ARTHROGRYPOSIS MULTIPLEX CONGENITA

G. C. LLOYD-ROBERTS and A. W. F. LETTIN, LONDON, ENGLAND

Fromn the Ortliopaedic Departmnemit, I/ic Hospital for Sick Cliildreii, Great Ormnond Street, LoiuIoi

Arthrogryposis multiplex congenita (amyoplasia congenita) is an infrequent cause of’

severe crippling. Although it is often possible to alleviate tile grave disability which it cOtllrflOtlly
causes, its rarity impedes the accumulation of enough clinical experience to establish guiditig
principles and indications for surgical treatment.
We have been able to examine fifty-two patients with this disorder who attended the
Hospital for Sick Children between 1955 and 1968. Of these, thirty-nine have been treated
by one of us. The others were seen personally in consultation. We will discuss some features
of the disease, speculate upon etiology and diagnostic criteria, and make certain observations
upon management with particular reference to the indications for, and techniques of, surgical
treatment. Our statistics refer to the thirty-nine patients treated personally, for only in these
are the records complete. We have also excluded those with coexistent but unrelated
abnormalities which might have influenced treatment.
Our general approach to treatment resembles that of Mead, Lithgow and Sweeney (1958)
alld Friediander, Westin and Wood (1968), who have published admirable reviews. We
differ 011 certain points of emphasis, timing and technique.

THE NATURE OF THE DISEASE

The disease is characterised by multiple and protean deformities ofjoints determined by

abnormalities in the muscles and the nerves which supply them. These abnormalities are variable:
ill some, weakness may predominate, whereas in others fibrosis may infiltrate or even replace the
muscle. Sometimes muscle groups fail to differentiate intotheircomponent parts, so that individual
muscles are totally absent, being represented by homogenous scar tissue. One or other of these
manifestations may predominate in individual patients, but in general there is a mixture of
the different types. Sheldon (1932), writing from this hospital and recognising the paramount
importance of the muscle abnormality, proposed the excellent synonym amyoplasia congenita.
The deformities are produced by contracture and weakness of the fibrotic muscles,
modified by the positions imposed by intra-uterine environment-circumstances comparable
with those commonly seen in myelomeningoceie, in which congenital spinal paralysis inhibits
ilitra-uterille movenieilt so that tile child is often born fixed in the foetal attitude.

ETIOLOGY

The cause is unknown. Although a similar disease in merino sheep is genetically

determined, a genetic role is unknown in man. Three sets of identical twins have been reported.
In two of these only one baby was involved (Hillman and Johnson 1952), whereas in the other
both were affected (Lipton and Morgenstern 1955). These reports, although inconclusive for
the genetic factor, dispose of the proposal that uterine compression with or without
hydramnios or oligohydramnios is the only cause, for it is hardly credible that one twin could
be immune to an environment so destructive to the other.
The etiological hypothesis which most nearly explains the observable facts proposes that
arthrogryposis is a syndrome caused by muscle weakness, myopathic or neurogenic, developing
during intra-uterine life. Adams, Denny-Brown and Pearson (1953) developed this argument,
and Drachman and Banker (1961) reported narrowing of the spinal cord, with reduction in
tile number of’ anterior horn cells and peripheral axons at the cervical atld lumbar levels, in a

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 ARTIIROGRY POStS MULTIPLEX CONGFNITA 495

patient dying from tile disease. This proposition presupposes that the cause arises and is
spontaneously arrested during intra-uteriile life, because after birth it does not progress. We
have already mentioned the similarity between tile deformities of arthrogryposis and those
Comlllolily seen in infants witil myelodysplasia in whom the Ileurogellic influence is obvious.
Similar del’ormities are sonietinles seen in association witll benigtl congenital hypotonia.

CLINICAL FEATURES

There are obvious deformities varying in number, situation, severity and pattern (Fig. I).
These will be described when their treatment is considered individually.
At birth it may appear that these infants are not only deformed but also mentally retarded.
It is important to suppress this suspicion, for among their most important characteristics are

FIG. 1
The typical appearance of neonatal arthrogryposis multiplex congenita. Note the straight
elbows, flexed wrists and knees and severe equinovarus deformities. Such babies are
sometimes wrongly regarded as mentally defective.

high intelligence coupled with determination to overcome their disability. These features
dominate their later development and response to treatment (MacKenzie 1959).
A consistent and valuable sign is lack of normal limb contours. The limbs are sometimes
almost tubular. The skin, which is often glossy, may be dimpled over the prominences of
deformities and lacks creases. It ensheaths the diminished muscles tightly, there being but
little subcutaneous fat. Folds may be raised to form webs bridging the concavity of flexed
joints, especially the knee and elbow.
Radiologically the joints are at first normal but later the condyles become misshapen if
deformity and loss of movement become established. Sometimes the bones are abnormally
slender and porotic, especially if activity is greatly impaired. In contrast to the limbs, the trunk
is relatively unaffected.

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Lastly, it is worthy of emphasis that there is a group of patients ill whom widespread

liluscIe weakness, tronl wliicll the trutik is hot spared, dolilillates tile prognosis regardless of’

treatilient (Figs. 2 and 3).

DIFFERENTIAL DIAGNOSIS

Diagnosis is usually obvious if both arms and legs are abnormal. Severe examples of
benign hypototiia with joint laxity sometimes simulate arthrogryposis in the newborn. Later,
if contractures develop, the same difficulty may arise, but normal limb contours are usually
maintained in contrast to the featureless limbs of arthrogryposis.
The slender bones, rendered fragile by immobility, may break repeatedly, so ititroducing
a suspicion of osteogenesis imperfecta. Diastrophic dwarfism and juvenile rheumatoid arthritis
of early onset have also caused difficulty.

FIG. 2 FIG. 3
Figure 2--This hypotonic patient is unable to support herself in spite of only moderate
deformity. Figure 3-Radiograph of the spine of the same patient: the scoliosis is
paralytic in type.

If arthrogryposis is indeed a syndrome representing the effects of overt motor-neurone

disease, arrested in utero, it is not surprising that other neurological diseases with a motor
component can cause confusion in infancy.
In practice, difficulty arises only when the lower limbs are involved and the upper limbs
spared. Deformities from myelodysplasia, without recognisable radiological signs in the spine,
may be indistinguishable from those of arthrogryposis if the child is too young for sensory
testing. Arthrogryposis is a 11011-progressive lesion: so delay in diagnosis is not harmful.
Spinal dysraphism (Till 1969), however, is potentially a cause of neurological deterioration.

Since this is usually detectable by myeiography tilis investigation should he carried out wilen
doubt arises.

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Tempting though it is to withhold the diagnosis of arthrogryposis when the lower limbs
alone are involved this is not possible, for sometimes only the upper limbs are affected. In both
these circumstances the diagnosis is made only by exclusion.

MANAGEMENT DURING FIRST SIX MONTHS

During this time we must consider the parents no less than the child.
The parents-It is a shattering experience for a mother to be presented with a baby severely
affected by arthrogryposis, and our first duty must be to mitigate her natural anxiety as best
we may. We can assure her that her child is now at its worst and that the condition will not
progress : indeed it can only improve. We may truthfully say that in spite of appearances she
can expect intelligence to be above the average. Her child will almost certainly walk unless
generalised weakness prevents this-a rare event not detectable at this age.
Genetic counselling is important, for her anxiety about the outcome in future pregnancies
may thereby be allayed. Lastly we should give her an outline of our intentions and emphasise
the role she must fulfil in management of the child. We must warn her that treatment will
continue at least until maturity and that this will not only be surgical but will also demand her
prolonged and dedicated cooperation as an amateur physiotherapist and manager of splints.
The infant-The physiotherapist should practise and teach stretching exercises from the first,
maintaining correction by suitable splints. At first all deformities are treated, not omitting
the common medial rotation of the shoulder. Success is unpredictable but with the exception
of severe hand and foot deformities we have seen correction achieved. Usually we find that
some are corrected or greatly improved whereas others remain obstinate. Thus of two knees
with similar deformity one may respond favourably and the other not at all.
The surgeon should pay particular attention to the hip joints because fixed flexion and
abduction deformities and some dislocations demand early and vigorous treatment. Diagnosis
of dislocation may however be difficult when there is fixed flexion deformity, a transradiant
femoral head and reduced movement.

GENERAL CONSIDERATIONS INFLUENCING SURGICAL TREATMENT

We now adopt the following principles.
Timing ofoperations-It is our aim to correct all lower limb deformities that can delay walking
before the age of eighteen months. These include severe equinovarus and knee and hip flexion
which would inhibit walking in calipers. If dislocated hips are to be treated it is hoped that
stable reduction will be achieved during this time. Operations on the upper limbs may usually
be postponed for several years. This is a great advantage because it allows time to assess
functional capacity, thus reducing the risk of taking decisions which by interfering with the
modes of movement that the child has or would have developed if left alone, may reduce
rather than augment performance. These intelligent and determined children repeatedly
surprise us by their dexterity in spite of their severe handicap.
Type of operation-We have found osteotomy alone to be unrewarding in early childhood
though it is satisfactory when the child is approaching the end of growth. In contrast,
capsulotomy with lengthening or division of deforming structures is more likely to achieve
lasting correction.
Generalised hypotonia-ln patients with arthrogryposis and severe weakness of all muscle
groups operation is unlikely to be helpful. The patient is not only unable to move his legs
even when correctly positioned but sagging of the trunk and weakness of the arms prevent
the use of crutches. It is in these that scoliosis of a collapsing paralytic type develops.
Unfortunately it is usually impossible to recognise this complication in the very young, and
treatment having begun may have to be abandoned later.

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(#{149}. ANt) A. ‘IV. F. LL 1 FIN

Splinting-Whenever deformities have been corrected prolonged spiillting and stretcllillg are
necessary to prevent relapse. Thus after surgical correction of a deformity of the knee we
would advocate the use ofa walking caliper and a night splint for at least five years-continuing
thereafter with a night splint for a further similar period.

SURGICAL TREATMENT OF INDIVIDUAL DEFORMITIES

LOWER LIMBS

The feet: Equinot’arus-This is by far the commonest deformity (fifty-seven feet). It is usually
severe and associated with stiffness at the ankle and tarsal joints. The object is to convert a
deformed rigid foot into a rigid plantigrade platform.
Non-operative treatment is unlikely to succeed unless the deformity is exceptionally mild.
Only five feet derived permanent benefit from manipulation (with or without anaesthesia) and
splinting (Table 1).
TABLE I
RLSUL;IS 01 CONSERVAtIVE TREATMENT IN FIFTY-SEVEN FEEt WITII TALII’Es EQUINOVARUS

Number of feet

Method of treatment Permanently Temporarily Never

plantigrade plantigrade plantigrade

Physiotherapy and splints . 2 23 13

Manipulation under anaesthesia 3 23 2

Many feet were treated by more than one method and so the total numbers in Tables I and II
exceed fifty-seven.

TABLE II
RESULTS OF OPERATIVE TREATMENT IN FIFTY-SEVEN FEET WITH TALIPES EQU1NOVARUS

Number of feet -- -
Method of treatment - Permanently Temporarily - Never
plantigrade plantigrade plantigrade -

Soft-tissue release 3 -

-
Wedge resection . . . 10 3 9

_______________
Talectomy . ______. 17 2* 3

* Later repeated with success.

Medial soft-tissue release has not been reliable and with tendon transplants, tenotomies
alld elongations failed permanently to influence so rigid a deformity in eleven of fourteen feet.
In children under five wedge tarsectomy succeeded in only ten oftwenty-two feet (Table II).
In the older child triple arthrodesis is the natural choice. In the very young child beginning
to walk and handicapped in that calipers cannot readily be fitted this is not feasible and we
110W prefer talectomy and calcaneal tenotomy. By this means the foot can certainly be made
plantigrade though sometimes partial excision of the navicular is also needed. We do not,
however, gain any useful ankle movement.
Certain aspects of technique require empllasis. We prel’er an anterior incision because
teildolls are usually futlctioniess alxl may he sacrificed as necessary. StifT’ness, deforniity atld
the small size of the foot may make recognition of tile talus difficult and it is best to identify
it at the ankle joint. With care it may then be enucleated complete but if it is necessary to
remove it in pieces it is of great importance to ensure that none remains to grow and reproduce

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FIG. 8 FIG. 9
Correction of equinovarus deformity. Figures 4 and 5-Equinovarus deformity. Lengthening of the tendo
calcaneus has failed to correct equinus. Figures 6 and 7-The result after talectomy. Figures 8 and 9-Lateral
radiographs before and after talectomy.

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500 G. C. LLOYI)-ROI3ERTS AND A. \V. F. LETTIN

the original or some other deformity. In dividing the calcaneal tendon it is advisable to remove
a segment to prevent a return of continuity which may cause a recurrence of equinus. We
stabilise the foot beneath the tibia by transfixing both with a Kirschner wire. This is removed
after three weeks when the plaster is changed, to be replaced for a further three weeks. By
this means we ultimately obtained a plantigrade foot in seventeen of twenty feet (Figs. 4 to 9).
Other deformities-We have observed congenital vertical talus twice and calcaneo-valgus
three times. Congenital vertical talus is not likely to inhibit either walking or caliper fittitig

TABLE III
RESULTS OF TREATMENT IN TWENTY-EIGHT KNEES WITH FLEXION CONTRACIURE

Number of knees
Method of treatment Lasting Temporary No
improvement improvement improvement

Physiotherapy #{149} #{149} ‘ 0 0 6

Manipulation and splinting 6 9 0

Posterior release #{149} #{149} 9 1 0

Supracondylar* osteotomy 4 8 2

* Fourteen osteotoniies in twelve knees, two repeated later and six amputated.

in tile early years and so requires no treatment during this time. Being already rigid it may be
corrected by triple arthrodesis later. Calcaneo-valgus has responded to non-operative
treatment.
The knee joint: F/exion-Flexion contracture (Figs. 1 and 12) is more common than hyper-
extension in the ratio of twenty-eight knees to five. Of fifteen flexed knees treated by serial
plasters six achieved full and lasting correction. The remainder improved but subsequently
deteriorated and required operation.
Supracondylar osteotomy has consistently failed if persistent deformity compromises

walking or splinting in the young child. There were two disasters when popliteal vascular
occlusion dictated urgent amputation. Generally the deformity recurs rapidly and repeating

FIG. 10 11
The deformity produced by supracondylar osteotomy twice repeated at an early age.

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FIG. 12 FIG. 13
Figure 12-The appearance shortly after birth. Note the extended arms, flexed wrists and knees and severe
equinovarus. Figure 13-After bilateral capsulotomy of the knees and excision of the tali the legs are in
functional positions.

the operation causes further posterior subluxation ofthe tibia so that it finally articulates with
the posterior quadrant of the femoral condyles. In two cases stiffness and deformity increased
to such an extent that through-knee amputations
were the only solution (Figs. 10 and 1 1).
Supracondylar osteotomies were performed
on twelve knees, two being repeated. Excluding
the amputations there remain six knees of which
four operated upon before skeletal maturity were
not permanentlycorrected. The operation was in
two cases repeated at the age of fifteen years with
lasting improvement. A similar result followed in
the case of a patient primarily treated by this
method at this age (Table III).
Posterior capsulotomy with division of
deforming muscular, tendinous and fascial
contractures has been successful in correcting
deformity without loss of stability but not in
restoring movement-indeed, some may be lost.
This may be a difficult operation in a young child
with marked flexion, ample subcutaneous tissue
and distorted anatomy. We prefer to use two
incisions-medial and lateral-and establish the
capsular plane on both sides, avoiding a tendency
FIG. 14
to search too proximally. A blunt instrument may
In early infancy the shoulders are internally rotated,
next be passed in contact with the capsule from theelbows and knees are straight, the wrists flexed and
medial to lateral side and the neurovascular the feet in equinovarus.

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502 G. C. LLOYD-ROBERTS AND A. W. F. LETTIN

structures lifted away by a gauze swab sling. The posterior capsule can now be divided under
direct vision followed by division of tight hamstring tendons and usually the superficial popliteai
fascia. Extension is now limited by tension in nerves and blood vessels, so it is prudent to
apply the plaster in some flexion. Full correction is usually achieved at the second or
third change of plaster. Treated in this way nine of ten knees were corrected and thus far
remain so (Table III and Figs. 12 and 13).
Hyperextension-T his is less frequent but when present (Fig. 14) it is resistant to non-operative
treatment and is in all respects similar to recalcitrant idiopathic genu recurvatum (Laurence 1967).

Unilateral dislocation of the hip. The acetabulum is well formed.

FIG. 16
The dislocation was reduced by operation and a varus osteotomy was
performed later.

Operative treatment, elongation of quadriceps and anterior capsulotomy, is rarely indicated

because this deformity does not delay walking, for the knee flexes at least to neutral (0 degrees)
and calipers may be fitted.
The hip joint: Deformities without dislocation-There were twenty hips with fixed flexion
deformities. Flexion is often associated with abduction and sometimes with lateral rotation.
This is the position that a baby with flexed knees must adopt whether lying supine or prone.
It follows therefore that these deformities will be perpetuated and aggravated by persistent

knee flexion and are likely to improve once the knees are straight.

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Our experience confirms this proposition. Once the knees are corrected the child may be
nursed prone with a short crossbar between the splinted knees. Flexion deformities in all but
four hips were corrected in this manner to within 20 degrees of full extension.
When deformity is resistant and if, as is usual, the lumbar spine is mobile, treatment of fixed
flexion is difficult. Osteotomy in the young is of transient benefit and soft-tissue operations
seldom achieve full correction. Persistent deformity must be accepted in some children until
they are old enough for osteotomy to offer some hope of permanent correction.
Dislocation-There were sixteen dislocated hips, six being bilateral. We have gradually
developed a more aggressive attitude and now hesitate to reduce the dislocation only in those

TABLE IV
RESULTS OF TREATMENT IN SIXTEEN DISLOCATED Hips

Number of hips

Method of treatment Reduced Reduced Unreduced

and mobile and stiff and flexlon
contracture

None. . . ‘ ‘ 0 0 6

Closed reduction . . ‘ I I 2

Open reduction and osteotomy 5 2 0

Colonna arthroplasty . . 0 I 0

* Includes the two hips which failed to reduce with closed methods.

TABLE V
DEFORMITIES FOUND IN FORTY-SIX INVOLVED HANDS

Number of hands

Deformity Metacarpo- Proximal

phaIangeal interphalangeal Thumbs
joints joints

Extended . . . 10 4 0

Flexed. . . ‘ 14 26 0

Adducted . . . - - 12

Abducted . . . - - 6

rare bilateral dislocations in which rigidity ensures stability while at the same time making it
improbable that useful movement will be achieved even if the hips are reduced. These six
hips, however, developed an obstinate fixed flexion deformity.
Conventional non-operative treatment succeeded only twice, indicating that open reduction
is usually necessary. This we prefer to postpone until the child is twelve months old or
thereabouts (Figs. 15 and 16). Further delay is undesirable (Table IV).
The technique of and the findings at operation do not differ from those in uncomplicated
dislocations (Lloyd-Roberts and Swann 1966). Reduction once achieved is stable but movement
is less than that to be expected in simple congenital cases.
We must emphasise the difficulties. These included supracondylar fractures after operation,
intractable myositis ossificans and obstinate stiffness after immobilisation. We must mention

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504 G. C. LLOYD-ROBERTS AND A. W. F. LETTIN

in particuLar a Colotlna arthroplasty performed on a patient aged eight which led to ankylosis,
and ill-conceived extension osteotomies which in one hypotonic patient made sitting very
difficult.
TRUNK
Scoliosis-This usually has the features of a paralytic collapsitig curve and when presellt
should alert the surgeon to the possibility of hypotonia so severe that the child is unlikely
to learn to walk. Hitherto this factor has contra-indicated correction and stabilisation (Fig. 3).

UPPER LIMBS

Patterns of deformity and weakness are more variable in the upper than the lower limb,
and treatment is more difficult. Thus in the lower limb our aim is to provide a straight,
stable and if possible moderately mobile limb, whereas iti the upper limb we must

TABLE VI
RESULTS OF TREATMENT IN THIRTY-SEVEN WRISTS WITH FLEXION CONTRACTURES

Number of wrists

Method of treatment Lasting Temporary No

improvement improvement improvement

Physiotherapy and splinting 18 4 15

Anterior capsulotomy . 4 1 0

Osteotomy, radius and ulna 0 4 0

Arthrodesis of wrist . . 3 0 0

TABLE VII
RESULTS OF OPERATIVE TREATMENT IN TWENTY-EIGhT ELBOWS WITH LIMITED FLEXION

Number of elbows

Treatment Permanent Temporary No

improvement improvement improvement

Physiotherapy and splinting . 15 0 13

Posterior cupsulotomy . - 7 0 0

Pectoralis major tendon transfer 6 0 2*

Steindler flexorplasty - . 1 0 0

* Subsequently repeated with permanent improvement.

concentrate on the hands whichmust always function as mutually dependent instrumetlts.

We must never forget the remarkable adaptability of intelligent children. Furthermore, in
providing means whereby the hands can be placed in functional positions we must be cautious
lest we sacrifice some other vital property such as the ability to use a crutch.
The hands-There were forty-six abnormal hands, the commonest deformity beitig flexion of’
the proximal interphalangeal joints (twenty-six hands), the metacarpo-phalangeal joints being
flexed or extended in equal proportion (Table V). Associated deformity ofthe thumb occurred
in eighteen of which twelve were adducted. It is remarkable that involvement is usually
symmetrical and function so good as to be frequently near normal.
The stiff spider hand with extended interphalangeal and flexed metacarpo-phalangeal
joints merits further consideration. There is usually enough movement at the metacarpo-
phalangeal joints to demonstrate that the interphalangeal deformity is due to contracture of
the interosseous muscles (intrinsic plus syndrome). No benefit follows release, for although

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interphalangeal movement is improved there is insufficient power in the long finger flexors
f’or grip
IlOt prevented
to be restored.
from meeting
We must
each
therefore
other by any
accept the deformity
subsequent procedures
and ensure

1’
on the
that
arms.
the hands are

FIG. 17
The right wrist, which originally had a Ilexion contracture similar to that
seen on the left, has been treated by anterior capsulotomy.

The wrist joint-Fiexion contracture with or without ulnar deviation is common, being present
in thirty-seven of forty-eight wrists. A further eight lacked dorsiflexion and three lacked
palmar flexion. When the hands are stiff we accept the position because the forearms
are usually pronated so that the hands can oppose
their radial borders to provide a pincer grip of a
strength and mobility dependent upon that of the
arms.
If the hands have mobility and power, disabling
wrist flexion needs correction. Non-operative treat-
ment was helpful in eighteen. Anterior cupsulotomy
corrected the deformity permanently in four of five
( Fig. 1 7) but we have learnt once again that osteotomy
is of only transient benefit (four failures). In three,
arthrodesis during adolescence resolved the problem
(Table VI).
The elbow and shoulder joints-These should be
considered together because correction of deformity
and restoration of mobility in one is dependent upon
the position and mobility of the other. Furthermore
the usefulness of disabled hands is largely determined
by the manner in which they can be placed in mutually
useful positions by the arms, which should be regarded
as a simple functioning unit extending from shoulder
to wrist. Although this seems to present difficulties
in deciding what to do, where, and in what order,
this is not so in practice, for the few possibilities limit
the scope of selection. FIG. 18
in practice restoring the ability to lift the hand Same patient as that shown in Figures 1 2 to I 3.
After posterior capsulotomy at the elbow and
to the mouth ensures that the hands, however a medial Steindler flexorplasty the hand can be
disabled, can be placed in favourable positions for carried to the mouth. The wrist flexion
contracture was corrected by stretching and
their limited function. Furthermore this helps to splinting.

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alleviate one of’ the great social handicaps for otherwise they must eat and drink by bringing
their mouths to plate or glass.
We believe that otie side should be completed and the result assessed before the other is
begun. It may be then found unnecessary or imprudent to proceed similarly, if at all, on the
other. For example, active flexion of one elbow may be used (if the other is mobile) to lift
the opposite hand to the mouth, thus achieving the
desired effect by indirect means. Again. and most
significantly, it may become apparent that with
disabled lower limbs the child needs one straight stiff
elbow to push himself out of a chair.
The shoulder joint-There were thirty-three shoulders,
all with medial rotation deformity (Figs. 1, 12 and 14)
and twenty-nine with limited abduction. if active
abduction is also lost (and it is very rarely present)
the resulting severe limitation of passive abduction is
unimportant but fixed medial rotation is important
for it directs the flexed elbow to the opposite shoulder
rather than the mouth. Stretching may correct this
deformity if begun in infancy (Fig. 18).
Later we have corrected it by rotation osteotomy
in the proximal third ofthe humerus using a medullary
nail and a plaster shoulder spica in 90 degrees of
lateral rotation (six shoulders). Previously we used
a plate, the arm being supported in a sling. This
was less satisfactory for the enforced medial rotation
was to some extent perpetuated after bony union.
FIG. 19
Following lateral rotation osteotomy of the It is possible that division of the subscapularis tendon
humerus, posterior capsulotomy of the elbow would prevent this. The operation is simple and
and a pectoral flexorplasty the hand can be
carried to the mouth. Note the bright, uncomplicated provided that it is remembered that in
intelligent eyes illustratingan essential feature medial rotation the radial nerve lies in front of the
of arthrogryposis.
humerus at the level of bone section.
The elbow joint-There were forty-five abnormal elbows in twenty-three patients (Figs. 1, 12
and 14).

Correction of deformiti’-Variable passive mobility is possible from positions of deformity

varying from extension to about 90 degrees of flexion. Most commonly there is a very limited
passive range of no functional value because it starts from full extension (twenty-eight elbows).
Active elbow flexion is rarely present in these circumstances. Even a limited range is, however,
of value if it is possible in an arc around mid-flexion.
Should it be decided to attempt to introduce active flexion to an extended or stiff elbow
it is first necessary to flex the joint to a right angle and retain or improve any pre-existing
movement.
Splinting and stretching improved flexion significantly in half the patients so treated.
When the problem persisted our unfavourable experience with osteotomy elsewhere discouraged
us from using it for this purpose and we have relied upon posterior capsulotomy with elongation
of the triceps (Fig. 18). We found this a most satisfactory operation for correction with
preservation of, or even increased, movement in seven elbows. There are no technical difficulties.
Timing is, however, of some importance, for whereas other operations on the arms may with
advantage be delayed until patterns of activity are established, delay in flexing the elbow may
prejudice the outcome. This is because growth ofarticulating surfaces for too long in extension
may mean that there is poor congruity in flexion.

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Flexorplasty-We have used Steindler’s proximal transposition of the flexor origin once and
a modification ofCiark’s (1946) pectoral transplant six times (Fig. 19). We have no experience
of anterior transplantation of the triceps.
Steindler’s operation is effective when the hand approximates to normal, which implies
that the wrist and finger flexors have adequate power. Ideally the child should be able to
achieve some flexion of the elbow before the transplant. Unfortunately, however, very few
patients are suitable for this procedure.
Flexorpiasty is more often required in those with ineffective wrist and hand flexors so
that pectoral transplant is more commonly indicated. This muscle is almost invariably present
and usually has sufficient power for this purpose.
It is necessary to turn the muscle upwards from below rather than downwards (Brooks
and Seddon 1959) because the biceps is unlikely to be sufficiently defined for use as a tendon
of attachment.
We have introduced two modifications to the technique ofClark’s operation in this special
context. First we make our attachment the ulna as far
to distally as possible rather than to
the radius. This is simpler and is dictated both by the frequent absence of the biceps tendon and
the fixed pronation which prevents the supination effect that a radial attachment might achieve.
Distal attachment has the added advantage of increasing mechanical efficiency. This requires
a longer fascial prolongation than in the standard operation and so dissection ofthe tongue of
anterior rectus sheath begins more distally-that is, below the umbilicus. The sheath is lifted
in continuity with the origins of the pectoralis major from the costal margins. The rest of the
procedure follows the standard technique. The rectus sheath is finally made into a tube before
fixation to the ulna.
The long fascial tube is vulnerable and our two failures were caused by loss of attachment
to bone. Secondary repairs with fascia iata succeeded but the final result was less satisfactory
than in those not requiring a further operation. Nevertheless all could get their hands to
their mouths against gravity and some resistance (Fig. 19 and Table VII).

SUMMARY

I. We have reported our experience in fifty-two patients with arthrogryposis multiplex

congen ita.
2. The nature ofthe disorder, its possible cause, the clinical features and differential diagnosis
are discussed.
3. Early management is described with special reference to the infant, his parents, and general
principles ofseiection and timing. The treatment ofthe individual deformities which commonly
occur is outlined.
4. We have emphasised that lower limb deformities should be treated vigorously in the first
year, whereas in the upper limb treatment is better delayed until an accurate assessment can
be made.
5. Correction in the young child should be by soft-tissue release rather than by osteotomy.
6. Prolonged splinting after operation is necessary.
7. Severe weakness may dominate the problem and make operation unrewarding.
8. The intelligence, determination and adaptability of these children flatters even modest
surgical success.

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