541458

research-article2014
POI0010.1177/0309364614541458Prosthetics and Orthotics International X(X)Gür et al.

INTERNATIONAL
SOCIETY FOR PROSTHETICS
AND ORTHOTICS

Case Report

Prosthetics and Orthotics International

One-year follow-up study of serial orthotic 2016, Vol. 40(3) 388­–393

© The International Society for
Prosthetics and Orthotics 2014
treatment in two cases with arthrogrypotic Reprints and permissions:
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syndromes who have bilateral knee flexion DOI: 10.1177/0309364614541458
poi.sagepub.com

contractures

Gozde Gür1, Suat Erel2, Yavuz Yakut1, Cemalettin Aksoy1 and

Fatma Uygur1

Abstract
Background: The aim of this pilot study was to investigate the effectiveness of serial splinting in two children with bilateral
knee flexion contractures due to arthrogrypotic syndrome.
Case description and methods: We evaluated the infants’ passive knee extension limitation and motor development levels.
Serial orthotic treatment was applied to decrease bilateral knee flexion contractures in the knees of the subjects. The
follow-up period was up to 1 year.
Findings and outcomes: At the end of serial orthotic treatment, improvement in bilateral passive extension limitation (for
the first case, the increase in passive range of extension was approximately 75°, for the second case it was 45°) was
achieved in both cases.
Conclusion: We believe that serial orthotic intervention is effective in patients with arthrogrypotic syndrome at the
preoperative period or in patients who cannot be operated on. Further studies are needed for evaluation of effectiveness
of this method.

Clinical relevance
Our pilot study aimed to investigate the effectiveness of serial orthotic treatment in knee contractures due to
arthrogrypotic syndrome in two infants which showed an improvement in range of extension.

Keywords
Arthrogrypotic syndromes, knee flexion contractures, serial orthotic treatment

Date received: 1 August 2013; accepted: 2 April 2014

Background
Arthrogryposis is a non-progressive syndrome of multiple
joint contractures that are present at birth. The etiology of
the syndrome remains uncertain.1 Although a similar form
was described by Otto in 1841, the term “arthrogrypotic
syndrome” was used first by Stern in 1923. The incidence
of arthrogryposis is one in 5000–10,000 live births.2
The most common cause of arthrogrypotic syndrome is
lack of fetal movement. It is thought that the lack of fetal
movement and formation of extra connective tissue around
joints causes contractures. Contractures in arthrogryposis
can be seen either on its own or as part of the syndrome as
well as neurological, myopathic, and systemic disease.3
Joint contractures in arthrogrypotic syndromes are usu-
ally symmetrical and divided into three groups. These are
as follows: (1) with limb involvement only, (2) with limb
involvement and other malformation or anomalies, and (3)
with limb involvement and central nervous system dys-
function or mental retardation.1,3
Most common deformities in extremities are pes equi-
novarus, calcaneovalgus, vertical talus, flexion and ulnar
1Physical Therapy and Rehabilitation Department, Faculty of Health
Sciences, Hacettepe University, Ankara, Turkey
2School of Physical Therapy and Rehabilitation, Pamukkale University,
Denizli, Turkey
Corresponding author:
Gozde Gür, Physical Therapy and Rehabilitation Department, Faculty
of Health Sciences, Hacettepe University, 06100 Samanpazari, Ankara,
Turkey.
Email: gosdegr@hotmail.com
Gür et al. 389
deviation of the wrist. In the knee and elbow, either flexion
or extension contractures can be seen.4,5 Owing to the fact
that developing deformities impair the child’s motor abil-
ity, it is important to intervene in a timely manner in order
to minimize these deformities.1
Case description and methods
This study was carried out on two consecutive infants who
had bilateral knee contractures due to arthrogrypotic syn-
drome. Other problems accompanying the diagnoses,
based on the motor development levels according to Gross
Motor Function Classification System (GMFCS), and
demographic characteristics were recorded. Also, bilateral
passive knee extension limitations were measured with
universal goniometry by the same physical therapist (PT)
experienced in such measurements at each session.
Case 1
The first was a 7-month-old infant with arthrogrypotic
syndrome who had hand and foot deformities and various
problems such as Young–Simpson syndrome, congenital
hypothyroid, congenital heart disease, severe mental retar-
dation, hypotonia, facial dysmorphism, and postnatal
growth deficiency. She could control her head during sup-
ported sitting for only a few seconds. Her motor develop-
ment level according to GMFCS was 5. Physical
examination showed hyper mobility of upper extremity
joints, bilateral pes planovalgus, and bilateral knee flexion
contractures.
Case 2
Our second case was a 6-month-old infant with arthro-
grypotic syndrome in addition to corpus callosum agene-
sis, mental motor retardation, and optic defect. In the prone
position, she could turn her head bilaterally. Motor devel-
opment level was 5 according to GMFCS. There was pes
equinovarus on the right side and pes planovalgus on the
left side; also bilateral knee flexion contractures were
observed.
Assessment protocol
The universal (standard) goniometry has been used by
physiotherapists to measure joint range of motion and
decide limitation. The intertester and intratester reliability
of the universal goniometry were found to be high,6 and
also its validity for the knee is established.7 During meas-
urements, the center of the fulcrum of the goniometry was
placed over the lateral condyle of the femur. The proximal
arm was aligned with the lateral midline of the femur,
using the greater trochanter for reference. The distal arm
was aligned with the midline of the fibula using the lateral
malleolus and fibular head for reference and the maximum
degree of extension attained was measured in degrees.8 All
measures of knee range of motion were carried out by
experienced physiotherapists who were also co-authors
(G.G. and S.E.). For the measurement to be precise, the
infants were held in the prone position at the edge of a
treatment table and supported at the hip by S.E. while G.G.
passively brought the infants calves from 90° flexion to the
maximum degree of extension attained and carried out the
measurement as described.
GMFCS divides subgroups according to individual
functional levels, thereby analyzing the child’s ability in
defined age groups. According to this system, the first
level includes children who walk without restrictions; sec-
ond level includes children who walk without assistive
devices with limitations in walking outdoors and in the
community; third level includes children who walk with
assistive devices with limitations in walking outdoors and
in the community; fourth level includes children who have
self-mobility with limitations, children are transported or
use powered mobility outdoors and in the community; and
in the fifth level, self-mobility is severely limited, even
with the use of assistive technology. GMFCS has been
shown to be a valid and reliable method in determining
children’s ambulatory ability.9
Current treatment options
In the treatment of joint contractures, physiotherapy and
splinting are important as well as orthopedic surgery.10
Despite serial casting being a common method used for
hand and foot deformities, its effectiveness in the long
term is controversial.4,5 In most cases, orthopedic surgery
is required in the long term. However, the severity of the
contracture affects the success of surgery.
Contractures in arthrogrypotic syndromes are structural
contractures that include cutaneous along with subcutane-
ous and muscle tissue, and serial casting has been shown to
be helpful.3,11
Serial casting is a method commonly used to improve
contractures and maximize range of movement.4 This inter-
vention can be preferred especially in very young children
to create tension, which can be tolerated for long periods.
However, for serial casting method can also cause atrophy
and shortening in the antagonist muscle.12 Therefore, the
patients should be carefully supervised. While in serial cast-
ing to remove the cast is impossible, in serial splinting you
can take off the splint whenever you want for care and exer-
cises.12,13 Although stretching of joint contractures by means
of serial splinting is advocated in many textbooks, we have
not come across studies explaining the application proce-
dures in detail and giving numerical values attained by
means of this application. Serial splinting is not an innova-
tive procedure; however, its use for treating flexion contrac-
tures in the arthrogrypotic knee joint has not been
investigated. Physiotherapy including exercises and activity
training is also important.
390 Prosthetics and Orthotics International 40(3)
Figure 1.  Positive cast model.
The aim of this study was to present our serial splinting
protocol and to investigate its effectiveness in two children
with bilateral knee flexion contractures due to arthro-
grypotic syndrome.
Our treatment protocol
Serial orthotic treatment was applied to infants to decrease
bilateral knee flexion contractures. Although serial
orthoses have been used to treat contractures and deformi-
ties, we did not come across any study in which serial
orthotics was used to treat knee flexion contractures of
arthrogrypotic syndromes in such young children. Before
each intervention, cast measurement was taken in the max-
imum passive extension degree attained. After required
corrections were made on the positive model, a static knee
orthosis from the inguinal area to proximal of malleolar
area was molded on this positive cast model by using low-
density thermoplastic material that was heated in hot water
at 80°C–90°C for 5–7 min (Figures 1 and 2). Orfit of 3 mm
was used. This material was chosen because it is nonaller-
genic and easily tolerated by little children. Unlike the
plaster cast, parents can take off this splint whenever they
want for general care, hygiene, and exercises. The knee
orthosis was renewed by taking measurements at approxi-
mately 15- to 30-day intervals. If knee extension limitation
was the same following two consecutive measurements,
serial orthotic treatment was terminated.
The infants first wore their orthoses under the supervi-
sion of the physiotherapist. After the physiotherapist was
convinced that there were no problems due to compromise
of circulation, instructions were given to the family about
donning and doffing the orthosis and how to care for the
orthosis and how to be aware of any circulation problems
that could occur. The infants’ parents were advised to put
on the orthosis for 15–18 h a day and to take off the ortho-
sis when the child was active during daytime. At each
Figure 2.  Static knee orthosis.
control, the range of passive knee extension was measured
and recorded by the same physiotherapists. The families
were asked to write down how many hours the orthosis
was worn each day. This study was approved by the
Experimental Ethics Committee of the University of
Hacettepe.
Findings and outcomes
Case 1
Serial splinting was started in the left knee. When a
decrease in limitation was observed, serial splinting was
also started on the other side. Increase in range of move-
ment with serial orthotic treatment was 40° for the left
knee, 35° for the right knee, and the child gained active
head control in all positions and began to cruise. Change in
knee extension limitation for both knees with each orthotic
intervention session and average orthotic usage time is
shown in Table 1. Right and left knee extension limitation
alteration with time is displayed in Figure 3.
Case 2
Serial splinting was started with the left knee, and when a
decrease in limitation was observed, serial splinting for
the other side was added. The increase in range of move-
ment with serial orthotic treatment was 25° for the left
knee and 20° for the right one. Change in knee extension
limitation with the orthotic intervention session and aver-
age orthotic usage time is shown in Table 2. Right and left
knee extension limitation changes with time are displayed
in Figure 4.
At the end of serial orthotic treatment, improvement in
bilateral passive extension limitation was achieved in both
the cases. For the first case, the increase in passive range of
extension was approximately 40°, for the second case it
Gür et al. 391

Table 1.  Change in knee extension limitation with orthotic intervention session and average daily orthotic usage time (case 1).

Evaluation Orthotic application side Right knee Left knee passive Average daily
passive extension extension orthotic usage
limitation (°) limitation (°) time (h)
First evaluation First session left knee orthosis 45 50 *
Second evaluation Second session bilateral knee orthosis 45 34 18
Third evaluation Third session bilateral knee orthosis 34 28 18
Fourth evaluation Fourth session bilateral knee orthosis 28 20 18
Fifth evaluation Fifth session bilateral knee orthosis 26 18 15
Sixth evaluation Sixth session bilateral knee orthosis 20 18 16
Seventh evaluation Continue with the same orthosis 20 18 16
Eighth evaluation Seventh session bilateral knee orthosis 10 10 16
Ninth evaluation Continue with the same orthosis 10 10 16
Tenth evaluation Continue with the same orthosis 10 10 6–8

*Orthotic use not yet started.

Figure 3.  Right and left knee extension limitation alteration with time (case 1).

Table 2.  Change in knee extension limitation with orthotic intervention session and average daily orthotic usage time (case 2).

Evaluation Orthotic application side Right knee Left knee passive Average daily
passive extension extension orthotic usage
limitation (°) limitation (°) time (h)
First evaluation First session left knee orthosis 40 45 *
Second evaluation Second session bilateral knee orthosis 40 30 18
Third evaluation Third session bilateral knee orthosis 28 20 18
Fourth evaluation Continue with the same orthosis 20 20 18
Fifth evaluation Continue with the same orthosis 20 20 10
Sixth evaluation Continue with the same orthosis 20 20 6–8

*Orthotic use not yet started.

was 25°. When knee extension limitation was the same at
three consecutive measurements, the value was accepted
as the maximum degree of passive extension range, and
serial orthotic treatment was terminated. However, to pro-
tect the range that was attained, the parents were advised to
continue using the orthosis during naptime.
Discussion
Although serial orthotic treatment is not an innovation, our
study is the first study which investigates the effectiveness
of serial orthotic treatment in knee contractures in two
cases with arthrogrypotic syndrome.
392 Prosthetics and Orthotics International 40(3)
Figure 4.  Right and left knee extension limitation alteration with time (case 2).
The main goal of initial treatment in arthrogrypotic syn-
drome is to protect range of joint movement and to prevent
contracture development during growth.3 In the literature,
the timing of surgical and conservative treatment is contro-
versial. In case conservative management fails to treat the
flexion contracture, surgery is recommended.10 Most phy-
sicians agree that surgery should be recommended when
conservative management fails to treat the contrac-
tures.10,14,15 However, some physicians believe that time
should not be lost by conservative treatment and that the
initial treatment should be surgical.
In conservative treatment of the lower extremity, pas-
sive stretching, serial casting,3,10 and various foot and
ankle orthoses are used.16 In the long period, surgical inter-
vention seems to be inevitable.10
With multiple orthopedic and rehabilitative interven-
tions, the ability to walk and perform activities of daily
living has been reported to be as high as 85%. In adult life,
the majority of individuals need assistance with daily
living.3
Studies related with correction of knee flexion deform-
ity usually compare results of surgical intervention.10,14,17
Studies that investigate the effectiveness of conservative
treatment in improving knee contractures are sparse.15,17
Choice of treatment method depends on degree of deform-
ity and level of ambulation.18 A study showed that nonop-
erative treatment (including serial casting and manipulation)
had good results up to 62% in extension contractures of the
knee.17 However, in another study carried out on 43
patients, a total of 78 knees with arthrogrypotic syndrome,
only 6° increase was achieved in knee range of motion with
nonoperative treatment.15 In arthrogrypotic syndromes,
knee joint involvement has been reported in 70%, with
flexion contractures being most common, followed by
extension contractures.16 There are some studies in which
the incidence of flexion contractures of the knee is as high
as 90%,10 and according to most researchers, conservative
management of flexion contractures is more difficult than
extension contractures.14,15,17 In a study by Hosny and
Fadel14, all patients with arthrogryposis were treated ini-
tially by physiotherapy and splintage which was success-
ful in all except one patient in the extended-knee group,
whereas only 7 of 26 knees responded to physiotherapy
and splintage in the flexed-knee group.14 In our study, the
decrease in knee flexion contracture was up to 80% in both
the cases. Also, our first subject could not achieve a long
sitting position before treatment; with increase in knee
extension, she could sit in the long sitting position and
began to cruise.
In patients with arthrogrypotic syndrome, muscles do
not have enough growth potential, and bone growth will
cause tightness in the muscle and consequently recur-
rence of the deformity.17,18 Continuation of the use of
orthoses and splints even after contractures improve is
required because of high recurrence rates.10 Continuity
of using orthosis is also important for surgical interven-
tion. By using an orthosis for adequate periods and
maintaining deformities at minimal degrees, less aggres-
sive procedures can be realized.14 In our study, the first
case continued to use her orthosis 4–6 h a day for
6 months, and after 1 year, the range of motion in the
knee joint was maintained. However in the second case
who gave up using the orthosis, there was a recurrence in
extension limitation.
Serial orthotic treatment has been reported to be costly
and time consuming.13 Yet, serial orthotic treatment is
preferable to serial casting because it is possible to take off
the orthosis and control the extremity, it provides active
participation of the parents by allowing them to do exer-
cises which will facilitate motor development, and it is
easier to tolerate by infants and compromise of circulation
is minimized.
Gür et al. 393
Conclusion
Our study was the first study to investigate the effective-
ness of serial orthotic treatment in knee contractures due to
arthrogrypotic syndrome in two infants. With orthotic
treatment, case 1 gained 75° and case 2 45° in knee range
of motion. It is not possible to generalize the results; how-
ever, our results imply that serial orthotic intervention can
be preferred in patients at the preoperative period or in
patients who cannot be operated on. Close collaboration
with the doctor who has undertaken the treatment of chil-
dren with arthrogrypotic syndrome is a prerequisite of suc-
cessful treatment.
Author contribution
All authors contributed equally in the preparation of this article.
Declaration of conflicting interests
The authors declare that there is no conflict of interest.
Funding
This research received no specific grant from any funding agency
in the public, commercial or not-for-profit sectors.
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