Syndactyly

Syndactyly means 'joined digits' and may involve webbing of the skin, or include fusion of the underlying bones. This may be along part or the whole length of the finger. It is the second most common congenital hand problem and occurs in around 1 in 1,000 births.

Hand development occurs in the early stages of pregnancy and the fingers separate in the ninth week. Syndactyly happens when two or more fingers fail to completely separate during development.

Sometimes syndactyly occurs by chance or it may be inherited. In rare circumstances, it may occur along with other signs as part of a syndrome (collection of signs).

Syndactyly can occur unilaterally (on one hand only) or bilaterally (affecting both hands), and can affect two or more fingers. Occasionally it may be associated with extra digits or other abnormalities in the hands.

Syndactyly can also affect the toes, involving webbing of the skin or fusion of the underlying bones along part or the whole length of the toe.

It is treated surgically under a general anaesthetic. Surgery is usually only possible to one side of a finger at a time, as any damage to the blood supply on both sides could result in the loss of the finger. So, if the child has syndactyly of three or more digits next to each other, they will need more than one operation.

Surgery is the only option to separate the digits. The aim of the operation is to improve function (how well the child can use their hand) and sometimes appearance (how the child’s hand looks). The surgeon will advise if the benefits to the child are likely to be functional, cosmetic or both.

Occasionally, some children could manage without treatment. Correction of toe syndactyly is usually for cosmetic rather than functional reasons.

Both the parent and the child will need to come to a pre-admission appointment shortly before the operation is scheduled. The purpose of this is to check that the child is well enough for the operation. It is also an opportunity to meet the surgeon again and ask any questions they might have.

The medical team provides written information about the care the child will need after the operation. They might request further X-rays or clinical photographs. At this appointment theywill ask the parent to give permission for the operation by signing a consent form.

On the day of the operation, parent and child should go straight to Puffin Ward, which is the same-day admission unit at Great Ormond Street Hospital (GOSH). The nurses will check that the child is well and ask the parent to complete some forms if they have not been filled in previously.

When the operating theatre is ready, parent and child will be collected and taken there by a member of staff. The parent is able to stay with the child until they have had the general anaesthetic, and then will be taken to the child’s postoperative ward.

The surgeon will recreate a new web with local flaps of skin from the sides of the digits. This usually involves making a series of zig-zag incisions along the digits, and wrapping the zig-zag pieces of skin to cover up the inside surface of the digits where they were previously joined.

This means that skin from the palm of the hand may end up on the back of the finger and vice versa. If a child’s palm is much lighter in colour than the back of the hand, this will be noticeable after the operation.

In most cases, the surgeon will need to use a skin graft to cover up any gaps. The skin for the graft is usually taken from the groin area, so that any scar is hidden in a natural skin crease. This will be a different colour on the hand than the rest of the skin. Occasionally, a skin graft will not be needed.

The operation lasts about three hours. The child will be away from the ward for about one extra hour to give time to prepare for the operation, and to recover from the anaesthetic afterwards. The nurses will tell the parent when the child is in the recovery area and take them there.

Around 150 major hand operations, including syndactyly repair, are carried out at GOSH each year and our surgeons are very experienced so will minimise the chance of any problems developing.

As with all surgery, there is a risk of bleeding, but the surgeon uses a tourniquet to reduce blood flow to the operation site, so there is rarely any serious blood loss. There is a risk of infection as the skin is opened, but the child will be given antibiotics to reduce the risk of this. If an infection does occur, it is usually minor and easily treated with a course of antibiotics.

There is a chance that the skin graft will not ‘take’, but looking after it carefully will help the operation site to heal as well as possible. Any infection or graft loss may affect the time the wounds take to heal and therefore the resulting scars.

The amount of scarring varies from child to child. If the child tends to scar easily from cuts and grazes, it is likely that they will have noticeable scars after the operation, but these will fade in time. If scarring is poor, then repeat surgery may be required when the child is older. A second operation might also be needed if the scar does not stretch as well as the normal skin as thechild grows.

There is a very small risk of nerve injury or of loss of the blood supply to the digit, but this is very rare with experienced surgeons.

The child will come back to the postoperative ward to recover. Usually this is Peter Pan Ward.

The child will have had pain relief in theatre but when this wears off, pain relief will be given in the form of medicines to be swallowed.

Their hand or foot will be covered in a large bandage to protect the skin grafts while they heal. The child will soon find a way to use their bandaged hand or foot in everyday activities. It is best to keep the limb operated on raised above the level of the heart as much as possible to reduce any swelling, which in turn reduces any pain.

It also minimises any oozing under the flaps and skin grafts. Thechild’s limb may be raised using pillows or soft toys, or the hand may be placed in a sling when sitting or walking around.

If the child’s fingertips or toes are visible, the ward nursing staff will check them on a regular basis for warmth, colour, sensation, movement and blood flow. These checks show that the blood is flowing properly. If the child’s digits are not visible, this means that the surgeon was satisfied that the blood was flowing properly at the end of the operation.

Children should be able to go home the day after the operation. They will have been given pain-relief while in hospital, but parents will need to continue giving this at home for at least three days. As well as medicine, distracting the child by playing games, watching TV and reading together can also take thei= mind off the pain.

The clinical nurse specialists will gives parents information about looking after the child’s hand or foot at home, particularly keeping the hand dry and raised above the level of the heart.

Parents should call us if:

• the child has a temperature of more than 37.5°C

• there is swelling or redness near the bandage

• there is staining coming from inside the bandage

• the child complains of a tingling feeling under the bandage

• there is an unpleasant smell coming from the bandage

• the child is in a lot of pain and pain relief does not seem to help

The child will need to keep their hand or foot bandaged until the wounds have healed completely, which usually takes four to six weeks.

If the child has had a skin graft, the first bandage change will be done while they are under general anaesthetic. This is to prevent the dressing change being too painful and to assess the skin graft properly. The first bandage change is usually done on a day case basis, so children come into hospital and return home the same day.

If the child has not had a skin graft, or when the skin graft is stable, the child will have the bandage changes done during a ward visit.

Once the child’s hand has healed completely, it will no longer require a bandage. The child will be able to have a bath or shower again, and also go swimming.

The team will review the child about three months after the operation during an outpatient appointment. This will give them the opportunity to assess the child’s progress and plan any further operations, if they are needed.

If thechild does not need any further operations, the consultant will see thechild at an outpatient appointment about one year later.

The child will then have regular outpatient appointments to check their progress until they have finished growing. As children continue to grow, the child may need a second operation later on. This is usually the case with about one in 10 children overall, but in some cases is more likely.

This depends on the degree of syndactyly and whether it occurred by chance or as part of a syndrome. The outlook for children with simple syndactyly is usually excellent. If the syndactyly occurred as part of a syndrome, the outlook depends on the particular syndrome and associated features.