bearing forces and diminish shear stress at the stump-socket interface. Minor
modifications in the prosthetic socket will usually relieve symptomatic pressure that is concentrated over
small areas of scarring in relatively non-weight-bearing areas of the amputation stump. Wearing a nylon
sheath next to the skin and beneath the stump sock or wearing multiple stump socks may prevent tissue
breakdown from stump-socket interface friction over small areas of scarring. More extensive prosthetic
modifications may be necessary when the scarred area is larger or is over weight-bearing areas of the
stumps. Check sockets made of transparent polycarbonate plastic and stump socks of pressure-sensitive
fabric allow precise identification of pressure-producing areas in the socket at both the transtibial and the
transfemoral amputation levels. For the severely scarred transtibial stump, the most commonly used
method of relieving excessive pressure and shear forces is attaching outside knee joints and a weight-
bearing thigh corset to a total-contact socket. Other successful techniques include the use of an air cushion
socket or a Silastic gel socket insert with a rubber sleeve for suspension. I have been most successful
in eliminating skin breakdown in the extensively scarred transtibial stump by using a meticulously fitted
hard-socket patellar tendon-bearing (PTB) prosthesis worn over two or three five-ply stump socks.
Suspension is by means of a supracondylar strap or, if necessary, with outside knee joints and a thigh
corset.

When upper-limb amputations are complicated by extensive trunk scarring, harnessing techniques
alternative to the figure-of-8 harness may be necessary. The shoulder saddle with chest strap is an
excellent solution in such cases (Fig 32-6.).
Fig 32-6. A shoulder saddle with a chest strap harness has been used to disperse pressure and
shear forces over extensively scarred soft tissues of this child with a transradial amputation due to
severe thermal burns. (From Tooms RE: The amputee, in Lovell WW, Winter RB (eds): Pediatric
Orthopedics, vol 2. Philadelphia, JB Lippincott, 1978, pp 999-1054. Used by permission.)

Neuroma formation in amputation stumps of children is seldom symptomatic enough to warrant surgical
treatment. In reviewing a large series of acquired childhood amputations, Aitken found that only 4%
required surgical treatment for neuromas, most being satisfactorily managed by socket adjustment.

The phantom limb phenomenon always occurs in children following acquired amputations. If the amputation
is performed on a child under the age of 10 years, the phantom sensation is rapidly lost. Painful phantom
limb sensation does not occur in growing children, but has been reported in the teenager.

SURGICAL TECHNIQUES

Except for the previously discussed surgical principles of limb length conservation and the need for
performing disarticulations in preference to transdiaphy-seal amputations, surgical techniques in the child
do not differ significantly from those used for the adult. Therefore specific surgical procedures will not be
outlined for any of the major levels of amputation in the upper or lower limb. However, several specialized
surgical procedures do deserve mention.

The Krukenberg, or "lobster-claw," operation, may well deserve consideration in the child with a long
transradial (below-elbow) amputation. The Krukenberg procedure provides a crude pinching mechanism
with preserved sensation by splitting a long transradial stump into radial and ulnar rays that are widely
separated and covered with skin possessing normal sensation. The forearm muscles that attach to the two
rays provide voluntary opening and closing of these rays. Children who have the operation performed early
in life learn to use the pincer and are not often emotionally disturbed by the unsightly appearance of the
stump. The procedure has its greatest application in bilateral upper-limb amputees, especially in the blind.

Syme ankle disarticulation is a frequently indicated level of amputation in the growing child. It should be
stressed that this procedure should be performed as a true disarticulation with a Syme-type soft-tissue
closure and not as a supramalleolar amputation as is done in the conventional ankle disarticulation in the
adult.

Lawn mower injuries and severe burns that result in partial limb loss occur in sufficiently large numbers to
justify specific comments on the surgical management of these problems. As in other traumatic incidents,
preservation of limb length is of major concern in both of these injuries. The physiologic tolerance of
growing children fortunately allows the surgeon to preserve limb length by using skin grafts, traction, and
soft-tissue-shifting plastic surgery procedures.

Most lawn mower injuries sustained by children result in partial-foot amputations. Some of these injuries
involve only the digits or the distal metatarsal area and present no great treatment problems. Others are
quite extensive and involve most of the foot with multiple deep and extensively contaminated wounds. In
these latter instances, proper surgical judgment is necessary to determine which injury can reasonably be
expected to provide a serviceable partial-foot amputation by using skin grafts and soft-tissue-shifting plastic
surgery procedures and which injury would require revision to a higher level. This decision is seldom
obvious, and when doubt exists, it is better to err on the side of conservatism. The initial debridement of
such injuries should be limited to excision of only that tissue that is absolutely nonviable, with any tissue of
questionable viability being preserved. Initial bone resection should be minimized until sufficient time has
elapsed to be certain how much viable soft tissue will ultimately be available for wound closure. Following
thorough irrigation and debridement, the wound is lightly packed open.

Five to 7 days later the wound is examined with the patient under anesthesia. After further debridement of
any nonviable tissue, a decision may be made to revise the amputation to a higher level or to continue with
a more conservative approach. For example, an amputation at the midtarsal joint level that requires
extensive skin grafting over the plantar surface of the heel and results in a loss of the foot dorsiflexors will
be less functional and require more subsequent treatment than will revision to a higher level. If revision to a
higher level (Syme ankle disarticulation or transtibial amputation) is indicated at the time of the first wound
dressing, an open amputation is preferable, followed by skin traction until secondary closure is performed 5
to 7 days later. If continued conservatism seems appropriate, it may be possible to partially close the wound
at this time with minimal additional bone resection. If extensive skin grafting or tissue-shifting plastic surgery
procedures such as a Z-plasty are needed, these are more safely done at the time of a second wound
dressing 5 to 7 days later, when granulation tissue begins to cover open areas and the danger of infection is
less.

Thermal or electrical burns may cause such widespread tissue destruction that amputation of a major
portion of the limb may become necessary. In such circumstances, conservative treatment should be
pursued until there is adequate demarcation of nonviable tissue to allow open amputation at the lowest
possible level. During this time, appropriate splinting of proximal joints is essential to minimize the
development of joint contractures in nonfunctional positions. Despite splinting, late soft-tissue releases may
be needed to improve joint motion. Extensive skin grafting is usually necessary in children with severe
burns, and the resultant scarred stumps may present very difficult problems in prosthetic fitting. The use of
pressure dressings over scarred and grafted areas helps to decrease scar hypertrophy. When healing has
occurred, gentle massage is often beneficial in mobilizing scar tissue that is adherent to bone. Successful
prosthetic use usually necessitates modification of prosthetic sockets and suspension systems as noted
previously.

The ISO/ISPO Classification of Congenital Limb Deficiency


H.J.B. Day, M.R.C.S. (Eng.), L.R.C.P. (Lon.)

In order to facilitate scientific communication about congenital limb deficiency, a logical, accurate system of
classification and nomenclature is needed, and this must be capable of translation into all languages. The
lack of a suitable system has allowed the use of the term "congenital amputation"-implying that a limb
segment has been lost before birth-to be used for cases that are patently failures of formation. The use of
terms derived from Greek or Latin roots may sound impressively scientific but are both inaccurate and
ambiguous and are often misused, none more frequently than "phocomelia," which has been used to
describe every level and type of deficiency. The history of classifications devised since 1961, including the
work of the International Society for Prosthetics and Orthotics (ISPO) "Kay" committee, has been described
previously by Kay, Swanson, and Day. Some further minor modifications, made more recently, have
enabled the ISPO classification to be drafted and accepted by the participating nations as an international
standard.
8548-1:1989 "METHOD OF DESCRIBING LIMB DEFICIENCIES PRESENT AT BIRTH"

The standard is reproduced here with the permission of the International Organization for Standardization
(ISO). Copies of this standard are available from the ISO Central Secretariat, Case Postale 56, CH-1211
Geneva 20, or from any ISO member body.

It has three constraints:

1. The classification is restricted to skeletal deficiencies, and therefore the majority of such cases are
due to a failure of formation of parts.
2. The deficiencies are described on anatomic and radiologic bases only. No attempt is made to
classify in terms of embryology, etiology, or epidemiology.

3. Classically derived terms such as hemimelia, peromelia, etc., are avoided because of their lack of
precision and the difficulty of translation into languages that are not related to Greek.

Deficiencies are described as transverse and longitudinal. The former resemble an amputation residual limb
in which the limb has developed normally to a particular level beyond which no skeletal elements are
present. All other cases are classed as longitudinal: a reduction or absence of an element or elements
within the long axis of the limb.

METHOD OF DESCRIPTION

Transverse

The limb has developed normally to a particular level beyond which no skeletal elements exist, although
there may be digital buds. Such deficiencies are described by naming the segment at which the limb
terminates and then describing the level within the segment beyond which no skeletal elements exist (Fig
33-1.). It is possible to use another descriptor in the phalangeal case to indicate a precise level of loss
within the fingers.

Fig 33-1. Designation of levels of transverse deficiencies of upper and lower limbs. Note that the
skeletal elements marked with an asterisk are used as adjectives in describing transverse
deficiencies, e.g., transverse carpal total deficiency. A total absence of the shoulder or hemipelvis
(and all distal elements) is a transverse deficiency. If only a portion of the shoulder or hemipel-vis is
absent, the deficiency is of the longitudinal type.
Longitudinal

There is a reduction or absence of an element or elements within the long axis of the limb, and in this case
there may be normal skeletal elements distal to the affected bone or bones. The following procedure should
be followed to describe such a deficiency (see Fig 33-2. and Fig 33-3.):

Fig 33-2. Description of longitudinal deficiencies of the upper limb. The asterisk indicates that the
digits of the hand are sometimes referred to by name: 1 = thumb; 2 = index; 3 = middle; 4 = ring; 5 =
little (or small). For the purpose of this classification such naming is deprecated because it is not
equally applicable to the foot.
Fig 33-3. Description of longitudinal deficiencies of the lower limb. The asterisk indicates the great
toe, or hallux.

1. Name the bones affected in a proximodistal sequence by using the name as a noun. Any bone not
named is present and of normal form.
2. State whether each affected bone is totally or partially absent.

3. In the case of partial deficiencies the approximate fraction and the position of the absent part may
be stated.

4. The number of the digit should be stated in relation to a metacarpal, metatarsal, and phalanges,
the numbering starting from the preaxial, radial, or tibial side.

5. The term "ray" may be used to refer to a metacarpal or metatarsal and its corresponding
phalanges.

Examples of transverse and longitudinal deficiencies are shown in Fig 33-4. and Fig 33-5., but it must be
understood that the stylized representation of the limb that is used in these figures is neither part of the
original ISPO "Kay" committee work nor of the new international standard, but the author has found it to be
the most useful way of illustrating deficiencies in clinical notes, and it can be used to indicate some
treatment as well as the deficiency.
Fig 33-4. Examples of transverse deficiencies at various levels are shown on
the skeleton and as the author's stylized version.
Fig 33-5. An example of longitudinal deficiency is shown on
the skeleton and as the author's stylized representation, which
shows not only the original deficiency but also the treatment
by knee disarticulation.

Upper-Limb Deficiencies: Surgical Management


Terry R. Light, M.D.

The birth of a child with an upper-limb deficiency leads to a myriad of confusing parental emotions. Parental
concerns and expectations must be dealt with in an honest and forthright fashion by both the physician and
the prosthetist caring for the child. Most parents have feelings of guilt and grieve that their infant is not as
perfect as they had anticipated through the course of the pregnancy. Many parents feel an intense need to
"do something," either surgical or prosthetic, to make their child "normal" and whole. Conflicting advice from
well-meaning friends and relatives may create further parental tension and confusion.

Initially, many parents seek a cosmetic prosthesis that will conceal their child's abnormality without regard to
its functional impact. The predictable lack of success when a purely aesthetic prosthesis covers sensate
skin in the hand should be openly discussed. As parents observe their growing child functioning without a
prosthesis, they gradually understand the potential encumbrance of a purely cosmetic prosthesis. Although
an aesthetic prosthesis may aid the rehabilitation of the traumatic amputee, it is usually a hindrance to the
congenital amputee. If prosthetic wear becomes a source of conflict between parent and child, the child
may even express the thought that the prosthesis is to be worn merely to please parents who cannot accept
them as they really are-without a prosthesis. If an active prosthesis is to be successfully integrated into the
child's life-style, it must provide the child with the ability to either accomplish otherwise impossible activities
or to carry out activities more easily or more rapidly. At times, cosmetic restoration, particularly in partial
hand and foot loss, may be considered a functional improvement.

The hand allows the child to explore his environment and manipulate objects within that environment. The
hand should be able to maneuver in space under volitional control and should be able to reach the body as
well as the area in front of the body. The child must be able to aim the hand so that it can precisely
approach an object by using visual as well as tactile clues. The object is then grasped by the closing fingers
and supporting thumb. The hand must also be capable of releasing the object from its grasp.

The two major types of grasp are precision prehension and power prehension. Precision prehension is
used to hold relatively small objects with modest force, while power prehension is used to hold larger
objects, often with somewhat greater force. In precision prehension, the object is secured between the
distal phalanx of the thumb and the index finger or between the thumb, index, and middle fingers. The
fingers are usually extended at the interphalangeal joints while the metacarpophalangeal joints are partially
flexed. The object itself usually does not contact the palm.

The three most common forms of precision grasp or pinch are palmar pinch, lateral pinch, and tip pinch. In
palmar pinch the flat palmar pads of the thumb and fingers secure opposite sides of the object being
grasped. In lateral pinch the palmar surface of the thumb's distal phalanx is brought against the radial
border of the index finger. Since this posture is often employed to grasp and twist a key, this pattern is also
known as key pinch. Tip pinch provides contact with the distal end of the distal phalanx of the thumb with
the distal phalanx of the index or of the index and middle fingers. Tip pinch is used to pick up a small coin
from a table top.

Power prehension involves the ulnar digits (most often ring and little fingers), while the radial digits (index
and middle fingers) are primarily used in precision prehension. Power grasp usually results in contact of the
object against three surfaces: the palmar aspects of the flexing fingers, the palm of the hand, and the thumb
metacarpal or proximal phalanx. Although the distal phalanx of the thumb may wrap around the object, the
majority of the thumb power is contributed by the stabilizing effect of the adductor pollicis, which resists the
pressure transmitted from the fingers through the object.

The hand also has an important role in nonprehensile activities. These activities usually involve the
transmission of force through the terminal portion of the limb to another object. Nonprehensile activities
include typing or button pushing. Other nonprehensile activities include the punch thrown by a boxer or
pushing open a swinging door.

SURGICAL TREATMENT

As one considers the treatment of an anomalous hand, it is well to contrast the effectiveness of surgical
reconstructions with or without prosthetic management as opposed to doing neither. Nonprehensile
activities are usually unaffected by surgical reconstruction. If a hand lacks a thumb (e.g., radial aplasia) and
digital motion is good, side-to-side pinch will allow the child to perform most precision activities with
reasonable facility. Without the buttressing effect of a thumb, however, power activities cannot be readily
accomplished. Compensation may be achieved by flexing the fingers and wrist and securing a large object
against the distal portion of the forearm. When the thumb is absent, shifting the index finger to the thumb
position by polli-cization will improve power grasp as well as facilitate precision activity.

The monodactylous hand consisting of only a thumb is capable of nonprehensile activities such as holding a
shoe lace in place but is incapable of either power or precision grasp. Construction of an ulnar-buttressing
digit by either distraction lengthening, toe-phalanx transfer, or free-toe-transfer may allow the hand to
achieve meaningful prehension. This may also be achieved by a prosthesis that provides a passive
buttress.

The infant is unaffected by his abnormality. As the baby begins to explore its environment, it learns to use its
unique physical capabilities to best advantage. The young child's goal is to reach the cookie, grasp it, and
bring it to its mouth. If this is most easily accomplished by one hand, the closest or most efficient hand will
be employed. If the object is large or if single-hand prehension is not possible, then both hands will act
together. The object may be secured against the chest. When upper-limb prehension is severely
compromised, a child may develop the capacity for foot prehension.
The child's growing awareness of his abnormality is usually the result of comments from playmates,
siblings, or well-meaning adults. The child usually does not become self-conscious until about the age of 6
or 7 years. At this age peer pressure may cause the child with a unilateral abnormality to conceal the hand
in a pocket or may lead the child to reject an otherwise successful prosthesis.

Other points of psychological stress occur during adolescence as dating begins and concerns arise over
attractiveness to the opposite sex. Feelings may be further complicated by impending marriage and the
prospect of offspring with similar abnormalities. Access to knowledgeable genetic counseling is essential,
particularly at that time.

Aesthetic considerations are important when weighing different therapeutic alternatives in the management
of congenital hand abnormalities. The hand and the face are the unclothed areas of skin most often
exposed to scrutiny. When anomalous parts have an abnormal appearance and function is not
compromised by their deletion, they should be removed. In this case, conversion of a malformed part to an
amputation may result in an aesthetic improvement.

On occasion, the removal of a functionless part may facilitate the fitting of a prosthesis. While approximately
half of lower-limb congenital amputees require surgical revision prior to prosthetic fitting, only about 10% of
congenital anomalous upper limbs fit for prostheses require surgical revision. Consultation between the
surgeon and prosthetist allows the surgeon to understand which anomalies will obstruct prosthetic donning
and wear. Portions of the affected limb that are useful for prehension without a prosthesis should never be
amputated. It is possible to fit a prosthesis around a short phocomelic limb. This will allow the child to also
develop functional capabilities out of the prosthesis. In some instances, the prosthesis may allow digits to
function while the prosthesis is worn.

TIMING

It is usually preferable to begin surgical reconstruction of the anomolous hand prior to or about the first
birthday. Anesthesia can usually be safely accomplished by 6 months of age. For children under 18 months
of age, it is possible to operate on both hands during the same surgical anesthetic. As the child ages, their
frustration when both hands are immobilized increases. In addition, early bilateral surgery may spare the
child an additional anesthetic.

Some procedures such as toe phalanx transfer must be carried out early for revascularization and
subsequent growth. Children undergoing digit-shifting procedures such as pollicization may benefit from
early integration of the pollicized digit into evolving patterns of grasp.

In some instances, it is wise to delay surgery until children are older because of systemic considerations. In
children with TAR syndrome (thrombocytopenia with absent radius), in whom low platelet counts at birth
gradually increase with age, it is usually wise to wait until the child's platelet count has increased to at least
60,000/mm before considering elective surgical reconstruction. In such cases, centralization of the wrist
may sometimes be delayed until the child is 3 or 4 years of age.

It is best to make major decisions regarding the reconstruction or deletion of digits when children are young.
It is inappropriate to place the burden for deciding whether a digit is to be deleted or pollicized upon an
adolescent. The parental temptation to wait until the child is older to let them make up their own minds
should be avoided since this places unrealistic pressure on the adolescent.

SHORT TRANSRADIAL AMPUTATION

This common level of terminal deficiency is effectively treated prosthetically. Surgical reconstruction is rarely
indicated. Initial prosthetic management begins with a passive hand. The sophistication of the prosthesis is
increased as the child matures.

WRIST DISARTICULATION

The Krukenberg procedure has been suggested as a reconstructive alternative for children with congenital
absence of the hand, particularly with profound contralateral abnormalities, associated blindness, or a lack
of access to prosthetic care. The radius and ulna are separated from one another by the Krukenberg
procedure. This creates a prehensile limb that will also allow prosthetic fitting. Because the cosmetic
disadvantage of this procedure is substantial, it is rarely appropriate for the unilateral case (see Chapter
36A).

SYNDACTYLY

Syndactyly, the joining together of digits, may be categorized as complete or incomplete, with subcategories
of simple or complex. When syndactylization extends the entire length of the digit, the condition is termed
complete syndactyly. In cases in which the web involves only a portion of the length of the digits, it is termed
an incomplete syndactyly. When skeletal and nail elements of the syndactylized digits are separate, the
syndactyly is said to be simple. When there is joining of digital skeletal and/or nail elements, the syndactyly
is termed complex. Acrosyndactyly refers to syndactyly in which the ends of the fingers are joined, often as
a result of congenital constriction band syndrome. Dobyns et al. have further suggested that syndactyly of
digits containing angulated phalanges be termed complicated syndactyly.

Surgical release of syndactylized digits is usually indicated to enhance digital independence. Even short
digits consisting of only a proximal phalanx may benefit from separation. Index finger radial abduction may
be increased and pinch improved when a short index finger is untethered from the middle finger.

Syndactyly release must provide skin coverage of the adjacent lateral surfaces of the released digits and
also create a proper web space floor. Because the surface area of two syndactylized digits is far less than
the skin surface area of two separated digits, a full-thickness skin graft is necessary to supplement local
flaps.

Many skin flap techniques have been advocated for the separation of syndactylized digits. Successful
surgical procedures surface both digits with durable skin, create an appropriate web space floor, and
accommodate growth of the digit without secondary contracture (Fig 34A-1.,A and B). Effective techniques
employ skin flap tissue to create a sloping web space floor of true anatomic proportions, both in width and
depth. This flap tissue may be derived either from the dorsum of the hand, from the palmar aspect of the
hand, or from a combination of both palmar and dorsal tissue. Dorsal flaps provide the best skin color match
when the web space is viewed from the dorsum but may result in a hypertrophic scar traversing the inter-
digital commissure. A palmar flap provides a better commissure contour but results in the shifting of pink
palmar skin into the web space. Since the web space is usually viewed from the dorsum, the difference in
color is particularly noticeable in dark-skinned individuals.

Fig 34A-1. A, dorsal view of simple incomplete syndactyly involving the left middle and ring fingers.
B, zigzag incisions have been used to separate the middle and ring fingers. A proximally based
dorsal flap is sutured to the palmar skin to create a floor for the web space with both depth and
width. A full-thickness skin graft harvested from the groin is applied to areas of digits not covered
by local flaps.
The web space floor normally begins just distal to the metacarpophalangeal joint and slopes to the edge of
the palmar commissure approximately one third the length of the proximal phalanx segment. The web
palmar commissure is supple enough to allow interdigital abduction of up to 45 degrees.

Skin incisions on the palmar and dorsal surfaces of the syndactylized digits should be planned to avoid
longitudinal scars crossing digital flexion creases because these scars tend to contract with growth. Zigzag
incisions may be planned to interdigitate skin flaps and defects to effect either full closure of one digit or
partial closure of two adjacent digits.

After the web space floor has been closed and digital flaps rotated into place, templates are made of the
residual defects. Templates are used to harvest full-thickness skin grafts from the groin crease. Skin grafts
are sutured in place. Interdigital dressings are maintained until all wounds have healed.

When multiple digits are syndactylized, it is usually wise to avoid releasing both sides of a digit during the
same procedure.

POLYDACTYLY

Polydactyly takes many forms. In black children, post-axial (ulnar) Polydactyly is the most common form,
while in white children preaxial (radial) Polydactyly is more frequent. Central Polydactyly is less common
than either preaxial or postaxial Polydactyly. Polydactylous digits are rarely supernumerary, that is, they
rarely represent parts additional to a normal hand. Most often polydactylous digits are abnormal and
suggest an abnormal segmentation or separation of digital ray condensations, as though one or more of the
five mesenchymal condensations was inappropriately longitudinally split. The challenge of surgery is not
simply to remove sufficient tissue but rather to retain tissue sufficient to optimally reconstruct the retained
digits.

Simply amputating one of the duplicate digits will usually result in a residual digit that is considerably
smaller than its counterpart on the opposite side. This effect can be lessened by soft-tissue coaptation (Fig
34A-2.,A and B). Incisions are planned to facilitate the coapting of soft tissues from both digits to provide
optimal soft-tissue bulk. Angular deformity in either phalanx or metacarpal should be corrected by
osteotomy. Surgical reconstruction aims to achieve a digit in which the carpometacarpal,
metacarpophalangeal and inter-phalangeal joints are parallel. The longitudinal axis of the metacarpal and
phalanges should be perpendicular to the three joints. Correction of angulation is usually achieved by a
closing wedge osteotomy and secured by Kirschner wires. An opening wedge osteotomy using a segment
of excised bone as intercalated graft is occasionally indicated.

Fig 34A-2. A, Wassel type IV thumb duplication preoperatively demonstrated angulation of the
larger ulnar thumb metacarpophalangeal and interphalangeal joint levels. B, surgical
reconstruction of the thumb Polydactyly depicted in A included a soft-tissue coaptation flap from
the excised radial digit, a closing wedge osteotomy of the thumb metacarpal, and an opening
wedge osteotomy of the proximal phalanx to achieve appropriate longitudinal alignment of the
digit. The radial collateral ligament of the metacarpophalangeal joint was reconstructed and the
abductor pollicis brevis muscle reinserted in the retained digit.
Preaxial Polydactyly takes many forms. Wassel has separated these abnormalities into seven categories,
six of which involve biphalangeal thumbs (Fig 34A-3.). Type I deformities may present as simply a wide
distal phalanx and nail, in which case no treatment is indicated. If two nails are present, two alternative
treatments may be considered. The first option is excision of one nail with the underlying bone, while the
second involves a central resection of adjacent nail borders and underlying bone, combined with
longitudinal phalangeal osteotomies to narrow the distal phalanx. When the latter technique, known as the
Bilhout-Cloquet procedure, is attempted, care must be exercised in matching the nail matrix to avoid an
unsightly longitudinal nail ridge. Osteotomies should be carried out distal to the physis to avoid growth
disturbance.

Fig 34A-3. Wassel's original classification scheme for thumb Polydactyly has been
modified to demonstrate the most common form of thumb Polydactyly (type IV) in
which two proximal phalanges each possess separate secondary ossification
centers.

Type II duplications consist of two undersized (in comparison to normal) distal phalanges seated atop a
somewhat widened proximal phalangeal distal articular surface. The radialmost digit possesses a collateral
ligament along its radial border, while the ulnar digit possesses a collateral ligament along its ulnar border.
The two digits abut with adjacent articular facets and are bound together by pericapsular tissue. It is
preferable in most instances to excise the more radial digit because it is usually less well developed. The
broad distal articular surface of the proximal phalanx may need to be tapered to a size appropriate to the
distal phalanx. The collateral ligament that initially secured the radial aspect of the deleted radial digit must
be retained to securely stabilize the radial aspect of the new interphalangeal joint. Retained flexor and
extensor tendons must be examined to ensure that the course and insertion of residual tendons are
centered.

Type III abnormalities are usually dealt with by deleting the radial digit. Type IV abnormalities usually require
deletion of the radial digit, narrowing of the metacarpal head, and collateral ligament reconstruction. The
intrinsic muscles that originally inserted into the more radial thumb are reinserted into the hood of the
residual ulnar thumb component.
Type V abnormalities usually require deletion of the more radial digit and reinsertion of the intrinsic muscle
insertion into the residual ulnar digit. Type VI abnormalities may require shifting of the more distal portion of
the radial digit onto the more proximal portion of the ulnar digit.

Central Polydactyly often presents in combination with syndactyly. Frequently an anomalous central digit
will be bound to the middle or ring fingers without normal metacarpal development. In such instances the
skeletal elements of the unsupported digit are excised, and skin flaps are designed to preserve or
reconstruct normal web space contour and digital bulk. When formal ray resection is required, web space-
preserving incisions should be selected.

Postaxial Polydactyly of the digit joined only by soft tissue may be treated by simple excision. When the
most ulnar digit articulates with the metacarpal head in a fashion similar to that in the Wassel type IV thumb
duplication, simple digital excision will result in an inadequate residual digit. It may be necessary to narrow
the metacarpal head, but it should be recognized that the little-finger metacarpal head, unlike the thumb
metacarpal head, contains a physis and that care must be taken to preserve physeal growth. If the
hypothenar musculature inserts into the more ulnar little finger, its insertion must be detached from the
skeletal elements being resected and reinserted into the retained radial little finger. Similarly, the ulnar
collateral ligament of the deleted digit must be retained and reconstructed to stabilize the ulnar aspect of the
residual little-finger metacarpophalangeal joint.

The mirror hand is an unusual abnormality in which there is duplication of the postaxial border of the hand
with seven or eight digits and two ulnae. Neither the thumb nor the radius is present. Surgery is useful in
expanding the arc of elbow flexion and extension but does not gain forearm rotation. Because there is an
overabundance of flexor musculature and relative paucity of extensor musculature, wrist flexion release
may be necessary. Deletion of two or three digits with pollicization of one of the digits along the "preaxial"
border will improve the aesthetic appearance of the hand and modestly improve function.

RADIAL DEFICIENCY

The radial-deficient upper limb demonstrates a variable extent of radial absence, aplasia, or dysplasia of the
soft-tissue and skeletal elements along the radial (preaxial) border of the limb. When the radius is absent
or severely dysplastic, the unsupported carpus will rest against the radial border of the ulna and cause the
hand to assume a posture at a right angle to the ulna. The thumb is frequently absent or, if present, is
dysplastic. The more radial digits are often stiff with limited active flexion. Because the ring and little fingers
are the most supple digits, they are frequently used for side-to-side prehension. The tendency to use a
pattern of ulnar prehension is usually a consequence of the greater mobility in the ulnar digits and their
position as the presenting part of the deviated hand as it is brought in front of the trunk.

Beginning shortly after birth, the hand is splinted in an effort to stretch radial soft tissues. Surgical
centralization stabilizes the hand at the end of the forearm by placing the hand and carpus on the end of the
ulna (Fig 34A-4.,A-D). Centralization allows the hand to reach out away from the body, more effectively
placing the radial digits in front of the body. The excursion of extrinsic digital flexors and extensors may be
focused upon digital motion and not frustrated by collapse of the hand and wrist at the ulnocarpal level.
Fig 34A-4. A and B prior to surgical correction, the hand of this limb with total absence of the radius
deviates severely radialward and palmarward. C and D, following surgical centralization of the hand
onto the end of the distal portion of the ulna, control of the hand in space is substantially improved.
It is possible for the thumb and radial digits to effectively reach out away from the body for
prehensile activity.

Although centralization does improve function in front of the body, it is not always indicated. If active elbow
flexion is lacking, the abnormal radial deviation at the ulnocarpal level provides the child with the ability to
bring the hand to the face. Because centralization frustrates this function, it is contraindicated if elbow
flexion is lacking.

The normal thumb participates in a wide variety of both precision and power prehensile activities. The hand
without a thumb is nonetheless capable of great dexterity. Side-to-side pinch between fingers allows
precision activity to be accomplished with relative facility. Normally, when an object is grasped in power
prehension, the object is forced against the palm by the flexed fingers and buttressed by the stability of the
thumb metacarpal. The ability of the individual without a thumb to hold large objects securely is thus
markedly compromised. Large objects can be held securely only by using both hands together or by flexing
the wrist and securing the object against the forearm or body. Pollicization shifts the index finger from its
normal position to the thumb position along the radial border of the residual hand so that it can participate in
power as well as precision activity. This shifted digit, however, is not a normal thumb.

If the index and middle fingers were used for precision pinch prior to surgery, it is likely the pollicized index
finger will continue to participate in precision activity against the middle finger. If the predominant pattern of
precision prehension was between the middle and ring fingers or between the ring and little fingers, it is
likely that this pattern will persist after polli-cization. In such situations the functional advantage of
pollicization is realized only with power activity.

Pollicization consists of four major elements, the skin incision, neurovascular dissection, skeletal
adjustment, and musculotendinous rebalancing. Skin incisions are designed to create a web space
between the shifted digit and the middle finger and to allow digital transposition without the need for skin
grafting (Fig 34A-5.). Neurovascular structures are preserved by ligating the proper digital artery to the
middle finger and splitting the common digital nerve to the index and middle fingers. Care must be taken to
preserve dorsal venous drainage of the digit. By resecting a major portion of the metacarpal, including the
physis of the metacarpal head, the resultant digit will be of a length similar to that of a thumb. The
metacarpophalangeal joint of the index finger is hyperextended and secured to the residual proximal
metacarpal. Musculotendinous balance is achieved by advancing the first dorsal interosseous and first
palmar interosseous muscle insertions into the hood and by shortening the extensor extrinsic tendons.
Shortening of the flexor tendons is not required. Spontaneous use of the digit is usually noted within a few
months and continues to improve as the child ages.

Fig 34A-5. This child with bilateral thumb aplasia has


undergone right hypoplastic thumb ablation and
pollicization. Two months following pollicization, he is
using the pollicized right index finger spontaneously for
large object grasp. The hypoplastic left thumb is ignored in
preference to side-to-side pinch between the left index and
middle fingers. Left-thumb ablation and pollicization were
subsequently performed.

ULNAR DEFICIENCY

The ulnar-deficient hand is characterized by an absence or hypoplasia of the ulna. Ectrodactyly is usually
present and may be manifested in the absence of any of the fingers, including the thumb. Syndactyly is
common, particularly between the ring and little fingers, and should be treated in the fashion described
earlier in this chapter. The wrist is usually stable and rarely requires surgical intervention. The elbow may be
stiff due to radiohumeral synostosis. In some instances, if the hand is positioned behind the body,
osteotomy of the radius is indicated to bring it into flexion (Fig 34A-6.). Another problem is the presence of
severe flexion contracture of the elbow. Because surgical releases are usually limited by tight neurovascular
structures, serial splinting or Ilizarov joint stretching have been employed to improve joint motion.
Fig 34A-6. This ulnar-deficient limb with congenital radial
humeral synostosis is awkwardly placed to naturally fall
behind the child's back. Humeral osteotomy may improve
the position of the hand in space.

CLEFT HAND

Confusion exists regarding the appropriate classification of children with a normal-length radius and ulna
and absence of the central (index, middle, and/or ring) fingers. The typical cleft hand or split hand is
usually bilateral and may be associated with bilateral cleft-foot abnormalities and an autosomal dominant
inheritance pattern. Morphologic variation is common. Digital absence, digital fusion, cross bones,
syndactyly, and distal Polydactyly may all be evident within affected hands. Because the morphology of this
condition may vary considerably from the right to the left side and from one generation to another, cases in
which a true cleft is not evident are often overlooked.

Digits frequently diverge from the central cleft with ulnar deviation of the ring and little metacarpals and
radial deviation of the middle and index metacarpals. The index metacarpal is often deviated toward the
thumb metacarpal, thus creating a narrow first web space. Syndactyly is frequently encountered, particularly
between the ring and little finger. Syndactyly should be released as previously described. Metacarpal
osteotomies may be necessary to gain parallel alignment of metacarpals at the time of web space closure
(Fig 34A-7.,A and B). In many instances, a flap derived from redundant skin in the central cleft may be
rotated into the first web space at the time of first-web space release.
Fig 34A-7. A, The index and ring fingers of this child with a bilateral cleft hand deformity were held
apart by transversely lying phalangeal components. The index fingers were rotated in relation to
the ring and little fingers. B, following excision of transversely lying phalangeal components and
transposition of the index ray onto the middle metacarpal, rotational alignment of the digits is
improved.

BRACHYDACTYLY

Treatment of the hand with short digits (brachydac-tyly) or absent digits (adactyly or ectrodactyly)
engenders much debate (Fig 34A-8.). Since these abnormalities are usually unilateral, affected children
possess considerable physical capabilities without surgical or prosthetic intervention.

Fig 34A-8. Effective prehension is possible in this child's


hand between the thumb and little finger components.
Surgical excision of the digital remnants of the index,
middle, and ring fingers eliminated the digital nubbins.
Interphalangeal joint arthrodesis in the little finger
improved the stability of the ulnar digit.

When digital soft-tissue sleeves are substantially longer than the enclosed skeletal elements, the skeleton
may be lengthened by a nonvascularized toe phalanx transfer. The proximal phalanx of the third or fourth
toe is "harvested" with its proximal volar plate and collateral ligaments. The toe phalanx is then secured in
the finger sleeve by Kirschner wires and by suturing the volar plate and collateral ligaments to the intact
proximal skeleton. Rudimentary flexor and extensor tendons may be defined and sutured to the palmar and
dorsal aspects of the transferred phalanx. Although these transferred phalanges are more likely to continue
to grow after transfer if the procedure is carried out between 6 to 12 months of age, the procedure may still
be beneficial in older children. Toe phalanx transfer is particularly helpful when the thumb lacks phalanges
or when the thumb is present along with metacarpals but the fingers lack phalangeal elements to pinch
against.

Free-tissue microvascular transfer of the second toe or of the second and third toes together has been
employed to augment hands without digits or digital soft-tissue sleeves. Vascularized toe transfers
continue to grow until skeletal maturity and yield a digital length approaching the predicted length of the toe.
The digit will retain the form of a toe and at best will have the range of motion of a toe. In most instances of
adactyly, the proximal musculature is poorly defined with restricted excursion. When these
musculotendinous units are sutured to a free-toe transfer, the resultant digital motion is often quite limited. It
is rarely worthwhile to attempt to achieve prehension in an adactylous hand by transfer of two separate
digits. When a well-controlled thumb is present without other digits, there may be benefit in transferring a
toe to the hand to provide counterpressure to the thumb and thus achieve prehension.

In some cases of brachydactyly, resection of the index or of the index and middle metacarpals may enhance
prehension through "phalangization," a procedure in which prehension is shifted proximally to facilitate
grasp between the thumb, ring, and little metacarpals.

CONGENITAL CONSTRICTION BAND SYNDROME

Congenital constriction band syndrome has many manifestations (Fig 34A-9.). These include band
indentation, distal edema, acrosyndactyly, and/or amputation of distal parts.

Fig 34A-9. This hand demonstrates multiple manifestations


of congenital constriction band syndrome. The paper clip
is placed through a small sinus proximal to the
syndactylized middle and ring fingers. Multiple areas of
band indentation are evident with amputation of the
terminal portion of the ring finger secondary to band
constriction.
Bands that encircle a limb or digit may result in distal edema. Surgical treatment requires excision of the
indented skin and of any deep fascial constricting tissue. The skin is closed after Z-plasty flap transposition.

Syndactyly release is described in the preceding section on syndactyly. Although a sinus with a web space
floor is often present, this web space floor is situated too distally and will require formal release to bring the
web space to the appropriate proximal level. Syndactyly usually is the result of a failure of normal interdig-
ital web space resorption during embryonic development. When syndactyly occurs in association with
congenital constriction band syndrome, however, a sinus is usually evident proximal to the area in which the
skin is joined. This form of syndactyly is termed ac-rosyndactyly or fenestrated syndactyly. In developmental
syndactyly, adjacent digits are joined side to side in a fashion similar to the intrauterine embryonic paddle
form. In acrosyndactyly, however, noncontiguous digits may be joined. The index finger might be joined to
the ring finger, while the middle finger is forced palmar or dorsal to the distally webbed digits.

When amputation occurs as a result of congenital constriction band syndrome, the amputation may occur at
the joint, metaphyseal, or diaphyseal level. Diaphyseal amputations usually occur through hand or foot
phalanges or through the tibia. The resultant tapered ends may require revision in adolescence or
adulthood because of the propensity of these amputations to appo-sitional overgrowth.

Although lower-limb amputation through the tibia occurs occasionally, amputation through the arm or
forearm is rare as a result of congenital constriction band syndrome. Deep indentation from band
constriction at the humeral level may result in an insensate paralyzed hand of no functional value and
subject to repeated infection. In some instances these hands may be amputated electively. Prosthetic
fitting of such limbs may be complicated by the insensitivity of the residual forearm.

When thumb length is insufficient for prehension due to congenital constriction band amputation, second-
toe or hallux transfer may provide effective augmentation of the thumb (Fig 34A-10.,A and B). Because the
blood vessels, nerves, and musculotendinous structures proximal to the level of the amputation are normal,
satisfactory ultimate neural, vascular, and motor function may be anticipated. The transferred toe will
continue to grow as the child's hand grows.

Fig 34A-10. A, congenital constriction band syndrome has resulted in amputation of the thumb
through the proximal portion of the proximal phalanx and amputation of the index and middle
fingers at the proximal interphalangeal joint level. B, the thumb is effectively lengthened by
microvascular hallux transfer.

SUMMARY

Children with upper-limb deficiencies may benefit from surgical reconstruction. The surgeon should consider
both functional and aesthetic impact. In most instances, prosthetic fitting does not require surgical
modification of the upper limb, that is, prostheses should be fabricated to fit the limb as it is. In other words,
function of the residual limb out of the prosthesis should not be compromised in an attempt to simplify
prosthetic fitting since children may spend considerable periods of their day without their prosthesis.
Upper-Limb Deficiencies: Prosthetic and Orthotic Management
Terry Supan, A.A.S., C.P.O.

The function and design principles of prostheses for children are similar to their adult counterparts. Whether
the child has an acquired amputation or a congenital anomaly, our goal should be to provide the most
functional and cosmetic design possible when and if the rehabilitation team and the family decide that the
child should be fitted with a prosthesis. The key difference is that children's prostheses must also be
developmentally appropriate.

Age guidelines for optimum fitting have changed as we have gained more experience. No longer must we
wait until the teenage years to fit a myoelectric prosthesis. Nor do we wait until the child has lived a year
before fitting after tumor surgery. Traumatic amputees now receive their first prosthesis in a matter of days,
not months.

RECENT DEVELOPMENTS

During the 1980s, a plethora of new components, materials, and techniques were developed for the adult or
geriatric amputee. A specific attempt was made at the end of the decade to redirect this effort toward the
pediatric patient. Myoelectric hands, elbows, and controls were miniaturized and simplified (Fig 34B-1.).
The lighter-weight, stronger new materials could be used for children. Thermoplastic socket designs allowed
for more adjustability and adaptability for growing limbs. Redesigned lower-limb components also became
available for the younger amputee.

Fig 34B-1. Miniaturized electronics, batteries, battery saver,


and myoelectric hand that are used for children under 4
years of age. The hand is from Variety Ability System; the
remaining electronics are from Universal Artificial Limb.

In a break with the past, the U.S. government did not underwrite this developmental cost. This resulted in
increased costs as the manufacturers amortized research expenses. Although we are fortunate that some
entrepreneurs have been willing to invest in such a small area as upper-limb prosthetics, the substantial
cost for newer technology is an ongoing concern.

The modularization of upper-limb components during this period offers the potential to recycle parts into the
child's next prosthesis. The preschool amputee may need a new socket annually because of growth, but the
expensive myoelectric hands and electronics should be reusable in the new prosthesis, provided that the
size remains appropriate.
The development of simplified myoelectric control and a battery-saver circuit allows the fitting of infants with
a practical control scheme. While hand opening and thumb adduction usually begin around 4 months, the
development of shoulder-hand coordination is delayed until after 9 months. Because of training difficulties,
cable-controlled prehension of the prosthesis is seldom feasible until 12 to 18 months. The new myoelectric
circuits provide controlled opening, automatic closing when the child relaxes, and a stall condition detector
to save battery capacity automatically. This type of prosthesis is easier for the infant to learn to use because
any electromyographic (EMG) signal detected within the socket creates hand opening. As the child
develops normal coordination of the contralateral hand, he also spontaneously improves his prosthetic
function.

WHEN TO FIT AND WITH WHAT

The key questions are when a child should be provided with a prosthesis and what the best type is. There
are no simple answers. "How soon?" is probably best answered by "when technically possible." The child
with a traumatic or acquired amputation should ideally be fitted within 30 days (Fig 34B-2.) to encourage
acceptance of the prosthesis and the continuation of bimanual activities. The child with a congenital
condition may be provided with a passive hand within 60 to 90 days after birth. This theoretically allows the
child to acquire more normal bimanual and quadripedal development.

Fig 34B-2. A preparatory myoelectric prosthesis for a


distal-third transradial traumatic amputation. The
prosthesis was fitted to the child 2 weeks postoperatively
and used for 6 months before its replacement by her
definitive prosthesis. types of terminal devices for different
functions in their life-style.

"What type of prosthesis?" is much more difficult to answer. The well-informed prosthetist can guide the
physician through this ever-changing area of new terms, techniques, and technology. "Should powered or
mechanical components be used?" "Both" is the best answer to that question. Each has its own advantages
and disadvantages, and they can often be used in combination.

Multiple prostheses with differing control patterns, however, can lead to confusion and frustration.
Therefore, only one type of prosthesis should be used for the very young child. As children mature, they
should be given the opportunity to experiment with different

Mechanical terminal devices are lighter, have fingertip prehension, and are less susceptible to damage.
Unfortunately, voluntary-opening hooks have much less pinch force than do electric hands and are not as
cosmetic. Voluntary-closing devices like the Adept have a graded pinch force that is controlled by the
individual. The streamlined design of hook devices permits visual inspection of the objects to be grasped,
which can be advantageous.

A myoelectric hand has greater pinch force and is capable of controlled opening and closing throughout the
full range of motion of the arm. It also can be operated independently of elbow function. However, it cannot
be submerged in water, is heavier, and is not as adept at picking up smaller items. The cosmetic gloves
must be replaced routinely to prevent moisture and dirt from entering the electrical and mechanical parts.

Passive, mechanical, and electric elbows are all available for the child amputee. Like the terminal devices,
each has advantages and disadvantages. Weight and function are the best guidelines to use when
recommending elbow components.

Passive elbows are light but must be operated by the other hand. Heavy-duty use would preclude the use of
a passive elbow.

Cable-controlled mechanical elbows are smaller versions of the adult models. One cable controls elbow
flexion, and the second controls the locking mechanism. The child must learn more complicated shoulder
motions to achieve both flexion and locking of the prosthetic elbow, which is sometimes difficult.

When a mechanical terminal device is used, the elbow must be locked for the cable's force to be transferred
to the hook. However, if a myoelectric hand is used with the cable elbow, then terminal device and elbow
function are independent.

Electric elbows for children are usually switch controlled, but myoelectric controls are available for the older
youth. They are heavier, more costly, and more complicated but are easier for the patient to control because
they require less force, excursion, and coordination. They are often used with short residual limbs or
congenital limb remnants.

The acquired unilateral amputee should be fitted with the most cosmetic, functional components available.
Children over 3 years of age can be trained to control all available devices. Younger patients or those with
congenitally deficient limbs sometimes do not have sufficient neuromuscular control to operate complex
devices. Although the fitting of a single-function myoelectric terminal device for infants is controversial, the
6- to 9-month-old can spontaneously develop control of the prosthesis without extensive training. Whether
they will continue to use the prosthesis or reject it later in life can only be determined by a longitudinal study.
It can be argued that fitting the child, despite the uncertainty of future results, will stimulate further advances
in the science of prosthetics. Reliability of electric prostheses has improved significantly and will continue to
do so as long as we continue fitting such devices.

RECOMMENDATIONS BY LEVEL

Infants with partial-hand amputations or congenital deficiencies probably would be best served by not fitting
them with a prosthesis. Children with unilateral conditions will readily adapt to their "one-handedness," with
the noninvolved side becoming the dominate hand. There is a lack of functional prostheses for the young
child with only digits or metacarpals missing. The available cosmetic partial-hand prostheses compromise
the sensory feedback to the limbs inside the prostheses. Combining this with an increase in length usually
results in a rejection of the prosthesis.

Transverse anomalies or amputations can be provided with an orthosis to prevent deformities or increase
prehension. Although attempts at fitting opposition posts and platforms can be made, long-term use of these
for the unilateral patient has been inconsistent (Fig 34B-3.). More effort should be concentrated on
adaptability without the use of prostheses or orthoses. The exception would be the child who has a limb-
length discrepancy that would allow the fitting of a functional prosthesis of equal length to their sound side.
Then the recommendation would be to give the family the option to fit the infant with a prosthesis when
possible (Fig 34B-4.). This would allow the prosthesis to become part of the child's natural development.

Fig 34B-3. Opposition platform used for a congenital partial-


hand anomaly.
Fig 34B-4. Three-year-old fitted with a myoelectric
transradial prosthesis for her short congenital condition.
The infant was fitted with her first electric prosthesis at 12
months. The first two prostheses had a single-site,
voluntary-opening control with her elbow fixed at 90
degrees. The present prosthesis has a self-suspending
flexible socket with full elbow motion.

The wrist disarticulation level raises concerns similar to those of the partial hands. Inequality of limb length
and loss of sensation related to prosthetic fitting are likely to lead to rejection of a prosthesis in congenital
conditions. On the other hand, traumatic injuries in older children should be treated very aggressively with
prosthetic fitting within the first 30 days to facilitate the incorporation of the prosthesis into the amputee's
lifestyle. Recent modifications to myoelectric components also have allowed this level of amputation to be
fitted before 18 months, if desired.

The transradial level has seen the most change in design for the younger patient. Newer components have
allowed the prosthetist to take a more aggressive approach with these patients. With the development of
"user-friendly" myoelectric controls, the child under I½ years can learn to develop control of the prosthesis
(Fig 34B-5.). This allows a more natural assimilation of the prosthesis without having to battle through the
"terrible twos." Although this concept has not been proved, enough positive anecdotal evidence has been
reported that it should be investigated further.
Fig 34B-5. A flexible-socket transradial prosthesis being
controlled by an 11-month-old during the delivery of his
prosthesis. The prosthesis has an auxiliary figure-of-8
harness and an external battery.

The younger child with a transhumeral condition should be fitted with either a static or friction elbow
mechanism. The approach for hand function should parallel the transradial case. The older amputee should
be provided with an active elbow joint. They can be expected to master either dual cable control of the
elbow lock or electric control of the motorized elbow.

Midshaft or longer transhumeral conditions with normal shoulder function do not require the heavier electric
elbows. These prostheses will function more quickly and quietly and with more proprioceptive feedback (via
the cable and harness) with mechanical elbows. The available electric elbows should be primarily
considered for patients with shorter residual limbs. At this level, the ease of control offsets the extra weight
and may increase the potential for prosthetic use. As with the traumatic transradial amputees, acquired
transhumeral amputees must be fitted very quickly to allow the individual to retain normal bimanual
functions.

The additional requirement for prosthetic shoulder function at the shoulder disarticulation and forequarter
level leads to more complications and more rejection. The prosthesis must provide a functional benefit for
the amputee, or he will reject it. A compromise between weight and control simplicity must be made. All the
available children's-size shoulder joints are passive, so the child must manually preposition the prosthesis'
shoulder in the desired location.

Elbow and terminal device choice is based on a needs assessment of the amputee. The evaluation of the
shoulder and forequarter amputee should ideally be conducted by an experienced clinic team due to the
large number of prosthetic component combinations possible.
The needs of the bilateral upper-limb amputee are also best met by the experienced rehabilitation team.
The child must learn to function both with and without the prostheses for the greatest independence.
Because of their proprioceptive feedback, cable control of the terminal device of the prostheses should help
the child's function (Fig 34B-6.). As the control and speed of electronic components improve, this trend may
be reversed.

Fig 34B-6. Eleven-year-old bilateral traumatic transradial


amputee fitted with Adept terminal devices and
multilayered sockets. This design allows greater
proprioceptive feedback and limb growth.

The bilateral amputee, who needs more than just prehension, will benefit from referral to a rehabilitation
center that has experience with this type of condition. The challenge of these children requires familiarity
with a variety of prosthetic components and the ability to combine them for the optimum function for the
amputee.

SPECIAL NEEDS OF CHILD AMPUTEES

Children are not just small adults. Their life-styles and attitudes are different. They want to be independent,
but they also want to fit in with their peers. They need to belong to the group and not feel like an outsider.

A child with a congenital anomaly or amputation will experience the reaction of others to their "difference."
The more cosmetic the prosthesis is, the greater the chance of a positive reaction to the child. "Captain
Hook" still conveys a negative image for most of our population. Therefore, cosmetic appearance does
become an issue for both child and family.

The physical changes that children normally go through must be considered when designing their
prostheses. The rapid growth of their limbs requires replacement of all or part of the prosthesis annually.
The use of multilayered sockets or thick limb socks will delay the replacement. The use of thermoplastics
that are adjustable will also help.

As noted above, the more expensive parts of electric prostheses can be reused in the next device. Several
centers use the concept of "limb-banking" electric components to help reduce the cost of the prostheses.
This only works if there is technical and financial support to maintain the parts.

Children can be very destructive in their normal active life-style. The prosthesis must be designed to take as
much abuse as possible. They also should be provided with the adaptive equipment to have as normal a
childhood as possible. The use of terminal devices designed for recreation should be encouraged (Fig
34B-7.).
Fig 34B-7. A transradial amputee using his prosthesis to
play hockey. (Courtesy of Therapeutic Recreation Systems,
Inc.)

CONCLUSION

Although technologies have changed, the challenges of the child amputee have not. The prosthetic fit of the
growing limb must be revised annually. The more complicated cases, i.e., high-level and bilateral
amputations, should be managed by clinic teams with more experience. The life-style of the child requires
that the prostheses be functional, durable, and cosmetic.

New materials and equipment are allowing the fitting of lighter and more advanced prostheses. Prospective
studies of these new methods of patient treatment are needed.

Upper-Limb Deficiencies: Externally Powered Prostheses


Francis J. Trost, M.D.
Dan Rowe, C.P.O.

Externally powered prostheses were first discussed by Reiter in a publication shortly after World War II.
Further work in this field, however, was not actively pursued until the late 1950s and 1960s.

At that time, fueled by technological advances and the need created by the thalidomide disaster, attention
was again focused on the possibility of producing externally powered prostheses. Efforts to accomplish this
were undertaken in the Soviet Union, England, Canada, Italy, and Germany.

Efforts to improve and refine these devices have resulted in the development of a valuable adjunct used in
the treatment of amputees. While much has been accomplished in this field, many problems remain to be
solved, and at this time, external power is not the complete solution for all of the amputees' problems but,
rather, is a valuable additional tool in the prosthetic armamentarium.
Because of the complexity of factors and components used in externally powered prostheses and because
of the considerable cost involved, it is felt that most of these prostheses are best prescribed in specialty
clinics composed of team members from the various disciplines concerned with the treatment of child
ampu-tees.

Initially, externally powered prostheses were fabricated for the adult amputee, and the components were
frequently too large for children. Because of the needs of the juvenile amputee and the influx of
thalidomide babies, various modifications, including miniaturization of components, allowed children to be
fitted with externally powered prostheses.

For unilateral amputees any prosthesis will always be an assistive device. Tasks requiring fine manipulation,
unless bimanual, will always be accomplished by the normal or nonamputated limb. In the case of bilateral
and high-level amputees (Fig 34C-1.), who have the greatest need for prosthetic assistance, externally
powered prostheses still fall far short of meeting their needs in regard to spatial placement of the prosthesis
and reliability. In view of this, it is not surprising that the most common externally powered prosthesis
used clinically is the unilateral transradial prosthesis (Fig 34C-2.).

Fig 34C-1. A high transhumeral amputee.


Fig 34C-2. A typical transradial myoelectric prosthesis.

EXTERNAL POWER COMPONENTS

In discussing external power components, emphasis will be placed on the child amputee and the
differences and special considerations that have to be given to them as children. Powered components for
the adult are discussed in Chapter 6A. It should also be noted that teenagers who have reached adult size
will have adult components available to them. Initially, components for small children were simply
downsized adult components, but currently, in addition to downsizing, some components have been
redesigned to meet the needs of the child amputee.

Control Systems

Basically, two external control systems are commercially available: myoelectric and electric switch. Other
methods have been tried experimentally but have not yet appeared on the market. Pneumatic power was
used initially, particularly in Europe, but despite some advantages, it has some major drawbacks and
currently is seldom used as a power source.

The myoelectric system works by picking up the electrical activity generated by the muscles like an elec-
tromyogram. This activity is picked up by an electrode, amplified, rectified and filtered, and sent to the
motor. The electrode is placed over that portion of the muscle that gives an optimum response as
determined by a myotester or, in the case of young children, small electric toys. The amplifier may be
contained in the electrode or be separate. The signal may be processed in a digital or analog fashion. If the
smooth filtered signal exceeds a certain basic threshold, the motor activates, and the component is
engaged and continues its movement until its limits are reached or the signal is discontinued. The
myoelectric signal therefore is simply a method of switching the component on and off

In a digital mode, regardless of the strength of the muscle contraction, the component is on or off. In the
analog mode, response is graded depending on the force of the muscle contraction. Electrodes are usually
one site, one function or one site, two functions. Attempts have been made to insert more functions in one
site, but these are not yet widely available.

The other commonly used system is that of an electric switch that opens or closes a circuit and activates a
component or turns it off. These are usually push or pull switches. The nudge control switch is a type of
push switch. Rocker and toggle switches are also available. Another type of three-position pull switch can
flex and extend an elbow and operate either a prehension activator or electric hook. Any of these switches
can be controlled in a variety of ways. Many are fixed to the harness to allow pull by body movements, while
some are pushed by use of the chin or other hand. In the high-level congenital amputee, functional limb
residuals may be used to operate switches. Switch location must be individualized and can be very
innovative (Fig 34C-3.).
Fig 34C-3. This amputee demonstrates the use of
vestigial limbs to operate a powered elbow.

Components

Most of the powered terminal devices for children are hands. There is one powered split hook commercially
available, but this is not often used. There are a number of hands available from different manufacturers.
Some are very small so that the very young can be fitted with an appropriate size. They are built with a
three-finger pinch, the active index and middle fingers opposing the fixed thumb and the remaining fingers
being passive. Although the cosmetic gloves are usually made of polyvinylchloride (PVC), there are some
made of silicone.

Characteristically, the fingers of the hand will open from 3.1 to 6.0 cm (1.34 to 2.36 in.) and will develop a
closing force of 9.9 to 12.1 kg (4.5 to 5.5 lb). The time to cycle the fingers depends somewhat on the type of
control system, temperature, moisture present in the socket, and other factors. The hand usually contains
the motor and other mechanical parts.

Powered elbows for children are primarily switch controlled, although myoelectric elbows are also available.
Elbows are added to a child's prosthesis some time after the terminal device is fitted just as with body-
powered prostheses. Occasionally the elbow will be too fast for small children until they get used to it.
Although cycling the elbow while holding something in the terminal device (live lift) is possible, the elbow is
primarily a positioning device, as is the wrist (forearm) rotator. These components put the terminal device in
a position where it can successfully accomplish a given task (Fig 34C-4.). Electric wrist (forearm) rotators
are available for teenagers. They are also a positioning device, and their weight, along with all the other
components, may make them burdensome for an amputee with a short residual limb. They are not used
nearly as often as hands and elbows. Without them, rotational positioning of the hand is done passively.
Humeral rotation and shoulder motion are done passively with friction joints.
Fig 34C-4. An externally powered elbow.

Interesting combinations of external power as well as external power combined with body power can be
used (Fig 34C-5.). A common combination is that of an externally powered elbow with a body-powered
terminal device. Another possibility is a powered hand on one side and a cable-operated hook on the
other. There are no large series addressing this issue of combinations of power, so fitting these various
components still tends to be individualized and subjective.

Fig 34C-5. A bilateral amputee using a combination of


myoelectric and body power for his prostheses.

The power source most commonly used for these prostheses is a 6-V nickel-cadmium rechargeable battery.
Other voltages are occasionally used. Whenever possible, for cosmetic purposes it is fabricated into the
prosthesis. Because of weight it should be kept as proximal as possible. In long transradial amputees it
may have to be placed in a pod that protrudes somewhat from the prosthesis. In certain selected
circumstances, the battery may have to be placed remotely on a belt or some type of harness. Resistance
to wire breakage with remote units has improved with the advent of new materials (Fig 34C-6.). The number
of cycles before recharging and the eventual life of the battery depends on the number of components it is
powering and the care it is given. A battery should never be completely run down before it is recharged, and
it should not be overcharged. To address this problem, many chargers now have an automatic shutoff when
the battery is fully charged. Typically, it takes about 12 hours to recharge a battery with a 50-mA charger. To
avoid the hysteresis effect a battery should be discharged sufficiently before it is recharged. With good care
a battery will last about 2 years. Extra charged batteries should be available for uninterrupted use.

Fig 34C-6. A myoelectric prosthesis fabricated with the


battery enclosed and one with the battery placed remotely.

Fabrication

In fabricating externally powered prostheses it is important that the prosthetist be familiar with the
prostheses and well trained in their fitting and repair. The placement of control sites, switches, and
batteries, in particular, may be highly individualized. In general, weight, (especially that of the battery)
should be as proximal as possible. The prosthetist should be skilled in the fabrication of self-suspending
sockets allowing the amputee as much range of motion as possible while maintaining adequate suspension.
Because of the weight of the components, suspension is especially important for powered prostheses. The
flexible socket has been very helpful for the high-level amputee and seems to give superior suspension.
Care must be taken to avoid "overgadgeting" amputees, especially children, to avoid frustration and
prosthesis rejection.

ADVANTAGES OF EXTERNAL POWER

The major advantage of externally powered prostheses for below-elbow (transradial) amputees is the ability
to combine cosmesis with function (Fig 34C-7.). This feature appears to be of major importance to the
parents of young children where the child has little or no input into the selection of the prosthesis. Another
major benefit in these prostheses is the lack of harnessing straps and control cables. Beside being
uncomfortable at times, the straps and cables detract from the appearance of the prosthesis. Additional
advantages claimed by amputees are ease of operation, comfort, the ability to operate the prosthesis in any
position, and superior grip strength, especially when compared with voluntary-opening devices.
Fig 34C-7. This child uses her myoelectric prosthesis in her
play activities.

With above-elbow (transhumeral) prostheses using an externally powered elbow, "live lift" is possible. In
children with limited shoulder or chest excursion, an externally powered elbow allows the available
excursion to operate a body-powered terminal device.

Patients have shown a tendency to prefer the externally powered hand when grasping larger objects but to
prefer the body-powered terminal device when handling small or flat things. This may be related to a
number of factors including the larger grasping surface of the hand, the greater friction of the glove, the
ability to use visual cues, and the bulk of the hand.

An uncommon but definite advantage of the powered prosthesis is found in the fitting of amputees whose
residual limb is extensively scarred, particularly in areas where suspension straps or a control cable can
exert excessive friction or pressure, as in the axilla. These can be eliminated if electrode placement can be
successfully carried out. The same rationale applies to the amputee who lacks the body excursion or
strength to operate and control a body-powered prosthesis but can activate a switch or electrode in an
externally powered component. The externally powered prosthesis will provide a superior prehension
force, which is particularly useful in high-level amputees who lack the power to operate a terminal device
successfully (Fig 34C-8.). It should be noted that operation of externally powered components requires less
energy expenditure than operation of body-powered components does, which is, again, especially important
for the high-level amputee.
Fig 34C-8. A high-level congenital amputee fitted with an
externally powered elbow and hand.

DISADVANTAGES OF EXTERNAL POWER

Many of the advantages of externally powered prostheses are subjective and dependent on amputee
response. The disadvantages, on the other hand, are more objective and specific.

One of the biggest problems still to be solved in externally powered prostheses is that of durability of the
various components. Through years of research and improvement, the transradial myoelectric system has
achieved a high degree of reliability and durability. The electrical system rarely fails, although when it does,
it is expensive to repair. However, the use of these prostheses demands certain restrictions of an amputee's
activities, a feature that is particularly cogent when considering very young children. For example,
myoelectric prostheses cannot be immersed in water. They cannot be used to hammer, to pry objects, or to
play in water without some risk of damage to the device.

By far, the weakest link in this system is the cosmetic glove. Much work needs to be done to improve its
durability. It tears quite easily and becomes soiled. Certain stains such as ball point pen ink and newsprint
are virtually impossible to remove. The cost of replacing cosmetic gloves is significant (Fig 34C-9.).

Fig 34C-9. A cosmetic glove showing the soiling due to


normal use.
Batteries need frequent recharging and periodic replacement. They are still quite heavy and, if they cannot
be built into the prosthesis, must be located remotely. The thumb axis and hand frame commonly need
repair and realignment in children.

One series of 47 children wearing myoelectric transradial prostheses required 1.9 repairs per year, and this
group included only a few very young children. Very young children do not understand that they must
make certain concessions to avoid damaging these devices.

The durability of externally powered elbows has been rather poor to date. Over a period of time, this has
improved significantly, but the incidence of repair due to breakdown in children is still rather high and
frequently means that the amputee will be without a prosthesis while the repairs are being made. Many of
the powered elbows are not available in children's sizes and tend to be heavy.

The weight of externally powered prostheses is a frequent complaint of amputees using them, particularly
those with short residual limbs. It does not appear to be a frequent cause for rejecting the prosthesis, but
it is a common source of dissatisfaction. In addition, much of the weight is located distally in the limb, and
thus an even greater force must be overcome when using the prosthesis. The greater the number of
components used, the heavier the prosthesis becomes. This is particularly relevant for the higher-level
amputee and for small children.

It has been stated by some that proprioception, which is limited with any prosthesis, is poorer with externally
powered prostheses than with body-powered prostheses, although this is disputed by others. In tests
done on measured tasks, externally powered prostheses have been shown to be twice as slow as body-
powered prostheses and five times slower than the normal or nonamputated limb.

While large-grasp functions with the externally powered hand are equal or superior to split hook terminal
devices, they appear to be inferior when used for fine motor activities or manipulating small objects.
Powered hook-type terminal devices for children are rarely used, and only one model is commercially
available.

Amputees at times will complain of inadvertent cycling of a powered component. Because of the bulk of the
hands, it is sometimes difficult to get the prosthesis through the sleeves of garments.

A very important factor to consider in prescribing externally powered prostheses is the cost, both initially
and for repairs. Although this varies in different parts of the country and is dependent on the type of
prosthesis, the minimum cost for these devices is several thousand dollars. There has been reluctance on
the part of many third-party payors to assume the cost of externally powered prostheses. An additional
problem in children who are still growing is that this expense will have to be repeated as the child grows out
of the prosthesis. This growth factor can be somewhat ameliorated by using socket liners. In clinics fitting a
number of children, establishing a limb bank and recycling components can defray the cost somewhat.

At this time, the functions that can be fabricated into an externally powered prosthesis for small children
include prehension and elbow motion. Not yet available commercially for small children are forearm (wrist)
rotation, wrist flexion and extension, upper-arm rotation, or shoulder motion. These are passive only. This
is not an indictment of powered limbs since these functions are not available in body-powered prostheses
either, but one would anticipate that with further research these functions could be made available to child
amputees using externally powered prostheses.

AGE CONSIDERATIONS

At what age should a child be provided with an externally powered prosthesis? Probably no question in the
field of prosthetics in recent years has evoked so much controversy.

It has been shown that children benefit from early (under 1 year) fitting with a body-powered prosthesis in
terms of prosthetic acceptance and use and in the development of bimanuality.
Based on the work of Sorbye and others, there are those who would recommend fitting children at a very
young age with externally powered prostheses. Working in a fully funded program under ideal
circumstances, they showed a high rate of compliance among their patients. Other investigators,
however, have not been able to reproduce those results, and so the controversy continues.

All the evidence would suggest that very young children can learn to operate externally powered
prostheses, so this is not the issue. Age alone should not be the criterion by which a certain prosthesis is
selected. The main requirement to be addressed in selecting a specific type of prosthesis for any amputee
is the patient's needs. These needs have to be met in the most appropriate manner possible. There is no
one type of prosthesis that is optimal for every amputee. To make an intelligent choice and give valuable
advice to the amputee one must be aware of and consider a myriad of factors. These include life-style and
activities of the amputee, available components and their advantages and disadvantages, financial
considerations, availability of knowledgeable prosthetists, distance from a prosthetic facility, availability of
training, characteristics of the residual limb and supporting structures, as well as the motivation,
expectations, and goals of the child and parents. Additionally, in young children, comprehension, strength,
and attention span must be considered. Needs of the amputee change, and one must never be fixated on a
certain type of prosthesis but be flexible and sensitive to the needs of the amputee at a given time in his life.
The ability to evaluate all these factors is one of the advantages of the multidisciplinary amputee clinics.

It is a common occurrence for infants and very young children to wear and use a prosthesis for some time,
even for several years, before they begin to use the prehensile capabilities of the prosthesis in a meaningful
fashion. Fishman and Kruger in their survey of children with myoelectric and body-powered prostheses took
special note of the children who simply wore their prostheses, those who used them functionally, and those
who rejected them. Among the children under 6 years of age there was a much higher percentage of
"passive" wearers, that is, children who wore the prosthesis but did not use it functionally.

In another series of children initially fit with body-powered prostheses there did not appear to be any
difficulties encountered when they were switched from body power to external power at a later age,
providing that the motivation to use external power was present.

In summary, when externally powered prostheses have been developed to the point that they are clearly
superior in every facet of prosthetic care to any other type of prosthesis, widespread prescription will be
warranted, but until then each amputee should be evaluated on an individual basis.

CONSIDERATION OF AMPUTATION LEVEL

The majority of externally powered prostheses have been fitted to transradial amputees. One reason for this
is that there are many more child amputees in this category than there are at higher levels, whether
acquired or congenital. Another reason is that the externally powered transradial prosthesis is functionally a
better prosthetic device than the prosthesis for high-level amputees. The reasons for this relate to the
available components and particularly to the residual-limb characteristics and the number of functions that
need to be replaced. Unfortunately, the amputees with the greatest need (i.e., the higher-level amputees)
are the least served by external power due to technological shortfalls. Because of this, the rejection rate
and incidence of failure to use these prostheses are highest in this group. It is hoped that future research
will solve these shortcomings and provide these amputees with a better, more useful assistive device.
Initially, the following observation will be directed at unilateral amputees. Bilateral amputees will be
specifically discussed later.

Historically, the transmetacarpal level of amputation has been a difficult level to fit with any type of
prosthesis. The incidence of rejection is high. In amputees of this level, length usually is not a significant
problem because the amputated limb is almost as long as the nonamputated limb. In addition, they retain
that marvelous sense that any prosthetic device eliminates, namely, sensation. With retained carpal and
metacarpal segments, they have some wrist and hand motion and lack only finger prehension. This function
is usually accomplished with the opposite hand. Externally powered prostheses have practically no use at
this level because, with the addition of the usual components, the forearm becomes disproportionately long
and also because the function of the residual limb at this level, on balance, is equal or superior to that of a
prosthetic device.

At the carpal level, an externally powered prosthesis, usually myoelectric, can be fabricated without creating
an unsightly long arm. Some of the same prosthetic problems mentioned above are encountered,
specifically in regard to length and sensation, especially if some of the carpal elements are retained. One
advantage of this level is that suspension can be achieved with a modification of the expandable wall
socket, thus eliminating the need for a Miinster-type socket and allowing the amputee to use whatever
forearm rotation remains while retaining full elbow flexion and extension.

The transradial is by far the most common and most successful level of amputation that utilizes external
power (Fig 34C-10.). Components for children are readily available. The powered hand with a three-finger
pinch is the most common terminal device used. Only one powered hook-type terminal device is available
for small children, and this is seldom utilized. Suspension is usually achieved by means of a modified
Munster socket, although this socket configuration eliminates any residual forearm rotation. The prosthesis
can be donned either by simply inserting the residual limb or with the use of a pull sock. The amount of
elbow flexion varies with the height of the anterior trim line, which in turn, depends somewhat on the length
of the residual limb and the purchase necessary to stabilize it.

Fig 34C-10. A transradial amputee with a myoelectric


prosthesis.

Long transradial amputees may have problems in concealing the battery pack so that it will not protrude
from the volar aspect of the socket. Conversely, amputees with very short residual limbs, such as in the
short transverse deficiencies, may have problems with suspension and in supporting the weight of the
prosthesis. Midforearm length is the ideal length for a myoelectric prosthesis. Forearm rotators are available
for the larger child, but clinically they are not used a great deal since they add weight and battery drain.

Elbow disarticulation amputees again have problems with relative residual limb length in that the space
required for an electric elbow will create an excessively long upper-arm segment and an asymmetrically
short forearm segment. As children assume adult proportions in their adolescent years, they are able to use
adult components, and this increases their options for external power.

The long to midshaft transhumeral amputation is ideal to accommodate an electric elbow. If the humeral
segment is long enough, they will have functional shoulder motion and be able to support the weight of the
prosthesis. With the longer residual limb, selection of control sites, especially for myoelectric use, is also
easier (Fig 34C-11.).
Fig 34C-11. An amputee with a long transhumeral
residual limb.

High transhumeral, shoulder disarticulation, and forequarter amputation levels have numerous problems:
the rejection rate is relatively high, and functional use is diminished. Weight is a problem, particularly in
small children because the surface area available for body support of the socket is reduced (Fig 34C-12.).
Location of adequate myocontrol sites may also be difficult. Training may be harder because these sites are
situated on muscles that do not normally control the comparable prosthetic function. Sockets for these
levels tend to get bulky and oppressive. They can become hot, although this can be alleviated somewhat by
fenestration. The flexible socket and frame have also been helpful for these levels. It is felt by many
amputees to be more comfortable, to give better suspension, and for some reason, to be cooler. Shoulder
motion at these levels is only passive. A powered shoulder has yet to be made available.

Fig 34C-12. This externally powered prosthesis for a


shoulder disarticulation amputee is bulky and heavy.
Successful fitting of the bilateral amputee, at any level, with externally powered prostheses has been
disappointing to date. There is an extremely high rejection of external power by these amputees who are so
dependent on their prostheses. In addition, bilateral amputees usually reject prosthetic hands and prefer
alternative terminal devices. This choice on their part probably relates to the comparative weight, durability,
reliability, and ease of operation of bodyand externally-powered types of prostheses.

MISCELLANEOUS CONDITIONS

Congenital amputees often present the clinic team with unique characteristics that require imagination and
ingenuity to fit prosthetically. Vestigial limbs, as in the case of high-level transverse deficiencies (phocome-
lia), may be used to control microswitches or myoelectric controls (Fig 34C-13.). In some cases, socket and
suspension fabrication may have to be very innovative. Although congenital amputees are classified
prosthetically as to certain functional levels, the unusual features of their vestigial limbs frequently present
opportunities to fabricate a unique prosthesis. It is always better to customize the prosthesis to meet the
needs of the amputee than to modify the amputee to fit a preconceived prosthetic design.

Fig 34C-13. This congenital amputee is able to operate the


controls of his prosthesis with his vestigial limb.

Various authors recommend using a myoplastic closure in upper-limb amputations to facilitate externally
powered control. This is easily accomplished in elective amputations, but in traumatic amputations this
consideration may have to be sacrificed to the maintenance of length and skin coverage. There are other
special considerations for amputations in children, but they are covered elsewhere in this text. However, it is
worthwhile to mention again, in the context of externally powered limbs, the importance of retaining length
and normal skin coverage, even if it means shifting skin flaps. This is done to cope with the weight and
control site placement requirements of externally powered prostheses. Another surgical consideration is the
decision about the fate of vestigial limbs or residual-limb anomalies. In general, they should not be ablated
unless they have demonstrated themselves to be detrimental to the amputee's rehabilitation or prosthetic
progress or unless they can be shown, after very careful consideration, to be of no value to the amputee
(Fig 34C-14.).
Fig 34C-14. This high-level quadrimembral congenital
amputee can operate the control of her wheel chair
with her vestigial foot.

TRAINING THE CHILD AMPUTEE WITH EXTERNALLY POWERED PROSTHESES

It has been well demonstrated that children of all ages, including toddlers, can be trained to operate
externally powered prostheses.

The training method comprises several phases. These include testing for controllable myoelectric signal and
training in the care, maintenance, control, and finally, the functional use of the powered prosthesis.

Although very young children can be taught to operate an externally powered prosthesis, much of the
responsibility for care, maintenance, and actual training will fall to the parents, who have to be motivated to
accept this role. In fact, it has been our experience that the motivation of a very young child with any
prosthesis generally resides primarily in the parents, gradually giving way to the amputee as he
incorporates the prosthesis into his normal living patterns. In addition to this, certain concessions have to be
made to the very young child because of his age, attention span, and conceptual abilities. These will be
referred to specifically as the process is described. The format followed is similar to that followed in the
training of an amputee with any type of prosthesis, except that location and control of the switching device
is unique to external power.

In fitting with a myoelectric prosthesis, location of control sites and muscle training is done by the pros-
thetist, therapist, or both. A myotester is used to accurately locate the optimum control sites. The muscles
to be used are palpated, and then the test electrode is moved until a maximum response is obtained on the
myotester. In very young children, the use of toys that move when activated by the myoelectric activity of
the child should be substituted for the myotester.

The amputee must learn independent contractions of muscle groups and, in the case of one site-two
function controls, differential or graded muscle contraction. This can be facilitated by the use of movements
of the phantom hand, although this is not available in very young or congenital amputees. One site should
be taught at a time, and after they are all learned, combined function can be taught.

Training sessions should be kept to 30 minutes or less, depending on the attention span and endurance of
the child. These sessions may have to be very short for the young child but can be supplemented later by
watching the child play and encouraging him to use the prosthesis in his play activities. Short training
sessions avoid muscle fatigue and soreness.
Other factors to consider are the developmental age of the patient, his ability to follow instructions, and the
complexity and speed of the prosthetic components. While terminal devices can be fit at a very early age,
elbow function, when needed, is usually added somewhat later.

Training time will vary, although most amputees can be taught to generate signals, and this does not seem
to depend on the length of time since the amputation. In the young child the parents should be incorporated
into the training program at the onset since their participation and cooperation is essential to a favorable
outcome.

Once the prosthesis has been fabricated, its care and maintenance should be thoroughly discussed with the
amputee and the parents. The location and function of each component of the prosthesis should be fully
described. The amputee or parents are then instructed on how to don the prosthesis. This is done by
inserting the residual limb, although sometimes a pull sock can be used to facilitate entry. If there is difficulty
in inserting the residual limb, compounds such as talc or surgical lubricant can be used. The amount of time
the prosthesis is worn each day should be gradually increased. The residual limb and socket should be
cleaned each day to avoid irritation and odor. At the first sign of any significant irritation or breakdown,
prosthetic wear should be discontinued until the problem is rectified. Various compounds are available to
control odor or excessive sweating. Antiperspirant sprays are sometimes used successfully. Wearing a
prosthesis in summer can be exceedingly hot, and it is not uncommon for the unilateral amputee to
discontinue or reduce prosthetic wear during this season, especially if he is on vacation from school. This
should not be a cause for undue alarm or fears of prosthetic rejection.

Cosmetic gloves are made of PVC. These gloves are quite easily stained or torn. Stains from ball point
pens and newsprint are particularly hard to get out. Gentle soaps and hand lotions can be used to clean
and keep the glove supple. The gloved terminal device can be immersed in water only if the glove is intact
with no cuts or tears. If additional protection of the cosmetic glove from soiling or staining is needed,
another ordinary glove should be worn over it. Rechargeable nickel-cadmium batteries are used to power
the prostheses. The length of time they will keep a charge or their longevity is dependent on use, but in
general a charge will last about a day, and the battery lasts about 2 years. Batteries occasionally can be
totally discharged but should be recharged promptly. When the powered component slows down or
operates erratically, the battery should be removed from the prosthesis and charged. Normal recharging
time is about 12 hours. Activities that cause excessive jarring of the prosthesis should be avoided. Stress
the fact that the prosthesis is a helping hand.

After the operation of the individual components of the prosthesis has been learned, the next step is to
apply this to the control of the assembled prosthesis on the amputee. This phase of training focuses on
accomplishing individual tasks with the prosthesis. Amputees learn to operate the components in various
positions. They learn that the elbow and wrist (forearm) rotators are primarily positioning devices so that the
terminal device can accomplish the task in the best possible position. Object training can then be started
beginning with grasping objects of different shapes and sizes and moving them from place to place and
then progressing to objects of varying densities and learning to moderate the force of grasp. Children over
the age of 5 years can follow this pattern. Age-appropriate games and toys are useful in the very young
child. Teenage boys frequently respond to challenges to accomplish various tasks. In multifunctional
prostheses, each function should be learned individually and then combined or alternated. Basic control and
function must be learned before proceeding to the functional use of the prosthesis. It is at this point that
parents can be effectively used as an effective and cost-efficient extension of the training program since
they spend much more time, especially with the young child, than the therapist can.

In the final phase of training, these learned functions are extended to include the more complex activities of
daily living at whatever level is appropriate for the child's age. It is here that individuality should be stressed
as well as the interaction of one limb with the other (e.g., stringing beads). Parents are invaluable in giving
encouragement and suggestions to the amputee on how he might use his prosthesis in daily activities.
Interaction and playing with siblings is helpful. Children 5 years and older can be taught to dress
themselves, eat independently, and perform various other tasks. Older children can be instructed in the use
of the internal hand switch, the method of "live lift" with a powered elbow, and how to "troubleshoot" simple
malfunctions of the prosthesis.

RESULTS OF FITTING WITH EXTERNAL POWER

Fitting amputees with externally powered prostheses has previously been more prevalent in Europe and
Canada than in the United States, although it has become much more common here in recent years.
In one study in a group of 40 children using transradial prostheses with external power, only 2 children
rejected the powered prosthesis and preferred the split hook. It should be noted that all these children are
unilateral amputees and that half of them have been monitored for 1 to 3 years. No one else has shown this
high rate of success. More typically, acceptance rates for transradial powered prostheses is from 60% to
90%. Many of these studies are difficult to compare because of different variables in the study. In an
interesting study done by Fishman and Kruger with a 3-year follow-up, of 120 children, 44% preferred the
myoelectric prosthesis, 34% preferred the body-powered one, and 22% rejected all prostheses. They also
noted that 68% were active users of their prostheses and 32% were passive wearers. H.J.B. Day found
that in a study done on young children, only 25% actively used their prostheses and the rest wore the
prostheses passively. Fishman and Kruger's study had 23 very young children, and the rejection rate
among these children was higher than age average, which casts some doubt on the premise that the earlier
the children are provided with external power, the less likely they are to reject it. The ability of the child to
have the cost of his prosthesis underwritten probably also significantly affects whether or not external power
is continued.

Bilateral amputees are obviously very dependent on their prostheses. The results of fitting them with
external power, if they have a choice of body power, has been disappointing.

In higher-level amputees, that is, transhumeral and proximal, the rate of rejection also varies from series to
series, although, if anything, it tends to be somewhat higher than in transradial amputees. The needs of
the higher-level amputees are more complex and with current prostheses are not as well served as are the
prosthetic needs of transradial amputees. Few studies of children with higher-level amputations have been
done, but in one a 50% rejection rate was encountered. At the very high levels (very short transhumeral,
shoulder disarticulation, or forequarter), rejection is high among unilateral amputees. Use of prostheses,
whether body powered or externally powered, is poor in small children with very high-level limb loss, as for
example, phocomelia or amelia, whether unilateral or bilateral. The prostheses are heavy, cumbersome,
and hot for these little children with a small body mass, as well as awkward and imprecise for them to
operate. In addition, their parents will frequently look after their bodily functions, or they become particularly
adept with the use of their feet if they have usable lower limbs. Available funds would be better utilized, at
least initially, for adaptive equipment for this group of small children rather than spending them for externally
powered prostheses, except perhaps in research situations.

Upper-Limb Deficiencies: Developmental Approach to Pediatric


Upper-Limb Prosthetic Training
Joanna Grace Patton, B.S., O.T.R.

*This chapter is adapted from material presented in Comprehensive Management of the Upper Limb
Amputee, published by Springer-Verlag. Chapters in that book by Joanna Grace Patton entitled
"Developmental Approach to Pediatric Prosthetic Evaluation and Training" and "Upper-Limb Prosthetic
Components for Children and Teenagers" are used as a basis for this format. The author wishes to express
appreciation to Springer-Verlag for permission to use the selected content.

THE CHILD WITH A UNILATERAL TRANSVERSE FOREARM DEFICIENCY

Child Development-Foundation For Early Prosthetic Fitting and Treatment

Principles of child development are important as a foundation for evaluating, prescribing prostheses, and
training children who have limb deficiencies or acquired amputations. The staff at the Child Amputee
Prosthetics Project (CAPP) at both the University of California at Los Angeles and Shriners Hospital for
Crippled Children, Los Angeles Unit, adhere to this philosophy of care. They feel that pediatric prosthetic
treatment is not and should not be a duplication of adult treatment.

Sypniewski reviewed the literature in 1972 and reported on the various rationales for early upper-limb
prosthetic fitting for children. Early fitting is clearly cited as an important concept. However, there is and
always has been a difference of opinion about which developmental milestones are most appropriate as a
basis for planning treatment. Clinicians still debate what is meant by "early fit." In a 1976 article by Fisher,
she discussed both the definition and practice of early fitting. She cited research that supports the belief
that the development of visually guided reaching is dependent on the opportunity to see the limb moving in
space. She questioned whether fitting the baby before 3 or 4 months of age would aid visually guided
reaching and thus influence future prosthetic wearing and use patterns.

These same questions concerning when to fit, what to fit, and how to do training will continue to have an
impact on both health professionals and the general public in the 1990s. For example, the development of
myoelectrically operated components for children in the 1970s and the manufacture of lightweight electric
hands and miniature circuits are changing the prescription and training process. In selected centers
that have well-established myoelectric programs, babies between 4 and 15 months of age are receiving
their first myoelectric prosthesis. The approach has gained media attention and generated much interest
and controversy among professionals and families who have limb-deficient children.

At CAPP, the first prosthesis with no active control is fit when the baby achieves independent sitting
balance. Since the baby has progressed beyond the stage of rolling, the prosthesis is less likely to
hamper movement. The practice at CAPP is based on clinical experience as well as a study by Brooks and
Shaperman that identifies the developmental period prior to 2 years of age as a good time to fit the first
prosthesis. In fact, children who received the prosthesis before the age of 2 years develop better wearing
patterns and skills than do those children who received one between 2 and 5 years of age. Most
clinicians agree that fitting the first prosthesis when the child is entering the "terrible twos" can be a very
negative experience for everyone and should be avoided when possible.

Family's Introduction to the Prosthetic Program

Early intervention and support are most important for families. They are encouraged to see the clinic team
as soon after the baby's birth as possible. Some parents are totally overwhelmed by the birth of their child
with a limb deficiency, while others seem more able to cope with the birth crisis. The early opportunity to
discuss feelings and concerns is most valuable to the family. They also need to receive appropriate
information about the baby's limb deficiency and future treatment. Parents are asked to bring siblings and
extended family members so that the CAPP staff may provide the following types of information:

1. Reassure the family that the baby with a transverse forearm deficiency has the potential to develop
normally unless there are other medical or neurologic problems unrelated to the limb deficiency.
2. Explain that the child will be able to perform most activities of daily living, including self-help,
school, recreational, vocational, avocational, and household tasks with or without a prosthesis.
Skills will develop because of the child's natural abilities and interests.

3. Reassure the family that the child should be treated the same as other siblings or children in terms
of performing daily tasks, receiving discipline, and enrolling in regular school.

4. Provide accurate information about prostheses, and clarify misconceptions about state-of-the-art
technology. Explain how the child performs activities with and without a prosthesis.

Parents frequently ask whether a prosthesis has a real value. Certainly a child can use substitute grasp
patterns like clasping an object under the arm or against the body with the residual limb. With the
prosthesis, the child can hold the object away from the chest and at the midline of the body. Then he is able
to complete the more complex part of the task with the sound hand.

Families need time to assimilate the information provided by the team because they are the ones who must
make informed decisions concerning the prosthesis for the child. When parents have the opportunity to
select the type of terminal device they want, they may be more committed to the total program. Even though
the team provides information, support, and training, the parents must follow through on a day-to-day basis.
They help the child develop both a consistent wearing and use pattern when performing daily activities.

Infant Prosthesis/No Active Controls

At CAPP, the prosthesis for the infant is a lightweight endoskeletal or exoskeletal transradial (below-
elbow) prosthesis with a nonactive terminal device. An infant chest harness is used instead of the traditional
figure-of-8 harness (Fig 34D-1.). A polyvinylchloride (PVC) gauntlet covers the socket to provide a friction
surface. It prevents the baby from sliding when the prosthesis is used for support on a hard surface.
Parents are given the choice of a CAPP Terminal Device No. 1 (CAPP TD), a Dorrance 12 P hook, a
Steeper foam-filled infant hand, or a Centri infant hand. The advantage provided by the CAPP TD and hook
is that they are both able to hold an object. In addition, the CAPP TD with its large grasping surface and
good friction cover provides a secure hold on objects. Even though the little passive hands provide no
grasp function, families do request them because of appearance.

Fig 34D-1. The child wears a transradial prosthesis


with an infant chest harness and an infant passive
Centri-hand.

Evaluation of and Training With the New Prosthesis

The prosthesis should be evaluated when it is delivered to the patient to make sure that it conforms to the
prescription and standards of the clinic. The occupational therapist observes fit, comfort, and function of the
prosthesis during normal use. By watching the baby move and play, the therapist evaluates the stability of
the socket and harness. Any signs of restriction or discomfort are reported to the prosthetist so that the
necessary changes and adjustments can be made.

The therapist sees the baby and family two or three times during the first month to provide both information
and support as well as tell parents how to do the following tasks:

1. Correctly apply and remove the prosthesis.


2. Maintain the prosthesis in good condition by washing the harness, clean the inside of the socket,
and use clean stump socks each day.

3. Encourage the baby to use the prosthesis in normal play activities.

4. Learn to recognize when the socket and harness are tight. Take the child to see the prosthetist for
necessary adjustments.

Grandparents, siblings, baby-sitters, and other extended family members are encouraged to attend one or
more sessions. Their role is vital in cooperating with parents to establish the development of a consistent
wearing pattern. It is reasonable and desirable for the prosthesis to be worn the entire time the baby is
awake. It may be removed when the baby sleeps, takes a bath, or swims. "However, wearing patterns
may vary with climate changes and individual parental needs."

When the baby receives the first prosthesis, his body and arm movements may be awkward for a few
weeks or so. The family need not be overprotective, but should provide assistance if the prosthesis
becomes pinned under the baby's body or caught in furniture. The baby is encouraged to include the
prosthesis to stabilize body weight when creeping on all fours (Fig 34D-2.) or when pulling to stand.
Large balls or stuffed animals are presented so that the child learns to clasp between the sound arm and
the prosthesis (Fig 34D-3.). Parents are also asked to place a toy or cookie in the terminal device. The 8-
to 12-month-old may try to remove the item or totally ignore it. Over time, as the parents continue to place
objects in the terminal device, the toddler will become aware of the holding function and mimic this
behavior. Gesell and Ilg describe a similar developmental activity in which babies learn to place cubes in
and out of a cup. This adaptive behavior becomes more meaningful and engrossing as the baby gets closer
to 18 months of age. To make it easy for the toddler to open the terminal device, the CAPP TD should
have a soft spring. Since the manufacturer issues the terminal device with a regular spring, the prosthetist
should be reminded to make the change.

Fig 34D-2. The baby uses the prosthesis to support


body weight.

Fig 34D-3. The toddler includes the prosthesis to clasp


a large toy.

Readiness to Activate the Terminal Device-The CAPP Approach

At CAPP the control line or cable is added to the transradial prosthesis when the child demonstrates certain
developmental behaviors that indicate the ability to learn how to use an active terminal device. The
readiness criteria are as follows:
1. The child is able to follow simple directions that have no more than two steps.
2. The child demonstrates an attention span of at least 10 minutes.

3. The child demonstrates an awareness that the terminal device can hold an object and attempts to
open it with the sound hand.

4. The child shows an interest in activities that require the use of two hands.

5. The child shows some willingness to be handled by the parent or therapist.

Some of these behaviors become apparent near the child's second birthday and are based on principles of
development. Pulaski says "the child at two makes a transition from sensory motor experience to mental
activity." The mental activity is demonstrated by the child's ability to follow simple directions and to
understand cause and effect. According to Gesell et al., the 2- to 2½-year-old has the beginning of
interest in constructive activity such as building with blocks and fitting simple toys together. He also has an
elementary interest in imaginative play. It is therefore appropriate to capitalize on this period of cognitive
and motor development by introducing the active control line or cable. The child integrates the opening of
the terminal device with fine motor bimanual activities and then relates it to everyday play. However,
these cognitive and motor skill changes do take place during the middle of the "terrible twos" when the child
may resist being handled. Sometimes after the control line is added and everyone gears up for the training,
the child is no longer cooperative. No harm is done if the therapist and family remain flexible. They may
postpone the training time or pursue alternative nonstruc-tured training methods.

Evaluation of the Prosthesis With the Active Terminal Device

The control line and figure-of-8 harness are added to the current prosthesis, or a new prosthesis may be
fabricated. The terminal device may be a CAPP TD No. 1, a 10X Dorrance hook, or a child's size
mechanical hand.

The size and contour of the child's anatomy as well as the range of motion and strength of the shoulder
girdle are certainly not the same as the adult's. Therefore the following components and adjustments should
be standard for a child's prosthesis to maximize efficiency and ease of operation.

1. The cross point of the harness is stitched in the center of the back a little lower than C7 rather than
toward the sound side.
2. The control attachment strap and lower axilla loop strap should pass over the lower third of the
scapulae.

3. The CAPP TD needs a soft spring, the 10X hook should have a quarter to half of a rubber band,
and both should open and close smoothly.

4. The cable housing should be lined with Teflon to increase the efficiency of the cable system.

Whenever a new prosthesis is issued, the following care and maintenance procedures are reviewed and
reinforced with the family.

1. Clean the inside of the socket each night. Wash the harness at least once a week.
2. Brush dirt or sand from the pulley system of the CAPP TD. Use an air hose at the gas station if
necessary. Immerse the ball bearing of the hook in alcohol to clean.

3. Change the CAPP TD covers when they wear out. Remove all rubber bands from the hook when
the elastic deteriorates. Replace with new ones.

4. Retread the neoprene lining of the hook when it wears out to maintain complete closure of the hook
fingers.

5. Clean the glove of a mechanical hand with alcohol, a manufacture's glove cleaner, or a special
detergent such as "Simple Green."

6. Adjust the wrist friction so that the terminal device does not inadvertently move during use.

7. Go to the prosthetic shop every 4 months. Have the harness adjusted as the child grows.
Control Motion Training

Controls training or initial training is conducted by the occupational therapist to teach the child to open the
terminal device. The therapist sits behind the child or next to the arm with the prosthesis. The child holds
a toy in the sound hand while the therapist moves the shoulder on the side of the prosthesis into humeral
flexion (Fig 34D-4.). Pressure is exerted by the harness under the axilla on the sound side. The child
usually moves the shoulder or upper part of the body to avoid the pull of the axilla loop. The result is slack in
the control system, which prevents ease of operation of the terminal device. To counteract the problem and
minimize frustration, the therapist stabilizes the shoulder on the sound side and encourages the child to
reach forward to place a toy in the terminal device. The therapist helps with the secure and correct
placement of the toy and extends the child's shoulder to relax tension on the control line to close the
terminal device. Once the child has completed the activity, he may remove the item with the sound hand.

Fig 34D-4. The therapist helps the child to learn the


control motion.

If the child is less inclined to sit at a table to learn the controls, the control attachment strap may be
tightened. When the child bends over or reaches forward toward a toy, the terminal device opens. The
therapist or parent then calls attention to the open terminal device.

The therapist definitely provides "hands-on" assistance during this period. Two or three short training
sessions a week for 2 to 3 months are reasonable to reinforce learning the control motion and to provide a
successful experience for the child. The parents must be included in the therapy sessions in order to assist
the child at home.

Although the focus is on learning the control motion, the child does not respond well to "drills." It is best to
use developmentally appropriate bimanual toys and games in order for the child to relate the control motion
to purposeful play. At CAPP we do not ask the child who has a unilateral limb loss to use the prosthesis
to perform dominant hand skills, but rather to assist the sound hand in two-handed tasks. Appropriate
activities that provide repetitive opening and closing of the terminal device may be used for this phase of
training.

1. Use threaded nesting barrels such a "Kitty in the Kegs" or small jars and bottles with toys or treats
inside. (Hold the barrel or the jar in the terminal device. Remove and replace the top with the sound
hand).
2. String large wooden or plastic beads with a strong cord or leather lace. (Hold the bead in the
terminal device and hold the string with the sound hand).

3. Use fat felt-tipped pens with loose caps. Hold the marker in the terminal device, and remove the
cap with the sound hand to scribble on paper.

To master the basic control motion the child must learn to independently perform the following skills:

1. Open the terminal device, and place an object securely inside.


2. Extend the shoulder to relax tension on the control line to close the terminal device. Time the
closing to prevent the object from falling out.

3. Release an object from the terminal device. Either pull it out with the sound hand, or actively
release it by using the same control motion.

To open the terminal device at the midline of the body the child must use biscapular motion. Some children
learn automatically. Others may need assistance.

Use Training

During use training the child acquires prosthetic skills that facilitate use of the prosthesis as he works to
develop a natural and spontaneous use pattern. While functional use of the prosthesis cannot be
compartmentalized, young children do not assimilate all facets of training at once. Stages of prosthetic
training overlap with each other, and learning takes place over a period of time along with the acquisition of
other developmental skills.

The focus during this period is on the prehensile use of the terminal device. However, the child should
continue to use the prosthesis as a unit to stabilize or support objects (Fig 34D-5.).

Fig 34D-5. The child uses the friction cover on the CAPP
TD No. 1 to stabilize the paper.
Skills that relate to prehensile function are introduced as the child is ready to learn them. Initially the
therapist focuses on the following skills:

1. Place an object securely and accurately in the terminal device. Reposition the object as needed.
2. Refine the size of terminal device opening, especially for small or thin items (Fig 34D-6.).

3. Reposition or change the position of the terminal device as required for different activities.

4. Actively release an object from the terminal device by using the control motion. (Learn to drop the
object on the table or floor. Actively toss the object from the terminal device into space.)

To assist the child to learn specific skills the therapist demonstrates the activity and provides verbal
instruction. In time only verbal cues may be necessary. As new, fine-motor, manipulative tasks are
presented, the child is encouraged to motor-plan and problem-solve for the new activity without assistance.
If the child has difficulty, the therapist asks how the task may be done another way. This opportunity allows
for a trial-and-er-ror approach before the therapist intervenes. Performance will vary depending on the
child's natural abilities and motivation. The therapist may need to do part of a more complex task and then
allow the child to complete the activity.

There are many ways to approach an activity. If the child appears awkward when doing the task, the
therapist should correct the method of prosthetic performance. For example, many children either avoid
prepositioning the terminal device or repositioning an object in the terminal device. They frequently
substitute shoulder motion to place the terminal device in a position of function and need reminders to
perform the task more efficiently. A helpful hint about the CAPP TD No. 1 is to position it so that it mimics
the sound hand.

Although it will always be appropriate to place an object in the terminal device with the sound hand, the
child must learn to actively grasp an item from a surface with the terminal device, especially if the sound
hand is occupied. Likewise, to achieve a fluid movement pattern with the prosthesis, the child may practice
reaching forward with both the terminal device and sound hand to grasp a stationary object in space. It is
best to avoid "one-handed drills" and use a more spontaneous two-handed approach with toys such as, tike
bike, tricycle, rocking horse, see-saw-swing, shopping cart, doll carriage, wheel barrow, rolling pin, and
Play-Doh (Fig 34D-7.).
Fig 34D-7. The child is encouraged to actively grasp the
steering wheel of the truck.

Since the child's work is play, toys, games, crafts, and other purposeful bimanual tasks are used to integrate
these skills into the child's use pattern (Fig 34D-8.). In fact the preschool youngster does well with
activities that encompass imaginary play such as a tea party, washing dishes (Fig 34D-9.), washing doll
clothes and hanging them on a line, planting flowers or seeds in a pot (Fig 34D-10.), grocery shopping, and
dress-up play with costumes and makeup. Playing baseball with a plastic bat and a large ball as well as
outdoor fun on playground equipment is also highly recommended.

Fig 34D-8. The child's work is play.


Fig 34D-9. Imaginary play activity is used to encourage
the prehensile use of the terminal device.

Fig 34D-10. Planting a flower is a fun bimanual activity.


Before the child goes to kindergarten, the therapist introduces or reviews specific prosthetic and self-help
skills. Practice in the following type of activities may help the child to function more independently in the
classroom (Fig 34D-11.).

Fig 34D-11. Practicing school skills.

1. Hold paper to cut with scissors.


2. Open and close glue bottles and jars.

3. Open a milk carton and package of cookies.

4. Stabilize clothing to zip a jacket or button a shirt.

5. Don and doff the prosthesis independently.

6. Begin to learn to tie shoelaces.

The school-age child learns additional skills that are a refinement of prosthetic use. In order to hold a soft or
fragile object in the terminal device, the child controls the pressure grip by maintaining a slight amount of
tension on the control line. This skill is important in order to hold a sandwich or crack an egg without a
mishap. To keep the terminal device closed when bending over or extending the shoulder, the child must
pinch the scapulae together or shrug the harness high up on the back. These maneuvers will help to relax
tension on the control line to keep the terminal device closed to tie shoelaces or shoot a toy bow and arrow
' (Fig 34D-12.).
Fig 34D-12. The child relaxes tension on the control
line as he leans forward to tie his shoelaces.

When the child acquires some degree of skill with the prosthesis, the focus in training shifts to developing
more spontaneity. Although some children become more spontaneous than others, there is no magic to
the training process. Practice and repetition are a definite part of building a habit pattern. While the 2-year-
old delights in repetitive bimanual tasks, the therapist does much to enhance training by being creative.
Parents must be present during training sessions because their involvement and cooperation are essential
for ongoing success. By the time the child enters kindergarten, formal therapy is no longer necessary.
However, at different developmental periods the child may need assistance with specific self-help,
recreational, athletic, and avocational activities. Therefore the youngster should always have access to the
prosthetist, therapist, or other appropriate team member.

Functional Need and Prosthetic Options

As the child grows and develops, function, cosmesis, interests, and skill levels change. The therapist is
usually aware of these needs and may be in a position to introduce different prosthetic components or
recommend changes to existing ones. Early in the use-training period, the therapist or child will identify a
need for more prehension force. The child may become repeatedly more frustrated when he is not able to
hold an object securely in the terminal device as resistance is applied by the sound hand. The child who
has the CAPP TD No. 1 will need to replace the soft spring (Hosmer 71622) with the regular spring (Hosmer
71623). This exchange is made as soon as the child has enough operating force to pull against the
increased resistance. A third and much harder spring is available for the older school-aged child.

The CAPP TD No. 2 with its automatic thumb-locking mechanism and heavy-duty closing spring provides
excellent grip force and function. This component is appropriate for the teenager and adult and will soon
be commercially available.

The Hosmer-Dorrance voluntary-opening hook uses rubber bands to provide grip strength. For the young
child, a quarter to half of a band may be added at a given time according to the youngster's available
excursion and operating force. Some preteen and teenage boys may actually switch to a hook from some
other device because they like the configuration, the fine tip prehension, and the option of adding rubber
bands to increase grip strength. The hook is especially versatile for grasping the handle bars of a bike, for
tool use, and for heavy-duty work.
Voluntary-closing Adept terminal devices from Therapeutic Recreation Systems (TRS) also provide a variety
of benefits. Crandall and Hansen report a study in which 16 amputees out of 20 with transverse forearm
deficiencies who originally used voluntary-opening hooks switched to the Adept devices. The children cite
specific advantages for themselves.

1. The voluntary-closing device provides increased prehension force as well as greater control over
the amount of force exerted to do an activity.
2. The voluntary-closing device makes it easier to lift heavy objects and to grasp cylindrical shapes
such as a bicycle handlebar and a baseball bat.

Electric hands that are myoelectrically controlled provide excellent grip force, ease of operation, elimination
of the cable and harness system, as well as good cosmesis. Use of this type of transradial prosthesis
usually depends on available funding resources. Because myoelectrically-operated prostheses are more
expensive than the cable-operated ones, state agencies and some insurance companies may not routinely
pay for them. CAPP does not have a myoelectric program for infants. However, when funding is available,
we use the Otto Bock, two-state, two-site control system and the appropriate electric hand to fit children as
young as 3 years of age.

An increasing number of parents will not accept any other terminal device except a hand. Therefore the
child's-size mechanical hands from Steeper, Ltd., may be pleasing in appearance and may be acceptable
as an alternative to other devices (Fig 34D-13.). Unfortunately, the hands do not have the same power
pinch as the electric hands and do not provide the same potential for function as the CAPP TD No. 1 and
the Dorrance hook. The child must use excessive operating force to achieve only minimal opening.
Depending on the spring setting, the Steeper 2-in. hand may not close completely. For the 2-year-old who
is just learning active operation of the cable-controlled terminal device and for the therapist doing the
training, this hand may provide more frustration than function.

Fig 34D-13. This school-aged child wears the Steeper 2¼


-in. mechanical hand.

Older youngsters who are involved in school or community-based athletic programs are usually required to
remove the prosthesis for body-contact sports. For certain activities, the youngster may wear the socket,
but the terminal device is perceived to be a problem. The TRS Super Sport Hand can be used as an
alternative. "This device is made of soft, flexible polymer and is shaped like a "cupped hand."

The type of prostheses and components that are offered in a particular amputee center may depend on the
following:

1. The center's history and experience with certain components and control systems.
2. Research components developed in a particular center.
3. Available financial resources and subsidized funding for prostheses and components.

Nonetheless, patients and their families have an ongoing need for up-to-date information about new and
available components and should have some say in the prescription process. Over time, if the patient
and/or family decides not to pursue prosthetic fitting, the clinic team should accept that decision and keep
the "door open" for future assistance.

THE CHILD WITH BILATERAL UPPER-LIMB AMELIA

Support and Guidelines for the Family

When a child is born with bilateral upper-limb ame-lia or other severe limb deficiencies, the family needs
emotional support, guidance, and information. As one CAPP mother so poignantly said, "We were in so
much shock we did not know how to act or what to do." An experienced, sensitive team can share the
family's fears and concerns and, over time, help them to refocus on the child's abilities.

The occupational therapist evaluates the infant's development and explains what to expect as the baby
matures. Guidelines for handling the baby's special needs are also presented. For example, babies with
severe limb loss have less skin surface from which to disperse body heat. They are frequently very hot
and perspire excessively around the head and neck. Parents are instructed to use lightweight clothing and
allow the baby's lower limbs to be exposed. The benefits are twofold. The baby remains cooler, and the feet
are free to touch and explore the environment.

The Baby's Ability to Adapt

It is important to understand that a baby with no arms has a structural deficit and not a neurologic
impairment. Unless there is a secondary problem, development should follow a specific sequence
according to the maturation of the neuromuscular system. However there are variations in the
establishment of specific fine and gross motor milestones. Obviously, babies with no arms or with short
transverse deficiencies above the elbow will not be able to creep on all fours. However, these children do
come to a sitting position, scoot on their bottoms, and push to a standing position to walk. Some babies
experience delays, while others perform developmental skills within the normal time frame. Although no
special therapy is needed, the parents need to know when and how to assist the baby through various
stages of neuromuscular development.

Gesell describes adaptive behavior "as the child's ability to make adjustments in perception, orientation, as
well as manual and verbal skills, which then allows him to initiate new experiences."

With neuromuscular maturation and voluntary control over body movements, the baby explores ways to
move in space and to manipulate objects in the environment. With encouragement, the baby may learn to
use the parent's leg as support to push to an upright position. Later the child may come to standing from a
prone position by pushing up with head and legs or use the body as a lever against a stationary object
(Fig 34D-14.).
Fig 34D-14. The toddler uses the body as a lever to
push to a standing position.

Children with high-level upper-limb loss learn very early to substitute foot use for missing arms. Schmid
says "that the foot skills of these children develop in a sequential pattern which imitates that of the upper
extremity. Even grasp between the great and second toe occurs within the same time frame as the grasp
between the thumb and finger." In addition to using the feet, children also hold and carry objects in the
mouth or between the chin and shoulder.

Rationale for Prosthetic Fitting

For the child with bilateral upper-limb amelia, use of prostheses is presented as an option or an alternative
way to perform selected activities. The team provides the family with information on appropriate
components and discusses the real function of prostheses. Before a prosthesis is prescribed, the following
questions must be addressed:

1. Does the child have a functional need for a prosthesis? Will the prosthesis interfere with the child's
development?
2. Is the child able to use the components and control systems currently available?

3. Do the parents want a prosthesis for the child? Are they ready to commit to the training process?

If at all possible, the baby should receive the first prosthesis before the developmental period of the "terrible
twos." It may be prescribed when the baby achieves independent sitting balance as long as it does not
interfere with the ability to move freely. At CAPP we sometimes wait until the child is walking. When the
feet are used for ambulation, the child may be more enthusiastic about wearing the prosthesis.

One or two shoulder disarticulation prostheses may be suggested for the first fitting. One socket covers less
of the chest area and may keep the weight, bulk, and heat to a minimum. However, it may be easier to
suspend two sockets on the body and give the child a greater sense of balance. These options depend
on the child's size as well as the contour and muscle mass of the chest and trunk. Essentially, the clinic
team must determine which fitting will provide the best comfort and meet the child's functional needs.
The First Infant Prosthesis With No Active Controls

At CAPP the prosthetic fitting of choice for the child with no arms is an endoskeletal shoulder disarticulation
prosthesis. It may include an aluminum frame or vented laminated socket and a 10X hook or CAPP TD with
no control cable. The elbow joint is usually assembled from CAPP components, but a new push-button
elbow joint is commercially available from the U.S. Manufacturing Company. The forearm and humeral
segment lengths are made of pieces of PVC tubing (Fig 34D-15.). These segments must be the appropriate
length to allow for the following functions:

Fig 34D-15. The child with an endoskeletal shoulder


disarticulation prosthesis uses trunk motion to push the
toy car.

1. The terminal device must reach the mouth at the midline of the body when the elbow is fully flexed.
2. The forearm must clear the table top when the elbow is flexed at 90 degrees.

The Training Period

After the prosthesis is evaluated for fit, comfort, and function, the parent must learn how to encourage the
child to use it. The daily wearing pattern may be full-or part-time, but the prosthesis should not interfere with
foot use or the development of other gross motor skills. The key, however, is a consistent wearing
pattern. As the parents repeatedly place toys and finger food in the terminal device, the child will learn about
the holding function. Parents will need to assist the child with many of the activities such as unlocking the
elbow to bring food to the mouth.

Since children without arms do not have active shoulder motion, they cannot reach out and clasp large or
small objects between the segment lengths. It is possible, however, to carry a large lightweight toy that is
wedged between the forearms. Likewise, the child must use body motion to push or move an object with the
prosthetic forearm or terminal device. Because the toddler can perform most age-level play activities with
the feet, it takes creativity and perseverance on the part of the therapist and parent to encourage use of the
prosthesis. When the child wears shoes so that his feet are not accessible, the following types of activities
may be used in the training process (Fig 34D-16.):
Fig 34D-16. The pull toy encourages the appropriate
use of the prosthesis.

1. Scribble with a crayon that is secured in the hook.


2. Push or maneuver cars, trains, or other toys on a table surface.

3. Pull a toy with a string that is secured in the hook.

4. Paint in a "Paint with Water Book." Secure a brush in the hook.

5. Hit a drum or xylophone with a mallet secured in the hook.

Rubber band loading on the hook is minimal during this phase of training, and objects may easily fall out
during use. An extra band placed over the hook tip will secure most thin items and prevent them from
slipping.

At 15 to 20 months of age the toddler begins to feed himself with a spoon. Children without arms may
also show signs of readiness to perform this task with the feet. It is difficult to learn to eat with a spoon in
the terminal device, but if there is interest and motivation, the activity may be introduced during this
developmental time period. A swivel spoon is placed in the hook and secured by a rubber band. The push-
button elbow is unlocked and placed in the free-swing mode. The child uses trunk motion to place the spoon
in the dish. Soft food will stick easily. With practice the youngster will learn to scoop food by pushing the
spoon against the high rim of a special dish. To bring the spoon to the mouth, the child leans the forearm on
the table and forces the elbow to flex. This method of eating is very complex and requires not only
practice but assistance from the parent (Fig 34D-17.). The child will not gain independence in this skill for
several years. The method of bringing the spoon to the mouth depends on the control system that is used to
power the elbow unit. The older child who uses either an electric elbow or one with a dual-control cable
system will be able to lift the forearm to the mouth with much less effort.
Fig 34D-17. A swivel spoon is secured in the electric
hook with a rubber band. The therapist assists the
child to flex the forearm to bring the spoon to the
mouth.

Activating the Terminal Device and Learning the Control Motion

The passive prosthesis provides only minimal function for the child without arms. The need to do selected
dominant-hand activities with the prosthesis prompts the activation of the terminal device as soon as the
youngster has the cognitive ability to learn the controls. The terminal device of choice for the active
prosthesis is a Dor-rance 10X hook because it provides fine tip prehension, nonprehensile hooking action,
and a clear view of the objects to be grasped.

The terminal device may be activated with either a Michigan External Power System (electric hook) with a
push-switch control or a body-powered single control cable and a thigh strap. Each system has its
advantages and disadvantages. The team must decide which one will meet the individual patient's needs
and abilities. The Michigan External Power System can be used with the endoskeletal type of prosthesis
and retain the manual elbow unit. The push-button control is placed inside the top of the socket, and the
child hits the button to open the hook. When the child relaxes away from the switch, the hook closes by
rubber band action. This method allows the terminal device to open and close in a stationary position.
Unfortunately, this system provides limited pinch force because the motor tolerates only about three fourths
of a rubber band. If more bands are applied, the motor usually malfunctions. While the hook is easy to
operate, the lack of pinch force soon becomes a problem when toys and other items are easily dislodged.

The other alternative is to use an exoskeletal prosthesis with a single control cable to close the hook. This
type of prosthesis has a nudge control on the socket to lock and unlock the positive-locking internal elbow
unit. Even with a half of a rubber band on the hook, the child does not have sufficient chest expansion to
move the cable. Therefore a thigh strap control is necessary to open the hook. The potential excursion
provided by the thigh strap allows for the following results:

1. Full or at least functional opening of the terminal device without undo exertion on the part of the
child.
2. Potential to add rubber bands to increase grip strength.

3. "Feedback" through the cable system.


In the beginning it may be more time-consuming and a little more frustrating for the child to learn the control
motion with this system. First, the therapist stabilizes the child's pelvis and assists the child to bend the
trunk to open the hook. The youngster then assumes an upright position. Because the terminal device
cannot open and close in the same position, manipulation of objects on a table surface is impossible. The
therapist must place the toy in the hook instead of asking the child to actively grasp it. With practice the
child learns to use shoulder elevation and trunk rotation to open the hook. This refinement allows the
youngster to perform tabletop activities at the midline of the body.

Use Training

Once the control motion is refined, specific skills are integrated into the use pattern. Initially the child learns
to grasp, lift, and carry toys from place to place. Later he will learn to push an object into the desired
position with the unopened hook before attempting to grasp it (Fig 34D-18.). Sometimes an item will slide
across the table when the child tries to grasp it with the hook. To minimize this problem, it is necessary to
place the stationary hook finger against the object before closing the terminal device. Rubber tubing
applied to the stationary finger will also provide a better friction surface. The child works best when
motivated with toys such as wooden puzzles with large knobs, simple card games with cards in a rack, play
food and dishes, as well as small trucks, cars, and trains. The Fisher Price barn, garage, zoo, schoolhouse,
etc., provide imaginary play opportunities that encourage fine prehension and manipulation of objects with
the hook terminal device.

Fig 34D-18. The child positions the object before grasping


it.

Initially, the therapist prepositions all the friction components to place the terminal device in the desired
position of function. The child must master this skill as soon as possible to lessen dependence on adult
intervention. The shoulder, elbow turntable, and wrist must be tight enough to maintain friction and not move
unnecessarily when the child walks, plays, or uses the prosthesis for function. However, these components
must be loose enough for the child to reposition them when necessary. The child pushes either the
humeral or forearm segment of the prosthesis against a stationary object in the environment to move the
shoulder or elbow turntable. To preposition the hook or to secure an object like an eating or writing utensil in
the terminal device, the child frequently uses the foot (Fig 34D-19.).
Fig 34D-19. The foot is used to place the pencil in the
hook.

The child may use the shoulder disarticulation prosthesis to eat, write, and carry objects. Without active
shoulder motion this type of limb provides limited function. In fact, it requires extensive practice to achieve a
degree of skill, speed, and proficiency with this type of prosthesis. Over time, wearing patterns vary. Some
children will always use prostheses to perform selected activities, while other individuals will wear and use
them for only a specific developmental time period.

Activities of Daily Living and Alternative Methods of Performance

The concept of adapted performance is essential to the child with severe upper-limb loss. The prostheses
never provide total independence and were never meant to replace foot skills (Fig 34D-20.) and other
problem-solving methods. Celikyol describes adapted performance "as encouraging the patient to
approach and solve tasks by using the entire body adaptively and to look beyond conventional methods of
arm and hand use to stabilize, grasp, and move objects in space." The occupational therapist helps the
child to participate in activities of daily living by demonstrating adapted techniques and experimenting with
adapted equipment (Fig 34D-21.). An activity may be introduced during the same time frame in which an
able-bodied child attempts the task. The limb-deficient child may accomplish part or all of a task quite easily.
Because dressing and toileting skills require considerable practice, coordination, and effort, the individual
may not achieve independence until the teen years. For some activities, assistance may always be needed.
In addition to experimentation and practice, these children need to maintain slim, flexible bodies. Any
excessive weight gain or limitation or loss of range of motion may compromise adapted performance and
independent function.
Fig 34D-20. Foot skills develop early and are essential
throughout a lifetime.

Fig 34D-21.Adapted performance. This dressing task


requires practice, skill, perseverance, and good range
of motion.

CONCLUSION

Most children with upper-limb loss have the potential to live full and productive lives whether they wear a
prosthesis or not. These youngsters go to regular school, participate in neighborhood and community
activities, and develop skills based on their natural abilities and interests. They respond positively to a
treatment program that provides the following:

1. A developmental approach.
2. An understanding and experienced clinic team.

3. A treatment program that considers the psychosocial, functional, and prosthetic needs of the child
and family.

Fig 34D-6. The child holds a thin object securely in the


terminal device.

Lower-Limb Deficiencies: Surgical Management


Leon M. Kruger, M.D.

BASIC PRINCIPLES

The physiologic differences between children and adults have already been discussed, as have general
surgical considerations and planning for the care of the juvenile amputee. Emphasis should be placed on
early prosthetic fitting and rehabilitation whenever possible. In general, it can be stated that the child with a
unilateral lower-limb transverse deficiency can and should be fitted when he shows any tendency to stand.
One should anticipate that the child will become ambulatory promptly, whether the deficiency is distal or
proximal. By the same token, in unilateral longitudinal limb deficiency, in which it is likely that surgical
revision will be necessary, a treatment plan should be developed so that, if possible, all surgical procedures
can be carried out in one stage. The child should be brought to independent walking as early as possible,
with surgical intervention and resultant scarring kept to a minimum and with the best physiologic restoration
available.

Surgical intervention on the limb-deficient child, particularly on those with longitudinal deficiencies, should
be undertaken only by the experienced orthopaedic surgeon and preferably in those centers that are
accustomed to dealing with these children. By their very nature and fortuitously, these deformities occur
infrequently and therefore are not likely to be seen on any recurring basis in the office practice or general
hospital.
While in the past, limb lengthening for congenital longitudinal deficiencies has been considered and largely
abandoned (with the possible exception of the congenital short femur), the introduction of the Ilizarov
technique of limb lengthening by callus distraction has once again appealed to some orthopaedic surgeons.
It should be pointed out that prior to discussing limb lengthening for longitudinal deficiency of the femur
or fibula, the total predicted discrepancy must be calculated in advance, and the impact that this choice will
have on the childhood of the patient must be considered. The family must recognize that when the child is
just beginning to walk, a lift will be required until the child is old enough for lengthening; that at least 9 to 12
months will be required, during which period the patient will be either in the lengthening apparatus or
immobilized or braced after healing; and that if over-lengthened he will require a lift on the normal side. This
cycle will probably be repeated as he approaches the teenage years until definitive equalization can be
attempted. In effect, this path would occupy the entire childhood. Prior to embarking on such an ambitious
surgical program, the family must completely understand the difficulties they will face. This problem will be
further discussed under the individual deficiencies where lengthening may be an option.

Surgical intervention on these children requires that certain basic principles be understood and applied: (1)
early communication and explanation of treatment concepts to the parents as well as to the pediatrician; (2)
maintenance of muscular development in the residual limb; (3) prevention of progressive deformity,
especially in joints proximal to the deficiency; and (4) retention of all long-bone growth plates.

Early Communication With Parents and Pediatricians

Those clinics caring for limb-deficient children should attempt to establish rapport with pediatricians and
obstetricians so that when such a child is born, the clinic chief will have the opportunity to examine the child
in the newborn nursery. Recognition of the shock, grief, and guilt experienced by the new parents of a child
with one or more deficient limbs dictates the physician's responsibility to assuage these feelings by
presenting to the parents not just the diagnosis or anatomic description of the deficiency, but a positive
approach to the child's immediate status and future capabilities as well. The physician should emphasize
the remaining normal limbs, absence of brain damage, and expectations of the child's physical
development. The new parents should be assured of the child's potential for ambulation, independence of
daily living, and normal mental development. Assistance in these discussions may be sought from the social
worker and pediatrician. If there is a problem with the parents, psychological or psychiatric consultations
may be indicated.

Many parents, on visiting a limb deficiency clinic, are impressed with the athletic accomplishments of
children with limb deficiencies. These parents should be encouraged to raise their child as "a normal child,"
and they should encourage normal physical activity, including any sports activity that the child is capable of
carrying out and in which he is interested. Competitive sports should not be prohibited for a child with a limb
deficiency but, in fact, should be encouraged. Experience has shown that a child who is missing a leg can
play competitive football, basketball, and tennis or, for that matter, any other sport (Fig 35A-1.,A and B).
Even when an individual is missing a hand, baseball, basketball, and golf are not outside his abilities.
Fig 35A-1. A, a patient with a total longitudinal deficiency of the fibula who has had a Syme ankle
disarticulation was fitted with a low-profile Syme prosthesis and is able to play competitive tennis
while wearing her prosthesis. B, no. 13, wearing a low-profile Syme prosthesis, plays field hockey
on her high school team.

It is particularly important to stress to the family that most limb deficiencies occur sporadically and are not
genetically transmitted. Genetic consultation is important, however. In those instances in which there is a
known heritable defect such as deficiency of the tibia, genetic consultation is mandatory. Should
pregnancy occur, parents should also be encouraged to advise the obstetrician of the history and to be
certain that a sonographic study is carried out in the early stages of the pregnancy.

Once out of the hospital, and particularly if surgical conversion of the limb deficiency is anticipated, the
parents should be invited to the "clinic.'' There they should be encouraged to observe older children with the
same or similar deficiencies, and particularly to discuss the child's physical and social development with the
parents of these older children. They should be encouraged to ask questions about the child's participation
not only in family activity at home but also in social, play, and school activities. Concerns about social
acceptance of the deficiency and, particularly, of indicated prosthetic restoration may subconsciously
prejudice a parent against any recommended treatment program. Airing their concerns to parents of other
children with the same problems eases new parents through this difficult transition period and assimilates
them into the clinic team. They become integral members in the planning and implementation of the
program to habilitate their child. Such "group therapy" enables the parents to comprehend the need for and
accept the recommendation to proceed with ablative surgery when it is indicated. Without this open
communication between parents and clinic team, the more complicated problems of the limb-deficient child
may be insoluble.

Maintenance of Muscular Development in the Residual Limb

A congenital limb deficiency may not be simply the absence of a long bone or the peripheral joint distal to
the deficiency; it may also include inadequacy of the proximal musculature and deficits in the skin, nails,
and nerves. Torode and Gillespie as well as Johansson and Aparisi have reported on the missing cruciate
ligaments in patients with congenital short femur and fibula deficiency. Recognizing the deficits in the
proximal musculature at the initial evaluation is important if progressive deformity is to be prevented. To this
end a program must be developed that includes not only institutional physical therapy but also education for
the parents in the techniques of development and maintenance of muscle strength. The parents should be
brought into the physical therapy department for instruction by the therapist in setting up a home program.
Prevention of Progressive Deformity

Prevention of deformity in the child with a lesser deficiency may pose no problem. In fact, even with a major
deficiency such as an amelia, there is no concern with progressive deformity. However, in the more
complicated limb deficiency such as proximal femoral focal deficiency (PFFD) and longitudinal deficiency of
the tibia and fibula, an exercise program for strengthening or stretching of the remaining musculature may
be inadequate to prevent deformity of the remaining joints. Orthotic management for the control of foot,
ankle, and knee deformities can be pursued until the child is ready for definitive surgical conversion (Fig
35A-2.). In many patients, particularly those with PFFD, orthotic management without revision may be
indicated until the optimal time for surgical intervention is reached.

Fig 35A-2. A, a young patient using a caliper orthosis for walking until definitive surgical
intervention. B, after amputation and awaiting her prosthesis. C, this patient with her
transfemoral prosthesis has cosmetically and functionally excellent results. She has since
married and has normal children.

Prevention of progressive deformity is an important ingredient of the long-range planning for these patients.
When surgical intervention is considered, plans should be laid out in such a manner as to anticipate the
result in terms of the adult patient. To this end we must consider the many facets of surgical intervention,
including the retention of all long-bone epiphyses as indicated. On occasion, as in carrying out a knee
fusion in the patient with PFFD, it can be anticipated that the amputation level will leave one with a
prosthetic knee joint that is below the level of the knee joint on the normal side. In such a case,
appropriately planned, the epiphyses may be destroyed. We should also think in terms of the preservation
of functional proximal joints, stabilization of proximal joints where necessary (i.e., knee fusion), and the
judicious use of skin grafting when necessary.

Retention of All Long-Bone Growth Plates

The percent contributions to the longitudinal growth of the distal femoral, proximal tibial, and distal tibial
growth plates have been determined (Fig 35A-3.). Unnecessary sacrifice of any of these three longitudinal
growth centers in the infant or very young child may result in major prosthetic problems in adult life. In some
instances, the sacrifice of such an epiphysis in the very young child can be catastrophic. As an example, the
patient with a longitudinal deficiency of the tibia should be treated by disarticulation at the knee level, with
the distal femoral epiphysis left intact. Assuming normal growth in the distal and proximal femoral growth
plates, in adult life the patient would have a slightly shortened knee disarticulation, which is ideal for
prosthetic fitting. Should the surgeon elect to carry out a transfemoral (above-knee) amputation with
sacrifice of the distal femoral growth plate, it would deprive the child of 70% of the eventual length of the
femur, and in adult life the patient would have a very short transfemoral residual limb. Disarticulation
ensures a long residual limb with all of its advantages in adult life.

Fig 35A-3. Epiphyseal contributions to longitudinal bone growth. Percentages represent


contribution of each epiphysis to the total growth of each bone. B, G, and A = Bisgard, Gill, and
Abbott. (From Aitken GT: Inter-Clin Info Bull 1968; 7:6. Used by permission.)

Similarly, in a longitudinal deficiency of the tibia, it is important to determine by ultrasound or magnetic


resonance imaging (MRI) the possibility of a nonossified proximal epiphysis of the tibia, which would
indicate a partial deficiency. In such a case, disarticulation should not be carried out so that the below-knee
segment of the limb can be preserved.

Preservation of Proximal Joints

Particularly in transverse deficiencies of the leg in the upper quarter or higher, preservation of the knee joint
is important. Functional restoration of the trans-tibial (below-knee) amputee is far superior to what can be
expected of the transfemoral amputee. The sacrifice of a knee joint in the small child may severely limit his
ability to climb stairs, manage ramps, and take part in many physical and sports activities. By the same
token, efforts to preserve such a very short tibial segment (Fig 35A-4.) may be rewarded in adult life with a
competent, functional knee joint and a good, sturdy, sufficiently long transtibial stump.
Fig 35A-4. A, at the age of 18 months, the diagnosis of the left leg is total
transverse deficiency of the leg; for the right leg it is a partial transverse
deficiency of the proximal quarter of the leg with a 50-degree flexion
deformity. B, a radiograph shows a short tibial segment that measures
less than 5 cm (2 in.). C, at the age of 11 years, 6 months, there has been
continued longitudinal growth of the tibial segment with evidence of
ossification of a small segment of the fibula. D, at 19 years of age, the
patient still has good left knee disarticulation. The right transtibial
residuum now measures 10 cm (4 in.) in length, and excellent knee
function is preserved for a transtibial prosthesis. E, at 19 years of age the
patient is employed as a welder and enjoys riding motorcycles.

Patients with classes A and B PFFD are recognized as having a hip joint at birth. It is not possible at the
time of birth to be sure of the integrity or the stability of that joint. Although Lloyd-Roberts and Stone had
recommended early exploration of all of these joints, they later conceded that it may not be necessary and
that early exploration may damage the joint.

Stabilization of Proximal Joints Where Necessary

Stabilization of proximal joints is particularly applicable to patients with PFFD. Steel et al. have described
iliofemoral fusion for PFFD-fusing the femoral segment to the pelvis so that the femoral segment will be
parallel to the floor. The knee is thereby flexed at a right angle when the patient stands. Extending the
knee would then, in effect, flex the hip. We have not used this procedure. When hip instability is present due
either in type A to the subtrochanteric defect or in type B to the lack of contact between the head, neck, and
shaft fragments, osteotomy and bone grafting or osteosynthesis between the two fragments will stabilize the
hip. In Aitken types C and D, when the femoral segment is quite short, knee fusion may be necessary in
order to have a good prosthetic result. These procedures will be described in more detail in the section
on PFFD.

Bilateral Limb Deficiencies

These decisions will be somewhat altered with regard to the bilateral limb-deficient child, but in general an
effort should be made to fit prostheses as early as possible, based on the team evaluation of the child.
Emphasis must be placed on the therapist's evaluation of the child's muscular coordination and ability to
manage prosthetic devices. Prosthetic fitting may have to be staged or delayed until surgical intervention
can reasonably be accomplished. The physiologic and, particularly, psychological differences between the
child with bilateral limb deficiencies and the adult with a bilateral amputation must be recognized when
contemplating the bilateral fitting. When bilateral surgical conversion or a revision procedure is planned,
rehabilitation goals for the infant or juvenile should take these differences into consideration. The child who
requires bilateral Syme ankle disarticulation should be expected to walk independently without crutches or
canes (Fig 35A-5.). He should be expected to take part in all normal activities, including athletics, and
should be able to don and doff the prostheses independently early in life. Similarly, the child with a bilateral
transtibial fitting should have very high rehabilitation goals (Fig 35A-6.). Even as the levels of amputation go
higher, one should anticipate that as long as the child has functional upper limbs with which to improve
balance, he should be independently ambulatory. It is generally appreciated that the adult bilateral
transfemoral amputee, if he is to walk, will require crutches or canes. The child, on the other hand, can be
expected to walk independently when properly trained. Early fitting and appropriate training can be a very
rewarding experience in such a patient. In the very young child, as in the very old patient with bilateral
transfemoral amputations, initial fitting with stubbies is recommended. Stubbies are modified sockets with
either a rocker or rubber-soled bottom. The use of stubbies permits the patient to develop balance in the
erect position. When independent walking has been accomplished, the stubbies may be lengthened,
thereby increasing the child's height and confidence in the erect position. The final prescription is for
articulated limbs (Fig 35A-7.). Ambulation without crutches or other external aids should be expected.
Fig 35A-5. A patient with bilateral longitudinal
deficiency of the fibula treated by disarticulation at the
ankle is fitted with bilateral Syme prostheses with
plastic laminate sockets, and partial soft inserts for
suspension.
Fig 35A-6.Bilateral partial transverse deficiency of the forearm and bilateral partial transverse
deficiency of the leg (transradial and transtibial amputations). A, at 1 year of age, the child is
prepared for the first fitting with prostheses. B, at 2 years of age the child is independently
ambulatory. C, at 12 years of age the patient is completely independent with his bilateral,
transradial prostheses and bilateral, patellar tendon-bearing supracondylar (PTBSC) transtibial
prostheses with auxiliary waistband suspension. Eventually, he was able to discard the auxiliary
suspension.

Fig 35A-7. Bilateral total transverse deficiency of the leg (bilateral knee
disarticulations). A, patient standing on stumps. B, patient in stubbies and
standing by her articulated limbs. C, the patient at 9 years of age is fully
independent, uses public transportation, and attends school. D, at 20 years of
age the patient is driving her own car and is completely independent. She is
now 30 years of age and remains completely independent.
The parents of the bilateral lower-limb-deficient child must be made aware of the importance of weight
control. Instruction and dietary regulation should be available to the family and their responsibility at home
stressed (Fig 35A-8.).

Fig 35A-8. A, a patient with bilateral PFFD has been fitted with prostheses without amputation or
revision surgery. B, the thigh segment of the prosthesis is carved willow wood with lacers over the
feet and a Velcro closure over the shank portion. A single-axis knee and a solid-ankle, cushion-heel
(SACH) foot complete the prosthesis. C, the clothed patient has a normal appearance even though
his gait demonstrates severe waddle. D, at 15 years of age he has become obese, a major problem
for such a patient. E, despite obesity, he remains fully ambulatory. F, at the age of 41 years the
patient is fully employed and continues to wear the same type of prostheses.
Although the problem of the patient with bilateral PFFD will be dealt with later in this chapter, it cannot be
repeated often enough that this is the one situation in which any consideration of amputation of the feet
should be deferred.

Skin Grafting

Skin grafting in the child is very well tolerated. Split-thickness skin grafts on the residual limbs of children
will mature and withstand the shearing or frictional forces of socket contact. Denuding of a short transtibial
residual limb is no indication to proceed with higher amputation in a child. Instead, skin grafting should be
carried out. The resurfaced limb should then be toughened up in anticipation of prosthetic application.

The surgeon should keep this philosophy in mind when dealing with the limb-deficient child. If preservation
of a knee joint requires a posterior release and skin is a problem, split-thickness grafting may be carried out.
The surgeon should not hesitate to use a skin graft to preserve an epiphysis where there is a deficiency of
skin. Split-thickness grafting in weight-bearing areas may ultimately require revision and/or a pedicle graft
(Fig 35A-9.), but most split-thickness grafts mature and tolerate prosthetic wear well. Newer improved
materials for sockets have been developed to reduce shear forces at the stump-socket interface and lessen
the possibility of breakdown of grafted surfaces.

Fig 35A-9. A, transfemoral amputation with the entire adductor surface covered
with a skin graft. B, transfemoral prosthetic fitting. The patient required later
revision of the grafts.

Selection of a donor site for a skin graft in the lower-limb amputee should not be casually undertaken.
Consideration must be given to the ultimate amputation level and the type of prosthesis that the patient will
ultimately wear. No area should be chosen as a donor site if there is the possibility that it may later interfere
with prosthetic wear. As an example, the ipsilateral thigh should not be chosen as the donor site for skin
grafting for a transtibial stump. There may subsequently be the need for a thigh corset, in which case the
scarred thigh would be a problem. In a transfemoral amputation, the pelvic brim area should never be
chosen as a donor site for skin grafts since a pelvic belt or Silesian bandage may be necessary for
suspension, in which case this would be directly over the scarred area.
When there is a need for a pedicle or flap graft, the operating surgeon should take into consideration the
patient's ultimate amputation level. These procedures should be planned so that there will be no
unnecessary scarring in areas of weight bearing or in areas where a strap or stump-socket interface may
occur. Such scarred stumps require special attention from the pros-thetist; with appropriate prescription and
prosthetic restoration, the skin graft will mature and be able to withstand the stress of a socket.

TRANSVERSE DEFICIENCIES

Phalangeal Deficiencies

Transverse deficiencies of the phalanges, whether partial or total, do not usually require revision surgery. If
associated with congenital constriction bands, surgical intervention should be directed at the constriction
bands. Occasionally, proximal amputation of the toes will be necessary.

Partial or Complete Metatarsal Deficiencies

As in transverse deficiencies of the phalanges, revision surgery is not usually necessary for metatarsal
deficiencies. Occasionally, it is necessary to remove vestigial phalanges that have either no bony
component or insufficient bony component for functional value. These may present not only cosmetic and
hygienic problems but also a serious problem in shoe insert fitting. The vestigial phalanges may become
irritated and even ulcerate. With such problems, excision is recommended. When the residual metatarsal
elements are extremely small, whether short or atrophied, shoe fitting problems are the major concern. If
there is insufficient residual foot on which to fit a shoe with an insert, consideration must be given to other
prosthetic restoration. Those patients with very short remaining metatarsal elements and those with
complete transverse metatarsal deficiencies must be treated as though they have a tarsal deficiency.

Complete or Partial Tarsal Deficiencies

For the patient with a complete transverse tarsal deficiency (apodia or congenital ankle disarticulation), the
deficit may be managed by prosthetic restoration. No surgical intervention is necessary.

Partial tarsal deficiencies with a normal distal tibial epiphysis and no length discrepancy may require
conversion surgery. In the very early years, these children may walk with a high-top laced shoe. Prosthetic
devices are available, but function and cosmesis are less than ideal (Fig 35A-10.). Proximal revision for
functional as well as cosmetic reasons will be considered (Fig 35A-11.), with Syme ankle disarticulation as
the procedure of choice. This procedure is frequently referred to as a modified Syme's amputation
because the articular cartilage is left intact. The Boyd amputation may also be considered as an alternative
to disarticulation. In the very small child, up to 3 years of age, the malleoli may be left intact and will
present no problem in prosthetic fit or restoration. In the older child, the malleoli may be transversely
sectioned at the level of the tibial articular surface to provide a broad, flat, end-bearing stump. Care must be
taken not to damage the distal tibial epiphysis in this procedure. In an older child in whom the epiphyses
have closed, one may have to model the stump or shave the malleoli to achieve acceptable cosmetic result
in a Syme-type prosthesis. Patients so treated are left with an end-bearing stump that is the most functional
type. The patient may walk without the prosthesis. There is no concern for the phenomenon of bony
overgrowth so frequently observed when transtibial amputation is performed.
Fig 35A-10. A, partial transverse tarsal deficiency. B, Chopart-type partial-foot
prosthesis with posterior closure. With shoes on, the cosmetic result is less than ideal.
Fig 35A-11. >A, child aged 2½ years with a partial transverse
tarsal deficiency. B, Syme-type prosthesis fitted at the age of 2½
years without further amputation.

Procedure for Syme Ankle Disarticulation

The procedure for Syme ankle disarticulation should be carried out with tourniquet control. The incision
starts anteriorly at the tip of the medial malleolus and is carried directly across the ankle joint to the lateral
side. The second half of the incision is then carried from these two points across the plantar surface of the
foot in a slight arc to carry the incision just distal to the calcaneocuboid level (Fig 35A-12.). The anterior part
of the incision is carried down through the subcutaneous tissue and the superficial vessels clamped and
ligated. The anterior tibial tendon and toe extensors are divided and held with a clamp or suture for later
attachment. The dissection is carried medially, and the posterior tibial vessel and nerve are identified and
traced distal-ward to preserve the circulation of the plantar flap. The anterior ankle capsule is opened, and
the medial and lateral ligaments are divided. The plantar portion of the incision is carried directly down to
the bone and the plantar flap dissected off the inferior aspect of the os calcis by sharp and blunt dissection.
With the foot pulled forward, the posterior capsule is divided and the heel cord dissected off the superior
aspect of the os calcis. The dissection is completed by separating the os calcis from the plantar flaps and
care taken not to puncture the skin posteriorly. All major bleeders are ligated, the tourniquet released, and
bleeding controlled. The heel cord is now sutured to the posterior capsule. The anterior tibial tendon is
sutured to the anterior capsule, and the plantar flap is brought forward. The heel pad is stabilized with a
Kirschner wire or Steinmann pin through the heel pad and the articular surface and across the epiphysis.
The plantar fascia flap is sutured to the anterior capsule. Drainage may be accomplished with a soft-tissue
drain or suction drainage if desired. The skin is closed with loose interrupted sutures. Dressing may be done
by using either the rigid dressing or dry compression technique. Drainage is discontinued at 48 hours.
Fig 35A-12. Syme ankle disarticulation. A, the incision starts anteriorly at the tip of medial malleolus
and is carried directly across the ankle joint to the lateral side. The second half of the incision is
then carried from these two points to the plantar surface of foot and describes a slight arc. B, with
the foot pulled forward the posterior capsule is divided and the heel cord dissected off the superior
aspect of the os calsis. C, dissection is completed, care being taken not to puncture the skin
posteriorly. D, the wound is closed.

Transverse Deficiency of the Leg, Partial

Upper Third

A partial transverse leg deficiency (congenital transtibial amputation, partial transverse hemimelia) may
occur as a true deficiency or in association with Street-er's dysplasia (congenital constriction band
syndrome). When it is associated with Streeter's dysplasia, surgical attention to other constriction bands
may be necessary, either above the level of amputation or on other limbs. In those true deficiencies,
vestigial digits may be present, and these may require surgical removal. Such appendages may range from
one metacarpal or metatarsal ray to five vestigial digits.

The deficiency is apparent at the time of birth, and attention should be directed to the maintenance of range
of motion in the proximal joints, particularly the knee joint. If the child is born with a flexion contracture of the
knee joint, it may be necessary to surgically relieve this condition prior to considering prosthetic restoration.
If the knee joint extends completely at the time of birth, implementation of a range-of-motion program
should be maintained until the child is ready for the prosthesis.

Until recently it was thought that these congenital amputations were not subject to the phenomenon of bony
overgrowth. Pellicore et al. have now reported the observation of bony overgrowth in the remaining tibia
of these children; this phenomenon has been observed through the period of symptomatic overgrowth until
surgical revision was required.

Children with transverse deficiencies of the leg in the proximal portion will demonstrate good standing
balance on their sound limb somewhere between 9 and 15 months of age. They are ready for prosthetic
restoration at this time (Fig 35A-13.).

Fig 35A-13. A, child with Streeter's dysplasia who was born with a partial transverse
deficiency of the leg. B, fitted with a PTB prosthesis socket with cuff suspension and a
SACH-type foot, he is completely independent.

Transverse Deficiency of the Leg, Total and Transverse Deficiency of the Thigh, Lower Third

Transfemoral deficiencies occur less frequently in children than do transtibial deficiencies. Surgical
intervention is seldom if ever indicated. Management is prosthetic restoration (Fig 35A-7.).

Transverse Deficiency of the Thigh, Total (Amelia)

Children with total transverse deficiency of the thigh (amelia) (Fig 35A-14.) should be fitted with their first
prosthesis as soon as they are standing independently on the normal opposite side. As with other
deficiencies, there may be a vestigial digit attached. This usually is not a problem since the vestige has no
rigidity and, hence, can be incorporated into the socket without difficulty. Surgical intervention is seldom
indicated.
Fig 35A-14. A, and B, child at 1 year of age with a diagnosis of total transverse deficiency of the left
thigh (amelia). C, with a hip disarticulation prosthesis.

The infant with bilateral amelia will require a plastic "bucket-type" socket fitted to a firm base to achieve
sitting or "standing" balance. As he grows older, he may progress to the swivel walker. If the child has
normal upper limbs, bilateral articulated hip disarticulation prostheses may be prescribed at about 4 or 5
years of age.

LONGITUDINAL DEFICIENCIES

Longitudinal Deficiency of the Fibula, Partial

Partial longitudinal deficiency of the fibula may take on many forms, from a shortening of the fibula, which
will frequently be associated with a ball-and-socket ankle, to almost complete absence of the fibula with
only a small segment present. In between, there may be a very hypoplastic short fibula, which will be
associated with a leg length discrepancy. The very small remaining segment of the fibula will frequently be
treated as a total longitudinal deficiency of the fibula, whereas the full-length fibula with shortening is treated
primarily as a length discrepancy problem.

Longitudinal Deficiency of the Fibula, Total

Because of the frequency of its occurrence, longitudinal deficiency of the fibula has received a great deal
of attention in the literature. The titles are varied- congenital absence of the fibula, fibular deficiency,
paraxial fibular hemimelia, dysgenesis, etc. O'Rahilly reported on 296 cases of this deficiency that he had
reviewed in the literature prior to 1935. Since that time several hundred cases have been reported. In
1952, Coventry and Johnson reported 29 cases of congenital absence of the fibula and mentioned
amputation as a form of treatment but stressed the reconstructive approach. Since Aitken's report of
1959, most authors have emphasized amputation and prosthetic restoration. Although O'Rahilly was
able to find only 296 cases in the literature prior to 1935, by 1991 there were over 2,200 cases in the
Shriners Hospitals for Crippled Children. Our experience at the Springfield Unit increased from 60 patients
in 1961 to 120 in 1991.
Clinical Picture

As with other longitudinal limb deficiencies, one is impressed by the fact that fibular deficiency is a true limb
deficiency, not simply the absence of a single bone (fibula). The tibia may be bowed and usually has an
abnormality of the distal epiphysis. There may be a minimal shortening of the femur or coexistent PFFD. A
congenitally short femur may be present. Deficiency also exists in the muscles, tendons, nerves, and even
the skin, as evidenced by the dimpling frequently present over the deformed tibia. Hootnick et al. described
a midline metatarsal dysplasia associated with an absent fibula in addition to the deficiency of the lateral
rays. The classic clinical picture is a foreshortened limb with an equinovalgus foot, with or without
absence of the metatarsal rays and tarsal anomalies (Fig 35A-15.). For the patient with total unilateral
longitudinal deficiency of the fibula, progressive length discrepancy is the major clinical problem. In the
patient with bilateral fibular deficiency, this problem translates to a failure to attain normal height.

Fig 35A-15. A, classic unilateral longitudinal deficiency: fibula, total; tarsal, partial; fourth and fifth
metatarsal-phalangeal, total. Note the anteriorly bowed tibia, which is short and has a dimple in the
skin over the bow. Note also valgus at the ankle and the absent fourth and fifth rays. B, side view of
the same patient. C, at the age of 2 years, after disarticulation he is fitted with a patella tendon
bearing prosthesis with a toddler harness. D, at the age of 5½ years, he is asymptomatic and fully
active at the level of his peers.

Definitive treatment of patients with fibular deficiency will depend on two major considerations: (1) is it
associated with PFFD, and (2) is it unilateral or bilateral? Patients with longitudinal deficiency of the fibula
and associated ipsilateral PFFD must be treated primarily as having a PFFD, with the fibular deficiency as a
secondary consideration. The patient with unilateral fibular deficiency and PFFD on the contralateral side is
an exception to this.

Treatment of Unilateral Longitudinal Fibula Deficiency, Total

Leg length discrepancy is the major problem in the patient with total unilateral longitudinal deficiency of the
fibula. Experience has shown that this is a progressive discrepancy and that the progress of this
discrepancy can be predicted on growth charts. Efforts at equalizing the discrepancy by tibial lengthening
have generally been unsatisfactory.

With the advent of the Ilizarov technique of lengthening by callus distraction, there has been a
reappearance of interest in preserving the foot and lengthening these limbs. As mentioned earlier, if, in
fact, an effort is made to lengthen the tibia in such a child, he will have to wear a lift to compensate for his
discrepancy until he is old enough for his limb to be lengthened. He then has a protracted period of wearing
the lengthening apparatus and protecting the limb, and this process will probably have to be repeated a
second time in order to obtain adequate equalization. Efforts at equalizing discrepancies in excess of 7.5
cm (3 in.) by contralateral growth arrest or shortening are contraindi-cated because this amount of loss in
overall height of the patient is too great a price to pay. Therefore, it would appear that only in the unusual
patient can leg length equalization, by a combination of lengthening and growth arrest, be accomplished. To
consider an effort at leg length equalization the patient should have a straight tibia with no anterior bow, as
well as a normal or near-normal foot and a length discrepancy that is not expected to exceed 7.5 cm. In our
experience, if the child has a 5-cm length discrepancy at birth and an abnormal foot, with or without
shortening of the femoral segment, then the treatment of choice remains ankle disarticulation (Syme type)
and prosthetic restoration. Reconstruction has been recommended but, in our opinion, is not optimal.
Therefore, it is recommended that early conversion be carried out (Fig 35A-16.)-between 9 and 12 months
of age, or as soon as the child is able to stand and indicates a readiness to commence walking.

Fig 35A-16. A, preoperative diagnosis: unilateral longitudinal deficiency of the fibula total; tarsal,
partial; fourth and fifth metatarsal-phalangeal, total. B, after ankle disarticulation. C, child fitted with
her first prosthesis-PTB with cuff suspension. She has immediate independent ambulation.

Indications for early amputation and prosthetic restoration may then be summed up as (1) length
discrepancy in excess of 5 cm (2 in.) and progressing, (2) foot deformity, and (3) a normal femur. It is
important that the procedure be a Syme ankle disarticulation or Boyd amputation, never transtibial
amputation. Trans-tibial amputation is contraindicated for two major reasons: (1) loss of longitudinal
growth at the distal tibial growth plate and (2) bony overgrowth at the amputation site.

The distal tibial epiphysis contributes 20% of the growth of the limb as a whole and 45% of the growth of the
tibia itself. Even if it is known that the distal tibial epiphysis may not be normal, it is still important to
appreciate that if this epiphysis is sacrificed at 1 year of age, the child will be left with a short transtibial
residual limb when he attains full growth. On the other hand, if the growth plate is retained, one may
anticipate that there will be, at worst, a long transtibial stump or, at best, a good Syme-type ankle
disarticulation. The disarticulation procedure also provides an end-bearing stump on which the child may
walk without the prosthesis.
Prior to 8 years of age, transtibial amputation is accompanied by a high incidence of bony overgrowth at the
amputation site (Fig 35A-17.). Much has been written about bony overgrowth, its prevention, and treatment.
When symptomatic, revision of the amputation is necessary.

Fig 35A-17. Bony overgrowth at the site of a transtibial


amputation.

In summary, most children with unilateral fibular deficiency require ankle disarticulation and prosthetic
restoration. During the operation the articular cartilage should be left on the distal portion of the tibia and the
heel pad fixed to it. One should also look for the presence of an anterior tibial bow. This has been described
as a kyphoscoliotic tibia and becomes a problem in prosthetic fit. It may be a result of contracture of a
lateral band but is more appropriately recognized as a central defect of the tibia. One sees dimpling of the
skin over this anterior bow. When indicated, this anteriorly bowed or kyphoscoliotic tibia should be corrected
by rhomboid resection of bone (Fig 35A-18.). The presence of a lateral band should also be taken into
consideration. Excision of this lateral band, which may be a deforming force, can be carried out at the
same time as the rhomboid resection and straightening of the tibial bow. The procedure for ankle
disarticulation has been described.
Fig 35A-18. A, longitudinal deficiency of the fibula (total) with a severe anterior bow of the tibia
(kyphoscoliotic tibia). B, and C, after rhomboid wedge resection of the anterior bow and fixation
with two K-wires. D, healed osteotomy, which facilitates prosthetic fitting.

In the older child, consideration should be given to modification of the disarticulation procedure. Many of
these children have already undergone triple arthrodesis or other surgical procedures in an attempt to
restore a functional foot. Length discrepancy is progressive and is the indication for amputation. In these
patients, fusion of the ankle joint and amputation through the mid-tarsal level, or a modified Boyd
procedure, retains the extra length and normal attachment of the heel pad to the os calcis. The broad stump
contour does require a more bulky prosthetic socket and is cosmetically less desirable. The procedure is
therefore usually reserved for boys, in whom cosmesis is not so important (Fig 35A-19.). Ankle
disarticulation is the procedure of choice for girls.

Fig 35A-19. A, Boyd amputation stumps. B, radiographic appearance.

Bilateral Longitudinal Fibula Deficiency, Total

There are two major considerations for patients with complete bilateral longitudinal deficiency of the fibula
and a normal femur: (1) the condition of the feet and (2) the anticipated overall height of the patient.

Patients with five-rayed feet that are reasonably aligned beneath the tibia will have no problem in shoe
wear. On the other hand, those patients with a three-or four-rayed foot and associated severe equinus and
valgus may require considerable surgery to align the foot plantigrade, Even after repeated surgical
procedures, normal shoe wear may be precluded by the shape and deformity of the foot. Severe foot
deformity may then be an indication for amputation and prosthetic restoration.

With fibular deficiency there may be a deficiency of the distal end of the tibia and its growth plate. When the
tibia at birth is disproportionately short as compared with the femur or when there is a severe anterior bow
in the tibia, one can anticipate that the discrepancy will be progressive. In these patients early ankle
disarticulation at the age of 1 year to 18 months is indicated.

A retrospective study of a small group of unampu-tated patients at Shriners Hospital in Springfield,


Massachusetts, concluded that 50% should have had amputation (Fig 35A-20.) and prosthetic restoration
to provide normal stature. When length discrepancy between the tibia and femur is sufficiently great to
make this decision at an early age, it is desirable to introduce parents to the limb deficiency clinic. There
they can see similar children with prosthetic restoration and have an opportunity to talk with the parents of
these children rather than having to rely completely on the recommendation of the clinic chief.
Fig 35A-20. Longitudinal deficiency of the fibula, total
and bilateral. A, at the age of 6 years, 7 months, there is
a disproportionate shortening of the tibias. B, a
posterior view confirms the shortening and reveals a
residual valgus of the feet and ankles. This patient
should have been treated by disarticulation and
prosthetic restoration.

If the discrepancy in proportional length of the tibia and femur is small and there is a good foot,
consideration of amputation should be deferred, but growth charts should be maintained for the patient. If
the discrepancy is a progressive one and it is apparent that the patient is going to be unduly short in adult
life, bilateral ankle disarticulation and prosthetic restoration may be recommended prior to school age (Fig
35A-21.). As the child grows older, and particularly if he has been permitted to enter his early teens without
surgical intervention, the child himself should enter into the decision making concerning such ablative
surgery and prosthetic restoration.
Fig 35A-21. A, longitudinal deficiency of the fibula, total and bilateral; tarsal, partial; fourth and fifth
metatarsal-phalangeal, complete. B, child after bilateral ankle disarticulations at the age of 1 year, 1
month. C, the child is fully ambulatory with bilateral patella tendon bearing, supracondylar (PTS)
prostheses at the age of 1 year, 2 months. D, at the age of 4 years, he is completely independent in
his PTS prostheses. E, at 13 years of age, he remains fully active and keeps up with his peers. Note
that he now wears low-profile sockets.

Longitudinal Deficiency of the Tibia

Longitudinal deficiency of the tibia may occur either unilaterally or bilaterally, and it may be either partial or
complete. Its occurrence is much less frequent than fibular deficiency. There have been several reports of
the association of longitudinal deficiency of the tibia with deficiency of the first metacarpal (absent thumb),
as well as with Polydactyly. Although Eaton and Mc-Kusick in reporting their four cases stated that "no
familial cases have been recorded," Clark has cited 13 case reports of familial occurrence of tibial
deficiency that she found in the literature. She added to it her report of a patient with nine affected
descendants in three generations.
The characteristic clinical picture of tibial deficiency can usually be recognized at birth (Fig 35A-22.).
Gross instability of the knee is evident, with or without flexion contracture and lateral displacement of the
fibula. Severe varus of the foot is present, with the sole of the foot facing toward the opposite leg or even
toward the knee or perineum. The knee joint is so unstable that the foot can almost be brought up against
the medial portion of the thigh. Length discrepancy is the most obvious part of this deficiency. Several
patients have an exostosis or osteochondroma of the femur (Fig 35A-23.). The deficiency may be total or
partial, but at birth it is difficult to be sure of this differential diagnosis. A pterygium may be present.

Fig 35A-22. A, total longitudinal deficiency of the tibia. The boy's foot faces toward his groin.
There is severe length discrepancy and no stability at the knee or ankle. The foot is in varus. B, a
radiograph shows the fibula lateral to the femoral condyles and the foot medial to the fibula. The
tibia is completely absent.
Fig 35A-23. Radiograph of the femur in a patient with
deficiency of the tibia and a large exostosis from the
shaft. The base of the exostosis has fractured.

Partial Tibial Deficiency

Since the presence or absence of a proximal segment of the tibia is crucial in the treatment decision, it is
important to ascertain this knowledge early in life (Fig 35A-24.). If there is a suggestion that a proximal tibial
segment is present, even though it is not visualized on a radiograph, it is important to rely on the clinical
examination. Ossification of a proximal tibial segment may not occur for months or even up to 2 years of
age. In such patients, arthrography and other techniques may be of assistance in making a diagnosis.
Grissom et al. have described the use of sonography in the management of tibial deficiencies since
ultrasound examination can define the cartilaginous anlage of the proximal tibial segment that cannot be
visualized radiographi-cally at birth. MRI may also be used, but ultrasound is a simpler and less expensive
examination and does not require the sedation necessary for the small child who is to have an MRI
examination. Partial deficiency of the tibia should be treated with an orthosis or an orthopros-thesis until one
is certain of the ossification of the proximal fragment and of the presence of the knee joint.
Fig 35A-24. A, radiograph of a partial longitudinal deficiency of the tibia. Note the fibula
subluxed proximally and varus of the foot and distal part of the fibula. B, child at the age of
23 months, after disarticulation at the ankle. C and D, patient without and with his prosthesis
at 18 years of age. He plays high school football.

Having established the presence of a proximal fragment and knee joint, the size of this tibial fragment is the
next concern. If the tibial segment is short, less than one third the length of the normal tibia, the procedure
of choice is synostosis of the fibula to the tibia and disarticulation of the foot (Fig 35A-25.). This produces a
long transtibial residual limb. On occasion, the fibula may migrate proximally above the knee joint (Fig 35A-
26.). In our experience, this has occurred in only one patient. Resection of the proximal half of the fibula
was carried out, and stability of the knee joint was maintained. Tibiofibular synostosis should prevent this
from occurring if it is done early enough.

Fig 35A-25. Partial deficiency of the tibia with a


synostosis to the fibula.
Fig 35A-26. Proximal migration of the fibula in a
patient with a partial longitudinal deficiency of the
tibia.

Procedure for Tibiofibular Synostosis

The proximal tibial segment is exposed through an elliptical incision between it and the fibula. The distal
portion of this tibial segment is dissected subperi-osteally and completely exposed. The fibula is now
exposed subperiosteally at a level opposite the remaining tibia. With a sharp gouge, the medial surface of
the fibula is turned proximalward, and the lateral aspect of the tibia is turned distalward. Bone graft is then
packed into the defect between the two. The fascia is not sutured. The subcutaneous tissue and skin are
closed in layers. Disarticulation at the ankle is then accomplished in the usual fashion. Marquardt has
described implantation of the fibula into the os calcis to create an end-bearing stump. This procedure may
be employed instead of simple disarticulation at the ankle. The limb is immobilized in a long-leg plaster cast
with the knee in full extension for 8 weeks, or until there is evidence of firm cross-union between the tibia
and fibula.

If the proximal segment of the tibia is sufficiently long, tibiofibular synostosis is not necessary. Syme ankle
disarticulation or a modified procedure (Boyd type) and fitting with a transtibial-type prosthesis may be
carried out early in life. When the proximal tibial fragment is very small, one must wait until adequate
ossification occurs and then attempt to implant the fibula into the tibial segment to create a one-bone leg.
After this is done, the os calcis can be implanted into the distal end of the fibula and amputation carried out,
thus treating the patient as a Syme disarticulate (Fig 35A-27.).
Fig 35A-27. A, patient at 1 year of age with a partial longitudinal deficiency of the tibia, Polydactyly,
and what amounts to a diplopodia. B, radiographic examination of the lower limb shows an intact
femur with a distal femoral epiphysis present. There is no evidence of ossification of a proximal
tibial segment; the fibula is bowed, proximally displaced above the knee joint, and abnormal foot
duplication of metatarsals is visible at the bottom of the radiograph. C, an arthrogram at the age of
3 years shows a small ossification center of the proximal part of the tibia; the dye definitely defines
a joint about the distal part of the femur and proximal end of the tibia, including the unossified
section medialward, the fibula remains proximally displaced after implantation of the fibula into the
tibial segment and an attempt at fusion of the os calcis to the distal part of the fibula. D, the fibula
has hypertrophied, and the patient has a good end-bearing, transtibial residual limb. E, the patient
at 6 years of age. F and G, patient with a prosthesis. Note the slight medial bulge from the os calcis
fused to the distal part of the fibula.

Longitudinal Deficiency of the Tibia, Complete

With complete longitudinal deficiency of the tibia, the clinical picture is the same as just described.

In 1965, Brown first reported his experience with construction of a knee joint in patients with complete
longitudinal deficiency of the tibia. His experience dated back to 1957, when he first implanted the fibula
beneath the femur and disarticulated the foot. The residual limb was fitted into a "transtibial" socket with
outside hinges. A limb deficiency that had always been considered for transfemoral amputation was
converted into a "transtibial" prosthetic restoration. Since that time, many of these procedures have been
accomplished. However, at a long-term review,[*Cooperative Clinic Chief's Meeting, Seattle, 1975.] the
high incidence of recurrent deformity and the need for repeated operative intervention was such that only a
few centers continue to perform this procedure. On occasion, the picture is altered by the presence of a
pterygium between the femur and the fibula. This pterygium may determine the incision location for surgical
intervention.

The treatment of choice is disarticulation at the knee. Resection of the fibula may also be necessary when
there is a severe pterygium. In those patients with unilateral deficiency, this procedure may be considered
as soon as the individual is standing on the normal limb. Prosthetic restoration and good functional
rehabilitation should be anticipated.

Procedure for Knee Disarticulation

In the procedure for knee disarticulation, a skin incision is marked out with a long anterior flap, which is also
wide at its distal portion, and carried to well below the level of the femoral condyles. The posterior portion of
the incision should be marked out at or just below the popliteal crease. The anterior incision is made and
carried down through the subcutaneous tissues. The anterior capsular structures are divided to enter the
knee joint. There may not be a patellar tendon. Posteriorly, the incision is made and deepened, and the
hamstring tendons, if identifiable, are divided somewhat long and tagged for later suture. The great vessels
are clamped and doubly ligated and the nerves drawn distally, divided, and allowed to retract. The
gastrocnemius origin (if discernible) is severed from the femoral condyles. The capsule and ligaments are
divided and the limb removed. At this point, the tourniquet is released and all bleeding controlled. The
hamstring tendons are sutured to the capsule or ligamentous structure as available. If a patella and its
tendon are present, the patella is left in place, and the tendon is sutured to the hamstring tendons or
posterior capsule. The capsule is closed over the cartilage of the femoral condyles. Drainage with either a
soft-tissue drain or suction is instituted, and the closure is completed. It should be noted that the long
anterior flap provides good coverage of the femoral condyles and good weight-bearing skin for subsequent
prosthetic wear. The postoperative care may be with rigid dressing or soft dressing according to the
surgeon's preference.

Disarticulation in the presence of a pterygium may pose a surgical dilemma for placement of the incision.
We have resolved this problem by making an L-shaped incision from the base of the pterygium down to the
"knee joint." The fibula is then filleted out extraperi-osteally and removed and the L incision closed to leave
a good end-bearing cover of the femoral condyles. A lateral scar is also left, which may be bulky proximally
but shrinks down rapidly thereafter (Fig 35A-28.).
Fig 35A-28. A, patient with bilateral total longitudinal deficiency of the tibia who has walked in boots
on his knees since infancy. He is now 15 years of age. B, same patient. Note the pterygium at each
knee. C, after knee disarticulation. D, with bilateral transfem-oral prostheses and Canadian
crutches, he is now fully and independently ambulatory.
As the patient grows older, a length discrepancy of the remaining femur may occur that will permit use of a
knee joint other than the outside hinge. If this discrepancy does not occur, distal femoral epiphysiodesis
may be considered when the child is 10 to 12 years of age. If the child is seen when it is considered too late
for epiphysiodesis and spontaneous differential femoral shortening has not occurred, the distal end of the
femur may be modified to allow for a more cosmetic prosthetic fitting. This can include distal shortening of
2.5 to 3.0 cm, partial condylar ostectomies medially and posteriorly, and patellectomy. Femoral shortening
may be considered. Newer design of joints for the knee disarticulation prosthesis may render this option
unnecessary.

Longitudinal Deficiency Femur, Partial (Proximal Femoral Focal Deficiency [PFFD])

In 1939, Langston described a "congenital defect of the shaft of the femur." Aitken recognized "the
existence of a group of partial deficiencies of the proximal femur involving the iliofemoral joint" and, since no
appropriate terminology existed, used the designation "proximal femoral focal deficiency." Amstutz has
defined PFFD as the "absence of some quality or characteristic of completeness of the proximal femur,
including stunting or shortening of the entire femur." Aitken described four classes (Fig 35A-29.). In class A
there is an acetabulum. The head of the femur is within the acetabulum, and there is a subtrochanteric
deficit. Class B presents with an acetabulum and a capital fragment within the acetabulum. There is a short
shaft fragment with no bony connection between the two. In class C there is no acetabulum. One finds a
short femoral fragment with a tuft on top. In Class D there is also no acetabulum. A very short triangular
femoral segment is present. Amstutz further expanded this classification and developed five types. His type
1 included those patients with what has been described by Aitken as a congenital short femur or coxa vara
with bowing. His other four types approximate the Aitken classes A, B, C, and D. These morphologic
classifications offer assistance in treatment decision primarily with regard to the hip.

Fig 35A-29. Four radiographic subclasses of partial longitudinal


deficiency of the femur (proximal femoral focal deficiency [PFFD]).
(Courtesy of the National Academy of Sciences, Washington, DC.)

Most reports of PFFD have included only small numbers of cases. Prior to Jan 1, 1968, the Area Child
Amputee Center in Grand Rapids, Michigan, had only 35 cases. In his report, King was able to review
over 100 cases of PFFD collected from various juvenile amputee clinics throughout the United States. In
1968, Westin and Gunderson were able to gather 165 cases collectively recorded from Shriners hospitals.
More recent reports have included a larger number of patients.

Westin and Gunderson reported a 65% incidence of other defects, with 50% of the patients having
deficiency of the fibula in the same limb. Amstutz and Ait-ken noted a high incidence of fibular deficiencies
in the same limb, as well as other skeletal deficiencies. Kruger and Rossi reported 31 of 38 patients to
have other limb abnormalities, with fibular deficiency in 50% of their patients. Koman et al. described their
50-year experience in the management of PFFD. Sen-Gupta and Gupta reported four patients with PFFD
in one family and stated, "In the absence of a history of maternal diabetes; premature child birth; exposure
to known teratogenic factors such as irradiation, toxic chemicals, and drugs; mechanical trauma; or
maternal viral infection, the defects appear to be genetic in origin." Only male members were affected.

Shatz and Kopits reported that "A review of radiographs obtained on one patients maternal great aunt
showed that she had a PFFD with absence of the ipsilateral fibula." Hillmann et al. reported that a review
of 49 patients showed no genetic basis for the diagnosis. Steel et al. in 1987 suggested iliofemoral fusion
for stabilization of the hip in PFFD. However, others have not been pleased with this procedure since
fusing the femoral segment to the ilium at 90 degrees and permitting the knee to act as a hip joint puts the
weight-bearing line of the limb anterior to the body.

In his early description of the biomechanical losses of lower-limb skeletal deficiency, Aitken has stressed
four points: (1) inequality of leg length, (2) malrotation, (3) inadequacy of proximal musculature, and (4)
instability of proximal joints. PFFD has all four of these elements of biomechanical loss. Although
malrotation, inadequacy of proximal musculature, and instability of proximal joints all contribute to the poor
gait and complexity of treatment, it is the length discrepancy that is the ultimate indication for definitive
surgery-amputation and prosthetic restoration.

Amstutz and Wilson recognized this progressive discrepancy in the length of limbs thus affected and
developed a concept of proportionate inhibition of growth in all patients over the age of 5 years. Amstutz
believed that precise prognostication of the expected discrepancy in limb length was possible and that
definitive treatment could be planned on these children as early as 2 years of age. The theory of
proportionate growth has been expanded by Mosley in his technique of charting the proportionate
discrepancy, as opposed to the growth of the normal limb. Although in most patients with PFFD the
discrepancy early in life is sufficient to suggest that eventual amputation will be indicated, growth charts can
be of assistance in decisions as to the total treatment plan.

The clinical picture of PFFD (Fig 35A-30.) is that of a short femoral segment that is positioned in flexion,
abduction, and external rotation. If ignored, this condition may become a fixed deformity with secondary
knee flexion deformity. When associated with fibular deficiency, the length discrepancy will be much greater,
and foot deformity will be present. If the tibia and fibula are essentially normal, then foot and ankle function
will be normal. It is important to institute an early stretching program to prevent knee and hip deformity.
Fig 35A-30. A, PFFD; the femur is short. The hip is flexed, abducted, and externally
rotated. The knee is flexed. B, orthotic management to prevent a fixed deformity
until surgical intervention.

The Hip Joint

As described by Aitken classes A and B PFFD have an acetabulum present by radiographic examination
at birth, which indicates the presence of a femoral head within the acetabulum. Types C and D have no
acetabulum present by radiographic examination at birth, which suggests that no femoral head will develop.
It is usually impossible to distinguish between types A and B at birth. As ossification of the capital epiphysis,
femoral neck, and trochanteric portion occur, the distinction can be made.

Class A PFFD can be recognized by radiographic examination when the cartilaginous anlage of the head
and neck have ossified. The head and neck will be connected to the shaft fragment with a subtrochanteric
varus deformity that may be progressive. This subtrochanteric varus may be associated with
subtrochanteric pseudoarthrosis. Correction of the deformity by subtrochanteric valgus osteotomy may be
carried out, and if a pseudoarthrosis, is present, resection and bone grafting are indicated. If correction is
deferred, the subtrochanteric valgus may increase to such magnitude that it will be difficult to correct it
completely at a single stage, and a two-stage procedure may be necessary. In the first stage the deformity
is partially corrected, and then after 1 or 2 years the operation is repeated to complete the correction. The
goals should be the creation of a neck shaft angle of between 120 and 135 degrees.

The Aitken class B PFFD is characterized by the presence of a head and neck fragment that has no
continuity with the shaft fragment that rides proximal to it. Lloyd-Roberts and Stone and Lange et al.
have found a connection either by fibrous tissue or cartilage between the two fragments. In either case,
between the ages of 3 and 6 years osteosynthesis is indicated to create a stable hip (Fig 35A-31.). These
efforts should be delayed until there is adequate evidence of bone in the neck fragment so that with grafting,
a firm union between the shaft and neck fragment can be attained. On the other hand, if osteosynthesis is
delayed too long, there is a possibility that the capital fragment may fuse to the acetabulum; in this case,
osteosynthesis should not be carried out since an unstable mobile hip is preferable to a rigid hip.
Fig 35A-31. A, Aitken class B PFFD. The head and neck are ossifying. There is no connection
between the shaft and the head-neck fragment. B, after osteosynthesis and knee fusion. Note that
the proximal tibial and distal femoral epiphyses have been destroyed to control longitudinal
growth.

Procedure for Osteosynthesis

A lateral incision is made over the proximal shaft of the femur with extension proximally to the anterosupe-
rior spine of the ilium, as with the Watson-Jones approach. The femoral shaft is exposed subperiosteally
and osteotomized at the level of the head-neck fragment. Muscle insertions, especially if abductors are
attached to the proximal fragment, are left intact. The neck is now exposed, and the capsule is opened to
inspect the hip joint. The lateral portion of the neck is usually covered with fibrous tissue and/or a
cartilaginous cap. This lateral aspect of the neck is nibbled away with a small rongeur to expose a raw bony
base in the center of the cartilage. If the patient is very young, only a small area of bone is available. The
shaft fragment is now brought into opposition with the exposed neck by wide abduction and fixed to the
neck fragment with Kirschner wires. In the case of the older child in whom advanced ossification of the neck
fragment is present, a Coventry screw or small Smith-Petersen nail and plate may be used. A bone graft is
desirable in an effort to obtain union. The proximal fragment of the femoral shaft is now allowed to fall back
into place and may be fixed with wire to the shaft at a more distal point. The wound closure is routine.
Immobilization should be in a hip spica with wide abduction for a minimum of 8 to 12 weeks, or until good
bony union is verified by radiographic examination.

Steel et al. have described fusing the femoral segment to the pelvis in classes C and D PFFD, with success
in four patients. In their technique, the femoral segment is fused to the pelvis with it parallel to the floor so
that when the patient is standing, the anatomic knee is flexed 90 degrees and the tibia functions as a femur.
After ankle disarticulation the transfemoral-type prosthetic knee is extended. When the patient sits, the
anatomic knee extends to permit flexion of the prosthetic knee. In our opinion, this procedure offsets the
weight-bearing line of the limb anteriorly and aggravates the hip limp in these patients.

Unilateral Partial Longitudinal Deficiency of the Femur (PFFD)

Since length discrepancy is the major factor in patients with unilateral PFFD, they may be divided into three
groups: (1) femoral segment less than 20% of the normal side, (2) femoral segment 20% to 70% of the
normal side, and (3) femoral segment greater than 70% of the normal side.
Short Femoral Segments

Patients with PFFD whose femoral segment is less than 20% of the length of the normal side are usually
either in the Aitken class C or class D. No hip joint is present, and even though the discrepancy may be
moderate at birth, it will become progressive with growth. Definitive treatment in these patients will be
disarticulation at the ankle level and prosthetic restoration (Fig 35A-32.). As with other limb deficiencies, if
the parents have been prepared and especially if they have attended a clinic and had the opportunity to
observe other children with similar problems, acceptance of this recommendation can be expected. If the
parents are reluctant to accept ablation, then as an alternative an articulated extension prosthesis can be
prescribed. This may be either a temporary measure or permanent if the parents so desire. As a further
option, the Van Nes rotation-plasty, described below, may be considered

Fig 35A-32. PFFD (20% femoral segment). A, 6-year-old child before amputation. B,
postamputation. C, with prosthesis. D, fully clothed.

Medium-Length Femoral Segments

There are many options available in the treatment of patients with PFFD who have a femoral segment
measuring 20% to 70% of the length of the normal femur. A treatment plan should be developed with the
emphasis on early management for the prevention of deformity at the hip and the knee. Orthoses may be
prescribed, as well as a stretching and exercise program. If the parents are resistant to surgical intervention
or if surgical intervention is to be deferred beyond the time of expected ambulation, the child may be fitted
with a platform orthosis or an extension prosthesis without amputation.

When the femoral segment approaches 70% of the length of the femur on the normal side and full knee
extension and quadriceps power have been maintained, disarticulation at the ankle and prosthetic
restoration with a Syme-type prosthesis are indicated (Fig 35A-33.). Although knee levels are unequal and
therefore stride length is unequal, these patients manage very well.
Fig 35A-33. A, following amputation to equalize leg length for a prosthesis in a
patient with PFFD, the knee levels are unequal, and there may be residual hip
flexion and knee flexion deformity. B, prosthetic length will be determined by
that length that provides the patient with maximum knee and hip stability
rather than an effort at exact equalization of leg lengths.

Children with shorter femoral segments may have developed a flexion-external rotation deformity of the hip
and associated knee flexion deformity. The weight-bearing line is therefore anterior and lateral to the
weight-bearing line of the body, and these children do not do well. Knee fusion, which should be carried out
in full extension, will establish a single skeletal lever. King and Marks have pointed out that with knee fusion,
the flexion-abduction-external rotation deformity at the hip will spontaneously correct without surgical
intervention. The single skeletal lever then comes into the weight-bearing line, and not only does the gait
improve, but also the strength of the muscles about the hip. Recurrent flexion may later appear through the
epiphyses. King and Marks have attributed this reoccurrence to the Hueter-Volkmann law. Recurrent
flexion deformity through the knee fusion should be corrected by osteotomy (Fig 35A-34.).
Fig 35A-34. A, partial longitudinal deficiency of the femur (PFFD). Definitive treatment has been
delayed. B, after knee fusion and ankle disarticulation, there has been increasing flexion deformity
at the knee. C, flexion deformity demonstrated with the patient lying prone. D, after correction, with
the patient prone, full correction is demonstrated. E, the patient with a single-axis knee and good
ischial bearing. Two years after surgery, correction is maintained.

Options in definitive treatment are disarticulation at the ankle with transfemoral prosthetic restoration or Van
Nes rotation-plasty. Disarticulation at the ankle may leave the prosthetic knee center below that of the
normal side. In this case, distal femoral epiphyseal arrest or shortening at the time of knee fusion may be
desirable.

The Van Nes rotation-plasty may be considered in conjunction with knee fusion. The Van Nes procedure
requires resection of a segment of the tibia and fibula sufficient to permit rotation of the distal portion of the
leg 180 degrees so that the foot then faces backward. Following solid healing, the ankle joint can then be
expected to function as a knee joint, with dor-siflexion of the ankle providing "knee flexion" and plantar
flexion of the ankle providing "knee extension." Prior to making the decision for Van Nes rotation-plasty,
there must be a reasonable expectation that at the completion of growth the ankle joint will be
approximately at the knee level of the sound side. Use of the Van Nes rotation-plasty provides the patient
with a "knee joint" and a transtibial-type prosthesis (Fig 35A-35.). The nonconventional prosthesis will have
a socket modified to accept the foot as the shank portion, with weight bearing primarily on the heel and
longitudinal arch of the foot. Placement of the knee axis must be precise and the thigh corset constructed
and padded to prevent pressure over the pretibial region.

Fig 35A-35. A, left leg, PFFD, Aitken class C. B, early prosthetic fitting around the
deformity and without surgery. C, after definitive treatment: knee fusion and Van
Nes rotation-plasty. The foot and ankle are turned 180 degrees. D, transtibial-type
prosthesis with an outside knee hinge. With the foot in equinus, the prosthetic
knee is fully extended. When the foot dorsiflexes, the prosthetic knee will flex 90
degrees. (From Rossi TV, Kruger LM: Orthot Prosthet 1975; 29:37-57. Used by
permission.)

Torode and Gillespie have performed rotation-plasty through the knee joint and, if 180 degrees of rotation
was not obtained, completed the rotation through the tibial osteotomy. Fricia et al. reported on 13 patients
with rotation osteotomy for PFFD and noted that 5 required repeat osteotomy, 6 of 12 unilateral patients had
excellent results and 4 had good results. They stated that 5 girls with excellent results had no objection to
cosmetic appearance.

Procedure for Van Nes Rotation-Plasty

Tourniquet control is optional in the Van Nes rotation-plasty, but preferred. After the usual preparation and
draping, an incision is made from the anteromedial proximal portion of the tibia slightly obliquely to the
anterolateral aspect of the ankle level. The diaphysis of the tibia and fibula (when present) are exposed.
Short segments of Kirschner wire may now be inserted into the proximal and distal ends of the tibia to be
used as a reference point for rotation, or if desired, the tibia can be marked prior to resection for this
purpose. According to the prior plan, depending on the age of the patient and the amount of shortening
desired, 5 to 7.5 cm of tibia is resected and approximately 2.5 cm more of the fibula. The fibula may be
reserved to use as a central bone graft for added stability. The tourniquet is released and bleeding
controlled.

If adequate bone has been resected, rotation of the distal segment 180 degrees poses no circulatory
problem, and fixation of the tibial fragments may be accomplished with a compression plate and screws. If
there is any question of circulatory impairment, either additional tibia may be resected, or parallel Kirschner
wires may be used above and below the osteotomy resection site for control of the fragments and the
reserved fibula used as a central bone graft to prevent displacement. The skin wound is closed, with no
effort made to close the deep structures. Again, circulation of the foot is observed, and if satisfactory, the
limb is immobilized in plaster.

Long Femoral Segment

Patients with femoral segments greater than 70% of the length of the normal side usually have a variation of
coxa vara with bowing, or what Aitken terms a congenially short femur. He does not include either of these
conditions in the PFFD category, even though they are a manifestation of a deficiency in the proximal end of
the femur. These patients must be observed and growth charts maintained, and as a rule they may be
treated as having a length discrepancy problem. Options to be considered include (1) lengthening of the
short femur, (2) growth arrest at the normal knee epiphyses, (3) shortening of the normal femur, or (4) a
combination of lengthening and growth arrest. If there is no reasonable hope for equalization of leg lengths,
disarticulation at the ankle and a Syme-type prosthesis are considered as a method of leg length
equalization rather than resorting to excessive shortening of the patient's overall height.

Although patients with coxa vara and bowing of the femur or a congenitally short femur appear to benefit
from subtrochanteric valgus osteotomy, development of the acetabulum must be carefully observed. If
acetabular dysplasia is present, valgus osteotomy is contraindicated.

Bilateral Partial Longitudinal Deficiency of the Femur (PFFD)

Patients with bilateral PFFD may be divided into two groups : the symmetrical, in which the length of the
limbs is approximately equal, and the asymmetrical, in which there is a discrepancy in length between the
two limbs so that to stand on the feet the patient is required to excessively flex one hip and knee while the
other is extended. Asymmetry may result from a difference in length of the remaining femoral segment or
from a concomitant fibular or tibial deficiency on the short side.

Symmetrical

Patients with bilateral PFFD and approximately equal-length limbs ambulate despite the severity of their
limb anomalies, provided that they have functional upper limbs. When seen in infancy, they can begin a
physical therapy program to maintain their musculature and prevent fixed deformity. Trunk-strengthening
exercises should be instituted early in life, and the parents should be encouraged to permit the child
maximum activity. When bilateral PFFD is associated with other lower-limb deficiencies such as fibular
deficiency, it may be necessary to consider surgical intervention and/or orthotic management to control the
deformity. Although for those with severe upper-limb deficiencies walking may be deferred until as late as 7
or 8 years of age (Fig 35A-36.), most of these patients with functional arms assume independent
ambulation sometime between 2 and 3 years of age. It is interesting that these children spontaneously
become hand walkers. When the child reaches 5 or 6 years of age, an effort should be made to allow him to
use "stilts."
Fig 35A-36. A child with bilateral symmetrical PFFD
and bilateral upper amelia is first able to stand and
walk at 7 years of age.

It is at the time of entering school that a child's deficiency in stature becomes important to him. Prosthetic
restoration is directed toward cosmetic rather than functional improvement. With development of good
balance, the child ultimately can be fitted with articulated limbs without amputation to providing him with
adequate height. It must be recognized that the energy consumption required for ambulation is great, and
since the child's activity level is usually reduced, weight is gained easily. The parents must recognize that
weight control is very important and institute dietary control at home.

This group of patients should not be considered for amputation. The feet and ankles should be preserved so
that the patient remains independent without prostheses. We have observed patients continuously using
their prostheses up to 35 years of age, but what their ability will be at the age of 55 or 65 years we cannot
say. It is therefore important to preserve the feet. It is wise to use a helmet for head protection for children
on stilt prostheses, particularly those with upper-limb deficiencies. Children with bilateral upper-limb amelia
and PFFD in the lower limbs prefer to have their feet free so that they may use the feet for prehensile
activities. These activities should be encouraged and are of sufficient importance to contraindicate a
prescription for prostheses.

Asymmetrical

Patients with asymmetrical PFFD and one leg significantly longer than the other walk independently despite
the severe deformity of both legs. When seen in infancy, the major effort should be directed toward
preservation of joint function and prevention of deformity. Orthotic management will be necessary to
maintain knee extension, and lifts should be prescribed as necessary to equalize leg lengths. When the
discrepancy is great, as occurs when the short side has an associated fibular deficiency, disarticulation at
the ankle joint on the short side is indicated. Provision of a Syme-type prosthesis will equalize length and
give reasonable stature. Improvement in height by means of bilateral prosthetic restoration has been
attempted in these children. The short side is fitted with an articulated limb, whereas the long side is fitted
with an extension prosthesis (Fig 35A-37.). Since the combinations of deficiency may be varied, the
treatment plan for each patient must be individualized.

Fig 35A-37. A, patient with multiple limb deficiencies including bilateral asymmetrical
PFFD. B, disarticulation at the ankle on the right side is followed by fitting with a Syme-
type prosthesis for length equalization. C, for cosmetic purposes, to increase height the
patient may be fitted with a right transfemoral prosthesis, and the left leg and foot are
fitted into a molded plastic socket, with patient using his own knee above this
prosthesis.

Lower-Limb Deficiencies: Prosthetic and Orthotic Management


Daniel G. Oglesby, Jr., C.P.O.
Carmen Tablada, C.P.

Providing prosthetic and orthotic care for the limb-deficient child involves more than simply supplying the
proper componentry. The psychological impact on the parents and family must also be considered. In most
cases the very young child has no awareness of being different from his peers. It is only from the reactions
of his parents and others that he learns about his disability.

The prosthetist is often able to help the family deal with these issues by discussing their concerns openly
and calmly and by providing prosthetic care that encourages a normal developmental sequence for the
child. Fitting and delivery of new devices must be timely to avoid adding to the child's feelings of being
unable to keep up with his family and friends. Referral to a local support group can often help the family
faced with the new challenge of limb deficiency. Only occasionally is referral to a professional for counseling
necessary.
For the child with an acquired limb loss due to tumor or accident, there may be the added factors of fear of
death or guilt over the circumstances of the amputation. The emotional scars can be even more devastating
than the physical loss. The prosthetist's role is to help keep the child actively involved in normal daily
activities. A well-fitted fully functional prosthesis can help keep the child and the family focused on
constructive adaptation.

DEVELOPMENTAL STAGING OF COMPONENTRY

The basic principle in pediatric prosthetics is to provide components that are appropriate to the child's
developmental stage. Although sophisticated mechanisms have a role in prosthetics, children's devices
usually emphasize simplicity and reliability. General guidelines based on experience with many children
over time can be given for when to provide a particular level of complexity. The optimum time to utilize a
component for a specific child, however, must be individually determined by the clinic team.

Birth to 6 Months

Most centers do not provide lower-limb prostheses prior to 6 months of age because the child is growing so
rapidly that it would be quickly outgrown. Furthermore, the child must master sitting balance and other
developmental tasks before standing and walking become realistic goals. On occasion, the family may insist
on prosthetic fitting, and very lightweight materials can be used. Such fittings probably have more to do with
the parents' or grandparents' idea of "body image" than the child's.

7 Months to 14 Months

Most centers recommend fitting when the child is almost ready to pull to stand, which varies among
individuals. The major prosthetic considerations are as follows:

1. A socket that allows for rapid linear growth


2. A suspension system that does not encumber the child

3. Regular checkups to monitor growth and proper prosthetic length

The chief biomechanical function of the foot at this age is to fill the shoe. The child is so lightweight and the
gait pattern so primitive that commercial feet are optional. Many prosthetists fashion infants' feet from
lightweight and flexible polyethylene foam material that provides good balance without stress on the
residual limb or next proximal joint. Knee mechanisms are unnecessary for children of this age. Most
infants' prostheses are fashioned from one solid piece of balsa wood or rigid foam and covered with a
lightweight plastic shell. Endoskeletal construction is also possible; one solid tube from socket to foot
reduces weight by omitting the knee mechanism. When the infant with a hip disarticulation is fitted with a
prosthesis, a hip joint permitting flexion is necessary to allow sitting. Once again, the knee joint is
customarily omitted, and the foot is often formed of lightweight foam materials.

15 to 36 Months

During the period from 1 to 3 years of age, limb growth is the most evident change. There are several ways
to accommodate the need for a longer prosthesis. At the time of initial delivery, children's prostheses are
sometimes made 1.5 cm (½ in.) longer than is correct with an equivalent buildup on the opposite shoe to
level the pelvis. When the parents purchase new shoes in a few months (without a lift attached), the
effective length of the prosthesis is increased. It is also possible to place spacer blocks between the ankle
and foot mechanism up to a point; eventually, it may be necessary to cut the prosthesis in half and add
material to the shin plus relaminate. Endoskeletal designs can add longer tubes until the cosmetic cover no
longer stretches any further. Although foot size will seem to lag behind as the uninvolved foot grows, this is
seldom significant for the growing child. Most prosthetists advise selecting the largest possible foot size for
the initial fitting in anticipation of the child's future growth.

As the musculoskeletal system matures, the family may notice variations in the toe-in or toe-out of the
prosthesis. This seldom affects the child's gait significantly and can be simply observed. Only rarely will it be
necessary to realign the prosthesis due to significant postural or structural changes in the maturing child.
Through careful prosthetic planning and follow-up adjustments, it is common for pediatric prostheses to
remain serviceable for a full year or more despite the rapid growth that is anticipated.

37 to 72 Months

During the child's preschool years, from ages 3 to 6 years, manufactured components become available in
simple and basic styles. The preschooler's active lifestyle commonly results in lost or tattered clothing after
a hard day at play. The prosthesis is subjected to similar rigors and must therefore be simple, rugged, and
repairable.

A functional knee is commonly introduced at this age, often with a manual locking option initially. As the
child masters the prosthesis, the knee can be unlocked at home and later at preschool. An extension assist
aids knee stability but may need repairs from time to time. Endoskeletal designs are readily available, and
the components generally fare well; the covers are another matter. Some parents gladly accept the need to
replace the outside covers at intervals and eagerly embrace endoskeletal prostheses. Others prefer the
ultimate durability of the exoskeletal type. Function is similar regardless of the external configuration. A few
families will accept an endoskeletal device without the covering during the more destructive phases of
childhood.

Commercially available feet are preferable starting at this age. Almost all pediatric designs use
nonarticulated feet; the high-level or bilateral case is the possible exception. The solid-ankle, cushion-heel
(SACH) design is inexpensive and reliable; dynamic-response alternatives have recently become available.
Initial results suggest enthusiastic acceptance of the more responsive designs by both children and parents,
although the functional differences for children are as yet undocumented.

7 to 12 Years

The elementary school child goes through minor but continual growth changes. Fit and function should be
maintained by regular follow-up, at least quarterly. As the preteen years approach, both boys and girls
develop interests in new "outside" activities including sports, arts, and social activities such as dancing. The
youth's interests begin to have an impact on the prosthetic design as limitations of the previous artificial limb
become apparent. Each new fitting offers the opportunity to vary componentry to address the increasing
activities of this age group.

13 to 18 Years

During the preteen and particularly the teen years, physiologic and psychological changes are intensified-
for amputee and nonamputee alike. Cosmetic appearance naturally becomes an increasing concern for
both boys and girls. This is also a time of transition for the prosthetist inasmuch as he must recognize the
increasing independence of the maturing amputee and develop a good working relationship that will last on
into adulthood. During this period any number of considerations will arise, from abrupt changes in fashion
(e.g., heel heights) to pregnancy, and each must be handled with the seriousness they deserve.

The ability to conform by being a nonconformist can become a prosthetic factor too. Some will demand that
the prosthesis be inconspicuous and blend into the background, while others will insist on a garish neon
color lamination as a statement of personal expression. The need to provide some means of protection
from the stresses of competitive sports activities is common.

As a general rule, the prosthetic componentry and suspension will become increasingly sophisticated as the
teen approaches adulthood. By the time the youth is 18 years old, adult componentry and fitting principles
are fully applicable.

CONSIDERATIONS BY LEVEL OF AMPUTATION

As has been discussed, the primary factor in selection of prosthetic componentry is developmental
readiness. Beyond that, durability and function become significant considerations. During the "sandbox
years," simplicity and repairability may be prime requirements; as adulthood approaches, much more
sophisticated prostheses are increasingly common.
Partial Foot

Treatment for the partial-foot amputee varies according to the degree of loss. Since children are lightweight
and typically have excellent vascularity, they often do very well with partial-foot amputation. When only loss
of the toes is involved, a simple foam filler is usually all that is required. Unlike the case with adults, it is
seldom necessary to modify the shoes themselves. This is fortunate because shoe modifications would
quickly become expensive for the rapidly growing child.

Forefoot to midfoot amputations often do well with a modified University of California Biomechanics
Laboratory (UCBL)-type insert that incorporates a toe filler. Once the proximal third of the foot is involved,
suspension becomes a problem, and the modified ankle-foot orthosis (AFO) is one solution. Another
approach is to provide a flexible laminated rubber "boot" for both function and better cosmetic appearance.

Syme Ankle Disarticulation

The Syme and similar levels play an important role in pediatric amputations since the growth plates are
preserved (when compared with a transtibial level) and the risk of bony overgrowth is avoided. Furthermore,
most children with Syme or Boyd amputations can ambulate short distances without any prosthesis. For the
very young child there is often insufficient discrepancy to provide a commercial foot mechanism; the
discrepancy usually increases as the child matures due to more rapid growth on the uninvolved side.
Epiphysiodesis just prior to the cessation of growth is always an option. The SACH foot has been the only
option until recently. Dynamic-response feet have been well received by adults, and some types are
beginning to appear in pediatric sizes.

Transtibial (Below Knee)

Foot components are selected according to the function desired, as in adults. SACH remains most
common, but dynamic-response designs, particularly for the older child, have also been well received.
Many methods of suspension are suitable for this population. Cuff suspension alone may stress the
ligaments if not reattached regularly as the child grows. Although a loose cuff or fork strap attached to a
waist belt is more forgiving, such belts are seldom necessary and encumber the children. Many pediatric
amputees do quite well with supracondylar suspension, particularly those who need a little extra
mediolateral stability at the knee. Knee sleeves and the silicone suction socket (3S) design both provide
suction suspension. Joints and corsets are rarely seen, being reserved primarily for the child with marked
ligamentous damage to the knee.

Transfemoral (Above Knee)

The same controversies that exist regarding adult sockets apply to pediatric designs. Ischial containment
and quadrilateral contours have both worked well with this age group. Many prosthetists elect to use an
individualized shape for growing children that is based on the cast impression of their residual limb. The
presence of significant body fat or the use of bulky diapers dictates a unique shape for the small child's
socket. Knee criteria have been previously discussed; a free knee is unnecessary for the very young child.

Hip Disarticulation

One key factor for the hip disarticulation fitting is to plan ahead to accommodate circumferential growth of
the pelvis. The knee joint is usually omitted until the child is near school age. The hip joint is provided at the
outset, however, to permit sitting down. A SACH foot completes the prosthesis. Despite the simple
componentry, the pediatric hip disarticulate typically does very well with a prosthesis. Presumably this is due
to his small stature (short lever arms) and high energy level. Many progress to become excellent hands-free
ambulators even as adults. Most centers are experimenting with thermoplastics and more flexible socket
designs for all levels of amputation. Results to date look very encouraging, and further investigation is
warranted.
Proximal Focal Femoral Deficiency

This limb deficiency warrants individual discussion because of the complex clinical picture it presents. Not
only is there a significant limb length discrepancy, but hip joint involvement is also common. Hip abduction
and flexion contractures are common. The knee is often unstable and may be difficult to palpate within the
short, fleshy thigh tissues. All those factors complicate prosthetic management.

Prosthetic restoration is geared to minimize the excessive trunk bending and internal rotation of the hip that
typify proximal femoral focal deficiency (PFFD) gait. This is accomplished by meticulous casting of the
affected leg and pelvis while maintaining the proper rotation and hanging angle. Careful molding is
necessary to achieve good ischial weight bearing rather than posterior-thigh weight bearing. In cases where
the foot remains, it must be molded in a plantar-flexed position that allows good cosmesis yet permits some
weight bearing on the sole of the foot. For those with telescoping hip joints, maximum elongation during
casting improves ischial weight bearing during stance.

Transparent test sockets are invaluable in evaluating the above factors. Remodification of the positive
model and fitting with a revised test socket is often required. Dynamic alignment is individualized but must
take into account the child's age, physical findings, and any additional abnormalities. For those with
concomitant upper-limb involvement, the socket brim may be utilized as a control cable anchor point.

In some cases, the clinic may elect to initially fit the young child with shoe buildups and/or an AFO. Once
the child has grown somewhat, fitting with an extension prosthesis or ablation of the foot and fitting with a
modified knee disarticulation prosthesis may be undertaken. Rarely, Van Nes rotation-plasty may be
performed and the child is fitted with a modified transtibial prosthesis. Bilateral PFFD is a special
circumstance, and surgical intervention is generally avoided. Definitive treatment with orthotic devices or
with extension prostheses may be offered. As the child with bilateral PFFD grows and the extension devices
become longer, endurance and balance are reduced. Many abandon external devices as they approach
adulthood and choose to ambulate unencumbered.

SUMMARY

The young person with an amputation or limb deficiency will find that his life will be different in some ways
from the lives of his playmates. This does not necessarily mean that he is limited; he will be as functional as
society and circumstances allow. Prosthetic restoration may enhance or detract from the amputees
independence, depending upon the quality of fit and function provided. Careful attention to detail,
developmentally appropriate complexity, and faithful follow-up are the cornerstones of successful pediatric
prosthetics.