Congenital scoliosis surgery

This information is intended for children and young people who have been offered surgery for their congenital (present from birth) scoliosis. Your treating surgeon will go through all the information in this document in clinic during the consent process. This will hopefully help you understand the terms used and allow you to think of any questions before coming to your appointment.

This information is designed to give an in-depth overview of congenital scoliosis surgery, but still cannot go into detail on every aspect of your condition because there is great variation to the shapes of bones involved in congenital scoliosis and medical conditions associated with it, and so every patient is different. All quoted risks are from research published in peer reviewed journals. Please do talk to your surgeon and other treating consultants about your condition if you have questions related to them.

Benefits of surgery

In congenital scoliosis, patients have one or more bones in their back that are an abnormal shape and are causing the spine to grow curved. If these are left untreated, then the scoliosis will get very big and will affect the development of the lungs, which aren’t fully formed until the age of 8 years old. Treatments are aimed at stopping these bones from continuing to grow, and some will need removing, called a hemivertebrectomy or vertebrectomy.

Surgery is offered to those who have a scoliosis that is rapidly increasing in size, or a Cobb angle of 50 degrees or more, to prevent it getting bigger as you grow. It is often performed when you are younger than in other conditions to allow the lungs to develop, and reduce your chance of developing health problems related to poor lung function. In the past, very severe scoliosis has been thought to be life threatening if it causes major problems with lung function.

If you are older, then surgery is aimed at stopping the curve from getting bigger through adult life.

Although the operation does improve the shape of your back (by making the Cobb angle smaller), we may not be able to get the appearance as good as you would like. Also, the shape of your back may continue to change whilst you grow, even after surgery. Back pain may or may not improve, and in some people can be worse after their operation.

The surgery

All surgeries involve a general anaesthetic and spinal cord monitoring. Once the anaesthetist is happy and all monitoring is in place, you are turned onto your tummy so that the surgery can be performed. At this stage we ensure your eyes are not being pressed on directly, and your arms and legs are placed comfortably.

In most cases the surgery involves a long cut down the middle of your back. If the surgery involves getting to the spine from the front, your surgeon will explain this.

The muscles are peeled off the spine to allow placement of screws in the pedicles and vertebra of the spine. Hooks are also often used where necessary and attached to slightly different parts of the spine.

In a lot of cases, we will discuss removing one or more of the bones of the spine to stop this growing again. This is often done through your back, but means we have to go past the spinal cord in order to get to the bones at the front of the spine. Once the bone is removed, we will be able to get a much larger improvement in the scoliosis when we attach rods to the screws.

At times during the procedure, hammers and chisels are used to remove the joints at the back of the spine, and to remove the outer surface of the bone, to allow fusion. This is where the bone grows across the spine where the rods and screws have been placed, and prevents further change in shape of the spine.

After the operation, you may be asked to wear a brace for 3 to 6 months to help the spine heal in the position we gave it during the operation. This will be fitted prior to you leaving the hospital.

Hospital stay

Please follow the latest guidance on protecting yourself from COVID-19 in the 2 weeks leading up to your admission. Assuming your swab is negative, you will be admitted to Ward 2 the night before your surgery.

The day of your surgery (all timings are approximate)

You won’t be allowed to eat anything from 2am, and will be allowed sips of clear fluids until the time of your operation. The anaesthetic and surgical teams will normally come and see you between 7.30am and 8.15am to check you still want to go ahead, and answer any final questions. The nurses will also go through some final checks with you.

You will need to go to the High Dependency Unit (HDU) or Intensive Care Unit (ICU) when your surgery is finished, and so before we can start, we will need to check a bed is free for you. Unfortunately, if the HDU or ICU is full, then your surgery will have to be postponed. We will confirm with you as soon as we know whether the operation can go ahead.

The theatre team meet at 8.30am to discuss the cases on the theatre list and ensure we have enough staff and all the right equipment to proceed. Once everyone is happy, a member of the team will come and get you from the ward, normally between 8.30am to 9am. You will be taken down to theatre on a trolley.

Between 9am and 11.am you will be put to sleep and all necessary monitoring will be attached to your body. This will include the spinal cord monitoring: when you wake-up you will find spots of blood and pin-pricks on your arms and legs, and in your hair, from the tiny needles we use. A catheter (a tube that allows you to wee) will also be passed into your bladder.

Once all the monitoring is placed, you will be positioned on the operating table and the surgery will start: this normally occurs between 10.30am to 11.30am. It will normally take between 4 and 6 hours to perform your surgery, but unexpected events can make it last longer.

You will wake-up in theatre or in the Post Anaesthetic Care Unit (PACU) and spend 30 to 60 minutes being monitored there. You will have your first neurological checks and be made comfortable with any additional painkillers you may need. A member of your family will be allowed down once the whole team is happy it is safe for them to do so.

A member of the surgical team will talk to you and your parents once you are awake and inform them of how the surgery went and any difficulties or complications.

Once it is safe for you to leave PACU, and your pain is under control, you will be moved to HDU or ICU. Once there, you can move around in bed as much as you want to get comfortable, and you will be allowed to drink, but not eat, overnight. One of the orthopaedic doctors will check everything is going well later that evening.

Day 1

You will be seen on the orthopaedic ward rounds in the morning, and by the medical team looking after you on HDU or ICU. If your general health allows, you may be moved back to ward 2 on the first day. Some people need to stay on the HDU or ICU longer, to make sure you are looked after in the best possible way after your surgery.

A member of the team will listen to your tummy. If you have bowel sounds (a gurgling tummy), and are not being sick, you will be allowed to start eating. Start with something light such as soup, ice cream, yoghurt, toast. For those who are fed through a tube, we will start with small amounts and advise you to aspirate on the tube to make sure the feed is being absorbed. If it is, then the amount of feed being given can be increased.

People who are not in significant pain, and are well, will be encouraged to sit up in bed and move around the bed.

Day 2

The nursing checks will become less frequent on day 2, and hopefully any vomiting has stopped. Sitting up and moving around the bed will be encouraged if you are well enough.

Some patients will still be feeling very unwell from their surgery, and may not get this far.

If your pain is under control, the team will be looking at getting you onto painkillers you take by swallowing, rather than through a drip.

Day 3 to 7

You will be encouraged to get out of bed more and more, as long as you remain well. You will be allowed to go home once you are well enough, and can sit in a chair long enough for you to be able to get home in the car. You should also be confident that your pain is under control on regular painkillers. Most patients will be going home day 7 to 10, but we will not keep you in hospital if you are ready to go home sooner.

The catheter will be removed after a few days when you are eating and drinking, and there are no concerns about your general health. You can also shower once you feel ready. An x-ray is taken before you go home so that we can fully assess the operation.

You may find that you have not done a poo before going home, but as long as you feel well and are not being sick you do not need to worry about this. You will normally be able to go to the toilet by day 10.

After discharge

Once you are home, continue to build up your activities at a steady pace. You can decrease the number of painkillers you are taking as the pain improves. Increasing your activity levels will decrease your chance of certain complications.

Your wound should be dry and healed by two weeks after your surgery, and you do not need to wear a dressing after this time.

You should expect to be back at school or college by 6 weeks, depending on your pain levels and school circumstances. However, for those who rely on a wheelchair, this may need adjusting and could mean that you cannot get out and about comfortably until these adjustments have been made.

Risks of surgery

Sometimes things do not always go as well as expected, and approximately 1 in 10 patients who have an operation for congenital scoliosis are thought to develop a complication. For this reason, we have to tell you about things that could possibly go wrong. Some complications happen more often than others, and often these are treatable. Life-changing complications are thankfully less common. For the more complicated operations, we have two consultants performing the surgery, which has been shown to decrease the chance of things going wrong in complex surgeries.

To put any figures in context, we perform approximately 10 – 20 surgeries a year for congenital scoliosis at SCH (pre-Covid).

Medical complications

Having a general anaesthetic and an operation is a big event for your body. You can develop urine and chest infections which will be treated with antibiotics. Your heart and kidneys also have to work harder during the operation, particularly if you lose a lot of blood. Although rare in children, damage to the kidneys and heart muscle / heart attacks can occur and these will be treated by the anaesthetic and medical teams.

Chest and breathing problems are often easier to treat in patients with a good cough and who can follow instructions from a chest physiotherapist. If you have a weak cough then you are at higher risk of chest problems.

If your scoliosis or general health also affects the strength of your breathing, then the anaesthetist may not be able to wake you up after the operation and take the tube out of your throat that helps you breathe. If this happens, you will have to go to ICU rather than HDU, and you will be kept asleep. The ICU team will then look after you and decide when it is safe to allow you to breathe on your own and remove the tube.

If over several days, the ICU team find that you are still not able to breathe on your own, then you may need a tube placed through your neck into your windpipe, a tracheostomy. This will all be explained to you and your family before being put in, if it is felt to be needed.

Ileus

This is when the bowel slows down and food and wind is not moved from the stomach through your bowels at normal speed. This can lead to bloating and being sick. This is why we do not let you eat straight after your surgery. Once you have been told you can start eating and drinking, start with simple light food before returning to a full meal. For the first few days, if your bowel is working normally, you will find you pass wind, but no stools. This is entirely normal.

Ileus can last several days and is uncomfortable. There are no specific treatments, and we just have to wait for the bowels to start working again. If you are vomiting a lot, a tube can be passed through your nose into the stomach so that you do not need to actually vomit. It is taken out when the ileus settles.

Blood clots (deep vein thrombosis and pulmonary embolus)

Blood clots are rare in young people undergoing surgery for scoliosis. Your main defence is movement and so we will encourage you to start moving as soon as the first night of your operation. You must tell us if you are taking any hormone treatment or any contraceptives because we may need to stop these before your surgery.

If these occur only in the leg, DVT, then you may have leg pain or swelling. Treatment is aimed at preventing you from having another one. DVT’s happen after approximately < 0.01% (less than 1 in 10000) of surgeries.

Rarely (0.03%) these blood clots can move and travel to the lung. This can lead to chest pain, breathing problems and in severe cases death. Treatment is aimed at supporting your breathing and preventing you from developing further blood clots.

Infection

Infection occurs in 0.5% to 2% of surgeries. It can occur early in the first few days or weeks after the operation, but can sometimes take months or years to become apparent.

A superficial infection in the wound / skin will often be treated with antibiotics and nothing more is needed.

However, a deep infection around the metalwork and spine is much harder to treat. You are likely to need antibiotics given through a drip in the arm, and an operation to ‘clean’ the metalwork. Sometimes we need to do this more than once. If this doesn’t get rid of the infection, then you may need to have all, or part, of the screws and rods removed. Depending on how long it is from your surgery, we may need to put the metalwork back in. If this is necessary, we may have to keep you in bed in hospital for a period of time before replacing them to minimise the chance of the new metalwork becoming infected.

We will keep you informed of our treatment and decision making at all times.

Bleeding

All surgeries cause some bleeding, but more complex operations can lead to a large amount of blood loss. Bone contains many blood vessels, and so because we are placing screws in the bone, and peeling the surface of the bone off to achieve fusion, scoliosis surgery can lead to significant blood loss.

During the surgery we use cell salvage where we collect the blood from the wound. We then filter it to ensure it is clean, and give it back to you. We therefore only use blood transfusions, where you receive blood from someone else, when absolutely necessary if you lose a lot of blood.

The smaller you are, the less blood you have in your body and therefore the higher your risk of having life threatening bleeding during complex procedures. Blood transfusions are more likely in congenital and neuromuscular scoliosis surgeries.

Bleeding can also occur next to and around the spinal cord, an epidural haematoma. If this happens, then pressure will build up on the spinal cord and you may become paralysed. You will have regular checks on the movement in your arms and legs after the operation. If there are any concerns, then you will undergo further imaging of your spine. If there is something obvious pressing on the spinal cord, then an emergency operation may be needed to remove this and give the spinal cord chance to recover. This is thought to occur in < 0.01% of surgeries.

Nerve injury

We can damage nerves and vessels during the time it takes to expose the spine, but also when we are putting the screws in the bones of the spine. If we are unhappy with the position of a screw during the procedure, we will replace it, however some nerve injuries are not apparent until after the surgery.

If this happens in the top part of your spine (thoracic area) you may have pain coming around your chest wall or patchy numbness. In the lower spine (lumbar area) you may have pain shooting down the leg, areas of numbness or even weakness in some muscles of the leg.

Depending on the severity of the symptoms, we will check the position of the screws with X-rays, CT, or MRI scans. In discussion with you, we may decide to leave the screws alone and see if the symptoms settle, or we may decide to take you back to theatre and change the position of the screws that are in the wrong place.

CSF leak and dural tear

The nerves and spinal cord are surrounded by a thin layer called the dura. This helps protect them, and maintain the flow of cerebro-spinal fluid (CSF) around them. This thin layer may be injured by our instruments or the screw. Most dural tears will heal with repair in theatre, or with a period of bed rest after the operation.

If, however, they do not heal you may have a watery fluid leaking through your wound and develop headaches. We may need to do another operation to find the location of the leaking fluid and try and repair it. Please do contact the spinal nursing team if you are ever concerned about a wound healing problem.

Once the wound is healed, most dural tears will not give you any side effects in the long-term.

Lateral cutaneous femoral nerve injury

This is a small nerve that runs across the prominent bone at the front of your pelvis. When we position you face down during the operation you may be lying on this nerve and be squashing it for the whole of the procedure. We place you on soft cushions to try and make sure you are comfortable, but this can still happen despite our best efforts.

The nerve supplies sensation (feeling) to the outside of your thighs. If it is damaged from lying on it, you may notice numbness or tingling in these areas, and it can occur on only one side. It normally gets better over days and weeks, and can cause tingling (paraesthesia) whilst it recovers. If it doesn’t return to normal, you will be left with numbness on the outside of your thighs which most people get used to. If the tingling persists and is very uncomfortable, you may need painkillers to decrease how much it bothers you.

Metalwork mal-placement and failure

Metalwork may not always be placed in the correct position in the spine, and we have mentioned the possibility of nerve injury above.

Sometimes rods and screws move or detach from each other after the operation. You may not have any symptoms, but you may develop pain or be able to feel the screws and rods through your skin. If this occurs we may need to take you back for another operation to repair the problem.

If the bone doesn’t fuse together, then the rods may break. This can lead to increased pain in the back, but sometimes it is simply seen on a routine X-ray. Depending on where it occurs, how many rods break and how long it is from surgery, we may not need to replace it. We will usually perform a CT scan to help with this decision.

If you develop an infection, the screws and rods can become loose. The main aim of the treatment will be to get rid of the infection, and more details about this are discussed in the infection section.

Damage to adjacent structures

Although surgery focusses on the spine, there is a risk of damaging structures next to the spine. Although there is a greater risk with the anterior (front) approach, the posterior (back) approach also carries with it the chance of damage to the bowel, the lung, the kidney or the large vessels running in front of the spine. Any injury will be treated dependent on the nature of it.

Injuring a large vessel can cause major bleeding which can be life threatening and will mean you require a blood transfusion. You may also need emergency surgery to repair the damaged vessel and stop the bleeding.

Injury to the lung may mean you wake up with a chest drain that you were not expecting.

Non-union

One aim of the operation is to fuse the bones of the spine – this is when the bone grows around the screws and rods. If the spine does not fuse then this is called a non-union. Non-union will normally occur in a small area rather than across the whole spine. If this happens, then you may experience increasing back pain or prominent metalwork. It may also be only picked up on a routine X-ray showing a broken rod.

This will normally be investigated further with a CT scan and depending on your symptoms you may need further surgery to try and get the area of non-union to fuse.

Back pain

There is no guarantee that your back pain will improve, and some people are left with more back pain than they started with. The majority of back pain will improve over weeks and months, and you will be encouraged to increase your activities over this period because this has been shown to improve symptoms in the majority of people.

If your back pain does not improve you may be offered physiotherapy after your first follow-up appointment.

Shoulder imbalance and unhappiness with appearance

Depending on the type of scoliosis you have, you may notice that your shoulders aren’t level before your operation. During the surgery, attempts will be made to try and level your shoulders or make them look symmetrical. However, it is not always possible to get this absolutely right.

Also, the nature of congenital scoliosis means that it is generally not possible to achieve a naturally contoured spine. If you have multiple operations, you are also likely to have many scars, and some may not be thin and neat. It may be that as you get older you are unhappy with the appearance. However, it must be remembered that congenital scoliosis is very difficult to treat, and left untreated, could have caused significant health problems to you.

Proximal junctional kyphosis

This is when the spine suddenly bends forward over the top of the fixed spine, for example, where the last screws are at the top of the back. It can occur as early as straight after the operation.

In severe circumstances this can cause paralysis at this level of the spine. If there are any concerns after the operation with the function in your arms, your legs, or both, then you will undergo further investigations immediately. Rarely, a patient may need an emergency operation to correct the kyphosis.

Blindness

Surgery in the prone position (lying on your stomach) is associated with blindness. This is very rare described in less than 0.01 percent or 1 in 10,000 of surgeries.

During the operation your face is placed in a special cushion which is cut out to allow space for the eyes. The weight of your head is supported by your jaw, cheeks and forehead. During your operation the eyes are checked regularly to ensure the cushion has not moved and the eyes are not under pressure.

You must tell us if you are being investigated or being treated for any eye conditions.

Risk to life

There is always a small chance that a patient dies during their operation, or during their recovery (around 0.3 percent of surgeries).

Possible causes of death are major bleeding that the surgeon is not able to control, severe infections leading to sepsis, or other medical complications. The team looking after you will do everything they can to treat the cause, but sometimes they are not effective.

Crankshaft phenomenon and ongoing scoliosis progression

Despite our best efforts the scoliosis may continue to get bigger as you grow. Sometimes one part of the spine will grow more than the other, also known as crank shaft phenomenon. This may mean you need more operations to change the metalwork, or even fuse more of the spine earlier than expected.

Paralysis

This is thought to occur in approximately 2 percent or 1 in 50 surgeries for congenital scoliosis. It can occur due to putting rods and screws in the wrong part of the spine, during the correction of the scoliosis, or if the instruments we use during the procedure hit the spinal cord.

The critical point in the operation is when the curve is straightened as the rods are attached to the spine. The spinal cord monitoring team will let the surgical team know if there are any concerns over spinal cord function at this stage. The rods may need to be removed and the operation abandoned.

Despite the best efforts of the surgical and anaesthetic teams, you may be left paralysed by this procedure and the spinal cord may never recover. In this case you will be left with legs that are weak or don’t move at all, you may not be able to pass urine and need a long-term catheter, and you may need help with opening your bowels. For those patients who have limited function in their legs and arms, we may only realise a spinal cord injury has happened when the tube in your bladder is removed several days later.

If the spinal cord is injured in your neck, then your arms will be weak as well. This can occur because of PJK (discussed above), or if the spinal cord in the neck is injured during positioning you on the theatre table.