What is congenital scoliosis?

Congenital scoliosis is the name given to a type of scoliosis that develops because you are born with abnormally shaped bones in the spine. It can be due to a bone only partly developing or due to bones fusing to each other. In a growing spine, this can result in the spine tilting and growing abnormally resulting in a scoliosis.

Due to the way the body develops before birth, bony abnormalities in the spine can be linked with other problems such as abnormal kidneys, heart, limbs and occasionally even the digestive system. Multiple problems like this are often clumped together as one disorder known as VACTERL syndrome (vertebral-anorectal-cardiac-tracheo-esophageal-renal-limb syndrome).

A scoliosis means that the spine is curved abnormally when viewed from the front or the back. It can lead to the trunk and shoulders no longer being symmetrical. Sometimes the shoulders aren’t level anymore. Sometimes the head is not level or the pelvis is not level. The rib cage can also push out on one side so that it becomes prominent - this is known as a rib hump. The severity of the abnormalities can vary between people and can change with time.

How would I know there was a congenital scoliosis?

Sometimes congenital abnormalities are identified before your child is born on the antenatal scans. Otherwise either you, your child or someone else may notice that your child is not standing straight or that they have changes in the shape of their spine.

You may notice:

• Rib cage sticking out on one side especially at the back
• Shoulders not being level
• The hip or waist sticking out
• The head tilting over to one side of the trunk
• Clothes not fitting well anymore
• Not being able to stand up straight
• There may be pain as well as the changes above (rare)

Why does it matter?

Sometimes the shape changes can become a big concern. Some people do get very embarrassed about their shape. The shape can change as your child grows, particularly at the time of growth spurts.

If scoliosis worsens, it can affect the mechanics of your spine and result in aching or pain.

Occasionally, especially if the scoliosis affects someone early in life, it can affect the development and function of the lungs. This does need to be treated or at least monitored. The younger the age when the scoliosis starts, the more likely this may happen. If not monitored and treated when appropriate, this type of scoliosis can result in serious health problems related to the lungs in adulthood.

Generally, a scoliosis that has started early in life, can get a lot bigger due to the growth that the spine will go through. A bigger curve can cause more of the problems described above.

Congenital scoliosis can be associated with other abnormalities in other parts of the body such as the kidneys and the heart. It would be important to be have this checked out as well.

What should I do if I think there is a congenital scoliosis?

Speak to your GP. Your GP will be able to assess and see if there is a scoliosis.

If your GP is concerned, they will refer you to a Spine Surgeon, Paediatrician or Orthopaedic Surgeon at a hospital for further tests.

Once a scoliosis is confirmed, you will need to see a Spine Surgeon at a recognised spine centre who will assess you fully and talk about what you may need. The GP, Paediatrician or Orthopaedic Surgeon will refer you on to the Spine Surgeon.

Do not ignore it if you think there is a scoliosis. More often than not, it will be nothing serious and just require monitoring at the hospital. However it is important to be sure, so having someone check you properly is important.

How will the doctors check for a scoliosis?

The first thing the specialist will do is talk to you about your concerns. They will ask questions about health generally, how the problems are affecting your child and try to get an idea how much more growth and development there is left.

The doctors will want to examine the spine and so your child will need to get undressed for this. Often the hospital will give you a gown, but wearing shorts and a T shirt may make things easier. The examination will look at the back and see how flexible it is. They will often look at the back whilst bent forwards if possible, as this reveals the scoliosis more (Adam’s position). Your child’s arms and or legs will often be examined to make sure the spinal cord is working normally.

The Spine Surgeon will send your child for X - rays. These are necessary to see if there is a scoliosis and how bad it is. The Spine Surgeon will measure the angle of the scoliosis on the X - ray (Cobb angle). The X - rays also show if there are causes for the scoliosis. Sometimes the X - rays help to identify if there is a high risk of the scoliosis worsening.

After all of this, the doctors may want you to go for further tests such as an MRI and a CT scan. If your child is very young, an MRI scan may need a general anaesthetic to help them stay still in the scanner. The doctors will talk that through with you.

As congenital scoliosis can be associated with other abnormalities in the body, especially the kidneys, the specialist may send you for other tests such as ultrasound scans for the kidneys and sometimes an echocardiogram for the heart.

If the plan is to monitor your scoliosis, you will be asked to return in a few months (often 6 months) for another examination and X - ray.

How can you treat congenital scoliosis?

Observation

The commonest initial plan is to be monitored. This will be by 6 - 9 monthly appointments at the hospital by the Spine Surgeon. The appointment will usually involve being examined again and having an X - ray. This is to see if the scoliosis is worsening. If it remains roughly the same each time you are seen then they will continue to monitor you until you are fully grown.

Exercise

There is no need to avoid sport or exercise when you have congenital scoliosis. It is actually better for you to remain active and to have good core muscles. Unfortunately there is no strong evidence that exercise alone stops a scoliosis progressing or improves it. There are many ongoing studies that may help prove the role of exercise one way or another.

Casting or Bracing

Braces are not often used in congenital scoliosis. They are usually rigid braces and need to be worn 23 hours a day. They can be worn under your child’s clothes. It has been found that braces only work if they are used more or less constantly. You can take the brace off to wash, dress and during sporting activities.

Surgery

If the congenital scoliosis is felt to be at high risk of worsening or worsens whilst being monitored, surgery will be advised.

Surgery in a growing child’s spine aims to reduce the risk of the curve worsening at the same time as trying to ensure the spine overall still grows and lengthens with time.

In some situations, congenital scoliosis will be treated like an early onset scoliosis if your child is very young.

Further information can be found here.

Hemiepiphysiodesis is an operation specifically for congenital scoliosis. It involves finding the growing areas of the abnormal bone and stopping that growth. By doing this early in life, it is hoped that the remaining normal growth will slowly improve the scoliosis over time.

Hemivertebra excision is an operation which involves removing the abnormally shaped bone and fusing a small segment of the spine to stop the scoliosis worsening.

A surgical fusion of the abnormal section of the spine might be used to control the scoliosis. This may involve an operation on the front and back of the spine and putting rods and screws into the spine.