Radial club hand - Great Ormond Street Hospital

Radial club hand
Information for families
Great Ormond Street Hospital
for Children NHS Foundation Trust
2
clavicle
scapula
humerus
This information sheet
explains about the hand
anomaly called radial
club hand, what causes it,
how common it is, what
functional problems your
child might have and
how it can be treated.
It also explains what to
expect when your child
comes to Great Ormond
Street Hospital (GOSH) for
assessment and treatment.
radius
ulna
Radial club hand: Note missing radius and
hand turned inwards towards the body
Normal hand and arm
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What is radial club hand?
Radial club hand is a congenital
(present at birth) hand anomaly
where the radius bone in the arm is
missing or underdeveloped, causing
the hand to be bent towards the
body (radially deviated). It is also
known as radial ray deficiency or
anomaly. One in 75,000 children is
born with radial club hand.
Radial club hand can affect all the
structures on the radial side of the
upper limb, including the bones and
soft tissues (muscles, tendons, joints,
nerves and blood vessels). The wrist
is always affected to varying degrees.
The thumb may be absent or smaller
than usual with poor joints and
missing muscles and/or tendons.
Radial club hand is classified into
four types from mild to severe, but
within each type the degree of
severity can vary enormously.
The four types are as follows:
Type I – This is at the milder end
of the spectrum, with the radius
being only a little shorter than
normal and the hand mildly radially
deviated at the wrist.
Type II – The radius is much smaller
than usual with unusual growth
plates. The hand is mildly to
moderately radially deviated.
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Type III – A large part of the
radius is missing and the hand is
moderately to severely radially
deviated. The wrist lacks support
and the ulna is thickened and often
bowed. The forearm is short.
Type IV – The radius is completely
absent and the hand is severely
radially deviated and the forearm is
short. This is regarded as the most
common type of radial club hand.
In all types, the thumb may be
smaller than usual (hypoplastic)
or absent.
Your doctor will examine your
child closely and look at x-rays of
your child’s arm to work out the
classification of radial club hand.
This will influence the treatment
options available.
Type IV Radial Club Hand, post-surgery
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What causes radial club hand?
The majority of cases appear ‘out
of the blue’ (sporadically) with no
known cause. Radial club hand
can affect one arm (unilateral)
or both arms (bilateral). The
condition affects both arms in
about half of children born with
radial club hand. It occurs very
early in pregnancy between day 28
and 52. In very rare circumstances,
exposure to some substances
during pregnancy, for instance,
thalidomide, can cause problems
with the radial bones. Radial club
hand is only occasionally diagnosed
during routine ultrasound scans
before birth.
Unilateral radial club hand is not
generally associated with any
other problems and is less likely
to be passed on genetically.
Bilateral radial club hand is more
typically associated with other
problems, sometimes as part of
a ‘syndrome’ or collection of
symptoms often seen together.
Syndromes that usually feature
radial club hand include Holt-Oram
syndrome, TAR syndrome, Fanconi’s
anaemia and VATER/VACTERL
syndrome, some of which may have
a genetic component.
Your doctor will examine your child
closely to discover if they have
any other features associated with
syndromes. They may also refer you
to see a geneticist to advise about
further investigations for your child
and to plan future pregnancies.
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What is hand function like for children
with radial club hand?
The level of hand function will
depend on the severity of the
radial club hand and whether
one or both hands are affected.
Radial club hand with wrist
deviation and forearm shortening
is often associated with thumb
underdevelopment or absence, and
stiffness of the finger joints with
difficulty bending the joints.
Corrective surgery aims to improve
the appearance and function, but
function is always affected for the
child with a moderate to severe
radial club hand. There will be
reduced movement of the fingers
with poor pinch and grasp, reduced
strength and ability to position the
hand, for example to turn the palm
upwards.
A child with a severe, unilateral
deformity may be unable to
manage a few everyday tasks
which involve pinch or grasp with
both hands, for example, tying
shoe laces, doing up a zip, tying
hair into a ponytail, using a knife
and fork together and putting
on socks or tights. Some of these
tasks are managed by adapting
clothing or equipment or having a
little assistance.
There may be other tasks usually
performed with two hands which
the child learns to manage very
effectively with one hand. For
example, putting toothpaste on
the brush and doing up buttons.
These tasks may take your child
a little more time compared to
other children.
Other tasks may be managed
slowly and/or with difficulty using
both hands, for example, riding
a bike, where reach and grip is
affected.
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Type IV radial club
hand, post-surgery
demonstrating
functional use
However, most children find a way
around their problems. If your child
has unilateral radial club hand,
they may use the other hand to
carry out tasks. If both arms are
affected, your child may find ways
of picking up things with help by
gripping them to their body, using
both hands together or gripping
items between the fingers. Some
equipment can be adapted to
make activities easier or safer, for
instance bicycle handles can be
adapted, as can cutlery, clothing
and writing materials. You may
find difficulties at different stages
of your child’s development, but
generally, children are very good at
adapting to their condition and can
usually achieve most tasks.
Hand function can also be affected
by a child’s and their family’s
attitude towards the hand. If a
young child is very self-conscious
about their different looking limb,
they may hide it and not want
to use it. The less the affected
arm and hand is used, the less
functional it will be. From very
early on, the baby with radial club
hand should be encouraged to use
the affected arm(s) and hand(s).
If only one arm is affected place
toys on the affected side so that
they have to reach to grasp them
rather than always relying on the
unaffected arm.
Generally, most children manage
extremely well with functional
activities of daily living and they
find their own way of managing
their tasks to be fully independent.
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Dealing with feelings
It can be a big shock and very
distressing when a baby is born
with a hand anomaly. As a new
parent, you may be conscious of
your baby’s different hand, how
other people may look at your
child and the questions asked. This
is very normal and it is important
to address these feelings in order
to be able to develop a positive
attitude to your child’s hand
differences and over time, help
your child feel equally positive and
confident about their hand. We
are able to provide help with these
matters or put you in touch with
organisations that help and support
children and their families with
hand deficiencies.
How can radial club
hand be treated?
There are various options for
treatment and the doctor will
explain which are most suitable
for your child. This decision about
suitable treatments is influenced
by the severity of your child’s
radial club hand and their general
health including other symptoms.
It also depends on you and your
family’s feelings about treatment,
as some options involve a number
of operations and quite involved
care at home, which will require
determination from all involved.
The following options may be
available for your child.
Non-surgical hand
therapy and splints
Your child will be helped to
maximise their functional potential
through advice, therapeutic
exercise and splinting. Non-surgical
treatment with stretches and splints
is usually required in all types of
radial club hand.
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In mild cases, early in infancy,
you will be shown a series of
stretching exercises to carry out on
your child’s wrist with the aim of
increasing the range of movement.
These exercises are done every day.
Sometimes there is no stiffness in
the wrist but a tendency for the
wrist to rest in the radial deviation
and then a night splint may be
provided to keep the wrist in a
straight position.
In more severe types of radial club
hand, stretches and splints are
used. These are used before surgery
to stretch the soft tissues. Splinting
is also used after surgery to help
maintain the corrected wrist
position and protect the site of the
operation from knocks and bumps.
Type IV radial club hand, pre-op splint
Surgery
A series of operations is often
recommended to those children
with type ll, III and IV radial club
hand. Surgery tends to take place
in phases. This enables your child
to recover from one operation
and post-operative care before
moving on to the next. There are
also some operations that are best
done when a child is at a certain
age. Your doctor may suggest the
following operations:
Distracting the soft tissues of the
wrist with an external fixator
This aims to correct the position of
the wrist and is normally planned
for when your child is walking,
usually at around two years of
age. There are two parts to this
procedure. The first part involves
surgery to attach an external
fixator (a metal bar or frame) to
your child’s forearm and hand
bones. After the operation, the
soft tissues are gradually stretched
– this process is called distraction.
The two pieces of the external
fixator are gradually moved apart
to straighten the wrist, usually by a
millimetre or so each day.
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Repositioning the hand
Once the distraction phase has
finished, the fixator needs to remain
in place for several more weeks
while the bone grows stronger and
the tissues remain stretched.
External Fixator on Type IV Radial Club Hand,
and Post-surgery splint
During this time, you will be
expected to help clean where the
pins come through the skin and to
perform the distraction. You will
be supported and trained in how
to do this and you will need to be
seen regularly in the hospital to
check that all is going as planned.
The process of distraction is lengthy
and demands commitment from
you and your child. The usual time
that the distractor is on the arm is
between eight and twelve weeks.
This is an operation to remove
the fixator and maintain the
new position of the hand sitting
on the end of the ulna bone.
This will either be achieved by a
tendon transfer for a radialisation
operation where movement is
preserved, or a tendon transfer and
bone fixation for a centralisation
operation, where most of the wrist
movement is lost.
After the operation, the wrist is
held in position using K-wires for
a number of months. The wires
extend outside the bones but are
buried under the skin and your
child may be able to feel them.
Once the K-wires have been
removed, the arm and hand need
continuing support and protection
from a splint.
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Improving thumb function
Once the wrist surgery is complete,
the surgeon will focus on improving
thumb function. If the thumb is
present but weak, surgery can be
undertaken to stabilise any unstable
joints and strengthen the thumb by
taking a tendon or a muscle from
elsewhere in the hand. If the thumb
is absent, the index finger on that
hand may be moved to the thumb
position (pollicisation).
Type III radial club hand with absent thumb
before treatment (Right hand)
Type II radial club hand with absent thumb following treatment with pollicisation (Left hand)
Forearm lengthening
Most children with radial club hand
have short forearms in the limbs
affected. There are operations
that can be used to lengthen the
forearm but these are complex
and rarely produce forearms of the
same length. If surgery is possible,
this tends to happen during mid to
late adolescence.
What is the outlook
for children with
radial club hand?
The outlook depends very much on
the child’s original condition and
the degree of deficiency present
in the hand. For children with mild
radial club hand, surgery may not
be needed and they adapt well to
any difficulties. Children with more
severe radial club hand benefit
from surgery in the vast majority
of cases, wrist motion will never be
as full as the unaffected arm, but
most children adapt to carry out
everyday tasks and activities.
The radial deviation can recur as
the child grows older and may
require further surgery. For this
reason, all children require follow
up until adulthood when growth
has finished and the arm and hand
are stable.
Notes
Further information about radial club hand
If your child has radial club hand and you would like to know more about the condition
and how it can be treated surgically, you will need a referral to the Congenital Hand
Anomaly team. We can accept referrals from your local paediatrician or another
consultant. The Congenital Hand Anomaly team consists of the hand surgeon, clinical
nurse specialist, occupational therapist, physiotherapist and clinical psychologist.
You can also find out more about radial club hand by contacting the parent support
group REACH (Association for Children with Hand or Arm Deficiency). Visit their
website at www.reach.org.uk or telephone them on 0845 130 6225.
© GOSH NHS Foundation Trust September 2013
Ref: 2013F0962
Compiled by the Congenital Hand Anomaly team
in collaboration with the Child and Family Information Group
Photography by UCL Medical Illustration Services and appear in this booklet with the
consent of the child and parent, and must not be reproduced for any other purpose.
Great Ormond Street Hospital for Children NHS Foundation Trust
Great Ormond Street
London WC1N 3JH
www.gosh.nhs.uk