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
Abstract
The term arthrogryposis means contracted and
crooked joints. Many different variants of the
condition have been described and care must
be taken to identify any associated problems so
that an appropriate overall assessment of
the child can take place. The aim of the Ortho-
paedic management of these children is to
maximise independent living skills and mobil-
ity. A key feature is that none of the musculo-
skeletal tissues around the contracted joint are
normal and thus a normal joint with normal
function cannot be achieved with any form of
treatment either surgical or conservative. Both
the parents and clinicians must be aware of this
so that realistic treatment goals can be set.
A multidisciplinary team is essential in the
management of these complex cases.
Keywords
AMC  Amyoplasia and Orthopaedic
management  Distal arthrogryposis
Introduction
General Definitions
For nearly 100 years, the term arthogryposis has
been used to describe a group of disorders involv-
ing congenital joint contractures that affect two
or more areas of the body. The word itself means
hooked or curved joints and hence, in essence,
it is simply a description of a physical appearance
and the clinical picture has been recognised for
much longer than a century. Currently there are
some 300 recognised disorders that may present
as arthrogryposis [1]. To add to the confusion,
over the years a variety of terms have been used
to refer to specific subgroups of patients with
congenital joint contractures which has made
it difficult to compare case series and perhaps
hampered our understanding of the natural
history of these conditions and the influence of
treatment on them.
Arthrogryposis Multiplex Congenita (AMC) is
a historical term first used by Stern in 1923 and
A.J. Graydon and D.M. Eastwood
often used to refer to both the syndrome
of amyoplasia or classical arthrogryposis
and other more general arthrogrypotic
conditions [2, 3].
Amyoplasia refers to a particular form of
arthrogryposis with a characteristic phenotype
of internally rotated, extended upper limb pos-
ture, with ulnarly deviated, flexed and thumb in
palm hand deformities, hip dislocation, vari-
able knee deformity and severe foot deformities.
Associated features include normal intelligence,
mid-line facial haemangiomata, and ocular and
gastro-intestinal abnormalities [4].
Distal Arthogryposis, as the name implies,
involves predominantly the distal parts of the
limbs and is a congenital contracture of two or
more body areas without an associated neuro-
logical and/or muscle disease [5]. Bamshad
et al. [6] currently recognize 10 different pat-
terns of distal arthrogryposis.
Pterygia syndromes, where the webs crossing
the flexor aspect of joints (particularly the
elbow and the knee) add significantly to the
joint contractures.
Prevalence and Aetiology
The prevalence of an arthrogrypotic syndrome is
quoted variably between 1/3,000 and 1/5,000
live births depending on which definition of
arthrogryposis is used: for example, the preva-
lence of amyoplasia itself is usually given as
1:10,000 live births [3].
Although most causes of these syndromes
are unknown they are assumed to be multifactorial
in nature. Hall [3, 7] described a multitude of
groupings of potential aetiologic factors ranging
from maternal diseases to intrinsic muscle abnor-
mality. He emphasised the importance of
establishing the underlying disorder in order to
determine issues such as inheritance, the risk of
associated abnormalities and the natural history
for any individual patient (Table 1).
Overall, the group of arthrogrypotic disorders
is genetically heterogeneous but some subgroups
do demonstrate consistent inheritance patterns.
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
normal neurological examination)
Syndromes in
decreasing frequency Description
DA Type 1 (OMIM Clubfeet and camptodactyly
108120) with occasional involvement
of the shoulders and hips
DA Type 2 (OMIM Freeman-Sheldon
193700) syndrome
Hand and foot abnormalities
in addition to scoliosis,
oropharyngeal abnormalities
and a distinctive whistling-
face appearance
DA Type 5 (OMIM Ocular abnormalities in
108145) addition to the
musculoskeletal deformities
DA Type 7 (OMIM Trismus-
158300) pseudocamptodactyly
syndrome characterized by
an inability to open the mouth
fully
DA Type 3 (OMIM Rare disorders with varying
114300), 4 and 6 associations with cleft palate
Type 3 also known as
Gordon Syndrome
activation of the spinal cord neurons due to
a problem within the corticospinal tract. Abnor-
malities such as microcephaly and intra-uterine
CNS infection may cause such problems. In
a similar manner, chromosomal anomalies asso-
ciated with intrinsic CNS damage can lead to
arthrogryposis.
Similarly, any congenital alteration in
the function of the neuromuscular unit may result
in joint contractures. Congenital neuropathies
are uncommon but abnormalities of the neuro-
muscular junction are less so. Arthrogryposis is
seen more commonly in infants of mothers with
myasthenia gravis, as well as in infants born with
inherited mutations in genes encoding for
components of skeletal muscle acetylcholine
receptors [10, 11]. Congenital myopathies due
to mutations in skeletal muscle proteins can
also result in arthrogryposis. It is important to
recognize myopathies (such as congenital
muscular dystrophy) as a cause of arthrogryposis
because the contractures they produce may
increase in severity after birth [12].

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

There are very few studies on the natural history
of patients with arthrogryposis and furthermore
those published are often difficult to interpret
because of clinical heterogeneity and confusion
Amyoplasia
With modern medical techniques, it is generally
accepted that many children with disabilities are
living longer lives. Darin and colleagues [19]
Orthopaedic Management of Arthrogryposis Multiplex Congenita
demonstrated a 94 % 20-year survival rate for
children with amyoplasia. The natural history of
untreated amyoplasia is not clearly documented
but with treatment up to 85 % of children have
been reported to ambulate and participate in
activities of daily living [16] but at the cost of
an average of 67 operative procedures per
patient during their growing years [20]. Function
does deteriorate in later childhood despite treat-
ment. In a recent follow-up series, positive prog-
nostic factors for walking were active hips and
knees, hip flexion contractures less than 20 and
knee flexion contractures less than 15 (Table 4).
Only 7 of 11 patients did achieve walking ability
but this did not correlate with the severity of the
condition at birth. Six of 7 patients achieved
independent walking by 2.5 years [20].
Distal Arthrogryposis
Patients with distal arthrogryposes are expected
to have a life expectancy similar to their peer
groups. Essentially all patients will walk (after
any necessary correction of lower limb defor-
mity) and their independent living skills are gen-
erally good. The study by Darin [19] could not
detect any significant difference between the
functional walking abilities of a patient with
amyoplasia and one with distal arthrogryposis
although most clinicians would feel that the
prognosis for a better quality of walking pattern
was higher in the group of patients with distal
arthrogryposis.
Syndromic Arthrogryposis
Depending on the severity of the associated neu-
rological disorder, up to 50 % of infants born with
a congenital contracture associated with a central
nervous system (CNS) abnormality will die in the
immediate postnatal period [4, 19].
The natural history of those patients that sur-
vive the neonatal period is very much dependent
upon the severity of the CNS abnormality.
Their potential for independent ambulation and
activities of daily living also reflects this severity.
4633
Indications for Surgery
It is most important to remember that the char-
acteristic feature of arthrogryposis in all its
forms is a congenital contracture of the affected
joints. The joints (including bones, articular
cartilage, muscles, tendons and skin) have
never had normal movement and despite the
best of intentions never will. Even if joint
movement could be obtained, it is unlikely
that this movement would be well controlled
leaving the dilemma of whether a flaccid
uncontrolled (unstable) joint is better than a
rigid (stable) joint, albeit with deformity. It is
vital for both the surgeon and the family to
understand this aspect of the pathophysiology
of the condition as a failure to recognise this
will lead to unnecessary surgery and unrealistic
expectations. Repeated multidisciplinary assess-
ments are often required before a surgical treat-
ment plan is conceived. The goals of treatment
are not to achieve normal joint function but to
align the extremities to allow self care and to
maximise the potential to sit, stand and if pos-
sible ambulate. Overall, management is directed
at enhancing function and maintaining indepen-
dence throughout a lifetime.
The specific indications for surgery will
depend greatly upon the level and extent of defor-
mity, as well as any associated conditions that
may be seen with the arthrogryposis syndrome.
A more detailed discussion of the indications for
surgery on individual limb deformities is found in
the next section of this chapter.
Orthopaedic Management
General Considerations
Although this section primarily discusses specific
operative interventions for the individual defor-
mities associated with arthrogryposis, it is impor-
tant to remember the value of non-operative
strategies. The contractures are at their most sup-
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
Upper limbs are frequently involved and, in gen-
eral, the severity of the joint stiffness increases
from proximal to distal so that wrist and hand
contractures are worse. The limbs have normal
sensation and despite the significant deformities,
children show great adaptive ability in
maintaining a surprising amount of function
(Fig. 3). For example, poor elbow flexion may
effectively be augmented by neck flexion and
wrist flexion. The upper limbs are often used for
bi-manual activities and a careful multidis-
ciplinary assessment must be made before any
surgical correction is undertaken to ensure that
the new upper limb position does not detract
from function.
Achieving functional independence is the aim
of upper limb surgery. This involves use of the
upper limb not only for self-care activities but
also for achieving or maintaining the ability to
use crutches or a wheelchair for mobility. There-
fore one elbow may need active extension and the
other active flexion.
Fig. 3 Child with amyoplasia and significant upper limb
contractures demonstrating her ability to hold a pen
Shoulder
Surgery for the shoulder joint is rarely needed in
arthrogryposis, although the internal rotation
deformity classically seen in amyoplasia may
be amenable to an external rotation humeral
osteotomy. The aim of such surgery is to
place the hand and forearm into a more
functional position. As with all osteotomies in
arthrogryposis, care must be taken if the surgery
is performed before the age of 8 as the potential
for remodelling is high with recurrence of the
internal rotation contracture.
Elbow
Management of the elbow is challenging in that it
can be extremely easy to reduce the functional
status of the limb by taking an extended elbow
that is unable to flex, and turning it into a flexed
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

The aim of surgical management of the wrist and
hand deformity is to improve pinch, grasp
and release. Early management of the flexed,
ulnarly-deviated wrist deformity is becoming
more common. Tendon lengthening (of flexor
carpi ulnaris) may be helpful: a tendon transfer
to the extensors usually only provides a tenodesis
effect. Performed before carpal ossification,
a dorsal closing-wedge carpal osteotomy, in com-
bination with a flexor carpi ulnaris transfer and
centralization of the wrist extensors can achieve a
functional position of wrist extension with a pre-
served arc of motion [24].
The finger and thumb deformities can be diffi-
cult to manage. Correction of the thumb in palm
Fig. 4 Child with amyoplasia and 4 limb involvement.
Right elbow function is improved by use of an orthotic
device. Bilateral KAFO (knee ankle foot orthoses) allow
him to walk. He needs assistance to put his splints on and off
deformity to achieve post function for the thumb
and increase the web-space is probably most ben-
eficial. This may be achieved by an opponensplasty
and concomitant thumb metacarpophalangeal joint
arthrodesis. The camptodactyly of the lesser digits
associated with arthrogryposis is best managed
with physical therapy and splinting, as surgical
results are often disappointing and skin defects
requiring grafting are often created. Finger func-
tion may also improve following wrist centraliza-
tion and stabilization [24, 26].
4636
Fig. 5 Child with amyoplasia who has some passive
flexion of her left elbow but no active flexion. The right
arm is used as a support to keep the left elbow flexed
Lower Limb Surgery
The main aim of lower limb surgery is to facilitate
standing and maximise walking potential. As
with the upper limb, a multidisciplinary approach
is recommended in order to assess this accurately.
The pre-requisites for standing and walking are
plantigrade and stable feet, extended knees and
hips with less than 30 of fixed flexion [18].
Hip
Hip involvement in amyoplasia is common,
affecting 80 % of patients. Although the defining
characteristic is stiffness and contracture, actual
hip dislocation is not universal and occurs in only
1530 % of cases [27, 28].
Isolated external rotation or abduction con-
tractures improve with physiotherapy and time.
A pure flexion contracture may require surgical
correction when there is a fixed deformity of
more than 30 as at this level it may interfere
with ambulation. When the contractures occur
in combination, the typical external rotation pos-
ture may have turned the hip abductors into func-
tional hip extensors and effectively negated the
flexion contracture thus great care must be taken
when planning a soft tissue release to ensure that
function is improved and that hip extensor
strength is maintained for ambulation. A distal
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].
Treatment of bilateral dislocations is far more
controversial (Figs. 6 and 7a). Historically obser-
vation of bilateral dislocation has been advocated
given the presumed limited ambulatory potential
of this patient group. However, recent studies
have demonstrated the good long-term results
that can be achieved with open reduction [28].
Although the approach used should be that which
the surgeon is most comfortable and competent
with, many authors now advocate medial
approaches for initial reduction in the younger
child and anterior approaches for the older child
and revision procedures [17, 27]. All authors
appear to have a favourite technique: some advo-
cate careful capsular plication post-reduction and
minimal post-operative immobilisation, some
favour aggressive concomitant bony surgery
(Fig. 7b) and others suggest that a peri-acetabular
circumferential capsular release is a crucial fea-
ture of the operative procedure [2729].
There is considerable debate regarding the
timing of hip surgery but if stable hips can be
achieved prior to walking age this is advisable.
With associated knee deformities, extension
deformities of the knee can be managed concur-
rently with a hip dislocation, whilst associated
knee flexion deformities must be managed
separately due to the problems of post-operative
immobilisation.
Knee
Knee deformities are seen in around 75 % of
patients with amyoplasia: flexion deformities
Orthopaedic Management of Arthrogryposis Multiplex Congenita
Fig. 6 AP radiograph of a
10 yr-old child with
amyoplasia whose hip
dislocations were left
untreated
are significantly more common than extension
contractures and a few patients present with
knee dislocation (for example in Larsen Syn-
drome). Overall, irrespective of treatment, those
presenting with a flexion contracture are less
likely to be ambulators in later life. The goal of
treatment is a knee which has less than 20 of
fixed flexion and at least 60 of flexion (passive or
active). This allows a satisfactory sitting position
without compromising the ability to stand with or
without orthotic support [30].
Knee flexion deformities are the most difficult
to treat. Approximately 25 % of contractures
respond to physiotherapy and splinting or serial
casting but success is usually limited to the less
severe cases. If conservative treatment fails, fur-
ther management should be guided by the childs
ambulatory potential. Staheli and colleagues [21]
have recommended that this assessment is best
performed between 2 and 3 years of age. If the
walking potential is good and the flexion defor-
mity is greater than 20 , surgical treatment in the
form of a posterior soft tissue release and
tendon lengthening should be considered with or
without pre and post-operative serial casting. For
contractures of greater than 45 , a femoral
4637
extension and shortening osteotomy is
recommended [31], although the potential for
remodelling of the correction should be remem-
bered. Some authors advocate gradual correction
of moderate to severe deformities using Ilizarov
techniques [32] or guided growth techniques [33].
Whichever technique is used, post-operative
serial casting and prolonged bracing is an impor-
tant component of the management plan.
By contrast knee extension deformities (Fig. 8)
are usually easier to treat, responding well to
physical therapy and splinting [30]. The limb is
often externally rotated due to the hip position and
therefore great care must be taken in assessing the
plane of correction for the knee extension: all too
often the knee is cast into flexion in the exter-
nally rotated position meaning that the knee is
actually being cast into valgus causing unneces-
sary stretching of the medial (rather than the
anterior) structures. Often the extension deformi-
ties that fail to respond, are those associated with
knee dislocation. True knee dislocations should be
treated early: the surgical procedure often requires
an aggressive soft tissue release and in some cases
a concomitant femoral shortening operation. One
report [34] suggests that a very early (12 months)
4638
a additional difficulty: it is not unusual for the
b
Fig. 7 (a) Radiographs of a child with amyoplasia and
bilateral hip dislocations. She also had bilateral knee
extension deformities and bilateral vertical tali. (b) Same
child as in 6a, following bilateral open reductions and
bilateral varus, de-rotational and shortening femoral
osteotomies. The procedures were staged
percutaneous quadriceps tenotomy and serial cast-
ing obtains similarly good results. It should be
remembered that knee flexion should not be
achieved at the cost of the knee extension
required for standing but the long-term results of
quadricepsplasty have demonstrated that most
patients gain functional improvement and walking
ability, often without the need of orthotic support,
whilst maintaining quadriceps power [18].
Pterygia Syndrome
The knee flexion contracture associated with a
popliteal web syndrome presents a specific
A.J. Graydon and D.M. Eastwood
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.
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
associated with a high failure or recurrence rate,
a review by Drummond [36] noting an average
of 3.1 surgical procedures per foot. The most
common problems appear to be a failure to obtain
and/or maintain a complete correction from the
time of initial surgery leading clinicians to advo-
cate an aggressive circumferential soft tissue
release with tendon resection (rather than length-
ening), prolonged immobilisation and long-term
orthotic support [37]. Where possible, surgery
should be timed to coincide with the development
of a standing posture in order to maintain a plan-
tigrade position.
In selected cases, a young child with a severe
deformity, a well-executed primary talectomy
can lead to a satisfactory long-term result and
certainly the procedure has its advocates as
a revision procedure for the foot with recurrent
deformity. Incomplete excision and/or malposi-
tion of the talus with respect to the calcaneum is
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-
rection usually via osteotomies or arthrodeses:
revision soft tissue releases are rarely effective.
Treatment must be focussed on the most affected
joint but it is important to obtain a complete cor-
rection and therefore all levels (the hindfoot,
midfoot and forefoot and the supramalleolar
tibia) must be considered as part of the surgical
plan. Such a multi-level correction can be
performed as a staged procedure or performed
gradually using an external fixator device [39].
Despite the neuromuscular aetiology of many of
these foot deformities, muscle re-balancing or
tendon transfer procedures are rarely appropriate
due to the global weakness of the affected muscle
groups. Triple arthodesis is associated with a
good outcome and, if required, may be combined
with an ankle arthrodesis to obtain a plantigrade
and stable foot [17, 36].
In the past it has been felt the nature and sever-
ity of the soft tissue contracture in arthrogryposis
prohibited deformity correction by manipulation
4640
and casting: the thick capsular structures appeared
incapable of stretching with growth and thus
recurrent deformity was inevitable.
More recently, the success of the Ponseti
method of serial manipulation and casting in
obtaining excellent long-term results in the
idiopathic clubfoot has led clinicians to
re-evaluate the role of conservative treatment in
the arthrogrypotic foot. Boehm et al. [40] have
demonstrated that in the management of distal
arthrogryposis, the Ponseti method of correction
of CTEV deformity was as successful as in idio-
pathic cases, at obtaining a correction without the
need for a major surgical procedure. However a
significantly higher number of cast changes per
foot were required and the tenotomy rate was
100 %. The follow-up was too short to comment
on relapse/recurrence rates. Morcuende et al. [41]
reported similar results from a group of patients
who were probably more severely affected. 97 %
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
a significant posterior or posteromedial release.
They commented that, in most cases, it was not
feasible and indeed, unnecessary, to achieve 70
of forefoot abduction and that 50 was accept-
able: They also noted that even post-tenotomy,
ankle dorsiflexion was more limited and thus the
position of the shoes on the bars must mirror the
foot position in the final cast and should not be set
at the standard position for the idiopathic foot.
Van Bosse et al. [42] looked at 19 foot deformi-
ties in patients with a more amyoplastic than
distal arthrogrypotic appearance, and used a
modified technique which involved a percutane-
ous Achilles tenotomy at initial assessment and
prior to application of the first corrective cast.
Correction was continued until 60 of forefoot
abduction was achieved and a repeat tenotomy
was required in 53 % cases. 2 feet (10 %) required
a posterior release within the mean follow-up
period of 38 months [42].
In all situations it remains essential that the
child is treated in a holistic manner: many chil-
dren have associated knee contractures which
may influence the timing of treatment or its
A.J. Graydon and D.M. Eastwood
success, for example the Ponseti cast is usually
applied with the knee in 90 of flexion to
ensure that the foot position is maintained but
this is not always achievable in patients with
arthrogryposis. Similarly, the knee contracture
itself may require manipulation into extension
(rather than flexion). For the rigid flat foot defor-
mity or vertical talus similar concerns exist
regarding the reliability of a surgical correction
and the use of the reverse Ponseti technique. In
the review by Drummond et al. [36], the flatfoot
deformity required surgery less frequently as the
foot was plantigrade, stable and pain free. Aroojis
et al [43] believe that a vertical talus only occurs
in distal types of athrogryposis and does respond
to single-stage soft tissue correction: these
authors present Fig. 9 which demonstrates bilat-
eral vertical tali in a child with amyoplasia, both
feet were treated by the reverse Ponseti
technique.
Spine Surgery
The incidence of spinal deformity probably
varies with the exact aetiology of the
arthrogrypotic syndrome and may relate to the
severity of the muscle weakness. Typically,
patients with amyoplasia do not present with a
congenital scoliosis but the reported prevalence
[44, 45] for scoliosis during childhood and ado-
lescence is from 22.5 % to 66 %. There are no
comprehensive reviews of the natural history of
the scoliosis but most reported curves are struc-
tural thoracolumbar c-shaped neuromuscular
curves in appearance. They are often rigid and
severe and respond poorly to bracing regimes.
Most reports of scoliosis in this group of condi-
tions note that the deformity is associated with
hyperlordosis, a significant pelvic obliquity and
poor (or non-existing) walking ability.
Combined anterior-posterior spinal fusions
appear to give the best results and should be
considered if the curve is >30 particularly in
the non-ambulant patient [17, 46]. Extending
the fusion to the pelvis may not be indicated if
passive hip flexion is <90 as sitting posture may
Orthopaedic Management of Arthrogryposis Multiplex Congenita
be compromised [20, 47]. Amongst the distal
arthrogrypotic syndromes, scoliosis is much less
common and overall less severe: treatment is by
conventional means but the results may not be as
good as in non-syndromic conditions.
Peri-Operative Care
Accepting that the specific surgical indications
have been met, the arthrogrypotic child presents
certain other important issues for all members of
the surgical and rehabilitation team to consider
before informed consent can be obtained.
Co-Morbidities
Pre-existing malnutrition may be an unrecognized
issue in arthrogrypotic patients particularly the
young ones. Up to 50 % may have issues such as
poor enteral feeding secondary to their stiff
jaws and the relative immobility of their
tongue with failure to thrive and constipation
[17]. Chronic upper respiratory tract infections
are also more common, and these increase
the risk of post-operative complications such as
aspiration and atelectasis. Other issues may be
syndrome specific and dependant on the exact
aetiology of the joint contractures [48].
Specific Anaesthetic Issues
Several concerns affect the delivery of an anaes-
thetic in patients with arthrogryposis. Due to the
generalised nature of the condition both in terms
of number of body parts involved and the variety
of different tissues affected, venous access can be
limited. The muscle mass is significantly
decreased in affected children compared to their
peer group and this affects the volume of distri-
bution of many anaesthetic agents. Endotracheal
intubation can also be challenging given the
oropharyngeal dimensions of these patients and
the fact that the high cervical hypoplasia may be
associated with vertebral instability. The severity
4641
and number of the joint contractures may make it
difficult to position the patient appropriately on
the operating table, particularly if multiple
procedures are to be performed concurrently
and particular care must be taken to ensure that
pressure points are well-protected. Specifically
however, arthrogrypotic patients are at increased
risk of malignant hyperthermia. Although the
mechanism of this is poorly understood it proba-
bly relates to the intrinsic muscle abnormalities
of many of the distal arthrogryposes [48].
Rehabilitation Issues
Arthrogrypotic patients are typically smaller and
weigh less than age-matched controls. Neuro-
developmental studies have suggested that
although patients are typically of normal or
above normal intelligence, language skills may
not be age appropriate [17]. This has implications
for both pre-operative preparation and post-
operative rehabilitation in terms of ability to
comply with instructions and practical use of
the equipment required for rehabilitation. In addi-
tion the generalized nature of these disorders may
create its own mobility issues, for example upper
limb deformities may hinder the use of mobility
aids following lower limb surgery (Fig. 10). On
the positive side, these children are often very
well-motivated and show a natural ability to
learn substitution techniques and they may cope
surprisingly well with the rehabilitation process.
Post-operatively, it is imperative that children
have an appropriate and individualised
programme of physiotherapy and splinting
(Fig. 4). Bracing may be for function and/or for
maintenance of range of movement, therefore
there may be splints for daytime and for night
time use. Adherence to a bracing regime until
skeletal maturity reduces the risk of recurrent
contractures developing with growth. The recov-
ery of muscle activity after a return of passive
movement in a useful arc may take time and is a
good prognostic sign: it accounts for the increased
ambulatory ability in 4/7 children in the long term
follow up study by Fassier et al. [20].
4642
Fig. 10 Adapted walking frame for a child with amyoplasia
Complications
The Orthopaedic management of patients with
arthrogryposis in all its forms is subject to the
same set of general and local complications that
can affect any operative procedure. There are two
main pertinent issues to remember. The first is
that many surgical procedures, for example,
around the foot and the hip are associated with
post-operative stiffness and hence a reduced
range of movement. The second also relates to
the underlying pathology: whilst surgery can be
useful in obtaining a passive range of movement,
it is not so useful at restoring active movement.
Thus, although a flail joint may allow more
movement it may not necessarily improve
function. The use of an orthotic support to help
control movement may also make the limb too
heavy to move easily.
Overall, the greatest complication can be
disappointment for both the family and the sur-
geon when the surgical procedure does not
achieve its aims, and/or recurrent stiffness and
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
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
high in these children and often multi-level
surgery may be required particularly in the
lower limb: an experienced surgical team
with the appropriate back-up provides the best
environment for a good outcome.
References
1. OMIM. Online Mendelian Inheritance in Man. Johns
Hopkins University. http://www.ncbi.nlm.nih.gov/
sites/omim
2. Stern WG. Arthrogryposis multiplex congenita.
JAMA. 1923;81:1507.
3. Hall JG. Arthrogryposis multiplex congenital; etiol-
ogy, genetics, classification, diagnostic approach and
general aspects. J Pediatr Orthop B. 1997;6:15966.
4. Hall JG, Reed SD, Driscoll EP. Part 1. Amyoplasia:
a common sporadic condition with congenital contrac-
tures. Am J Med Genet. 1983;15:57190.
5. Bamshad M, Jorde LB, Carey JC. A revised and
extended classification of the distal arthrogryposes.
Am J Med Genet. 1996;65(4):27781.
6. Bamshad M, Van Heest A, Pleasure D.
Arthrogryposis: a review and update. J Bone Joint
Surg Am. 2009;91 Suppl 4:406.
Orthopaedic Management of Arthrogryposis Multiplex Congenita
7. Fahy MJ, Hall JG. A retrospective study of pregnancy
complications among 828 cases of arthrogryposis.
Genet Couns. 1990;1:311.
8. Sung SS, Brassington AM, Grannat K, Rutherford A,
Whitby FG, Krakowiak PA, Jorde LB, Carey JC,
Bamshad M. Mutations in genes encoding fast-twitch
contractile proteins cause distal arthrogryposis syn-
dromes. Am J Hum Genet. 2003;72:68190.
9. Narkis G, Landau D, Manor E, et al. Genetics of
arthrogryposis; linkage analysis approach. Clin
Orthop. 2007;456:305.
10. Morgan NV, Brueton LA, Cox P, Greally MT, Tolmie J,
Pasha S, Aligianis IA, van Bokhoven H, Marton T, Al-
Gazali L, Morton JE, Oley C, Johnson CA, Trembath
RC, Brunner HG, Maher ER. Mutations in the embry-
onal subunit of the acetycholine receptor (CHRNG)
cause lethal and Escobar variants of multiple pterygium
syndrome. Am J Hum Genet. 2006;79:3905.
11. Polizzi A, Huson SM, Vincent A. Teratogen update:
maternal myasthenia gravis as a cause of congenital
arthrogryposis. Teratology. 2000;62:33241.
12. Rankin J, Auer-Grumbach M, Bagg W, Colclough K,
Nguyen TD, Fenton-May J, Hattersley A, Hudson J,
Jardine P, Josifova D, Longman C, McWilliam R,
Owen K, Walker M, Wehnert M, Ellard S. Extreme
phenotypic diversity and nonpenetrance in families
with the LMNA gene mutation R644C. Am J Med
Genet A. 2008;146:153042.
13. Fuller DJ. Immobilisation of fetal joints: a cause of
progressive prenatal deformity. J Bone Joint Surg Br.
1975;57:115.
14. Moessinger AC. Fetal akinesia deformation sequence:
an animal model. Pediatrics. 1983;72(6):85763.
15. Swinyard CA, Bleck EE. The etiology of
arthrogryposis. Clin Orthop. 1984;1985:1529.
16. Sells JM, Jaffe KM, Hall JG. Amyoplasia, the most
common type of arthrogryposis: the potential for good
outcome. Pediatrics. 1996;97:22531.
17. Staheli LT, Hall JG, Jaffe K, et al. Arthrogryposis: a text
atlas. New York: Cambridge Univeristy Press; 1998.
18. Dillon ER, Bjornson KF, Jaffe KM, Hall JG, Song KJ.
Ambulatory activity in youth with arthrogryposis:
a cohort study. J Pediatr Orthop. 2009;29:2147.
19. Darin N, Kimber E, Kroksmark AK, Tulinius M. Mul-
tiple congenital contractures: birth prevalence, etiol-
ogy and outcome. J Pediatr. 2002;140:617.
20. Fassier A, Wicart P, Dubousset J, Seringe R.
Arthrogryposis muliplex congenita. Long-term fol-
low-up from birth until skeletal maturity. J Child
Orthop. 2009;3:38390.
21. Bevan W, Hall J, Bamshad M, Staheli L, Jaffe K, Song
K. Arthrogryposis multiplex congenita (amyoplasia):
an orthopaedic perspective. J Pediatr Orthop.
2007;27(5):594600.
22. Van Heest A, James MA, Lewica A, Anderson KA.
Posterior elbow capsulotomy with triceps lengthening
for treatment of elbow extension contracture in chil-
dren with arthrogryposis. J Bone Joint Surg Am.
2008;90:151723.
4643
23. Van Hest A, Waters PM, Simmons BP. Surgical treat-
ment of arthrogryposis of the elbow. J Hand Surg
[Am]. 1998;23:106370.
24. Ezaki M. Treatment of the upper limb in the child with
arthrogryposis. Hand Clin. 2000;16:70311.
25. Gogola GR, Ezaki M, Oishi SN, Gharbaoui I, Bennet
JB. Long head of the triceps muscle transfer for active
elbow flexion in arthrogryposis. Tech Hand Up
Extrem Surg. 2010;14:1214.
26. Mennen U, van Heest A, Ezaki MB, et al.
Arthrogryposis multiplex congenita. J Hand Surg
[Br]. 2005;30(5):46874.
27. Szoke G, Staheli LT, Jaffe K, et al. Medial-approach
open reduction of hip dislocation in amyoplasia-type
arthrogryposis. J Pediatr Orthop. 1996;16:12730.
28. Yau PWP, Chow W, Li YH, et al. Twenty-five
year follow-up of hip problems in arthrogryposis
multiplex congenital. J Pediatr Orthop. 2002;22:
35963.
29. Akazawa H, Oda K, Mitani S, et al. Surgical manage-
ment of hip dislocation in children with arthrogryposis
multiplex congenital. J Bone Joint Surg Br.
1998;80(4):63640.
30. Murray C, Fixsen JA. Management of knee deformity in
classical arthrogryposis multiplex congenital (amyoplasia
congenital). J Pediatr Orthop B. 1997;6:18691.
31. DelBello DA, Watts HG. Distal femoral extension
osteotomy for knee flexion contracture in patients
with arthrogryposis. J Pediatr Orthop. 1996;6:1927.
32. Brunner R, Hefti F, Tgetgel JD. Arthrogrypotic joint
contracture at the knee and foot; correction with
a circular frame. J Pediatr Orthop B. 1997;6:1927.
33. Klatt JK, Stevens PM. Guided growth for fixed knee
flexion deformity. J Pediatr Orthop. 2008;28(6):
62631.
34. Roy DR, Crawford AH. Percutaneous quadriceps
recession: a technique for management of congenital
hyperextension deformities of the knee in the neonate.
J Pediatr Orthop. 1989;9:7179.
35. Niki H, Staheli L, Mosca VS. Management of clubfoot
deformity in amyoplasia. J Pediatr Orthop.
1997;17:8037.
36. Drummond DS, Cruess RL. The management of the
foot and ankle in arthrogryposis congenita. J Bone
Joint Surg Br. 1978;60(B):969.
37. Widman RF, Do TT, Burke SW. Radical soft tissue
release of the arthrogrypotic clubfoot. J Pediatr Orthop
B. 2005;14(2):1115.
38. DSouza H, Aroojis A, Chawara GS. Talectomy in
arthrogryposis: analysis of results. J Pediatr Orthop.
1998;18:7604.
39. Choi IH, Yang MS, Chung CY, et al. The treatment of
recurrent arthrogrypotic clubfoot in children by the
Ilizarov method. J Bone Joint Surg Br. 2001;83B:
7317.
40. Boehm S, Limpaphayom N, Alaee F, Sinclair MF,
Dobbs MB. Early results of the Ponseti method for
the treatment of clubfoot in distal arthrogryposis.
J Bone Joint Surg Am. 2008;90(7):15017.
4644
41. Morcuende JA, Dobbs MB, Frick SL. Results of the
Ponseti method in clubfoot associated with
arthrogryposis. Iowa Orthop J. 2008;28:226.
42. Van Bosse HJ, Marangoz S, Lehman WB, Sala DA.
Correction of arthrogrypotic clubfoot with a modified
Ponseti technique. Clin Orthop Relat Res.
2009;467:128393.
43. Aroojis AJ, King MM, Donohoe M, Riddle EC,
Kumar SJ. Congenital vertiacla talus in arthrogryposis
and other contractural syndromes. Clin Orthop Relat
Res. 2005;434:2632.
44. Sarwark JF, MacEwen GD, Scott CI. Amyoplasia (a
common form of arthrogryposis). J Bone Joint Surg
Am. 1990;72:4659.
A.J. Graydon and D.M. Eastwood
45. Drummond DS, MacKenzie DA. Scoliosis in
arthrogryposis multiplex congenita. Spine. 1978;3:
14651.
46. Daher YH, Lonstein JE, Winter RB, et al. Spinal
deformities in patients with arthrogryposis. Spine.
1985;10(7):60913.
47. Yingsakmongkol W, Kumar SJ. Scoliosis in
arthrogryposis multiplex congenita. J Pediatr Orthop.
2000;20:65661.
48. Martin S, Tobias JD. Perioperative care of the child with
arthrogryposis. Paediatr Anaesth. 2006;16(1):317.
49. Beals RK. The distal arthrogryposes; a new classifica-
tion of peripheral contractures. Clin Orthop. 2005;
435:20310.