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.This page explains about 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.

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.
• 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.

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.

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.

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.

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.

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.

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.

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.

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.

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:

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.

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. 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.

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).

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.

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.