Neuromuscular scoliosis surgery

This information is intended for those who have been offered surgery for their neuromuscular 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 prior to your appointment.

Unfortunately, this information resource cannot discuss how every neuromuscular condition could affect how well the surgery goes. All quoted risks are from research published in peer reviewed journals. Please do talk to your surgeon and other treating teams about your condition if you have questions related to them.

Benefits of surgery

Surgery is offered to people who have a scoliosis that has a Cobb angle of 50 degrees or more to prevent it getting bigger through adult life. This is to prevent a much larger curve causing heart or lung problems in later life. If you use a wheelchair to get around, then this increases the chance of the curve getting bigger as you get older.

Surgery is also offered to those who are finding it difficult to sit in their wheelchair comfortably, despite adjustments. As the scoliosis gets bigger, patients can get pain if their ribs touch their pelvic bone (costopelvic impingement) or from leaning to one side and only sitting on one buttock. Sometimes people may also develop pressure sores from the way they are sitting.

Although the operation does improve the shape of your back (make the Cobb angle smaller), we may not be able to get the sitting position as good as you would like. Back pain may or may not improve, and in some people can be worse after their operation. In general, parents and carers report satisfaction with the results of scoliosis surgery.

The surgery

All surgeries involve a general anaesthetic and spinal cord monitoring. Once these have been started, 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.

The muscles are peeled off the spine to allow placement of screws in the bones of the spine and pelvis. Hooks are also often used where necessary and attached to slightly different parts of the spine. The spine is then straightened by attaching 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.

Hospital stay

Please follow the latest guidance on protecting yourself from Covid-19 in the two 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 to sip clear fluids until the time of the 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 11am 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 and 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) next to theatres 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 you 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. 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, or toast. For those being 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 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 sickness 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 on 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 2 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

There is no doubt that the majority of families who undergo scoliosis surgery are happy and feel the hard work is worthwhile. For those who cannot tell us themselves, carers report they are happy with the outcome of surgery in the majority of cases. However, sometimes things don’t go as well as expected, with complications occurring in approximately 13 percent to 20 percent of surgeries for neuromuscular scoliosis. 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 rare. For the more complicated operations, we have 2 consultants performing the surgery, which has been shown to decrease the chance of things going wrong during your operation.

To put any figures in context, we perform approximately 10 to 20 surgeries a year for neuromuscular scoliosis at Sheffield Children’s.

Medical complications

Having a general anaesthetic and an operation is a big event for your body. Unfortunately, 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 or 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 neuromuscular condition or syndrome 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, called a tracheostomy. This will all be explained to you and your family before being put in, if it is needed.

Ileus

This is when the bowel slows down and food and wind is not moved from the stomach through your gut 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 fart (pass wind), but not poo. 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 being sick a lot, a tube can be passed through your nose into the stomach so that you do not need to actually be sick. It is taken out when the ileus settles.

Blood clots (deep vein thrombosis and pulmonary embolus)

Blood clots are rare in young people having surgery for scoliosis. The main way to avoid blood clots 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 these may need to be stopped before your surgery.

If these occur only in the leg, (deep vein thrombosis or DVT), then you may have leg pain or swelling. Treatment is aimed at preventing you from having another one. DVTs happen after around <0.01 percent (less than 1 in 10,000) of surgeries.

Rarely (0.03 percent of the time) 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 around 5 to 10 percent 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 or 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 since 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 the blood 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. Surgery for neuromuscular scoliosis will often cause enough bleeding for you to require a blood transfusion.

Bleeding can also occur next to and around the spinal cord, called 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 have 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 less than 0.02 percent 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 and sitting imbalance, lumbar lordosis

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.

Looking from the side, there is a natural curvature to the low back called lumbar lordosis. Because of the way we correct the spine, this can be increased after surgery.

Despite our best efforts we may not get the shape of your back as good as you would like. Hopefully with wheelchair modifications, you will be able to find a comfortable supported sitting position.

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 0.02 percent (less than 2 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. In neuromuscular scoliosis this is thought to be in up to 2 percent (1 in 50) of surgeries but does depend on your condition.

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

Spinal cord monitoring difficulties

Spinal cord monitoring is when we pass messages up and down your spinal cord during the operation so that we are alerted if we are damaging it. We always make sure that we can do this once you are asleep and before we start the operation. In some neuromuscular conditions we are not able to send and receive messages along the spinal cord. This means we will not know if we are damaging the spinal cord during the operation. Before the operation you will discuss with your surgeon the advantages and disadvantages of continuing the operation at this stage.

Paralysis

This is thought to occur in approximately 1 to 2 percent of surgeries for neuromuscular scoliosis. However, this could be under reported because of the nature of neuromuscular conditions. It can occur if the screws and rods are put in the wrong part of the spine, during the correction 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 when 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.

In some patients we decide to continue the operation, despite the changes in cord monitoring, because it is more beneficial to their quality of life to have as straight a spine as possible and only one operation. Before your surgery we will discuss with you and your family about what we should do if this happens during the operation.

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 wee and need a long-term catheter, and you may need help with doing a poo. 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 proximal junctional kyphosis (discussed above), or if the spinal cord in the neck is injured during positioning you on the theatre table.