Acıbadem Comprehensive Spine Center
SPINAL DISORDERS CONGENITAL SCOLIOSIS

Scoliosis

Congenital Scoliosis

What is a congenital spinal deformity?

The development of a child's spine inside the uterus is completed during the first three months along with the development of the organs. If the structure of the spine is formed abnormally during this period or fails to separate, the vertebrae grow asymmetrically and spinal deformities develop. The type of deformity depends on the location and the direction of the abnormal vertebra.

A normal spine looks straight from the back and curved from the side. These curves are backward in the upper part of the back (kyphosis) and forwards in the lower part of the back (lordosis). In congenital cases, if the asymmetrical development is to the side it results in scoliosis, whereas if it is forwards this results in an increased kyphosis.

How does a spinal deformity develop in people with an abnormal vertebra?

As already mentioned, the most significant factor that causes deformation of the spine is the asymmetrical growth of abnormally formed vertebrae.

In cases where one side of a vertebra a fails to separate and remains fused, the fused side cannot grow, however the free side continues to grow, resulting in scoliosis. If both sides of the vertebra are fused, there is little or no growth in the fused area. This does not result in scoliosis, but this section can remain short. If the vertebra are fused from their front part (anterior aspect), a hunchback (kyphosis) will occur because growth continues from the back (posterior aspect).If the vertebrae are fused at the back, growth will continue from the front and a concavity (lordosis) will result.

Hemivertebrae cause one side of the spine to grow more than the other. While the vertebrae which are joined together (fused vertebrae) prevent the growth on their side, the other side grows normally and eventually results in a spinal curve . An important point is that although the abnormal vertebrae are present from birth, a curvature can be barely recognized initially. The curve develops with growth. Despite growth however, in a significant number of cases the spine may not develop a curve at all, or develop only a minor curve. If several abnormal vertebrae in the spine are distributed such that they balance each other, there may be limitation in truncal height rather than an increase in the curve.The curve can increase very slowly until the rapid phase of growth during puberty.

What are the symptoms of congenital scoliosis?

- A curve to the side, abnormal gibbosity or lordosis.
- Skin anomalies on the back: increased hair growth, dimples, discolouration.
- Abnormally long arms or legs.
- Unequal shoulders, waist or hips.
- Disproportionate shortness of the body compared to the legs.
- Balance disorders.
- Curving of the back which is noticeable when leaning forwards.

Is congenital scoliosis hereditary?

Congenital scoliosis is generally not considered to be hereditary. However, congenital scoliosis may be associated with the congenital disease in other possibly inherited conditions. Therefore, parents who have a child who has only congenital scoliosis and no other genetic diseases do not have an increased risk of having another child with congenital scoliosis. The cause of congenital scoliosis is not known exactly.

Some conditions may be observed more commonly together with congenital scoliosis due to some events that occur during the development of the embryo and fetus.

The most common are:

- Kidney and bladder system anomalies - 30%
- Spinal cord anomalies - 15%
- Congenital heart diseases - 12%
- These anomalies may or may not be functionally significant.
- Furthermore, spinal deformities are associated with various syndromes (Klippel-Feil syndrome, VACTERL, Goldenhar syndrome, fetal alcohol syndrome etc.)

Congenital scoliosis and diagnosis

Congenital scoliosis is initially diagnosed by examination. Special x-rays, MRIs, ultrasound and other tests may be necessary. The results of these examinations provide information on associated problems and the future behaviour of the deformity.

Congenital scoliosis and patient observation

In this stage of scoliosis, a phase known as "controlled observation" starts, in which a doctor observes how the deformity is behaving. This is done through regular examinations and x-rays. If the curvature continues to increase or other functional problems occur, a suitable surgical intervention must be carried out.

Congenital scoliosis and brace treatment

One of the most significant differences between congenital scoliosis and idiopathic scoliosis is the lack of efficacy of the brace treatment in congenital scoliosis. The body sometimes forms an additional curve above and below the congenital scoliosis curves in order to maintain balance. These curves can increase over time, and sometimes become even more serious levels than the congenital curve. A brace can be used in these patients in order to keep the second curve under control or prevent its progression.

Congenital scoliosis and treatment options

Controlled observation:

This is carried out at regular intervals through examinations and x-rays until the skeleton is fully grown (this is continued after the operation), and no special treatment is made unless there is an increase in the curve (scoliosis).

Surgery:

Today most surgical treatments applied for the management of congenital spinal deformities in still growing children aim to control the curve without any fusion or by with fusion of only a limited area.

Limited fusion operations

Hemivertebrectomy:

In some cases, the curve can be corrected by removal of the abnormal vertebra (hemivertebrectomy). This operation can be carried out from the back, yet some surgeons prefer to use both the front and the back. The patient is placed in a body cast for three to six months following the operation.

Management of the curve using growing rods:

In very small children if the curve is suitable for this method, correction can be achieved by rods connected to each other with screws placed above and below the curve. Then by periodic expansions of the device every 6 months, an attempt is made to keep the curve under control until the child has grown up, so that fusion is carried out when the patient has become an adult. The aim of this technique is to prevent the torso from remaining too short by preserving growth, and enable the lungs and the chest to grow to normal dimensions. Magnetic rods which have been introduced recently can be lengthened in the clinics without a need for repeated operations.

Operation to expand the chest (VEPTR):

Some of the patients with congenital scoliosis have associated rib anomalies and insufficient development of the chest. In these children, rods inserted into the thoracic cage provide both correction of the chest anomaly and management of the curve without fusion. These patients may require periodic expansions every 6 months.

Instrumentation and fusion, osteotomies:

The most difficult types of scoliosis are neglected curves above 70-80 degrees. Using modern techniques and experience, it is possible to correct these curves with or without removal of the deformed vertebrae and by fixation of the vertebra using titanium rods and screws.

When is an operation necessary in congenital scoliosis and is there an age limit?

Delay or disruption of the asymmetric growth of the abnormal vertebra is known as the most effective treatment for congenital scoliosis. This is often done by means of a fusion of the vertebrae (freezing the spine and eliminating movement). In order to control a progressive deformity, the operation may need to be performed when the child is fairly small. Parents are understandably concerned that early fusion (because the fusion prevents growth in the operated area) will prevent the growth in the torso. Although this is true to a certain extent, the torso height cannot regained by correction of a severe deformity once growth has ceased, and delaying surgical treatment solely for this anxiety may lead to severe problems in the patient's future life.

When early surgery is indicated in a patient with congenital scoliosis, the operation can be carried out at any age, however the oparation is generally delayed until the patient is one year old. If a scoliosis operation is necessary after this age, it should be done without a further delay..Despite an early initial operation, additional operations may be necessary if the first operation fails to control the curve. In actively growing children fusion from both the front and the back may be necessary to bring the scoliosis under control. These techniques can be used in both scoliosis and kyphosis. Autogenic (own) or allogenic (donated) bones, bone substitutes or a combination of the above sources can be used for the fusion.

If associated organ problems are identified, a separate plan must also be made for their management. Anomalies in the spinal cord should primarily be treated together with the spinal curves. While no treatment is necessary for some spinal cord abnormalities, others require an operation before or during the treatment of the curve.

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