ETIOLOGY

THE

M.

AND

P. KATZ,

B. J. S. GROGONO,

Congenital
thought

of

then

as compared

with

knee

in girls,

cases

equal
and

being

the

most

common.

Cleft

and

in

of the

harelip,

(1947)

review

(quoted

series

hip,

or one

chest

cage

by
It was

of cases

the

2,393
of the

dislocated

congenital
of the

knee

dislocation
hip and foot

deformities,

of the fibula and dislocation

of the elbow are some of the other deformities
dislocation
of the knee (Forgon
and Szentp#{233}tery 1961).
In some
of the families
studied
genetic
transference
of the condition

In Provenzanos

1822

Kopits
(1925)
reviewed
of congenital
dislocation

cent of the patients

with
congenital
dislocation

palate,

CANADA

Szentp#{233}tery 1961).

of a larger

1947).
cases

dislocation

Sixty per
anomalies,

Chatelaine

in a review

(Provenzano
found
eleven

of congenital

dislocated
hips.
other
congenital

by

MANITOBA,

Winnipeg

by Forgon

but

OF AMERICA,

WINNIPEG,

Children,

described

(quoted

DISLOCATION

STATES

Crippled

was

in 1834

common

923

UNITED

K. C. SOPER,

and

Hospitalfor

the

by Bord

was found
to be
congenital
anomalies

for every eighty-four

of the knee
have
deformities

the Shriners

to be more

sex distribution
patients
with
knee

and

OF CONGENITAL
KNEE*

ILLINOIS,

MANITOBA,

dislocation

1891)

at first

CHICAGO,

WINNIPEG,
From

Shattock

TREATMENT
OF THE

hypoplasia

seen

with

has

congenital

been

evident.

of 200 cases,

seven patients
had a family
history
of congenital
dislocation
of the knee.
McFarlane
(1947)
described
a family
in which
a mother
and her
three
children
by three different
fathers
all had congenital
dislocation
of the knee.
Etiology-Both

intrinsic

genetic

abnormalities

pointed

out

and
; the

that

it is often

extrinsic

causes

extrinsic
difficult

have

causes
to

been

are

distinguish

between

those
produced
by adverse
factors
occurring
during
the extrinsic
causes
suggested
have been abnormal
quadriceps

muscles,

gestation

and

(1891)

Shattock
as the

traumatic

and

cause

of congenital

had breech
deliveries
the chin.
This position

patients

under

dislocation

Drehmann

suggested.

mechanical

The

causes

Duraiswami

genetically

inherited

the development
foetal
position,

during

intrinsic

factors.

are
(1955)

anomalies

of the embryo.
primary
contracture

and

Some of
of the

parturition.

(1900)

postulated

dislocation

of

an

the

abnormal

knee.

In

with the knees in a hyperextended

produced
a hyperextension

foetal

cases

position

that

position
force
at the

they

during

reported

the

and the feet locked

knee,
causing
it to

dislocate.
This theory
failed
to explain
the etiology
in those
cases-the
majority-in
which
there
is not a breech
delivery,
although
the percentage
of breech
deliveries
with congenital
dislocation
of the knee is higher
than the expected
3 to 4 per cent of breech
deliveries
in the
general

population.
Middleton

(1935)

believed

the quadriceps
partly
replaced

muscle.
He
by fibro-fatty

failed

that

to

prove

of the dislocation.
Trauma
to the
Mauclaire
because
dislocation
*An
112

(quoted

the

muscle

knee

during

by

Forgon

that

version

of this

paper

deformity

this
This

changes
birth
and

it has been shown

in dead
of the knee, the femoral

abridged

the

came
to
tissue.

was

were
was

was

conclusion
has not
really

implicated

Szentp#{233}tery 1961).

the

result

after
been

of

primary

and

at

the

British

contracture

not

of the

dislocated

longer

an

Orthopaedic
THE

to be
author

secondary

This

is no

of

these
muscles
because
the

as a cause

infants
that when an attempt
epiphysis
will displace
before
read

a primary

finding
accepted

effect
knee

accepted

by

theory

is made
to produce
anterior
the knee will dislocate.
Association

JOURNAL

OF

Meeting,
BONE

AND

Bristol
JOINT

1965.

SURGERY

THE

Most

reported

means.
in

ETIOLOGY

Mayer

8 1 per

33 per

of

plasters,

traction

or

the
later

six

achieved

of

that

knees

age

it

is often

achieve
a reduced
position.
to lengthen
the quadriceps
and

to

release

in order

the

Mayer

ropsies

(1913)

and

similar

anterior

dislocation

cruciate

ligament

and

elongated.

tibial

plateau

found

covering

surface

of

ODell

and

cruciate

Holt

has

to

Less

been

completely

to

absent

knee.

considered

classified

configuration

ofthe

by the

to movements

foetus

demonstrated
foetus

after

the

shown

that

the

of an
and

in the

embryo
During
the
differentiated,
and
VOL.

mesenchymal
cruciate

ingrowth
cruciate

that

of tissue.

knee
ligaments
The

after
knee

older

to

infants

the

one

to
of
FIG.

congenital

into

knee the
condyles

joint-Henke

and

the intra-articular
a

joint
and

sequence

Congenital
dislocation
of the knee with the tibial
condyles
lying
anterior
to the femoral
condyles.
There
is also hypoplasia
of the fibula.

five
to be

of the

(Magnus

experimentally

in the

the

was

been used
deformities

and

it necessary

treated
of

1960).

King

dislocation

kneejoint

found

patients

structures

poorly
; in

(Fig. 1).
of the knee

(1960)

anterior

and

knee.
In this form of hyperextension
of the
condyles.
The posterior
surfaces
of the tibial
the femoral
condyles
Prenatal
development

of the

In some
the

to

to four

cruciate

of the knee
The category

is congenital

normal.

fixation

three

anterior

ligament

(1964)

deformities
entities.

here

the

hypertrophied
and

examinations

posterior

and

have

of external

of age ; follow-up

of

serial

was

(1929)

terms
hyperextension

Finder

hyperextension
distinct
clinical

of

of the

found

(Niebauer

form

using

and

cartilage

cruciate

in some

position

only

plan

attenuated

the anterior

be

a reduced

of

general

anterior

be

elongated

posterior

Classification-Many
describe
various
the

be

frequently

noted

the

patient

also

The

often

femur

margin

end

of age.

achieved

rate

reports

was

McFarland

(1954)

ligament

developed.

upper

tibia.

age ; a cure

nec-

; these

and

kept

been

with

The

rounded,

to

non-operative

had

capsule

patients

to

of

months

by

a cure

113

KNEE

position.

on the

found

that

months

knee

lengthen

and King
tendons,

There

posterior

the

to

thirty

knee

was

the

completely

of the

the

OF THE

successfully

found

three

were

were

contracted.

was

patients

reviewed

tibia

flex

six months

of

The

to

until

reports

of the
was

after

anterior

findings.

quadriceps

treated

in a flexed

also

dislocation

revealed

three

Niebauer
and patellar

knee

surgical

congenital

the

the

before

necessary

contracted

to reduce

managed

treated

Most

position

been

and

gradually

strapping.

revealed

months

have
patients

in those
was

reduced

early

were

DISLOCATION

OF CONGENITAL

sixty-eight

who

treatment

maintain
years

treated

reviewed

those

was

non-operative

TREATMENT

patients
(1913)

cent

cent

AND

1905).
normal
had
menisci

tibia dislocates
in front
of the femoral
lie adjacent
to the anterior
surfaces
of
Regher

structures

(1874)
were

Fell and Canti

(1934)
knee joint
continued

been
develop

of differentiation

transplanted
within
is first

to
the

believed
the result

the

bony

adaptation

disproved
this when they
to develop
in the avian
another
knee

the

that
ofan

and

menisci,

site.
are
then

It has
not

the
the

been
result

capsule

1910).
Chondrification
of the femoral
blastema
is complete
by six weeks;
at seven weeks
the cartilaginous
form
of the tibia is complete.
eighth
week
the anterior
and
posterior
cruciate
ligaments
are completely
and the foetal
knee joint
is a miniature
replica
of the adult
knee joint
(Gray

ligaments

Gardner

1950).

49 B, NO.

1,

FEBRUARY

(Bardeen

1967

1 14

M. P.

KATZ,

B. J. S. GROGONO

CLINICAL
There
boys

were

and

five

one

girl.

cases

of congenital

Their

ages

at

K.

C. SOPER

MATERIAL

anterior

the

AND

time

dislocation

of the

of operation

were

knee

in three

respectively

four,

children-two
four

and

nine

years.

There
illness

was

with

In

dislocated

of the

Two

was

limited

The

range

patients

had

that

subtalar

had

There

were

a very

short

Radiographic
of the

the

on

an

only

instance

mother.

of maternal

This

hips

or

was

knees.

treated
All

three

brought

from

all the

anterior
Two

absent

or

90

to

and

popliteal

fossa.

degrees

of

flexed

15 to

The

flexion.

tibia

Flexion

be hyperextended

sign

knees

had

hips.

subluxation

of

fibula.

and

all

a positive

or subluxated

hypoplastic

20

could

drawer

lateral

dislocated

the

by at least

painless.

of the

of dislocated

knees
in

knees

completely

position.
was

their

palpable

and
was

with

All

demonstrated
posterior

All three
the

patients

ankle

lateral
drawer

joint

in

of the

patients

had

in any

of the

patients.

sign.

had

signs

three

of

of
the

subluxation

of

4).

signs

of generalised

neck,
the

when

There

thoracic

findings-At

tibia

The
in one

of dislocating

easily

a definite

signs

(Fig.
no

case.

history

walked
were

femur

fibula.

joint

patients

condyles

had

dislocated

extremities

in any
pyelonephritis

a family

to 90 degrees

children

the

there

of movement

in the
of

was

the
on

hypoplasia

delivery

of recurrent

mentality.

anteriorly

the

that

case

femoral

knee

All

had

no

The

20 degrees.
instability

of breech
was

had normal
examination-All

degrees.

the

history

pregnancy

antibiotics.

patients
Physical
20

no

during

zero

femur

ligamentous

scoliosis

and

degrees

(Fig.

1).

laxity

pectus

of flexion

In

the

the five knees

lateral

One

patient

carinatum.
view

the

showed

tibial

an anterior

plateau

was

dislocation

severely

inclined

posteriorly.

intercondylar

view

notch

The

to be markedly

reduced

In

the

all

(Fig.

4).

patients
One

ofthe

patients

of the

third patient
The patient

had normal
with scoliosis

The
showed

of the
with

hips.
had

showed

that

in a child

subtalar

bilateral

joint

dislocation

5) ; another

(Fig.

femur
with

patient

Hypoplasia
of the
multiple
hemivertebrae

tibial
plateau
sloped
a shallow
intercondylar

posteriorly
notch
and

had a bucket-handle
tear
to the tibia in the anterior

was
ofthe

had

the

height

of the

of a similar

age

demonstrated
knees

by

had

a unilateral

fibula

at operation-Four
of the knees
showed
in the fifth knee this ligament
was thin
absence
ofthe
posterior
cruciate
ligament;

One knee
unattached

were

hips

dislocating

as compared

subluxation

dislocation

region.
Findings
ligament;
complete

of the

also

intercondylar
(Figs.

2 and

3).

cineradiography

bilateral

subluxation

congenital

of the

hip.

The

was present
in three of the five knees.
in the lower cervical
and upper
thoracic

30 to
poorly

complete
absence
of the anterior
cruciate
and elongated.
Two of the knees
showed
in one knee this ligament
was attenuated.
45 degrees
developed

of the medial
halfofthejoint,

in all five knees.

tibial
spine.

meniscus
so that

; in

three

All

knees

the menisci
abnormally

knees

the menisci

the

were

mobile.
All

the

knees

could

be

reduced

without

flexion
was limited
to 90 degrees.
Treatment
and results-Unfortunately,

lengthening

no patient

was

the

anterior

brought

for

three
years.
The knees
could
not therefore
be held reduced
plaster.
The aim of our treatment
was to reduce
the dislocation
After reduction
we allowed
function
; in doing
this we thought
collateral
ligaments
stable
knee.
In

all

cases

provide

the

knee

cruciate

ligament

and
the

bony

cruciate

configuration
ligaments

with

extra

stability

was

reconstructed.

were
so
For

that

would

develop

found

to

it would
this

purpose

be
remain

structures,

treatment

although

before

the

age

of

and functional
in a brace
or
and to allow weight
bearing.
that the joint
musculature,
the
normally

either

to

absent
reduced

we

used

THE

JOURNAL

give
or

slight
OF

BONE

patient

hypoplastic.

in a brace

with

the

an

modification
AND

JOINT

a
To

anterior
the
SURGERY

THE

technique

(Fig.

described

6).

medial
not

The
to

to

hole

ETIOLOGY

so

by

as to

A
the

be

taut

hole

was

epiphysial
to

CONGENITAL

repair

DISLOCATION

the

lateral

reconstructed

anterior

anterior

from

the

OF THE

ruptured

fashioned

through
The

prevent

of

was

drilled
plate.

enough

OF

(1963)
for
ligament

cruciate

patella.
across

TREATMENT

Jones

anterior

the

pass

AND

the
femoral

dislocation

cruciate

retinaculum
was

at zero

pulled

degrees

ligament

and tendon
so directed
as

condyle

ligament

115

KNEE

through

of flexion.

this
After

FIG.

Figure

and

3-Normal

Subluxation

operation

The
flexion

limb

1,

was

retained

fitted.

exercises

49 B, NO.

the

knee:

of the subtalar

was

was

isometric
VOL.

the

plaster

The

put

to strengthen
FEBRUARY

into

intercondylar
poor

joint,

a long

1967

the

view

shown

the

shows
of the

intercondylar

of the knee joint

FIG. 4
in all five limbs
of the knee.

plaster

after

were

view

development

intercondylar

for six weeks,

patients

3
reduced

FIG.

knee:

2-Dislocating
intercondylar
notch,

which

allowed

quadriceps.

with
up
The

of a child

affected

10 to

a long

braces

in
were

of

worn

dislocation

of flexion

allowing
their

the

Figure

of 9.

by congenital

15 degrees

leg brace

walking

height
eminence.

of the

knee.

20 to 90 degrees

braces
day

and
and

were
night.

of
given

116

M. P. KATZ,

Results-Four

of the

five

knees

B. J. S. GROGONO

treated

have

AND

remained

tibia

area
with

of horizontal
angulation

tibial
was

plateau

done

to

for

bring

tibial

FIG.

Bilateral

congenital

dislocation

SOPER

One

patient

was

able

to walk

any signs of subluxation

or dislocation;
The knee that redislocated
had a very

articulation

the

C.

reduced.

without
his brace
ten weeks
after operation
without
the other
knees are still being protected
in the braces.
small

K.

with
condyles

the

femur.

parallel

An

to the

osteotomy

femoral

of the

condyles.

of the hip

present

was

in one patient.

MUSCLE

PATELL

PATELLAR
L$GAMENT

FIG.
Technique

for
Reproduced

reconstructing
an
by kind
permission

THEORY

In our
hypoplasia
induced

opinion

the

of the cruciate
in the developing

In the adult
knee the
is greater
than their vertical

primary

cause

anterior
of the

OF

cruciate
Editor

and

ligament
Dr K.

(Jones
G. Jones.

1963).

ETIOLOGY

of congenital

ligaments.
embryo

The dysplasia
before
nine weeks

femoral
diameter

condyles
(Figs.

dislocation

of

the

may be genetically
of age.

are elliptically
shaped;
7 and 8). In the young
THE

JOURNAL

their
foetus
OF

knee

is absence

inherited

or may

horizontal
the femoral
BONE

AND

JOINT

or
be

diameter
condyles
SURGERY

THE

ETIOLOGY

AND

FIG.

Figure
round

TREATMENT

OF CONGENITAL

7-Foetal

FIG.

femur

compared

with

(twenty-two
those

of the

FEBRUARY

Figure

are elliptically

1967

tibial

plateau

weeks):
the
Figure
10-Adult

is

approximately

are

femur:

the

relatively
femoral

10

plateau
tibia:

KNEE

8-Adult
shaped.

tibial

OF THE

condyles

FIG.

tibia (twenty-two
young
foetus.

the

1,

adult.

FIG.
Figure
9-Foetal
35 degrees
in the

49 B, NO.

the femoral

weeks):

condyles

VOL.

DISLOCATION

the

10 to 15 degrees.

slopes
posterior

posteriorly
slope

of

117

118

M.

are not fully

(Figs.
7 and
slopes

developed
8). The

10 degrees

As the
condyles.

P.

KATZ,

B. J. S. GROGONO

; they are round

foetal
tibial
plateau

(Figs.

9 and

adult
knee
As the distal

AND

K.

C. SOPER

as compared
to the elliptically
slopes
posteriorly
35 degrees

shaped
whereas

adult condyles
in the adult
it

10).

extends
the
attachments

tibial condyles
follow
an elliptical
of the collateral
ligaments
move

path around
the femoral
in this elliptical
path they

are

stretched
to their limit at zero degrees
of flexion
and further
forward
gliding
of the tibia
femur
is prevented.
When
the foetal
knee is extended
the collateral
ligaments
move along
an arc of a circle.
This allows
the collateral
ligaments
to remain
relatively
lax in extension.
As the adult knee extends
the posterior

on

half

of the

femoral

tibial

As

sion.

posterior
the

plateau

condyles,

the

foetal

slope

tibia

abuts

blocking

knee

of the

to

against

exten-

extends

tibial

glide

the

further

the

plateau

around

allows

the

femoral

condyle
(Figs.
1 1 and 12).
The structure
shown
spanning
the posterior
aspect
of the
knee joint
in Figures
1 1 and 12 represents
the

posterior

the

and
extends

these

against

zero
assist
FIG.

Figure

I1

11-Foetal
of

figuration
prevented

knee:

because

anterior

joint

knee:

the
joint
the cruciate

by

Adult
joint

FIG.

of the

dislocation

structures

necessary
it was

because

is

to produce
that

thirty-fourth

the

week
Of

Figure

configuration
by

be

the
in

deformity.

that

of

femoral

flexion

the

of the

described

example

have

in

at

this

way
of

the

hyperextension

can

occur

before

abut

against

the

In a random
were

the

radiological

in a breech

thirty-fourth

in a breech

cruciate
ligaments
are
in a foetus
a dislocating

position

study

position

week

most

75 per

cent

of 3,875

at some

absent
force

pregnancies

time

before

underwent

spontaneous

were

to have

noted

or
is

the
their

is therefore
a force
18 per cent of all
the end result
will

knee.

a patient

in whom

routine

radiographs

of the

knee

showed

that

eminence
was absent.
Giorgi
further
noted
that this patient
had a positive
drawer
sign, but no signs of dislocation
or subluxation
of the knee.
This may be
of knee with congenital
absence
of the anterior
cruciate
ligament
that did not

a hyperextension

articular

force

structures

the

and

the knee sufficient

stability
Experimental
dissections-To
of

and

intercondylar

anterior

an

dislocation
(1956)

abut

condyles

hyperextension

in an extended
or hyperextended
position
(Vartan
1945).
There
to produce
an anterior
dislocation
of the knee in approximately
pregnancies.
If there is an absence
or hypoplasia
of the cruciate
ligaments
Giorgi

knee

the posterior
condylar
developed
and a greater

knees
tending

be a congenital

adult

the

foetuses

were

of

hamstrings

structures

the
posterior
structures
femoral
condyle.

posterior

and

After

projecting

preventing

If the
hypoplastic

of the

posterior

degrees

range

the

As the

12-

of the

the

ligaments,

cent

foetuses

con-

can

bony

of gestation.

those

bony

prevented

clinical

25 per

the

capsule,

knee.
In the foetus
projections
are not

12

dislocation
alone.

the collateral
cruciate
ligaments.

found

version.

of

anterior
ligaments

joint

gastrocnemius.

between

ligaments,

applied
to it during
intra-uterine
life.
bony configuration
of the knee developed
to allow normal
activities.
test our hypothesis
further

fourteen
and thirty-two
the collateral
ligaments

gastrocnemius

force was applied

that in themselves

origins

and

posterior

weeks
and the
capsule)

the

of development
posterior
joint
were

observed

knees

In this event
the paranormally
so as to give
of thirty

while

an

to the knee.
It was found
that the cruciate
ligaments
could
prevent
anterior
dislocation
; when
only the
THE

aborted

foetuses

were
dissected.
The
structures
(hamstring

JOURNAL

anterior

cruciate
tendons,

dislocating

were the only structures

cruciate
ligaments
were
OF

BONE

AND

JOINT

SURGERY

THE

cut

an

anterior

foetuses
and

ETIOLOGY

that

tibial

joint
found
adult

TREATMENT

dislocation
were

less

condyles

could

than

had

In dissections
structures

AND

approached

be

ruptured

and

ligaments,

that

the

origin

of the

fifty-six
skeletal

system.

The
present

was

deformities

of the

most commonly
in 45 per cent

feet,

the

most

of the children
had congenital
and subtalar
joint
that occur
due

to a common

absent

the

or

defective

head,

defect.

annular

common

and

deformity

being

knees

that

this

being

the

knees

we found

The

avulsed

posterior
were all
and in the
dislocation

cruciate
ligaments,
the biceps
femoris
from

talipes

the

the
was

femur.

we found

condition

respectively

interosseous

the

exists

the

dislocation
of the
cent had congenital

equinovarus.

of the elbow.
The dislocations
with congenital
dislocation

dislocating

ligaments

ligament

was

the
case

occurring
anomaly
was congenital
of the patients.
Thirty-one
per

It is possible

intra-articular

the

joint.

in adult

dislocation.

ruptured
in one

to

femoral

seventeen
Shriners
Hospitals,
1 55 children
with
been
treated.
There
were
ninety-nine
girls
and
patients
had associated
anomalies
of the musculo-

dislocation
in association

In the

hypoplastic.

only
the

ANOMALIES

the

hip : this

applied

tendons
and the gastrocnemius
findings
in the older foetuses
study
of traumatic
anterior

gastrocnemius

last thirty-eight
years
at the
dislocation
of the knee
have
boys.
Eighty-two
per cent of these

also

119

KNEE

week

knee

and
anterior

force
ligament;

ASSOCIATED

In
congenital

adult

weeks

prevented

findings

twenty-eighth

of the

twenty-eight

of his cases the dislocating

and the tibial collateral

in another

By the

configuration

than

OF THE

These

old.

capsule,
the collateral
ligaments
the hamstring
to be deterrents
to anterior
dislocation.
These
seem to be corroborated
by Kennedys
(1963)

of the knee.
In most
posterior
joint
capsule,

DISLOCATION

produced.

weeks
the

older

the cruciate

than

easily

twenty-eight

of foetuses

other

OF CONGENITAL

intra-articular
in the

per

ligaments

other

ligamentum

talo-calcaneal

Ten

cent

of the hip, the elbow

of the knee may all be
to be

dislocating

teres

joints,

of the

femoral

ligament.

SUMMARY

Five

knees

with

to be absent

found

ligament

appear

cruciate

ligaments

early

foetal

foetal
is

The

are

We

absence

or

dislocation

or hypoplastic.
to be good.
On

life ; this

knee.

an

congenital

the

main

deviation

structures
from

adult
the

postulate

that

hypoplasia

of

cruciate

their

explored.

the
thanks

to

an

pattern
basic

The

cruciate

by reconstructing
of foetal
knees

preventing

the

therefore

authors
wish to express
of North America.

were

The results
obtained
the basis of dissections

anterior

is due
defect

to

ligaments
an anterior
we believe

dislocation
the

bony

in congenital

of

were
cruciate
that the

the

knee

configuration
dislocation

in

of
of the

the
knee

ligaments.
W.

Dr

B. MacKinnon

and

Dr

A. E. Deacon

and

the

Shriners

Hospitals

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THE

JOURNAL

OF

BONE

AND

JOINT

SURGERY