INTRODUCTION

The term idiopathic scoliosis describes an anomalous curvature in the spine; this does not mean that any curve of the spine is scoliosis. According to the definition of the Scoliosis Research Society (SRS), scoliosis is a lateral deviation of the normal vertical line of the spine which is greater than eleven degrees. Scoliosis consists of a lateral curvature of the spine with rotation of the vertebrae within the curve. The cause of scoliosis may be congenital, growth-related or degenerative, but in most cases its cause is unknown and it is therefore described as idiopathic scoliosis.

It usually presents in the thoracic, thoracolumbar or, more rarely, the lumbar spine; it presents in a curve shaped like a C, or two consecutive C-shaped curves that result in the spine having the shape of an S.

The more frequent types of scoliosis are:

 

Congenital scoliosis

It is caused by a congenital abnormality in the formation of the vertebrae. Scoliosis exists in these patients from infancy.

 

Neuromuscular scoliosis

Abnormalities in muscles or nerves cause weakness of the muscles which cannot keep the spine aligned above the pelvis. This usually occurs during childhood in patients who are unable to walk due to muscular (such as muscular dystrophy) or neural (such as cerebral palsy) causes.

 

Degenerative scoliosis, also known as adult scoliosis

This develops later on in life either de novo or as the evolvement of scoliosis that existed from childhood but was not progressing. It is caused by the degeneration – arthritis – of the joints between vertebrae, in particular the intervertebral disc.

 

Idiopathic or adolescent scoliosis

This most common type of scoliosis appears during adolescence and progresses during the adolescent growth spurt. 
 

 

IDIOPATHIC SCOLIOSIS

 As mentioned above this is the most common type of scoliosis, it presents mainly in puberty and its name (idiopathic) means that its cause is unknown. It often occurs, however, to members of the same family which indicates some form of heredity. There are no statistical data for Greece but in the U.S. around half a million adolescents have scoliosis.

Idiopathic scoliosis is divided into three types, based on the age at which it presents:

 

• From birth to 3 years: infantile scoliosis

• 3-9 years: juvenile scoliosis

• 9-18 years: adolescentscoliosis
80% of idiopathic scoliosis cases belong to the third type.

 

The classification above basically separates infantile scoliosis from the other two types (juvenile and adolescent) that are relative in shape and response to therapy. There is another type of classification based on the development of the lungs; the lungs are fully developed by the age of 5 and therefore any abnormality in the pulmonary cage (formed by the spine and the ribs) that occurs after that age will not influence their capacity. According to this classification, idiopathic scoliosis is separated into two categories according to when it appears, before and after 5 years of age.

 

The risk of idiopathic scoliosis progressing, increases during puberty, especially at the time of the juvenile growth spurt that, in girls, occurs at 11 and in boys, at 13. Scoliosis with a significant curvature presents mainly in girls that need therapy 8 times more often than boys do, since curvatures in girls tend to progress. Generally speaking though, the majority of patients with scoliosis do not need to undergo any kind of therapy.


 

SYMPTOMS & DIAGNOSIS

Idiopathic scoliosis (in short, IS) causes deformity in the spine but no pain. Patients with IS may feel pain in their spine (e.g. backache), the way their peers may, without, however, being more susceptible to backache. In children that have both pain and scoliosis, the cause of the pain is something other than the scoliosis.

As scoliosis causes a change in the shape of the body (a deformity, as awful as that sounds) there are obvious signs that can lead to its diagnosis:

Α) one shoulder is higher up than the other

Β) oneshoulderbladeprotrudes more than the other

C) theribsprotrudeorsithigherupon one side of the body

D) one hip protrudes or sits higher up than the other

Ε) the waist is asymmetrical

 

The first to diagnose scoliosis in a child is often the school doctor or nurse. The most common test for the diagnosis of scoliosis, when it is small and without any obvious deformity, is the Adam’s test, where the patient is asked to bend over with their knees straight while the doctor or nurse watches their back, mainly from behind (image 2). This position clearly demonstrates any asymmetry in the spine and the trunk in general. The next diagnostic step is an x-ray of the spine.

Although in most cases scoliosis is idiopathic and there is no neuromuscular infrastructure, the correct and necessary procedure is to also have a clinical examination, the neurological part of it in particular.

The spine is x-rayed standing up (front and back) to confirm the diagnosis and to measure the scoliosis (in degrees). The Cobb method is used to measure it and it is accurate to 3-5 degrees. The x-ray needs to include the upper part of the pelvis; from the degree of ossification one can roughly estimate the remaining growth in a child (Risser Sign, scale 1-5). In certain, rare cases,

  • When the diagnosis is accompanied by neurological findings (increased reflexes)
  • Before each treatment, in the case of infantile scoliosis
  • When the scoliosis is in the left thoracic (in cases where the curvature turns the convex towards the left side of the body, whereas it is almost always expected to be towards the right), anMRI of the spine is also necessary.

Based on the findings, scoliosis can either be treated conservatively – observation or brace – or with surgery.

 

CONSERVATIVE TREATMENT

The decision about the treatment of IS is made based on the child’s skeletal maturity (how much more it will grow at the time of diagnosis) and the position and size of the curvature (in degrees). The behavior of each scoliotic curvature -whether it will progress or not- is influenced by specific factors; the younger the patient is, in other words the farther from skeletal maturity, and the larger the curvature, the bigger the chance of it growing further (in degrees). Other factors are the female gender, the lack of menstruation (which is the same as skeletal immaturity) and the presence of the scoliosis in the thoracic spine.

Forms of treatment such as exercise, chiropractic, osteopathy or physical therapy have been proven pointless by extensive studies on countless children. It is equally pointless to try to change a child’s daily habits, such as which side they hang their school satchel on, how they sit at their desk etc; curvatures are not influenced by any kind of activity.

 

OBSERVATION

Curvatures smaller than 10 degrees, are not considered scoliosis, rather an asymmetry of the spine. It is very unlikely that they would progress and generally don’t demand any kind of observation or treatment, except for in cases where the child is either very young or very immature regarding its growth; then, observation is required.

It is also advisable to regularly observe curvatures of 10-20 degrees, every 4 to 6 months, in order to check on the progression of the curvature. Curvatures are considered to progress when they grow by at least 5 degrees (which is the precision limit of the measurement): growth of fewer than 5 degrees is not considered progression and no measures are taken. If the curvature progression is larger than 5 degrees, in a child that is still growing, and if the scoliosis surpasses 20 degrees in total, bracing is recommended.

 

BRACING

The brace is considered to contain curvature progression but it cannot reduce the deformity (the size of the curvatures); in other words, it cannot “cure” scoliosis. Moreover, even when it is used, one third of scoliosis curvatures will continue to grow within the confines of the brace.

Bracing is absolutely necessary only during the juvenile growth spurt period which is when scoliosis might worsen. Menstruation signals the beginning of the end of the juvenile growth spurt period in girls; this is finally verified by x-raying (Risser sign and hand x-ray) and also the appearance of advanced, secondary gender characteristics such as the growth of the breasts and pubic hair.

Those curvatures that may continue to develop post-puberty (as adult scoliosis), are those that are larger than 50 degrees, which is why bracing’s objective is for the child to enter adulthood with a curvature smaller than 50 degrees.

There are two types of brace with different indications:

  1. The Thoracolumbosacralorthosis-TLSO is custom-molded to adapt with precision to each child’s body and can be worn under clothes.It works with three point pressure to the spine and is compatible with almost every type of curvature. It has to be worn 16-23 hours a day and can be removed for sports activities.
  2. The Charleston brace is a night-brace that basically curves the body against the scoliotic curvature. It is recommended for single, mild, thoracic curvatures.

Since bracing can stop the progression of a curvature in children that are not yet fully developed, it become obvious that using a brace on a child that has reach its skeletal maturity is unnecessary. This means that the use of bracing is limited to 11-13 year- old girls and 12-14 year-old boys. A child with a curvature of approximately 30 degrees who has almost reached skeletal maturity needs to be observed but not treated with bracing, since the chances of their scoliosis progressing are minimal.

All of the above regarding bracing relates to the American and Scandinavian philosophy. The British school, on the other hand, is totally negative to the use of bracing in IS. The British doctors’ theory is that the research conducted by the Americans and the Scandinavians on bracing contained methodological errors that make it unreliable. Moreover, their own research has shown that, with or without the use of a brace, the number of children that will finally be led to the operation theater for scoliosis treatment is analogically the same. They also claim that most children will not wear the brace that has been prescribed because the psychological repercussions are huge. Therefore, the primary check for IS in schools is considered pointless in Britain, as the natural course of the disease cannot be altered by intervention at an early stage (small scoliosis). To children with visible scoliosis (deformity), if appropriate, depending on measured degrees, British doctors recommend the surgical solution.

In 2013 an NIH founded and thoroughly planned research demonstrated the efficacy in AIS (in kids with scoliosis from 20-40 degress, in pre-menarchial or less then 1 year post-menarchial, 10-15 years old, less then 2 Risser sign). This was published in New England Journal of Medicine.

 

SURGERY

The objective of surgery in IS, is spinal fusion - the permanent joining together of the vertebrae that participate in the curvature – to put a stop to the progression of the scoliosis. At the same time, with the use of strong materials, we also achieve partial correction of the curves, in other words a better cosmetic result.

Scoliosis surgery is recommended for curves in excess of 40 degrees that appear to be progressing, despite the bracing, as well as for curves that exceed 50 degrees after growth has stopped (because it means that they will continue to progress into adulthood). The reason we operate on scoliosis, up to 70-80 degrees, is purely cosmetic. When scoliosis exceeds this limit, it not only causes a severe deformity, it also puts pressure on the heart and lungs. For this reason, when scoliosis exceeds the 80-degree limit, spinal fusion is absolutely recommended.

The expected correction, using contemporary spinal fusion materials, is in the vicinity of 50% (in degrees) and depends on the flexibility of the spine. In older patients the spine has lost its elasticity and it is difficult to achieve correction. The surgery’s objective is not so much to correct the curve/s by reducing the degrees, but to result in symmetry of spine and shoulders (which often stand at a different height prior to the operation).

During the typical posterior approach operation, the rods are used in pairs (left and right) and attached to the vertebrae with the use of hooks, screws or wires. Meanwhile, the bone (implant) is added – taken either from the patient’s pelvis or from a bone bank which fuses the vertebrae; the fusion process takes 3 to 6 months (sometimes more) and results in the permanent immobilization of the spine (the rods provide correction and temporary immobilization).

The surgeon can reach the spine in two ways, using either an anterior approach (via the thorax or the abdomen) or a posterior one.

 

The posterior approach

Access is achieved from the posterior of the vertebrae to which the materials that will be attached to the rod (screws or/and hooks, or/and wires). The rods are given the desired shape and adapted to the vertebrae; as a result, the curve finally adopts the shape of the rods. Lastly, the bone implant is added.

The anterior approach

There are some specific indications that point towards an anterior approach (either on its own or combined with a posterior one). The spine is reached through a side incision either in the chest or the abdomen after the noble organs have been moved aside. Then the discs are removed from in between the vertebrae, making the spine more flexible, and screws are placed on each of the vertebra to be fused. Again the rod (one is usually enough but two provide better στήριξη) is shaped outside the body and is adapted to the spine, locking on each vertebra so that the spine ends up taking the shape of the rod. The bone implant where the fusion will finally take place, replaces the discs.

 As a single operation it can be done on all types of scoliosis with a single curve (thoracic, thoracolumbar or lumbar), with the advantage of the same or a better result (correction-wise but also cosmetically due to the position of the scar on the body), fusing fewer vertebrae than in the posterior approach. That is of particular value to thoracolumbar and lumbar scoliosis where the effort is made to fuse as few vertebrae as possible so as to preserve movement in the waist and to avoid, in the future, the creation of arthritis and pain in the remaining free parts between the fusion and the pelvis.

 


RISKS AND COMPLICATIONS

The most significant risk is that of paraplegia that is, however, an extremely rate complication (2 out of 10,000 children: 0,02% according to SRS) that is caused to the disturbance of the spinal cord during surgery, either by one of the hooking materials or by a drop in the patient’s blood pressure that reduces the flow of blood to the spinal cord or even the change in shape of the spine and, consequently, of the spinal cord itself.

Inorderto prevent such an occurrence the spinal cold is monitored throughout the operation. The automatic activity of the nerves is recorded with the application of electrodes to the limbs and the head (spontaneous EMG_spEMG); so is the flow of electrical current from the limbs to the head (somatosensory evoked potentials_SSEP’s) and from the head to the limbs (transcranial motor evoked potentials_tcMEPs). The awakening/wake-up  test is also still used, although the electrical monitoring of the spinal cord is proven to be particularly reliable. 

For the awakening/wake-up test (called the Stagnara test in honor of its inventor) we wake up the patient after the rod has been placed – they feel no pain and they don’t remember the incident when they come out of surgery – and ask them to move their lower limbs.

If one of these two methods – electrical monitoring and awakening/wake-up test – show any pathology then the correction is loosened and if after the initial loosening there is still no movement in the lower limbs the spinal fusion materials are completely removed.

Another significant risk is great blood loss that is limited by careful technique so, usually, there is no need for a blood transfusion. Preventively the patient can choose to give a unit of their own blood, either right before the surgery or a month prior to it, so that the body can replace it in the interim. This blood is transfused back to the patient after the end of the operation. Also the cell saver system can be used that essentially recycles the blood that is lost during surgery.

 

POST-SURGICAL CARE

After the operation the patient can move whenever they feel comfortable in terms of pain and exhaustion; in most cases this happens on the day after the surgery. Patients are hospitalized for 4 to 7 days and can return to school in 2 to 4 weeks after the surgery.

It is important to stress that the longer the spine remains immobile the bigger the chance of the spinal fusion being successful. This is why use of a brace is often recommended post-surgery and movements such as twists of the torso, bending down and lifting weights are forbidden. Sports that involve contact, such as football or basketball are allowed after 6 months to a year, when the fusion of the implants is complete.

To observe the course of the spinal fusion we x-rays are made every 6 months, for 1 to 2 years. After the successful incorporation of the implant, no other treatment is needed. Spinal fusion in the thoracic spine especially, does not particularly affect the movement of the body. Spinal fusion materials rarely have to be removed. Scoliosis surgery – and scoliosis in general – does not in any way affect normal pregnancy and child-birth.

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