THE

PROSTHETIC

MANAGEMENT

OF

THE D. S. Fro,n

the

Congenital deformities amputation or when, without

DEFORMITIES

OF

EXTREMITIES

MCKENZIE,

Ministry

CONGENITAL

LONDON,

of Health

ENGLAND

Limb-fitting

Centre,

Roehampton

of the extremities may come within the amputation, there is sufficient shortening

limb fitter’s sphere after or deficiency to allow

the fitting of an artificial limb to the deformed limb. The classification of these cases is difficult because they present wide variations in anatomy, but certain broad groups emerge in relation to prosthetic management. The material for this paper was gathered from a series of

166

cases

the

Limb

case

are

with

a follow-up

Service shown

incidence

both

at

of

in parenthesis

of these

up

to

Roehampton

conditions,

eight and

years, in the

in each

section,

because

the

but

series

together provinces. they

are

not

is composed

with

many

The

numbers

others

to be taken

of highly

treated

of each

by

type

of

as indicating

selected

cases.

are

common.

the

CLASSWICATION LOWER

Deformities

primarily

appearance they may be present. as

a whole,

and

a degree

with at this

absent (Fig. Deformities The common

I). primarily deformity

is usually

of

the level.

in this

The from

amputation. limb

been

after rays

The

foot

Commonly

Occasionally

some

of which

39 B,

be knee and

Half the pelvis, and the long are sometimes underdeveloped. with varying degrees of equino-

may one

it is the may

NO.

buds

seen

may

of the femur. The hip and may show flexion deformity,

be almost or more

tibia

that

normal

digital

or it may

rays

are absent,

2,

is absent.

hypertrophy considerably, The ankle is grossly deformed

and weight bearing cannot be tolerated the limb in these cases. The skin dimple VOL.

In

toe

surgical may be

there may be syndactylism of two or more toes, and the tarsus may be deformed and incomplete. A skin dimple can usually be found at the front of the leg, often at the junction of the middle and lowest thirds, which is the common site of bowing of the tibia. the fibula unsatisfactory.

not

Rudimentary

tibia often shows deformity which may be a slight bowing, usually forwards, to complete

pseudarthrosis. be deformed.

has as digital

deformities

foot of the

the leg (twenty-seven cases)region is absence of the fibula.

shortening,

shortening but they

genu valgum is not uncommon. bones on the affected side, The ankle is usually unstable, varus. anything

DEFORMITIES

cases)-These

equino-varus

foot deformity, One or more

affecting

marked

due to associated are usually sound,

(two

may resemble a surgical partial There may be some shortening

in association amputations

There

LIMB

the foot

affecting

MAY

1957

Note

Although the and

limb is unstable,

(Figs. 2 and 3). is usually present.

FIG.

I

Congenital deformity of foot resembling Chopart’s amputation.

and

equino-varus (Mr Leon

perhaps

It is usually

and Gillis’s

the

advisable

toe

bud.

case.)

knee

likewise,

to amputate

233

234

D.

Deformities

primarily

affecting

the

thigh

S. McKENZIE

(nineteen

cases)-These

deformities

common. The femur is shortened and there is often a severe flexion knee, so that the thigh is held much in the position seen in quadrupeds.

of both

tibiae.

This patient

was treated

may

be deficient tapering

in various away

ways

to a point

(Fig. a few

4).

by disarticulation

It may

inches

relatively

at the knees.

(Mr

J.

M.

case.)

Turner’s

end,

also

2

FIG.

Absence

are

deformity of the hip and The shaft of the femur

be represented

proximally.

The

by a relatively head

and

normal

neck

may

lower

be absent

or underdeveloped, and there may be severe coxa vara. There is sometimes a pseudarthrosis of the femur. The hip may have good function, or it may be rudimentary and represented by a shallow or absent acetabulum with the attenuated upper end of the femur lying more less in its proximity. The skin dimple can usually be found. The common. limbs

knee The

is often leg and

are usually

sound, but flexion deformity is foot are usually normal. These

surprisingly

strong,

and,

be strikingly

hirsute

Congenital having the

amputation appearance

stumps-A of a surgical

rare

lower

but

in the

it occurs below-knee may

“amputation” This type seen

in comparison

limb,

and

of

as a solitary

of the

(Fig.

Toe lower

in girls,

other

may

limbs. deformity stump

is

in the

upper.

takes short.

the form of a The epiphysis

hip,

but congenital with a congenital

has been seen. deformity, in the defect

the

congenital amputation

common

the anatomy

fairly normal, in association

even

with

in the lower limb it usually stump which may be very

be present

is usually the hip

or

thigh

When

and

dislocation through-knee

buds limb,

are has

knee of

common. not been

5).

FIG. 3 Absence of tibia. This patient had been treated by a Syme’s type of amputation. He is wearing a conventional below-knee prosthesis, but it is doubtful how long the stump will tolerate weight bearing. (Mr J. I. P. James’s case.)

FIG.

3 THE

JOURNAL

OF

BONE

AND

JOINT

SURGERY

THE

Multiple

PROSTHETIC

MANAGEMENT

and bizarre

deformities

singly or in combination. deformity of the upper

OF

(thirty

FIG.

congenital.

The

primarily

to affect

dimple

is affected. (Fig.

Both 6).

symmetrical

the

asymmetry

in this

DEFORMITIES

cases)-The

deformities

They may be confined to one limb, in which case it is usually,

evidently seems

deficiency

the

Multiple cluding

shaft.

ties of both

A skin

Note

appearances

legs may One patient

constriction. to the

ring

which

being

atrophic

be

deformed, has been

in

by slightly

that

ring

they

were

is present,

the

to a varying

extent

limb 7).

by buds, or there amputation-stump part of the limb may

may be greater deformity. The be precarious. It

that

the also

mechanism produces FIG.

legs and left forearm. intelligent

facies.

development .

are lower of one .

may may

The

absent or deficiencies circulation

possible deformities

deformities intype deformi-

by

There the toes

be be

producing constriction the amputation-stump

(

6

Multiple congenital deformities. Right leg deformity is primarily femoral. Left leg is represented by a foot placed close to the buttock. Left arm shows “through elbow” amputation stump. Note skin dimple on right shin. Operative stabilisation of the right ankle was performed before limb fitting. (Mr E. W. Somerville’s case.) Fig. 6 VOL.

39 B,

NO.

2,

MAY

1957

exist

5

distal

ring is not constant. of the limb; thus

seems band

may

limbs

caused

(Fig.

above

characteristic

greater

tissues. of all

235

EXTREMITIES

which case the deformities with total absence of both

seen

site of the constriction loss of the extremities represented up to an of the distal

described

and attractive

represented

suggest

A constriction being

the

THE

There may be a concomitant not necessarily, the same side

congenital amputation

was present.

case

OF

FIG.

unusual

the pelvis and greater bulk of the overlying soft Two children have “amputation-stump” deformities four limbs. “Constriction band” deformities (four cases)-Some show

limb. but

4

Femoral deformity of rather type which was nevertheless

that

CONGENITAL

seldom limbs, side

of

236

D. S.

McKENZIE

FIG. Deformities

of

7

the

lower limbs, with tubing applied to the sulci to demonstrate cord’s having produced the constrictions. (Mr Leon Gillis’s

deformity

if the constriction

bones has Amputation

not been seen necessitated

is sufficiently

severe

(Fig.

8).

the possibility

Absence

or deficiency

in association with constriction band deformities by spina bifida (seven cases)-Although these deformities of the extremities, a congenital deformity included in this paper. resorted to in these

sensibility

incontinence

ulceration one of the fitter. The

completely that

of the

long

in this series. are not cases they arise out

of of

and are conveniently When amputation cases it is commonly

on account of trophic These patients present problems to the limb lack

of the umbilical

reconstruction.)

or

is so

often

is

of the feet. most difficult stumps often

in part,

and

the

present

adds

to

the difficulties of providing a prosthesis not cause chafing and further ulceration

that will of the

stumps. When the skin overlying the ischial tuberosities is also anaesthetic the problem may be beyond solution. Because of these difficulties in limb fitting, amputation should be undertaken only as a last resort, and the prognosis in respect of function with artificial limbs should be guarded (Figs. 9 and 10). FIG.

8

Severe constriction band deformities Relief of constriction by plastic because of circulatory be tolerated on the Fig.

of both lower

limbs.

surgery was required deficiency before a prosthesis could left side. (Mr R. J. V. Battle’s case.)

8 THE

JOURNAL

OF

BONE

AND

JOINT

SURGERY

THE

PROSTHETIC

MANAGEMENT

FIG.

OF

CONGENITAL

DEFORMITIES

9

FIG.

Figure 9-Congenital absence of sacrum and lower lumbar lesions of lower limbs. Treated by amputation of the disarticulation at the left knee. Patient is now walking prostheses. pylons.

Note

OF

(Mr Vincent Snell’s extreme development

case.) of mastered

Figure shoulder articulated

vertebrae. right

leg

on more

10-Same patient, girdle and arms. legs.

THE

EXTREMITIES

10 There were trophic

below the knee and or less conventional showing preliminary This boy ultimately

A. I FIG. Figure Note

Il-Through-elbow Figure 12-Severe absence of opposition

39B,

NO.

case.)

VOL.

11

2,

MAY

1957

FIG.

amputation type deformity. Note bilateral deformities of arms. Fusion of thumb and fingers. Skin dimple Arthur Porritt’s case.)

12

hand bud. (Mr E. of elbow joints and is seen on the right

E.

Harris’s shortening. arm. (Sir

237

238

D. UPPER

Congenital of an upper amputation,

amputation

“

stumps

limb is not uncommon. most of the patients

preponderance

of

this

type

patients with congenital had this type of arm amputation elbow, therefore

“

are:

below

affecting

DEFORMITIES

cases)-Congenital

absence

Since the deformity resembles are sent to a limb-fitting centre.

of

deformity

elbow, 1 1). in the

LIMB

(twenty-four

deformities deformity.

shoulder (Fig. not included

S. McKENZIE

among

referred In order through

Most present

of

our

cases.

Thus

to Roehampton of frequency

wrist,

carpal

these series.

cases

or are

of the

out

of

metacarpal,

part

surgical for the

twenty-eight

in the past the common

treated

distal

that resulting from This may account

new

six months, fifteen sites of congenital

through

by routine

elbow,

above

methods

and

are

(fifteen cases)-These usually take the form of syndactyly, come before the limb fitter. Multiple and bizarre deformities (sixteen cases)-The common feature of most other arm deformities is fusion of the elbow joint with varying degrees of shortening. The hand may be almost normal or may show deficiencies in digital rays, especially of the thumb. There may be only one digit, or there may be an aberrant digit sticking out at a grotesque angle. Deformities

microdactyly,

etc.,

the hand

and

seldom

Syndactyly is common. The skin dimple can incapacitating, especially as they are often bilateral. Complete absence of both upper limbs has been GENERAL

are

Children strikingly

exhibiting handsome.

equally at risk. this observation

often be detected. Fortunately they seen.

evidence in favour these deformities

are 12).

MANAGEMENT

these deformities are nearly always The sexes seem to be equally

Little puts

These deformities are uncommon (Fig.

of high affected,

of a hereditary trend in sharp contrast to

intelligence and all

and often social grades

has been found. such conditions

they are

If confirmed, as syndactyly

and polydactyly, in which a hereditary influence is accepted (Barsky 1951, Bagg 1929) and a further study of this important aspect of the problem is being made. Psychological adjustment to these deformities is usually good, especially when a reasonably good aesthetic and functional result is obtained by limb fitting. A few children have shown aggressive or exhibitionist traits, and one child developed a facial tic. Such reactions usually resolve when the child goes to school. Unless deformities are grotesque or severely disabling the children stead. Abnormal

should

go to a normal

psychological

There have been deformed child.

reactions

instances Marriages

of

school,

where

their

in the parents

suicide of one or have broken down

high

intelligence

of deformed

stands

children

are

both parents after the through one parent’s

them more

in good com4non.

birth of a severely implying that the

deformities in the child were the result of bad stock on the other parent’s side or through allegation of cruelty during the pregnancy, or from morbid obsessions of guilt. Some parents have deserted a deformed child or simply refused to have anything to do with it, necessitating its being cared for in a nursery or a school for the disabled. In most cases, however, the parents

accept

mixture to treat

of sympathy and firmness. the child as normal.

the

child

and

their

responsibilities They

are

PROSTHETIC

patient

with

a congenital

it and

is enough

patients

can

shortening

be fitted

with

to allow an extension

it well

little

with

of the

the

deformity

right and

LIMB

deformity

presents

an

clinical and radiological examination is necessary to assess limb, the range of movement of the joints and the capacity if there

manage

to make

MANAGEMENT LOWER

Every

to encouraged

of the

fitting

prosthesis

of at least without

individual

an artificial

surgical THE

problem.

Detailed

the capabilities of the deformed for weight bearing. In general,

JOURNAL

foot,

intervention, OF

BONE

AND

most

of these

and

in many

JOINT

SURGERY

THE

PROSTHETIC

cases

this

other

deformity

will

MANAGEMENT

give

the

amputation

may

best

functional

have

may

OF

CONGENITAL

result.

DEFORMITIES

In some

to be considered

before

cases

limb

OF

THE

operation

fitting

239

EXTREMITIES

to correct

is undertaken.

flexion

or

In others

an

be indicated.

Limb fitting for the lower begins to make efforts to stand. weight bearing, the deformities

limb is begun as early as possible, ideally when the child At this stage, when the limbs have not been subjected to are probably not in their final state. It may seem politic

to amputate,

in equino-valgus

for example,

a foot

with

absence

of the fibula.

But

an extension

prosthesis can usually be fitted, involving nothing that is irrevocable, and amputation can be resorted to later if the functional or aesthetic result is unsatisfactory. It is surprising how many parents think that the deformed leg will grow to be normal in time if it is left to itself, and may

no amount of explanation never accept that it was

have been themselves. both with

well had the One child

limb seen

will done

disillusion them. for the best, and

been left alone. A similar at the age of sixteen years

legs in infancy for deformities. Prostheses them. She was sullen and unco-operative,

hatred for her mother for agreeing to the amputations, When contemplating

her

for she amputation,

will

to remove freak anatomical children amputation should

the

will

child

but

illustrates.

gain

these

particularly, the Nevertheless,

in function. can

had

the fibula since the much to be desired.

age

self-conscious

been

met earlier. It should not

her

prospects

in the care

minds

in this husband of some

It follows that must be taken

especially extension fall short

39 B,

can

be

suitably

maintained an extension

refusing

should

reactions

may

reassuring too limb

anything

the

long,

operation

child

as the

for

natural

appearance. indications

make

the

was excellent, that she was

to wear

resist

of serviceable there are clear

and,

more

following

case

congenital

absence

of

but aesthetically it left a precocious child she but

slacks,

and

became

that

if she

has

a girl

reaching

a freakish

maturity deformity

is at a serious and,

although

disadvantage

in

amputation

is

an

respect, a relatively normal looking stump may be more acceptable to than a deformity which is more or less bizarre. It is probable that this is of the

girls

who

seek

each case must be treated in balancing the functional

amputation

for cosmetic

on its merits and, and psychological

reasons

as they

in borderline requirements.

cases,

grow

up.

the greatest Nevertheless,

in the younger patients, it is felt that nothing can be lost by an attempt to fit an prosthesis in the first instance, resort being made to amputation only if the results of requirements. Whenever possible surgery should be deferred until the child is

the position

VOL.

and

psychological by

wearing

appearance,

is made to a cast of the foot the equinus the less obtrusive

by fitting conventional

rarely

overcome

care

old enough to share in the decision. The extension prosthesis is constructed fitted, greater

psychologically

A Syme’s amputation was ultimately agreed to at the age of was good, and psychologically the child was restored to normal. a happier childhood if her parents’ request for amputation had

forgotten

of marriage

but she had not done well that she had conceived a

with deformities, and for her father for the limbs would have come right in time. the surgeon should satisfy himself that the

of eight. The functional result Through our failure to recognise of her

be

been fitted it transpired

world

or

attitude in our

unruly and unmanageable. fourteen; the aesthetic result This child would have had

also a handicap the prospective

be

in infancy they that all would

reaction has been seen in the children had had below-knee amputations of

structures and leave a stump be resorted to only when Very

usually

been

the

functionally

parents. a conservative

A girl

became

patient

into

had and

thought that therefore,

temptation In young necessary,

the

bringing

operation

that

help

If the leg is amputated may continue to believe

to the extent

that

the heel

as follows.

A boot,

into

which

in a position of equinus and fixed is the prosthesis, but care should slides

off the platform.

Some

assistance

the

toes in slight dorsiflexion. Below the platform. as much prosthesis is applied by means of side steels in much the

NO.

2,

MAY

1957

the

natural

foot

is

on a platform. The be taken not to force can

be obtained

as is possible of a same way as in the

240

D.

O’Connor

appliance.

The

side

steels

S. McKENZIE

may

be extended

can afford ischial devices as T-straps

bearing if required. and patella straps

cast of the wood, with

leg. For young children ankle and a felt forefoot.

deformed a fixed

Knee applied.

upwards

joints The

to carry

Deformities

ambition

of singing

primarily

affecting

on the

the

corset

which

steels and such and set up to a

the lower part of the appliance is made from For adolescents and adults a metal base may be

used, and in a few cases it may be possible to enclose the deformed within the shell of a metal shin with excellent aesthetic effect. to achieve her to do before.

a thigh

can be fitted to the side whole appliance is made

concert

platform,

foot-These

limbs

foot more or less completely One such patient was enabled

which

can

she

usually

had

been

be fitted

too

with

sensitive

one

of

conventional prostheses for Chopart’s amputation, with relatively minor modifications. there is shortening of the leg as a whole it may be necessary to make a modified platform This

gives

good

function

but

is unsightly

because

of the

bulk

at the

the

When limb.

ankle. ‘-

FIG.

13

Congenital absence of fibula. Drawing prepared by superimposing tracing of radiograph on sketch of prosthesis. The boy came third in his school high

jump

in open

normal

FIG.

Old-standing premature backward The hip corset-top

joints thesis.

tended

FIG.

Deformities

it is usually

The varus deformity Tibial bowing, flexion the

14

tuberculous

hip with

epiphysial arrest subluxation of the was ankylosed. Fitted caliper with free

and knee. with knee

mounted on platform prosCross knee strap and calf band

to correct

knee

deformity.

FIG.

shortening to allow weight bearing must

under

with

13

primarily

although

competition

children.

stimulus

are sound the used, by means

affecting the leg-These deformities are of the fitting of an extension prosthesis. be assessed; if it is in doubt an ischial possible

to retain

knee

function

that is commonly present contractures at the knee of weight

bearing,

boot does not need of a blocked leather

and

by fitting

at the ankle and hip, and

may

disappear

to be extended boot, to accept

with a prosthesis. in his school high

Many patients can jump in competition

even with

joints

can genu

to the

When

inserted

is uniformly

JOURNAL

(Fig.

13).

the

knee

and

hip

condyles, which can be load if this is desirable. between

good

run without a limp. normal boys. The THE

steels

be controlled by a T-strap. valgum frequently improve

entirely.

by a wedge

14

associated with sufficient capacity of the limb for corset should be fitted,

beyond the tibial some of the weight

Genu valgum can often be helped in young children and the foot of the prosthesis. The functional result with this type of deformity fitting third

usually The bearing

OF

after

the

satisfactory

One boy was aesthetic result BONE

AND

ankle

JOINT

placed is less SURGERY

THE

PROSTHETIC

MANAGEMENT

satisfactory, and amputation reason alone. Patients with amputation Patients other weak

are

fifteen

Some

cases

This ankle

usually joint.

felt

those

be

DEFORMITIES

at the ankle may be a tibial pseudarthrosis

fitted with to fit a rigid

such of

takes The

end of the amputation and

CONGENITAL

required which

through the pseudarthrosis to get satisfactory with shortening from arrest of growth after

causes can it is possible

There

OF

cases leg

a similar hip joint

in this

deformity

the form resulting

extension and pelvic

OF

THE

in girls at a later stage fails to unite probably

function. poliomyelitis,

for this require

tuberculous

disease

prosthesis. When control of band to the top of the outer

series

(Fig.

14).

have

been

referred

to

the

of a modified Syme’s amputation, stump is usually satisfactory and

limb

centre

241

EXTREMITIES

after

or

the hip is side steel.

amputation.

the foot being removed can tolerate end bearing.

at the The

stump is not so bulbous as is a true Syme’s stump. Patients with this type of are usually fitted with the metal enclosed type of Syme’s appliance, a fixed ankle

foot

being

used

of an extension

while

the

prosthesis,

child

but

is small.

of femur. Diagram thetic fitment. Note

showing ischial

and free swinging The

sary

pelvic

there

results maybe

are

comparable

difficulty

with

in preventing

prosbearing

artificialknee band

was

this case because instability.

in

functional

is absent

FIG. 15 atresia of upper parts

Congenital

joint.

The

if the fibula

neces-

of lateral

16

FIG.

Child aged two and a half in whom the right femur is absent, showing

normal

the

open to these

Fitted with extension

prosthesis Figure

of the except

band

15,

type shown in that a pelvic

was not required. Mr Eric I loyd’s

(The late case.)

15

FIG.

the

existence

patients.

prosthesis

FIG.

from

better children

and, indeed, who have

remains removal,

and they by those

rotating may been

on

the

be perfect. subjected

are frightened in attendance,

stump.

It must

Occasional to amputation and unco-operative. of the white coat

playthings and sweets. If the child will not permit attempt a few weeks later is usually successful. When the tibia is absent it is seldom possible disarticulation

at the

prosthesis. Deformities

primarily

problem. The less at the same that of the is unstable VOL.

39 B,

knee

will

probably

affecting

thigh-These

and a cast

the

aesthetic

result

to be taken

followed provide

at the first

a functional by the fitting a more

knee

2,

MAY

1957

is

with young experience

This can usually be overcome by suitable distractions in the

to conserve

cases

that

has been experienced the memory of painful

visit

by the way of a second

joint,

and

a

of a conventional difficult

prosthetic

flexion deformities of hip and knee are such that the knee is usually more level as the hip and lying anterior to it, so that the axis of the leg is anterior

bodily centre of gravity. and tiring to manage, NO.

the

be required,

be admitted

difficulty in that

16

If a platform prosthesis is fitted in the axis of the leg and a free swinging artificial knee cannot be controlled.

or to it

242

D.

If the “

whole

weight

jack-knifing

“

is carried

by

at the hip and

knee.

trunk is a complex at the pseudarthrosis

one

commonly if present.

the

S. McKENZIE

foot

The

on

the

movement

platform

there

of flexing

and

taking place partly If it is necessary

is a strong extending

tendency

the shank

to on the

at the hip but mostly at the knee, to use an artificial hip joint its

and best

position can be determined only by trial and error. It follows that the extension prosthesis for deformities of this type must usually afford ischial bearing. The side steels should be allowed to incline slightly backwards from above downwards so that, at the level of the platform, they are in the plane of the axis of the centre of gravity. The natural heel is then usually at about the same level as the normal knee. Knee joints

can

upper

section

good, and Aesthetically

be fitted

to the

steels

steels

(Fig.

of the

is comparable the anterior

at this

level,

15).

the

Function

boot

on

with

with that obtainable position of the knee

this

its platform type

being

of prosthesis

by a patient with produces a bulge

disarticulation which may

attached

to the

is usually

quite

at the knee. be more or less

FIG. 17 Right leg is similar to that shown in Figure 15, except that the femur is more nearly complete. Illus-

trates

the

difficulty

of

concealing

knee and foot. The apparently

been

left leg has

represented

by

some rudimentary structure of the type shown in Figure 18, but this unfortunately was amputated in infancy. A tiltingtable prosthesis has been fitted. (Mr F. G. St Clair Strange’s

case.)

FIG.

18

Congenital deformities of both lower limbs showing prosthetic fitments. The right prosthesis is an extension limb. On the left a more or less conventional aboveknee prosthesis was possible. This child goes to a normal school.

(Mr S. L. Higgs’s case.)

p

FIG.

17

conspicuous according project forwards and by setting the artificial In some cases the

FIG.

to show

the length of the through the trouser

knee joints prosthetic

as low problem

femur. In a sitting position leg or below the skirt. This

as possible has been

(Fig. 16). simplified

by surgical

18

the toes tend to can be minimised correction

of

the

deformities of the hip and knee flexion, with arthrodesis of the knee in extension. This may give an excellent limb in good alignment, with robust function on a platform prosthesis. Sometimes a Syme’s amputation, either alone or in association with a corrective operation, has been used. The stump can then be fitted into a conventional thigh prosthesis if the socket is modified

appropriately,

in such a way that rather than in the doubtful Van

but

care

must

the axis of the prosthesis line of the tibia. The

if the functional result Nes (1950) described

is ever another

be taken

that

is in the aesthetic

so robust method

the

socket

is aligned

in the

container

plane of the axis of the centre of gravity result of this procedure is good, but it is

as that obtained with the extension prosthesis. of treating these cases. Arthrodesis of the THE

JOURNAL

OF

BONE

AND

JOINT

SURGERY

THE

knee

PROSTHETIC

is performed

MANAGEMENT

and

OF

a femoral

CONGENITAL

osteotomy

is done.

180

degrees and retained in this position round to the front. The foot is fitted conventional below-knee limb prosthesis. the movements technique seem

of the artificial shin. good if the full 180

residual

deformity

flexion

the

rotation

the

femur

was

a few

was

refractured

difficulties in the flexion deformity part

with

of the degrees

his stick.

hip.

distal

I have

and rotation

243

EXTREMITIES

fragment

is rotated

seen with

functionally the can be ensured,

only

one

and

after

still

patient

through

that

the

relative

possibilities of this and if there is no

so treated.

a subsequent

more

loss

tracking” of the prosthesis. the boy never walked with

It is emphasised

THE

osteotomy unites. This brings the heel into a specially fitted socket within an otherwise The patient’s ankle then acts as a knee to control

occurred “

The

OF

the

of 180 degrees.

union

alignment and of the hip, and

while

Aesthetically degrees of

short

DEFORMITIES

failure

of

rotation,

There was great confidence of this

case

In this

accident

was

case

in which resulting

in

a slight residual and could not due

to alignment

Fi. 19 The firstset of prostheses supplied at the age of two years to the patient shown in Figure 18. The type of walking stick with flat base illustratedhas proved of value for young children. problems

and

does

this case to note of gait immediately

not

that

the plantar

sensory

Congenital

amputation

special hip.

The must

Multiple

latter

will

hip joint

prosthetic

work

if necessary 39 B,

VOL. E

on

with NO.

2,

(Whillis

can

there

probably

the

necessitate

be fitted the

the

shorter

1957

boot,

provision

two,

and

possibly

the

conventional such of an

position problems

lower

of the

with

factors

the correct

both

is unsound.

It was

interesting

in

quadriceps in the rhythm the reflex originates in

1953).

by trial and error. deformities-These produce When

principle

over the function of the re-education. Presumably

are complicating

be required.

a surgical MAY

that

endings

its merits. on

imply

stumps-These

should

be decided and bizarre

be taken

must

nerve

difficulties unless

prosthetic and

necessarily

the calf muscles took and without extensive

limbs

limbs

as congenital

ischial

for it may of great

are short

longer limb with a caliper

and

present

dislocation

no at the

bearing limb and, if a be difficult to determine complexity

it is often

and possible

each

case

to begin

being fitted without extension, if required for stability. When

244

D.

S. McKENZIE

mastery prosthesis

of this apparatus to the longer

physique, For

the prostheses for the shorter limb example, the patients with one limb

and

other

the

First

the

limb

foot

on

is achieved, progression limb to bring the limb

by a deformity the

short

an otherwise conventional been amputated, it was

was

thigh necessary

an appropriate extension with the general bodily

being lengthened correspondingly represented by a foot located

of the

side

is made by fitting into proportion

thigh,

fitted

have

undergone

on a specially

the

made

following

ischial

pylon. In one case in which to use a tilting table pylon.

socket

and was to have

beginning to a Z-plasty “

walk, done

“

that this patient will require amputation Spina bifida with amputation-Amputation partly The

anaesthetic, and general principles

be protected by full ischial fitting felt-lined slip socket When there is incontinence have to

The been

peel

so

be seen

but

thigh

is treated

Recent corsets

formed

have

remains

corrosion

eventually. stumps

the

been

with

to relieve these are applicable.

used

with

but

leather

unscratched.

method steel

this

Stainless

UPPER

It is not our practice to begin limb lower. The instinct to walk is practically a prosthesis to the lower limb at about

are

Various

and be

The

steels

value can

to corrosion. Anodising is also

is expensive and is also a possibility

coatings

skin for

has

this

usually

and must

a loosely stump. effects of

plastic

the plastic of

almost

bearing stump

area is adequate, and protects the anaesthetic against the corrosive

may

success.

susceptible

bifida

of weight below-knee

lacquer.

a

on

deterioration It is probable

spina

areas The

bonding

silicones

some

is very

it remains

set in.

with

that

Duralumin

from circulatory blood supply.

in association

satisfactory

suggests

intact,

has

by coating to obtain

work

by heavy plating. the metal provided

until

but ulceration to improve

it is necessary of limb fitting

it is difficult

flake.

adequately will protect skin

apart had

conventional prosthesis, and an The raising to full stature was very gratifying. Some patients

bearing when sensibility in that suspended to the stump by elastics the prosthesis must be protected

leather-work tried,

or

Polythene

equipment and control

band deformities-These cases are treated on similar general principles. Thus, ring constrictions at both ankles was fitted with a simple extension prosthesis

the shorter side made it necessary

urine.

within

the small foot had, unhappily, The length of the pylon was

been achieved the pylon was replaced by a fully articulated extension prosthesis was fitted to the other limb deformity. done in several stages. The results in these cases have been are even able to go to normal schools.

always friction.

programme.

bearing

adjusted to permit of weight bearing on the other limb with no prosthetic from a surgical boot and ischial bearing caliper if required. When stability

Constriction patient with

(Figs. 17 to 19). to the buttock,

close

tended purpose.

be protected

A good

cellulose so long

effective

paint as the

will not readily it is difficult to work.

scratches

but

LIMBS

fitting at such irrepressible, the age when

an early age in upper limb cases as in and use can be made of this in fitting the child could normally be expected

to start walking. In the case of the upper extremity, however, the deformed arm or stump is used freely and is usually sufficiently dexterous to meet the simple needs of the very young child, who is not self-conscious of the deformity. Nevertheless we regard it as important to have school. sensitivity

the child At this

fitted stage

with the prosthesis he is plunged into

to comment

by his

fellows,

and accustomed a new environment,

he should

look

to its use by the time he goes to and to reduce his inevitable

as normal

as is possible.

Apart

from

this we have no hard and fast rules but, by keeping these patients under observation, begin prosthetic work when the child’s intelligence has developed sufficiently to co-operate well. The youngest child that we have fitted with a functional arm prosthesis was two and a half. We have known instances in which premature limb fitting has caused the child to form a revulsion The

to a more

from usual

the

prosthesis

procedure

functional

and

to persist

is to fit first arm

as intelligence

in refusing

a simple

arm

to wear

of largely

it through aesthetic

the value,

years. and

to progress

develops. THE

JOURNAL

OF

BONE

AND

JOINT

SURGERY

THE

PROSTHETIC

MANAGEMENT

OF

CONGENITAL

represented

by

to give

Congenital

to the

amputation

length

single

powerful

opposition,

but

stumps-These

of stump.

and

digit. the

can

no special

Palm

case

appliance

with

was

be fitted

with

difficulties

artificial

of little

the

within

unless

the

lacks

an

mutilated

partly

that

finds

any

patient’s

of

provision

a purely “dress” poor aesthetic

VOL

39B,

NO.

useful

in

its

This natural

MAY

1957

may a

sort state.

give

usually

has

encumbrance. attempts

scars

at

in awkward

be

in purely

and

contractures.

unsuccessful

In

surgery

the

low. this

group

aesthetic

instance to replace absent too, may give disappointing

digits.

come

for

prosthesis.

the for

These cases, results because

of hand Even

prosthesis will be bulky and of appeal. (Professor T. Pomfret Kilner’s case.) 2,

have

These

often

hyperaesthetic

patients of

an

for

he possesses

after

with

such

morale

Some

more

appliance

case

pincer

20).

patient

patients,

associated

consequence

In

way

the digits

possibly

sometimes

fitter

grasp.

they

The

reconstruction,

situations,

this

(Fig. and

in using

some

surgical

infinitely

provided

so expert he

In

uncommon, results.

of

to fit a palm like that used

plate

be

seldom

limb

the

power

hand.

can

Moreover

is

the

opposition

are

of

be possible

it may

a

become

FIG. 21 of fingers.

appropriate

hand-These

province

hand

disappointing

absence

provided

prosthesis the

the

carrying function

Congenital

was

value.

affecting

come

cases

_________

245

EXTREMITIES

thumb

practical

conventional

these cases

-

THE

arise. Deformities

-

OF

20

FIG. Hand

DEFORMITIES

the such

structure that the

of the prosthesis .

look

natural

and

natural cannot

hand is be mounted

often to

.

inconspicuous:

in

any

case,

246

D.

the

prosthesis

is

inevitably

an

S.

McKENZIE

encumbrance,

and

it

is

often

quickly

discarded

(Fig.

21).

Multiple limb

and

bizarre

deformities-This

prosthetics.

usually

The

more

deformities position.

or

he

less

are

time,

and

and

such that has usually

of

prostheses,

which

the

less

useful

side

the

patient

necessarily

is

should

the

be

the

the

attempted

hands,

deformity

in upper

appearance

about

is The

this.

prosthetic

point

function of

view,

management

on

at first both at a

when

one

side

first.

of upper cap for

23

limb. Modified suspension. The

conventional

above-elbow

using shoulder elbow lock is operated by the digits by means of a toggle bar. Flexion at the elbow and manipulation of thumb or split hook is operated by separation of scapulae in rounding shoulders. The deformity was bilateral. Figure 23-Same case as shown in Figure 22. This shows the extent of the rehabilitation that may be achieved in cases of this severity. The

fused

elbow

or

nearly

full the

is full

the

head

function of

and

neck

without

this

when

sort,

and

Three

unusual

same.

arm

The

articulated

through

represented

the

problems

presents

patient

and the

deformity

of one

joint

of

is prevented

much

being

the

bones the

to limb,

problems general

so impairs this

FIG.

congenital

The sensitive

From

22

FIG.

Figure 22-Severe prosthesis fitted

difficult

inevitably

attempt.

approach

most sides.

to make a prosthesis hang in a natural skill with the deformed hands that the

enclose

abandon to

the both

affects

it is impossible achieved such

and may it is advisable

frustrated

deformed,

arms

grotesque

usually patient

feels

provides

commonly

are The

application

that

group

deformity

of the

from

upper

half There

disproportionate. provision

of

a surgical

its

own.

If the

reaching

length

of his body. is no

of

and

his mouth,

the

A prosthesis

satisfactory

pseudarthrosis

arm

indeed

cannot

prosthesis must

as a whole

from

be

reaching

provide

for

this

a deformity

considered,

especially

is bilateral. cases

were hands the upper

of

shorter

severe

double

than

lacked

one

or

wrist

with

a short

end

of

the

deformities

in the other,

ulna

two

but

digital

rays

forearm fused

to

have the

been

anatomical

but

otherwise

composed the

lower

In were

were

of a single end

THE

seen. features

of

JOURNAL

the

fairly bone

radius. OF

each

BONE

the

They

normal. which An AND

long

substantially probably elbow JOINT

joint SURGERY

THE

of sorts

PROSTHETIC

was

MANAGEMENT

present,

lay some distance so that the arms

and

OF

a short

humerus

below an indefinite were held in much

patients were highly fitted with prostheses Three patients

CONGENITAL

intelligent of definite, have been

DEFORMITIES

terminated

abruptly

felt

is fully rehabilitated is being undertaken case but, in addition

to offer

much

or

partly

flail

because

arms.

flail elbow by using elbow cage. If the that

so

a split

the

chest

or

instance,

other

that

merits, and

the

possibilities

and

many

demand

prosthetist

difficulties the

tools

an

at

surgically.

The

stages

are the

are

it

of all the joints, begging. These They the

have

been

upper

limb.

benefit

to

in these above The

able

be

sought

to

help

of

on

palmar

the

be taken between

on

its

surgeon

prosthesis

and,

possibility

in selected

splint

of the

comprehensive

must

liaison

the

a

to an

Control

cases. are by no means closest

of of

mounted

a suitable

case

cases flexion

operated.

Each

of the

in certain

voluntary

prosthesis

and

infinite.

of movement

provide

construction

should

surgeons

where

third is aged fourteen. has been considered mutilation, it was not

range

with to

ingenuity. in

opinion

plastic

the

be carried

of success described

technical

all

orthopaedic

applied

possible

can

variation

utmost

is demanded

arise,

limb

the

be

be

and

used for the above-elbow a device can be fitted

prosthetic

of individual

narrow

can

it may

scapular muscles is, however, a prerequisite It will be realised that the types ofdeformity and

end,

feet: one woman did exquisite needlework that were supplied because their function too set in her ways to learn. The second

was

technique

For

a harness like that hand is functionless,

hook

at its proximal

and in employment as an accountant. The and is promising well. Pectoral cineplasty to parental resistance to the consequent

success

247

EXTREMITIES

and used their short limbs remarkably well. if limited, usefulness (Figs. 22 and 23). seen with total or almost total absence of

tunnel would have been small. Flail arms (eight cases)-Prosthetic flail

THE

glenoid. There were sharp contractures the position of a dog’s forepaws when

They had acquired astonishing skill in the use oftheir in this way. She did not persevere with the prostheses was limited and she was, at the age of forty-two, patient Fitting in this

OF

of

when

improving

cases.

SUMMARY 1.

Congenital

infinitely

defects variable,

of a

the

extremities

broad

are

classification

described.

in

Although

relation

to

the

prosthetic

detailed

anatomy

management

has

is been

suggested. 2.

Most

patients

with

intervention.

With

amputation.

A plea

recourse

these

this

is made

functional

the

child

and

3.

The

prostheses

4. 5.

The

application

The

incorporation

is old

will for

should

to amputation

of

deformities

they

cosmetic enough

at least

applicable

to

of similar

the

and

features

a prosthesis as

in this

respect.

in which

that,

when

without

function

they It

prosthetic

possible,

major would

Leon

that

falls

be

types

of

to cases

deformity

of acquired

of artificial

arms

are

briefly

shortening

deferred

short until

in flail

arm

described.

is mentioned. splints

I am indebted to the Chief Medical Officer, Ministry of Health, for permission to publish thanks are also due to Mr H. J. Seddon for advice and encouragement in preparing the script to Mr J. S. Barham for the drawings, and to all those who have referred patients to Mr

after

is suggested

equipment it should

surgical have

decision.

various

techniques

of certain

attitude to cases

requirements, in the

with

as good

a conservative

be confined

to share

be fitted

can

have

is discussed. this paper. My and illustrations, me, in particular

Gillies.

REFERENCES BAGG,

H. J. (1929):

Hereditary

Abnormalities Anomalies

A. J. (1951): Congenital NES, C. P. van (1950): Rotation-plasty Surgery, 32B, 12. WHILLIS, J. (1953): Personal communication. BARSKY,

VOL.

39 B,

NO.

2,

MAY

1957

for

of the

Limbs.

of the Hand. Congenital

American

Journal Defects

of

Journal

of Anatomy,

of Bone and Joint Surgery, the Femur. Journal of

43, 167. 33-A, 35. Bone

and

Joint