Neonatal Screening For Cdh

OCCULT

SPINAL

DYSRAPHISM

IN ESTABLISHED

CONGENITAL

achieved heads

DISLOCATION

stability, were

of the further

surgically open

attempts surgical

degree

the

calf

osteotomy and femoral has still not stabilised.

spina

had Table of the

I. Results hip

in 1 17 children at five- to 20-year

with established follow-up

congenital

dislocation

Number

grade

of hips

I

55

2

42

3

18

4

8

5

7

Result

Per cent

Success

76

Intermediate

13

Failure

11

83#{176}c were

acceptable,

I7#{176} required

further

with

bifida

five had

Two

the three

unilateral

a normal

response

but the radiograph revealed some

three At this to a lesser

foot

taken drifting

who

sacral

of

to frame

similar they

degree

was

right

reduction

the

and

one

sided.

All

and

one

reduction head child

VOL.

70-B.

No.

5. NOVEMBER

rotation of the left leg, persisting after but the Trendelenburg test is positive

1988

posterior

usually out of a

sustained

test 4 and

also became 5). All five

‘

1”:..

Lateral is stable

to had

than

I

I

-

to

an inverted limbus. allowed to walk freely,

one year after of the femoral Only

failed of spinal

dislocations

dislocations

acetabulum.

had

stigmata

a lesser

bilateral

fixed, and the Trendelenburg on the affected side (Figs

\

Figure 3 right hip

the

a

spontaneous fracture after pelvic osteotomy, but in all cases, when these children stood and walked, the affected limb rotated laterally (Fig. 3). At first this posture could be corrected passively, but later it became

surgery

3

on

in the affected

)

Fig.

out had in

all

physical signs Radiologically,

though

had

poor

children no

developed first group.

occulta,

group.

femoral

drifted

Each child osteotomies

a smaller

was always

other

but in the

dysplastic

S

same and

hips. The prolonged to be the cause of the

arthrotomy in each case revealed Six months later, they were

MOdified

Severin

the

as nocturnal enuresis and constipato be secondary to their prolonged

to treatment

previous of

the affected were thought

The outcome

dysraphism, those seen

shorten-

year laterally

acetabula. and pelvic

(associated

hips (Fig. 2). Occult cases. The

open reduction, pelvic congenital dislocation

745

supracondylar fractures that after the removal of their plasters. wasting of the affected buttock and

treatment.

After repeated ing, the right

HIP

one

rotated

deepened reductions

side), as well were considered

respond

within

to have

to stabilise programmes

spontaneous sustained stage, the same tion,

but

found

OF THE

Fig.

4

completion on the

Fig. of surgical left.

management

of CDH

.

5

Figures

4 and

5

-

The

positive children

J. A. WILKINSON,

746

developed

the

wasting

as

dislocation

foot.

Most

degree

first

usually

children same

same

the

failed

suffered

had way

and

group,

from

nocturnal

those

with

spinal

overt

In

1965

and

as the

side

of

All these

surgery

our

total

in the

dysraphism.

30%

250

cases

the

hip,

diagnosed

of

was

one

usually

foot on the

the feet from

to grow

Within

side

of dislocation.

age as the

the next

length

three

of the

affected

such

foot

shortening

overt

and

occult

spinal posture

rotation

established posterior children

congenital are splinted

Browne

abduction this

The same

child

time,

Fig.

Radiograph of the “tear osteotomy,

rarely

more

the

increased

feet.

The

included

of the

group

of

in those or cavus with

concentric

in Lorenz

and

for a total

legs are

held

then

the in Denis

in a position

showing left CDH one drop” and an increased there is lateral rotation

6

above

Foot inequality.

any

will prevent

bringing adaptation

power selective

this

the knees depends

into the on the

in the

hip extensors

weakness

of these

muscle

and

the child

natural

of age and postoperatively weeks in a plaster spica with minimal abduction

recovery

and

of the plaster,

the legs are splinted for eight with the hip in full extension and medial rotation. After

rotators,

a

the

it is necessary

child

will

fail

to carry

to

out

respond

imbalance

with

associated (Fig. 9).

have

lateral

with

fractures. extensive

seen

rotator

a smaller Spontaneous

in children sensory

and

dominance

foot

on

or stress

with motor

fractures

denervation

Fig.

is 0.5 cm shorter,

THE JOURNAL

AND

one or muscle side

are most

who of the legs

8

is slight cavus deformity. drift of the femoral head, after plication of capsule

OF BONE

this

of the of the and

is usually

affected

meningomyelocele

7

The left foot

; this

the

both

to

treatment and once again develop lateral rotation affected leg with recurrence of the lateral drift femoral head out of the surgically deepened anteverted acetabulum (Fig. 8). Thus, a persistent lateral rotation posture of occasionally both legs, signifies a persistence of

and there year after posterior arthrotomy and excision of the limbus. There is lateral gap between the tear drop and the calcar. Figure 8 - Same case one year of the femoral head with drift out of the acetabulum. -

of

be left with persistent lateral rotation. This might be the mechanism of the lateral or outward drift of

commonly

Fig.

in Figs 3 to 5. Figure

position

congruity

the femoral head out of the acetabulum at this stage of treatment in a minority of the patients in our series (Fig. 7). Pelvic osteotomy is usually performed at two years

Stress

of six months.

6

as shown

of after

reduction,

plasters

harnesses

hip,

will well

actively, postural

of normal

rotators;

medial or

treatment

the

Lorenz

passive and active medial rotation exercises to maintain a normal gait. If there is any selective weakness of the

of patients

In our

This

experimentally,

removal

inequality

children

rotation.

to produce,

than

to 0.5 cm

the

leg.

in neutral

to be

dysraphism.

and

both

of

toe proved

however,

dislocation

arthrotomy

During

years,

this

Measurement was

patients

length;

there was greater inequality unilateral metatarsus varus

with

Lateral

inequality

in these

more (Fig. 6) and who had developed deformities

to its full

heel to the tip of the longest

difficult at this 0.1 or 0.2cm. of foot

failed

medial

groups

born

at birth

and at that time appeared to have normal feet of equal length. Subsequently, no measurable degree of inequality developed in the first year of life. At two years ofage, the time ofassessment for pelvic osteotomy, the majority were walking with a normal gait and their feet remained equal in length. In 7% of them, however,

and shown

development of of

were

angle been

and medially rotated sagittal plane. This

series

dislocation

1985,

has

the concentrically reduced femoral head within the acetabulum (Wilkinson 1962). After the period of splinting, the child is encouraged to kick freely and walk, allowing the hips to be extended

opposite

FEATURES

congenital

between

the

enuresis.

to primary

inequality.

on

as much

to respond

right

of muscle

foot

failed

as

established

the

to grow

CLINICAL Foot

distribution

and

E. M. SEDGWICK

JOINT

Figure 7 spreading and pelvic

SURGERY

-

OCCULT

SPINAL

DYSRAPHISM

IN ESTABLISHED

Strong medial

medial

rotation

Weak rotation

CONGENITAL

DISLOCATION

from

our

1985 ; Katifi

:T11::

Cadilhac weakness hip rotators

of

sensory

Fig. 9

whom Two physical signs hypoplasia resulting

in overt and occult from spinal dysraphism.

forms

of

neuromuscular

the

thirds has

most

common

of the been

femur.

sites

The

observed

dysraphism

the

same

in

associated

are

with

congenital

do not display

the excessive

that

seen

in

the

rare

in

our

fractures

were only

series

being

seen

hip

of 300 infants

formation

of

treated

well

to

her

surgical

five

spinal

Generally,

stress of

have a reputation the outcome of

the

for CDH,

treatment

fractures

are

management

only of this and

for

very of

the

uncommon

CDH,

but

of ominous significance treatment. Such a long-term

unlikely to be due to the simple fracture, caused by an underlying neurogenic spinal dysraphism.

96 children, stigmata of in Table

published

in incidence is shown.

is

but may well defect, such

be as

ofthe

abductor

structurally

and

normal

adductor

plane, the two opposing well balanced, because

of abnormal

gluteus

muscles.

rotatory the lateral

maximus

and

potential

Yet

in the

muscle rotators

70-B,

No.

5, NOVEMBER

1988

of rosterior

potential

tests,

tibial nerves diagram

upon

the

horizontal

groups are not (including the

short

rotators)

recordings

Ground

VOL.

et al.

findings

hip is dependent

Fig.

evoked

II and

(Sedgwick

are

three times stronger than the medial rotators (including the gluteus medius, gluteus minimus and tensor fascia lata). Thus before birth, this natural dominance of lateral rotators can produce subluxation and even dislocation of the flexed hip joint in the presence of hormonal joint

idealised

Somatosensory recordings.

54 of spinal

the balance between antagonist and protagonist muscle groups acting in three primary planes (Steindler 1973). The flexor and extensor muscle groups are strong and well balanced, and the same can almost be said for the

iliopsoas,

they

concerning effect

Stimulation

lemniscus system.

DISCUSSION Stability

patients.

dislocation. complications

been

and

reflect

medial

are summarised

has

in

one who had no clinical and neurological evidence spinal dysraphism sustained a similar fracture, and resulted from a fall out of her cot. She recovered responded

results

report

Georgesco

Such

occult

of the

and

is

et al.

response

spinothalamic

Recordings were made from had CDH alone with no The

(Gilmore

of the

the

the response normal data

studies

1986 ; Zhu, column

not

of and

spinal

condition.

cases in one

747

dislocation,

callus

former

dorsal but

1988). The difference in the various groups

of fractures

other

Sedgwick

of the

dysraphism.

proximal

overt

with

healing

dysraphism, In our

and

distribution

children

but they

is

distal

and

Abnormalities

pathway

a preliminary and

own

and

1987).

dysfunction

Selective medial

HIP

ankle (Fig. 10). The latency on the height of the subject

available

““

THE

Somatosensory evoked potentials. Somatosensory evoked potentials can be recorded from the scalp following an electrical stimulus delivered to the posterior tibial nerve at the depends

Inequality

OF

showing

levels

of stimulation

and

typical

10

J. A. WILKINSON,

748

laxity limbs

(Wilkinson medially

normal

1985). The is fundamental

function

for the held

final

in the

fetal

in inward

normal

hip

and

posture

knee

This

development

hip displacement time,

be increased

extended, legs

when

the

and

natural

an

infants,

imbalance

groups affected

they

lower of

is responsible

the

flexed

hips

are

accounts

for

the

of acetabular

and

femoral

capable of rotating plane, without any

first extend

dominance

of

WICK

their

lateral

their risk of

thighs.

At

rotation

can

by gravity, if the child lies supine with hips since this encourages lateral rotation of the favours the lateral rotator group.

In paralytic that

and

position

anteversion (Wilkinson 1985). Newborn infants are thus legs inward beyond the sagittal this

to rotate the the development

in which

rotation.

prenatal

ability to

E. M. SEDG

it has been

between

is responsible hips, with

the

suggested

abductor

previously and

adductor

for the spontaneous dislocation selective weakness of the abductors

of in

the presence of strong adductors reinforced by strong flexors in the presence ofweak extensors (Mustard 1952; Sharrard

1964).

there

appears

which

usually

In occult affects

one

The clinical and patients have reaffirmed natural

muscle

rotators

by of

the

persistent causes with

(Fig.

leading

horizontal

1 1), even the

of lateral

the

in the

being

medial

presence

of

maximus.

Thus

is due to recurrent

rotation

of the

a drift of the femoral head out of the the development of a secondary

leg,

or months

which

been

The lateral

found

ipsilateral

It

is

rotation

posture

to be closely foot

to the opposite length

the

have interesting

to grow

limb. been

associated

Extensive note

affected

with

to its full

undertaken to

of the

extent

ofthe

at all ages from

Table

these

II. The

congenital feet or an

a failure when

surveys

leg

of the

(Meredith

incidence

of abnormal of the hip response

foot 1944).

that

at

has

only

CDH patients that “in older

18

reference

CDH

only

CDH

and

occult

CDI-I

and

overt

CDH

and unequal

CDH

not

CDH

responding

*

differences

significant

spinal spinal

if they

dysraphism

feet

responding

in

dysraphism

to surgery

to surgery

who

exceeded

evoked

its adult

at four

to inequality

years

of foot

size the

(from typical

length

in

(1936), who noted the affected side is

evoked also

had

potentials spinal

was first detected at two 0.2 cm. Further inequality

in patients dysraphism,

with unequal

SEP

Total number

Number

Per

54

17

31

34

19

56

8

5

63

52

27

52

41

23

56

47

somatosensory

and

an inequality of foot length years and was no more than

somatosensory

alone and those to surgery

of patients

to half

cm)

was made by Fairbank children the foot on

With abnormal Group

grown

often smaller than its opposite number”. In our series, the majority of patients had equal feet, but in a minority

compared

studies

dislocation unsatisfactory

has

human

foot

8.0 cm at birth to 12.75 value is 16.0 cm.

acetabulum mechanical

dysplasia. This

11

Muscle imbalance in spinal dysraphism. Iliopsoas is the stronger lateral rotator of the femur, as compared to gluteus medius and minimus which are weaker medial rotators in the normal child. This difference is greater when there is selective weakness of the medial rotators, as in spinal dysraphism.

other.

plane

of

gluteus

to redislocation

posture

the

weakness

of

Fig.

rotators,

observations in our original concept, the

in the

wasting

however,

medial

than

radiological Steindler’s

a selective hip

and

subluxation

dysraphism,

of the

leg more

imbalance

exaggerated weakness

spinal

to be a weakness

potential

11 latency

figures

cent

23 were

taken

as

statistically

2 s.d.

THE

JOURNAL

OF

BONE

AND

JOINT

SURGERY