The skull is made up of several ‘plates’ of bone which, when we are born, are not tightly joined together. The seams where the plates join are called ‘sutures’. As we grow older, the sutures gradually fuse (stick) together, usually after all head growth has finished. When a child has craniosynostosis, the sutures fuse before birth.
It can affect one suture or several. When one or more sutures fuse too soon, the space inside the skull cannot expand as a child grows. This can put pressure on the brain (intracranial pressure) which can have significant long term effects. Front-orbital remodelling tends to be carried out at the age of 12 to 18 months, while the skull bones are more pliable so respond better to treatment. Children with the following types of craniosynostosis may benefit from this operation:
- Unicoronal craniosynostosis
- Bicoronal craniosynostosis
- Metopic craniosynostosis
- Crouzon syndrome
- Pfeiffer syndrome
- Apert syndrome
- Saethre-Chotzen syndrome
- Muenke syndrome
It may be the only form of treatment needed or it may be just one in a series of operations carried out throughout childhood and adolescence.
What happens before the operation?
Preparing for a planned operation, test or procedure before coming in to hospital avoids delays and reduces the risk of cancellation.
The results of any tests and investigations are available in plenty of time and can also be re-checked if they are not within the normal range. Your child may need various blood tests before the operation – this depends on your child’s medical condition and the nature of the surgery that is planned.
The doctors and/or nurses will meet you and your child to take down their medical history and any other information needed before your child is admitted to hospital. The nurses will explain about any care your child will need before and after the operation. If your child has any medical problems, particularly allergies, please tell the doctors about these. Please also bring in any medicines your child is currently taking.
You may be seen by one of the team carrying out your child’s operation and be asked to give permission for the procedure by signing a consent form. If you give your consent at the pre-admission appointment, you will need to confirm that you still agree to the procedure on the day of admission.
One of the team will explain about the types of anaesthesia that are used at the hospital, and also about options for pain relief after
the operation, test or procedure. If there any questions or concerns about your child’s anaesthesia, an anaesthetist may come to see your child in the pre-admission clinic.
The night before surgery
You will be asked to give your child a bath or shower and hairwash before surgery. It is important that your child does not eat or drink anything for a few hours before the operation. This is called ‘fasting’ or ‘nil by mouth’. Fasting reduces the risk of stomach contents entering the lungs during and after the procedure.
You will be informed the night before the procedure of the time that your child should be ‘nil by mouth’ – in other words, have nothing to eat or drink before the anaesthetic. In broad terms, this is six hours for food (including milk), four hours for breast feeding and two hours for clear fluids before the procedure.
It is equally important to keep giving your child food and drink until those times to ensure they remain well-hydrated and get adequate nutrition. This may involve waking your child in the night to give them a drink which we recommend.
On the day of surgery
Please come to Puffin at the time stated in your admission letter. One of the nurses will check that your child is well enough for the operation, complete some paperwork with you and take some baseline observations. If you did not give your consent for the
operation at the pre-admission appointment, a member of the surgical team will visit you to explain about the operation and ask you to sign a consent form. The site of the operation will be marked with a marker pen. All children are seen by the anaesthetist on
the day of the operation.
What anaesthetic is given?
Your child will be given a general anaesthetic by an anaesthetist who specialises in giving anaesthetics to babies and children. Both parents will be able to go with your child to the anaesthetic room and stay until they are asleep. This usually involves your child breathing some anaesthetic gas. Later, a tube is passed into the airway (trachea) to safeguard breathing. A cannula (thin, plastic tube) is put in a vein and usually left in place for a short time after the operation. Fluids can be given to your child
through this tube during the operation and afterwards if necessary.
What does the operation involve?
Fronto-orbital remodelling is carried out while your child is under general anaesthetic. When your child is under general anaesthetic, the surgeon will clip your child’s hair just over the incision site and fix the rest of their hair out of the way. They will make an incision over the top of your child’s head from ear to ear. They will then pull the skin and soft tissues over the forehead downwards to expose the skull.
The surgeon will next cut through the coronal sutures to remove the front portion of the upper skull and re-shape it by cutting and
trimming the bone to form a more normal shape. Once the skull bones are in place and the surgeon is happy with the result, the bone will be fixed in place using strong stitches that dissolve very gradually over the next few months while the bone heals. The skin is then closed over the incision site and held in place with dissolvable stitches.
Some children have a drain inserted which will be left in place to drain off any fluid that collects after surgery – this will be removed
a day or two later when no longer needed. Finally, the surgeon will put a head bandage over the operation site. Your child will then be taken to the Recovery area to start to wake from the anaesthetic.
Are there any risks?
Healthy children usually cope well with the anaesthetic, but the risk increases if your child has other problems.
All surgery carries a small risk of infection or bleeding. To reduce the risk of infection, your child will be given an initial dose
of antibiotics during the operation. This will continue as an infusion into a vein (intravenously) for 24 hours after surgery. Your child will be monitored very closely during and after the operation to identify any blood loss. A blood transfusion is sometimes required but the surgeon will have ensured that donated blood of the correct type is available if needed.
This surgery requires separating the skull bone from the protective layer covering the brain (the dura), a process which can, in a tiny proportion of cases, cause brain injury or bleeding inside. Therefore, any craniofacial operation carries a very small chance of causing serious complications such as seizures (fits) or stroke (brain damage), which may in some very rare cases be life-threatening. The overall risk of a major neurological event or death is much less than one per cent (less than a 1 in 100 chance).
Sometimes small holes can be made in the dura during the operation, which do not pose any risk to the brain, but which can occasionally be the source of a leak of cerebro-spinal fluid (CSF; a clear, watery fluid that surrounds and cushions the brain). The surgeons usually identify and repair (stitch) any holes during the operation. Despite this, sometimes children develop a CSF leak after surgery, which may show up as a fluid-filled swelling at the operation site or a leak of clear, watery fluid from the wound. It will be explained to you what to do if you notice these problems after discharge from hospital, as sometimes further procedures may become necessary to stop the leakage if one occurs.
Your child’s head and face will look swollen and bruised after the operation. This particularly affects the eyes following fronto-orbital remodelling and the nurses will clean them carefully in the days following the operation. Swelling tends to get worse for the first two to three days and then start to improve. The nurses will check your child’s head bandage every few hours and re-apply it if it is getting tight.
The incision site will start to heal and will eventually fade until it can hardly be seen. The hair will also start to re-grow in the days following surgery.
Are there any alternatives to this operation?
Fronto-orbital remodelling surgery is a headshape change operation rather than one to improve function.
What happens after the operation?
Once your child has started to recover from the anaesthetic, they will be brought back to Koala Ward to continue recovering. At the
end of the day, the doctors will visit you to talk about the operation.
They will spend the first night in the High Dependency Area on the ward, where a nurse will monitor them closely to watch for any signs of bleeding and fluid imbalance. They will carry out regular observations of their breathing, heart rate and temperature throughout the rest of the day and night. Your child may feel sick after the operation, but the doctors will give them medicines to reduce this. The sickness should pass within a couple of days. Your child will be able to start eating and drinking as soon as they feel like it.
The nurses will also assess your child’s pain and give them regular pain relief for the first few days – intravenously to start with and then by mouth when they are eating and drinking. The intravenous infusion of antibiotics will continue for the first 24 hours. Your child’s eyes will be very swollen after the operation so the nurses will clean them gently to make them more comfortable. Your child
should sit and lie in as upright position as possible to reduce any swelling. The swelling is temporary and will start to improve in a few days. The nurses will remove the head bandage and drains when they are no longer needed.
Over the next few days, your child will be able to start moving around. Your child is likely to be in hospital for around four days.
When your child is recovering well and eating and drinking as normal, you and your child will be able to go home. On the day of discharge, the nurses will wash your child’s hair gently with a mild shampoo and show you how to do it safely at home. They will also give you a copy of our discharge information which explains how to look after your child and what signs to look out for over the next few weeks.
Follow up appointments
Your child will have a series of appointments after the operation – we will give you details before you go home.
What is the outlook for children who have had fronto-orbital remodelling?
This operation is a head-shape changing operation rather than one to improve function. The results of surgery are variable but overall many parents are happy with the results.