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Orthopaedic Management of

Arthrogryposis Multiplex Congenita

Andrew J. Graydon and Deborah M. Eastwood

Contents Peri-Operative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4641


Co-Morbidities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4641
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4628 Specific Anaesthetic Issues . . . . . . . . . . . . . . . . . . . . . . . . 4641
General Definitions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4628 Rehabilitation Issues . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4641
Prevalence and Aetiology . . . . . . . . . . . . . . . . . . . . . . . . . 4628 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4642

Classification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4629 Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4642


Characteristics of the Differing Subgroups . . . . . . . . 4629 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4642
Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4630
Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4631
Natural History of Arthrogryposis . . . . . . . . . . . . . . 4632
Amyoplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4632
Distal Arthrogryposis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4633
Syndromic Arthrogryposis . . . . . . . . . . . . . . . . . . . . . . . . . 4633
Indications for Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . 4633
Orthopaedic Management . . . . . . . . . . . . . . . . . . . . . . . 4633
General Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4633
Upper Limb Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4634
Shoulder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4634
Elbow . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4634
Wrist and Hand . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4635
Lower Limb Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4636
Hip . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4636
Knee . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4636
Pterygia Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4638
Foot and Ankle . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4638
Spine Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4640

A.J. Graydon (*)


Starship Hospital, Auckland, New Zealand
e-mail: agraydon@xtra.co.nz
D.M. Eastwood
Royal National Orthopaedic Hospital, Stanmore,
Middlesex, UK
e-mail: d.m.eastwood@btinternet.com

G. Bentley (ed.), European Surgical Orthopaedics and Traumatology, 4627


DOI 10.1007/978-3-642-34746-7_172, # EFORT 2014
4628 A.J. Graydon and D.M. Eastwood

often used to refer to both the syndrome


Abstract
of amyoplasia or classical arthrogryposis
The term arthrogryposis means contracted and
and other more general arthrogrypotic
crooked joints. Many different variants of the
conditions [2, 3].
condition have been described and care must
Amyoplasia refers to a particular form of
be taken to identify any associated problems so
arthrogryposis with a characteristic phenotype
that an appropriate overall assessment of
of internally rotated, extended upper limb pos-
the child can take place. The aim of the Ortho-
ture, with ulnarly deviated, flexed and thumb in
paedic management of these children is to
palm hand deformities, hip dislocation, vari-
maximise independent living skills and mobil-
able knee deformity and severe foot deformities.
ity. A key feature is that none of the musculo-
Associated features include normal intelligence,
skeletal tissues around the contracted joint are
mid-line facial haemangiomata, and ocular and
normal and thus a normal joint with normal
gastro-intestinal abnormalities [4].
function cannot be achieved with any form of
Distal Arthogryposis, as the name implies,
treatment either surgical or conservative. Both
involves predominantly the distal parts of the
the parents and clinicians must be aware of this
limbs and is a congenital contracture of two or
so that realistic treatment goals can be set.
more body areas without an associated neuro-
A multidisciplinary team is essential in the
logical and/or muscle disease [5]. Bamshad
management of these complex cases.
et al. [6] currently recognize 10 different pat-
terns of distal arthrogryposis.
Keywords Pterygia syndromes, where the webs crossing
AMC  Amyoplasia and Orthopaedic the flexor aspect of joints (particularly the
management  Distal arthrogryposis elbow and the knee) add significantly to the
joint contractures.

Introduction
Prevalence and Aetiology
General Definitions
The prevalence of an arthrogrypotic syndrome is
For nearly 100 years, the term arthogryposis has quoted variably between 1/3,000 and 1/5,000
been used to describe a group of disorders involv- live births depending on which definition of
ing congenital joint contractures that affect two arthrogryposis is used: for example, the preva-
or more areas of the body. The word itself means lence of amyoplasia itself is usually given as
hooked or curved joints and hence, in essence, 1:10,000 live births [3].
it is simply a description of a physical appearance Although most causes of these syndromes
and the clinical picture has been recognised for are unknown they are assumed to be multifactorial
much longer than a century. Currently there are in nature. Hall [3, 7] described a multitude of
some 300 recognised disorders that may present groupings of potential aetiologic factors ranging
as arthrogryposis [1]. To add to the confusion, from maternal diseases to intrinsic muscle abnor-
over the years a variety of terms have been used mality. He emphasised the importance of
to refer to specific subgroups of patients with establishing the underlying disorder in order to
congenital joint contractures which has made determine issues such as inheritance, the risk of
it difficult to compare case series and perhaps associated abnormalities and the natural history
hampered our understanding of the natural for any individual patient (Table 1).
history of these conditions and the influence of Overall, the group of arthrogrypotic disorders
treatment on them. is genetically heterogeneous but some subgroups
Arthrogryposis Multiplex Congenita (AMC) is do demonstrate consistent inheritance patterns.
a historical term first used by Stern in 1923 and A detailed discussion of the genetic basis behind
Orthopaedic Management of Arthrogryposis Multiplex Congenita 4629

Table 1 Potential aetiological agents in the development Within these broad categories more specific
of arthrogryposis [3] subgroups are then defined. This approach has
Cause Example great merit in encouraging the clinician to define
Abnormality of muscle Muscular dystrophies the underlying disorder and then to promote
structure or function Mitochondrial diseases appropriate treatment goals.
Abnormality of neural Brain, spine or peripheral
structure nerve abnormality
Abnormality of Diastrophic dysplasia
connective tissue Various forms of distal
Characteristics of the Differing
arthrogryposis Subgroups
Space limitation Twins, oligohydramnios
Bicornuate uterus Non-Neurological Causes
Maternal diseases Trauma, myasthenia gravis Amyoplasia
Multiple sclerosis Hall [4] first described amyoplasia in 1983 and
Abnormality of intra- Anterior horn cell death the characteristic features have already been
uterine vascularity Abnormal neural described. This form of arthrogryposis accounts
development for approximately 40 % of all cases who present
with congenital joint contractures: 85 % have
the arthrogrypotic conditions is beyond the scope symmetrical upper and lower limb involvement.
of this text but two reviews [8, 9] give descrip- The distal joints tend to be more affected than the
tions of current thinking on the molecular basis of proximal ones. In Halls series [4], all cases were
the inheritance of the arthrogrypotic syndromes. described as sporadic in nature although an
increased prevalence was noticed in conditions
such as twin pregnancies, oligohydramnios and
Classification bicornuate uterus that were associated with intra-
uterine crowding. Clearly the multifactorial
Most attempts at classification have attempted to nature of the aetiology of amyoplasia may
group patients according to their phenotypic include some form of genetic predisposition but
similarities but the approach by Bamshad and as yet this remains unknown.
colleagues [6] simply describes two groups:
those with normal and those with abnormal Distal Arthrogryposis
neurological examinations (Box 1). Many of the distal arthogryposis subtypes demon-
strate an autosomal dominant inheritance pattern,
albeit with a large phenotypic variation dependent
Box 1
on the underlying coding mutation [5, 6]. In gen-
Normal neurological examination eral, the distal arthrogryposes groups 110, have
(non-neurological causes) been associated with various mutations in the
Amyoplasia genes that code for components of the fast-twitch
Distal arthrogryposis (types I-X) myofibril contractile apparatus. Specifically these
Generalised connective tissue disordero genes are TNNI2, TNNT3, TPM2, MYH3 and
Foetal crowding MYH8 a combination of Troponin I, Troponin T,
Tropomyosin and Myosin genes [8]. Although the
Abnormal neurological examination mechanism by which these mutations cause con-
(neurological causes) tracture abnormalities remains unclear, their asso-
Central or peripheral nervous system ciation is undoubted. For example, 90 % of DA2A
abnormality (Freeman-Sheldon syndrome) cases are associated
Abnormality at motor end-plate with MYH3 mutations and 40 % of DA2B (Shel-
Abnormality of muscle don-Hall syndrome) patients also have a MYH3
mutation (Table 2).
4630 A.J. Graydon and D.M. Eastwood

Table 2 Subtypes of distal arthrogryposis [49] (all with activation of the spinal cord neurons due to
normal neurological examination) a problem within the corticospinal tract. Abnor-
Syndromes in malities such as microcephaly and intra-uterine
decreasing frequency Description CNS infection may cause such problems. In
DA Type 1 (OMIM Clubfeet and camptodactyly
a similar manner, chromosomal anomalies asso-
108120) with occasional involvement
of the shoulders and hips ciated with intrinsic CNS damage can lead to
DA Type 2 (OMIM Freeman-Sheldon arthrogryposis.
193700) syndrome Similarly, any congenital alteration in
Hand and foot abnormalities the function of the neuromuscular unit may result
in addition to scoliosis, in joint contractures. Congenital neuropathies
oropharyngeal abnormalities
and a distinctive whistling- are uncommon but abnormalities of the neuro-
face appearance muscular junction are less so. Arthrogryposis is
DA Type 5 (OMIM Ocular abnormalities in seen more commonly in infants of mothers with
108145) addition to the myasthenia gravis, as well as in infants born with
musculoskeletal deformities inherited mutations in genes encoding for
DA Type 7 (OMIM Trismus-
components of skeletal muscle acetylcholine
158300) pseudocamptodactyly
syndrome characterized by receptors [10, 11]. Congenital myopathies due
an inability to open the mouth to mutations in skeletal muscle proteins can
fully also result in arthrogryposis. It is important to
DA Type 3 (OMIM Rare disorders with varying recognize myopathies (such as congenital
114300), 4 and 6 associations with cleft palate
muscular dystrophy) as a cause of arthrogryposis
Type 3 also known as
Gordon Syndrome because the contractures they produce may
increase in severity after birth [12].

Neurological Causes
Neurological causes can be due to central or Pathophysiology
peripheral problems in any part of the neuromus-
cular axis (Box 2). Although the specific causes of arthrogrypotic
joint contractures may vary, it is thought that
the final common effect of reduced intra-uterine
Box 2
movements of the foetus (foetal akinesia) leads to
Central neurological abnormalities
a failure of normal joint development and growth
Arthrogryposis in association with a CNS
and subsequently to the clinical features of
abnormality occurring anywhere along
arthrogryposis. The final common pathway for
the corticospinal tract for muscular
joint contractures is the collagenic response to
control
foetal joint immobility which, by extrapolation
from a number of animal models, is thought to
Peripheral neurological abnormalities
occur after about 3 weeks of immobility [13, 14].
(Neuromuscular)
At this stage, the joint contractures are considered
Arthrogryposis in association with an
irreversible and histologically, the joint capsule
intrinsic abnormality occurring anywhere
becomes thickened with collagen proliferation
within the neuromuscular unit
and fibrotic replacement of muscle [15]. This
pathological tissue is a major factor in the poor
results following surgery with high rates of recur-
CNS abnormalities that lead to arthrogryposis rent deformity: even if joint mobility could
can do so either by direct injury to the developing be restored, there is no normal muscle to power
spinal cord neurons themselves or by reduced the joint.
Orthopaedic Management of Arthrogryposis Multiplex Congenita 4631

Diagnosis

Clinical examination is the key to making the


diagnosis. The clinical picture of amyoplasia is
characteristic: the limbs appear featureless and
tubular with an obvious reduction in muscle
mass and with an absence of skin creases across
the joint. Deep dimples over the extensor aspect
of the joints are common. The classical limb
postures are easily recognizable (Fig. 1). Fifteen
percent will have involvement of either the upper
or lower limbs, whilst the remaining 85 % will
have all four limbs involved. In addition, well
over half the patients with amyoplasia will
have an associated genital malformation, whilst
approximately 10 % will have an intra-abdominal
malformation [16]. Naturally the degree and
nature of involvement of the syndrome tends to
reflect the degree of disability to the patient.
In contrast, the picture for distal arthrogryposes
may be much more subtle. It is important to be
aware of the clinical heterogeneity of the congenital
contracture syndromes and recognize for example,
that a recurrent deformity in a difficult clubfoot Fig. 1 Child with amyoplasia showing the typical pattern
case may in fact represent a case of distal of involvement of all 4 limbs: the arms are extended at the
elbow, the wrists are flexed and lie in ulnar deviation, the
arthrogryposis rather than an idiopathic CTEV thumb is in the palm and the fingers are less flexed than is
(congenital talipes equinovarus) deformity. Con- normal for a neonate, the perineum is wide due to the
genital vertical talus is commonly associated with bilateral hip dislocation, both knees are flexed and the
a distal arthrogryposis variant (Fig. 2). In an attempt feet are in equinovarus. The limbs appear featureless
and tubular
to bring some structure to the diagnosis of distal
arthrogryposis, major upper and lower limb diag-
nostic criteria have been described [6] (Table 3). infection and maternal health. Family history is
Typically two or more major criteria must be extremely important, as is enquiry about develop-
exhibited but when a first degree relative displays mental milestones and general medical health.
these criteria, other family members with only one All children should be seen by a geneticist and
major diagnostic criterion are considered affected. a paediatrician. The assessment of the child should
For a variety of reasons such as treatment be global, looking for associated problems such as
options, outcome expectations, genetic counselling central neurological disorders and issues such as
and natural progression or otherwise of the condi- intra-abdominal pathology that may accompany
tion, it is important to try and establish the under- amyoplasia. Particular attention should be paid to
lying diagnosis. In this regard the approach by the childs functional abilities and extent and dis-
Bamshad and colleagues [5, 6] to define broad tribution of all their joint contractures.
groupings of presence or absence of neurological Appropriate investigations may include not
abnormality, is extremely useful. With this in mind, only radiographs of the affected body region but
an approach to the child presenting with a congen- also other regions such as screening for hip devel-
ital contracture should include a full antenatal his- opment and advanced imaging of the central ner-
tory paying particular attention to intra-uterine vous system with magnetic resonance scans.
4632 A.J. Graydon and D.M. Eastwood

Fig. 2 Child with distal


arthrogryposis and bilateral
vertical tali (rocker bottom
feet). (see Fig. 9 for
radiographs, of a different
patient, that show the same
deformity)

Table 3 Major Diagnostic Criteria for Distal over classification. Clearly in such a heteroge-
Arthrogryposes [6] neous group of disorders, the natural history will
Upper limb Lower limb vary according to the presence or absence of asso-
Camptodactyly Talipes equinovarus ciated conditions and the number and the severity
Hypoplastic and/or absent Calcaneovalgus of the deformities. It is considered that, untreated,
flexor creases
the ambulatory and functional potential of
Over-riding fingers Congenital vertical
talus
patients with arthrogryposis is poor, but that with
Ulnar wrist deviation Metatarsus aggressive conservative and surgical management
adductus/varus and a supportive rehabilitation programme, most
children will develop a functional level of walk-
ing. A recent report [18] suggested that compared
Haematological studies such as creatine kinase to their normal peer group, children with
levels and acetylcholine receptor antibody assays arthrogryposis took less steps per day and spent
may help in identifying certain neuromuscular less time at high activity levels: however the study
causes. Similarly muscle biopsies and nerve con- was too small to identify significant differences
duction studies may help differentiate between between the children with amyoplasia and those
neuropathic and myopathic aetiology although with distal arthrogryposis.
the differentiation between these two causes of Whilst typically no new joint involvement
arthrogryposis has not yet translated usefully into occurs over time, the joint problems do progress;
the design of different management plans. joints become stiffer with age and degenerative
changes do develop.

Natural History of Arthrogryposis


Amyoplasia
There are very few studies on the natural history
of patients with arthrogryposis and furthermore With modern medical techniques, it is generally
those published are often difficult to interpret accepted that many children with disabilities are
because of clinical heterogeneity and confusion living longer lives. Darin and colleagues [19]
Orthopaedic Management of Arthrogryposis Multiplex Congenita 4633

demonstrated a 94 % 20-year survival rate for


children with amyoplasia. The natural history of Indications for Surgery
untreated amyoplasia is not clearly documented
but with treatment up to 85 % of children have It is most important to remember that the char-
been reported to ambulate and participate in acteristic feature of arthrogryposis in all its
activities of daily living [16] but at the cost of forms is a congenital contracture of the affected
an average of 67 operative procedures per joints. The joints (including bones, articular
patient during their growing years [20]. Function cartilage, muscles, tendons and skin) have
does deteriorate in later childhood despite treat- never had normal movement and despite the
ment. In a recent follow-up series, positive prog- best of intentions never will. Even if joint
nostic factors for walking were active hips and movement could be obtained, it is unlikely
knees, hip flexion contractures less than 20 and that this movement would be well controlled
knee flexion contractures less than 15 (Table 4). leaving the dilemma of whether a flaccid
Only 7 of 11 patients did achieve walking ability uncontrolled (unstable) joint is better than a
but this did not correlate with the severity of the rigid (stable) joint, albeit with deformity. It is
condition at birth. Six of 7 patients achieved vital for both the surgeon and the family to
independent walking by 2.5 years [20]. understand this aspect of the pathophysiology
of the condition as a failure to recognise this
will lead to unnecessary surgery and unrealistic
Distal Arthrogryposis expectations. Repeated multidisciplinary assess-
ments are often required before a surgical treat-
Patients with distal arthrogryposes are expected ment plan is conceived. The goals of treatment
to have a life expectancy similar to their peer are not to achieve normal joint function but to
groups. Essentially all patients will walk (after align the extremities to allow self care and to
any necessary correction of lower limb defor- maximise the potential to sit, stand and if pos-
mity) and their independent living skills are gen- sible ambulate. Overall, management is directed
erally good. The study by Darin [19] could not at enhancing function and maintaining indepen-
detect any significant difference between the dence throughout a lifetime.
functional walking abilities of a patient with The specific indications for surgery will
amyoplasia and one with distal arthrogryposis depend greatly upon the level and extent of defor-
although most clinicians would feel that the mity, as well as any associated conditions that
prognosis for a better quality of walking pattern may be seen with the arthrogryposis syndrome.
was higher in the group of patients with distal A more detailed discussion of the indications for
arthrogryposis. surgery on individual limb deformities is found in
the next section of this chapter.

Syndromic Arthrogryposis

Depending on the severity of the associated neu- Orthopaedic Management


rological disorder, up to 50 % of infants born with
a congenital contracture associated with a central General Considerations
nervous system (CNS) abnormality will die in the
immediate postnatal period [4, 19]. Although this section primarily discusses specific
The natural history of those patients that sur- operative interventions for the individual defor-
vive the neonatal period is very much dependent mities associated with arthrogryposis, it is impor-
upon the severity of the CNS abnormality. tant to remember the value of non-operative
Their potential for independent ambulation and strategies. The contractures are at their most sup-
activities of daily living also reflects this severity. ple in infancy and there is some evidence that
4634 A.J. Graydon and D.M. Eastwood

physiotherapy, joint manipulation, casting and


splinting of deformities initiated within the first
4 months of life can improve joint range of
motion, preserve and enhance muscle mass and
also reduce the need for surgery [21]. The out-
comes of surgical release of the contracted joints
is generally better if performed early (before 5
years of age) before articular incongruity has
developed but, in contrast, osteotomies should
be performed later otherwise remodelling with
growth can lead to more complex problems.
A recent long term follow-up study [20] on
patients with amyoplasia documented an average
of 6 lower limb operations per patient but only 0.8
upper limb procedures per patient. Surgical inter-
vention can frequently be followed by recurrent
deformity over time, unless an aggressive long
term bracing/splinting routine is followed. The
use of splints can be difficult on the somewhat
tubular and featureless arthrogrypotic limbs and
support for the family from the physiotherapists
and orthotists is important.

Upper Limb Surgery Fig. 3 Child with amyoplasia and significant upper limb
contractures demonstrating her ability to hold a pen
Upper limbs are frequently involved and, in gen-
eral, the severity of the joint stiffness increases Shoulder
from proximal to distal so that wrist and hand
contractures are worse. The limbs have normal Surgery for the shoulder joint is rarely needed in
sensation and despite the significant deformities, arthrogryposis, although the internal rotation
children show great adaptive ability in deformity classically seen in amyoplasia may
maintaining a surprising amount of function be amenable to an external rotation humeral
(Fig. 3). For example, poor elbow flexion may osteotomy. The aim of such surgery is to
effectively be augmented by neck flexion and place the hand and forearm into a more
wrist flexion. The upper limbs are often used for functional position. As with all osteotomies in
bi-manual activities and a careful multidis- arthrogryposis, care must be taken if the surgery
ciplinary assessment must be made before any is performed before the age of 8 as the potential
surgical correction is undertaken to ensure that for remodelling is high with recurrence of the
the new upper limb position does not detract internal rotation contracture.
from function.
Achieving functional independence is the aim Elbow
of upper limb surgery. This involves use of the
upper limb not only for self-care activities but Management of the elbow is challenging in that it
also for achieving or maintaining the ability to can be extremely easy to reduce the functional
use crutches or a wheelchair for mobility. There- status of the limb by taking an extended elbow
fore one elbow may need active extension and the that is unable to flex, and turning it into a flexed
other active flexion. elbow that is unable to extend. It may be more
Orthopaedic Management of Arthrogryposis Multiplex Congenita 4635

functional to have one elbow for flexion and the


other in the extended position. Dislocated elbows
are not uncommon.
If passive elbow flexion has not been achieved
by the age of 2, it is recommended that a posterior
elbow release and triceps lengthening is considered
in the most flexible or dominant elbow [22, 23]. If
no active flexion has developed by the age of 4, a
tendon transfer may be of use. Historical studies
documented disappointing results because of
donor site morbidity and poor tendon transfer
activity particularly if the muscle used was
affected by the arthrogryposis [24]. Recent
studies have been more optimistic with a triceps
to biceps transfer probably providing the best
results although care must still be taken to
ensure that this does not contribute to the
development of a flexion contracture [22]. The
loss of active extension can be devastating
for some patients, for example, those who
need to push out of a wheelchair and those who
were independent for toileting. A recent report
transfers triceps to the proximal ulna [25].
The pre-requisites for a tendon transfer include
passive elbow flexion to 90 and a muscle of
MRC grade 4 to transfer.
Patients with only passive flexion or a flaccid
elbow may be helped by an orthotic brace (Fig. 4) or
use their opposite upper limb as a prop (Fig. 5).

Wrist and Hand


Fig. 4 Child with amyoplasia and 4 limb involvement.
The aim of surgical management of the wrist and Right elbow function is improved by use of an orthotic
device. Bilateral KAFO (knee ankle foot orthoses) allow
hand deformity is to improve pinch, grasp him to walk. He needs assistance to put his splints on and off
and release. Early management of the flexed,
ulnarly-deviated wrist deformity is becoming
more common. Tendon lengthening (of flexor deformity to achieve post function for the thumb
carpi ulnaris) may be helpful: a tendon transfer and increase the web-space is probably most ben-
to the extensors usually only provides a tenodesis eficial. This may be achieved by an opponensplasty
effect. Performed before carpal ossification, and concomitant thumb metacarpophalangeal joint
a dorsal closing-wedge carpal osteotomy, in com- arthrodesis. The camptodactyly of the lesser digits
bination with a flexor carpi ulnaris transfer and associated with arthrogryposis is best managed
centralization of the wrist extensors can achieve a with physical therapy and splinting, as surgical
functional position of wrist extension with a pre- results are often disappointing and skin defects
served arc of motion [24]. requiring grafting are often created. Finger func-
The finger and thumb deformities can be diffi- tion may also improve following wrist centraliza-
cult to manage. Correction of the thumb in palm tion and stabilization [24, 26].
4636 A.J. Graydon and D.M. Eastwood

femoral de-rotation osteotomy can improve the


foot progression angle if this is required [21].
A unilateral hip dislocation should be reduced.
Closed reduction is generally unsuccessful [28]
and an open reduction may be performed
through either anterior (anterolateral) or medial
approaches. Anterior approaches are associated
with lower rates of re-dislocation but higher rates
of joint stiffness, whereas a medial approach, par-
ticularly if performed early, is associated with less
stiffness but a higher incidence of re-dislocation.
A concomitant femoral shortening osteotomy
reduces AVN rates in all series [21, 27, 29].
Fig. 5 Child with amyoplasia who has some passive Treatment of bilateral dislocations is far more
flexion of her left elbow but no active flexion. The right
arm is used as a support to keep the left elbow flexed controversial (Figs. 6 and 7a). Historically obser-
vation of bilateral dislocation has been advocated
given the presumed limited ambulatory potential
Lower Limb Surgery of this patient group. However, recent studies
have demonstrated the good long-term results
The main aim of lower limb surgery is to facilitate that can be achieved with open reduction [28].
standing and maximise walking potential. As Although the approach used should be that which
with the upper limb, a multidisciplinary approach the surgeon is most comfortable and competent
is recommended in order to assess this accurately. with, many authors now advocate medial
The pre-requisites for standing and walking are approaches for initial reduction in the younger
plantigrade and stable feet, extended knees and child and anterior approaches for the older child
hips with less than 30 of fixed flexion [18]. and revision procedures [17, 27]. All authors
appear to have a favourite technique: some advo-
cate careful capsular plication post-reduction and
Hip minimal post-operative immobilisation, some
favour aggressive concomitant bony surgery
Hip involvement in amyoplasia is common, (Fig. 7b) and others suggest that a peri-acetabular
affecting 80 % of patients. Although the defining circumferential capsular release is a crucial fea-
characteristic is stiffness and contracture, actual ture of the operative procedure [2729].
hip dislocation is not universal and occurs in only There is considerable debate regarding the
1530 % of cases [27, 28]. timing of hip surgery but if stable hips can be
Isolated external rotation or abduction con- achieved prior to walking age this is advisable.
tractures improve with physiotherapy and time. With associated knee deformities, extension
A pure flexion contracture may require surgical deformities of the knee can be managed concur-
correction when there is a fixed deformity of rently with a hip dislocation, whilst associated
more than 30 as at this level it may interfere knee flexion deformities must be managed
with ambulation. When the contractures occur separately due to the problems of post-operative
in combination, the typical external rotation pos- immobilisation.
ture may have turned the hip abductors into func-
tional hip extensors and effectively negated the
flexion contracture thus great care must be taken Knee
when planning a soft tissue release to ensure that
function is improved and that hip extensor Knee deformities are seen in around 75 % of
strength is maintained for ambulation. A distal patients with amyoplasia: flexion deformities
Orthopaedic Management of Arthrogryposis Multiplex Congenita 4637

Fig. 6 AP radiograph of a
10 yr-old child with
amyoplasia whose hip
dislocations were left
untreated

are significantly more common than extension extension and shortening osteotomy is
contractures and a few patients present with recommended [31], although the potential for
knee dislocation (for example in Larsen Syn- remodelling of the correction should be remem-
drome). Overall, irrespective of treatment, those bered. Some authors advocate gradual correction
presenting with a flexion contracture are less of moderate to severe deformities using Ilizarov
likely to be ambulators in later life. The goal of techniques [32] or guided growth techniques [33].
treatment is a knee which has less than 20 of Whichever technique is used, post-operative
fixed flexion and at least 60 of flexion (passive or serial casting and prolonged bracing is an impor-
active). This allows a satisfactory sitting position tant component of the management plan.
without compromising the ability to stand with or By contrast knee extension deformities (Fig. 8)
without orthotic support [30]. are usually easier to treat, responding well to
Knee flexion deformities are the most difficult physical therapy and splinting [30]. The limb is
to treat. Approximately 25 % of contractures often externally rotated due to the hip position and
respond to physiotherapy and splinting or serial therefore great care must be taken in assessing the
casting but success is usually limited to the less plane of correction for the knee extension: all too
severe cases. If conservative treatment fails, fur- often the knee is cast into flexion in the exter-
ther management should be guided by the childs nally rotated position meaning that the knee is
ambulatory potential. Staheli and colleagues [21] actually being cast into valgus causing unneces-
have recommended that this assessment is best sary stretching of the medial (rather than the
performed between 2 and 3 years of age. If the anterior) structures. Often the extension deformi-
walking potential is good and the flexion defor- ties that fail to respond, are those associated with
mity is greater than 20 , surgical treatment in the knee dislocation. True knee dislocations should be
form of a posterior soft tissue release and treated early: the surgical procedure often requires
tendon lengthening should be considered with or an aggressive soft tissue release and in some cases
without pre and post-operative serial casting. For a concomitant femoral shortening operation. One
contractures of greater than 45 , a femoral report [34] suggests that a very early (12 months)
4638 A.J. Graydon and D.M. Eastwood

a additional difficulty: it is not unusual for the


sciatic nerve to be lying superficially within
the web. The knee deformity limits mobility and
thus treatment must be considered. Resection of
the web and Z-plasty of the skin and soft tissues
rarely provide enough correction and acute
femoral shortening (and occasionally extension)
or gradual distraction techniques may be neces-
sary. Acute shortening appears to provide better
outcomes.

b
Foot and Ankle

Foot deformities in both amyoplasia and the con-


genital contracture syndromes in general are vir-
tually universal: the classic abnormality is the
clubfoot (talipes equinovarus) deformity, noted
in up to 8090 % of patients [35]. In some forms
of distal arthrogryposes, a short stiff foot may be
the only abnormality seen. The other deformities
that may be seen include congenital vertical talus
(Fig. 9) and calcaneovalgus. The aim of treatment
is a braceable, plantigrade, stable, and pain free
foot for weight-bearing.
Traditionally, management of the arthro-
grypotic foot deformity has been surgical and
Fig. 7 (a) Radiographs of a child with amyoplasia and associated with a high failure or recurrence rate,
bilateral hip dislocations. She also had bilateral knee a review by Drummond [36] noting an average
extension deformities and bilateral vertical tali. (b) Same
child as in 6a, following bilateral open reductions and of 3.1 surgical procedures per foot. The most
bilateral varus, de-rotational and shortening femoral common problems appear to be a failure to obtain
osteotomies. The procedures were staged and/or maintain a complete correction from the
time of initial surgery leading clinicians to advo-
percutaneous quadriceps tenotomy and serial cast- cate an aggressive circumferential soft tissue
ing obtains similarly good results. It should be release with tendon resection (rather than length-
remembered that knee flexion should not be ening), prolonged immobilisation and long-term
achieved at the cost of the knee extension orthotic support [37]. Where possible, surgery
required for standing but the long-term results of should be timed to coincide with the development
quadricepsplasty have demonstrated that most of a standing posture in order to maintain a plan-
patients gain functional improvement and walking tigrade position.
ability, often without the need of orthotic support, In selected cases, a young child with a severe
whilst maintaining quadriceps power [18]. deformity, a well-executed primary talectomy
can lead to a satisfactory long-term result and
certainly the procedure has its advocates as
Pterygia Syndrome a revision procedure for the foot with recurrent
deformity. Incomplete excision and/or malposi-
The knee flexion contracture associated with a tion of the talus with respect to the calcaneum is
popliteal web syndrome presents a specific associated with a poor outcome [38].
Orthopaedic Management of Arthrogryposis Multiplex Congenita 4639

Fig. 8 Radiographs of a
child with amyoplasia and
bilateral knee extension
deformities (she also had
bilateral hip dislocations
and bilateral vertical tali)

Fig. 9 Radiographs of a
child with amyoplasia
demonstrating bilateral
vertical tali (equinus
hindfoot and dorsiflexed
mid foot and dislocation
of the talonavicular joint)

The relapsed foot may require aggressive cor- Despite the neuromuscular aetiology of many of
rection usually via osteotomies or arthrodeses: these foot deformities, muscle re-balancing or
revision soft tissue releases are rarely effective. tendon transfer procedures are rarely appropriate
Treatment must be focussed on the most affected due to the global weakness of the affected muscle
joint but it is important to obtain a complete cor- groups. Triple arthodesis is associated with a
rection and therefore all levels (the hindfoot, good outcome and, if required, may be combined
midfoot and forefoot and the supramalleolar with an ankle arthrodesis to obtain a plantigrade
tibia) must be considered as part of the surgical and stable foot [17, 36].
plan. Such a multi-level correction can be In the past it has been felt the nature and sever-
performed as a staged procedure or performed ity of the soft tissue contracture in arthrogryposis
gradually using an external fixator device [39]. prohibited deformity correction by manipulation
4640 A.J. Graydon and D.M. Eastwood

and casting: the thick capsular structures appeared success, for example the Ponseti cast is usually
incapable of stretching with growth and thus applied with the knee in 90 of flexion to
recurrent deformity was inevitable. ensure that the foot position is maintained but
More recently, the success of the Ponseti this is not always achievable in patients with
method of serial manipulation and casting in arthrogryposis. Similarly, the knee contracture
obtaining excellent long-term results in the itself may require manipulation into extension
idiopathic clubfoot has led clinicians to (rather than flexion). For the rigid flat foot defor-
re-evaluate the role of conservative treatment in mity or vertical talus similar concerns exist
the arthrogrypotic foot. Boehm et al. [40] have regarding the reliability of a surgical correction
demonstrated that in the management of distal and the use of the reverse Ponseti technique. In
arthrogryposis, the Ponseti method of correction the review by Drummond et al. [36], the flatfoot
of CTEV deformity was as successful as in idio- deformity required surgery less frequently as the
pathic cases, at obtaining a correction without the foot was plantigrade, stable and pain free. Aroojis
need for a major surgical procedure. However a et al [43] believe that a vertical talus only occurs
significantly higher number of cast changes per in distal types of athrogryposis and does respond
foot were required and the tenotomy rate was to single-stage soft tissue correction: these
100 %. The follow-up was too short to comment authors present Fig. 9 which demonstrates bilat-
on relapse/recurrence rates. Morcuende et al. [41] eral vertical tali in a child with amyoplasia, both
reported similar results from a group of patients feet were treated by the reverse Ponseti
who were probably more severely affected. 97 % technique.
of their patients had an initially satisfactory cor-
rection, however at follow-up of a mean 4.6
years, a total of 4/32 feet had required Spine Surgery
a significant posterior or posteromedial release.
They commented that, in most cases, it was not The incidence of spinal deformity probably
feasible and indeed, unnecessary, to achieve 70 varies with the exact aetiology of the
of forefoot abduction and that 50 was accept- arthrogrypotic syndrome and may relate to the
able: They also noted that even post-tenotomy, severity of the muscle weakness. Typically,
ankle dorsiflexion was more limited and thus the patients with amyoplasia do not present with a
position of the shoes on the bars must mirror the congenital scoliosis but the reported prevalence
foot position in the final cast and should not be set [44, 45] for scoliosis during childhood and ado-
at the standard position for the idiopathic foot. lescence is from 22.5 % to 66 %. There are no
Van Bosse et al. [42] looked at 19 foot deformi- comprehensive reviews of the natural history of
ties in patients with a more amyoplastic than the scoliosis but most reported curves are struc-
distal arthrogrypotic appearance, and used a tural thoracolumbar c-shaped neuromuscular
modified technique which involved a percutane- curves in appearance. They are often rigid and
ous Achilles tenotomy at initial assessment and severe and respond poorly to bracing regimes.
prior to application of the first corrective cast. Most reports of scoliosis in this group of condi-
Correction was continued until 60 of forefoot tions note that the deformity is associated with
abduction was achieved and a repeat tenotomy hyperlordosis, a significant pelvic obliquity and
was required in 53 % cases. 2 feet (10 %) required poor (or non-existing) walking ability.
a posterior release within the mean follow-up Combined anterior-posterior spinal fusions
period of 38 months [42]. appear to give the best results and should be
In all situations it remains essential that the considered if the curve is >30 particularly in
child is treated in a holistic manner: many chil- the non-ambulant patient [17, 46]. Extending
dren have associated knee contractures which the fusion to the pelvis may not be indicated if
may influence the timing of treatment or its passive hip flexion is <90 as sitting posture may
Orthopaedic Management of Arthrogryposis Multiplex Congenita 4641

be compromised [20, 47]. Amongst the distal and number of the joint contractures may make it
arthrogrypotic syndromes, scoliosis is much less difficult to position the patient appropriately on
common and overall less severe: treatment is by the operating table, particularly if multiple
conventional means but the results may not be as procedures are to be performed concurrently
good as in non-syndromic conditions. and particular care must be taken to ensure that
pressure points are well-protected. Specifically
however, arthrogrypotic patients are at increased
Peri-Operative Care risk of malignant hyperthermia. Although the
mechanism of this is poorly understood it proba-
Accepting that the specific surgical indications bly relates to the intrinsic muscle abnormalities
have been met, the arthrogrypotic child presents of many of the distal arthrogryposes [48].
certain other important issues for all members of
the surgical and rehabilitation team to consider
before informed consent can be obtained. Rehabilitation Issues

Arthrogrypotic patients are typically smaller and


Co-Morbidities weigh less than age-matched controls. Neuro-
developmental studies have suggested that
Pre-existing malnutrition may be an unrecognized although patients are typically of normal or
issue in arthrogrypotic patients particularly the above normal intelligence, language skills may
young ones. Up to 50 % may have issues such as not be age appropriate [17]. This has implications
poor enteral feeding secondary to their stiff for both pre-operative preparation and post-
jaws and the relative immobility of their operative rehabilitation in terms of ability to
tongue with failure to thrive and constipation comply with instructions and practical use of
[17]. Chronic upper respiratory tract infections the equipment required for rehabilitation. In addi-
are also more common, and these increase tion the generalized nature of these disorders may
the risk of post-operative complications such as create its own mobility issues, for example upper
aspiration and atelectasis. Other issues may be limb deformities may hinder the use of mobility
syndrome specific and dependant on the exact aids following lower limb surgery (Fig. 10). On
aetiology of the joint contractures [48]. the positive side, these children are often very
well-motivated and show a natural ability to
learn substitution techniques and they may cope
Specific Anaesthetic Issues surprisingly well with the rehabilitation process.
Post-operatively, it is imperative that children
Several concerns affect the delivery of an anaes- have an appropriate and individualised
thetic in patients with arthrogryposis. Due to the programme of physiotherapy and splinting
generalised nature of the condition both in terms (Fig. 4). Bracing may be for function and/or for
of number of body parts involved and the variety maintenance of range of movement, therefore
of different tissues affected, venous access can be there may be splints for daytime and for night
limited. The muscle mass is significantly time use. Adherence to a bracing regime until
decreased in affected children compared to their skeletal maturity reduces the risk of recurrent
peer group and this affects the volume of distri- contractures developing with growth. The recov-
bution of many anaesthetic agents. Endotracheal ery of muscle activity after a return of passive
intubation can also be challenging given the movement in a useful arc may take time and is a
oropharyngeal dimensions of these patients and good prognostic sign: it accounts for the increased
the fact that the high cervical hypoplasia may be ambulatory ability in 4/7 children in the long term
associated with vertebral instability. The severity follow up study by Fassier et al. [20].
4642 A.J. Graydon and D.M. Eastwood

deformity occur due to either the underlying


pathology or problems with the post-operative
bracing regime.

Summary

Patients with arthrogryposis present their fami-


lies and their doctors with a variety of challenges.
Management of the musculoskeletal problems
can be particularly difficult and is best accom-
plished when the surgeon is part of a multi-
disciplinary team. This team should involve
developmental paediatricians, neurologists,
physiotherapists, occupational therapists,
geneticists and social services and is essential in
ensuring the child and their family receives not
only general care and support for a condition
associated with life-long difficulties but also
appropriate specific input to maximize the out-
come of any surgical procedures the child may
receive. The team is also invaluable in helping to
Fig. 10 Adapted walking frame for a child with amyoplasia identify the childs specific needs and in directing
the site and timing of surgery to ensure that real-
istic goals are achieved. The need for surgery is
Complications high in these children and often multi-level
surgery may be required particularly in the
The Orthopaedic management of patients with lower limb: an experienced surgical team
arthrogryposis in all its forms is subject to the with the appropriate back-up provides the best
same set of general and local complications that environment for a good outcome.
can affect any operative procedure. There are two
main pertinent issues to remember. The first is
that many surgical procedures, for example,
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