Congenital Scoliosis: Symptoms, Treatment, Outlook
What is congenital scoliosis?
Congenital scoliosis is a side-to-side curving of the spine caused by the bones of the spine (vertebrae) not forming properly in the womb. The spine may also be twisted or rotated, which can affect the ribs and shoulders.
This type of defect is present in a baby at birth, although it may not be noticed until later in life when the curve worsens. Not every infant with scoliosis has congenital scoliosis — some have a curve even though their vertebrae are normal.
Symptoms of congenital scoliosis include:
- Uneven shoulders with one shoulder blade sticking out more than the other
- Ribs appearing more pronounced on one side
- Uneven hip or waist
- Tilting of the head caused by a rotation of the neck
- Lean to one side while standing
- In rare cases, a child may have weakness, numbness or loss of muscle control caused by a problem with the spinal cord or nerves
Congenital scoliosis is a type of birth defect. Two kinds of problems may occur during a child’s development in the womb to cause this condition. Some children may also have multiple problems at different locations on the spine.
Vertebrae not forming normally. One or more vertebrae may be partially formed or completely absent in babies with congenital scoliosis. One type of this is known as a hemivertebra, in which part of a vertebra doesn’t form on one side of the spine. This can cause the spine to curve at that location.
Vertebrae not separating correctly. Vertebrae are normally separate bones connected by joints. In congenital scoliosis, two or more bones of the spine may remain joined on one or both sides of the spine. This causes the spine to grow more slowly on the affected side and can lead to a curve at that location.
Depending on what type of spine defect is present, a curve may develop at that location. Children may also have curves above or below the affected area — these help them stay in an upright position.
Children with congenital scoliosis may also have other health issues, such as problems with the heart, lungs, kidneys, bladder, stomach, or nerves. These organs develop in the embryo at the same time as the vertebrae.
Several risk factors have been linked to congenital scoliosis. Often, more than one factor is involved in increasing the chance that an infant will be born with congenital scoliosis. These include:
- Environmental factors. During the first two months of pregnancy, the vertebrae of the embryo (early fetus) are still forming. Exposure to certain factors during this time may cause problems with the vertebrae. These factors include lack of oxygen in the blood going to the fetus, and certain drugs and hormones such as thalidomide, lovastatin, and some types of progesterone/estrogen.
- Genetic factors. Genes inherited from one or both parents may increase an infant’s chance of having congenital scoliosis. This is less common than environmental factors. However, with certain rare medical conditions, genetic factors are the main cause of congenital scoliosis.
Children who have defects in their vertebrae at birth do not always have scoliosis at that time. The abnormal curving may not develop until later. Scoliosis is also not usually painful, so it may not be noticed until later. A doctor uses the following to diagnose congenital scoliosis and related medical conditions:
- Medical history. A doctor will ask about the child’s symptoms and possible exposure to medications or hormones while in the womb, as well as the family’s history of scoliosis or related conditions.
- Physical exam. A doctor will look for abnormal curves of the spine while the child is in a standing position and while bending. The doctor will also look for other problems, such as difficulty walking, slow or absent reflexes, or a loss of feeling in the legs or other parts of the body.
- X-rays. These use radiation to create an image of the vertebrae of the spine. They show the type, location and severity of the curves. A child may need x-rays every three to six months to look for worsening of the curves.
- Magnetic resonance imaging (MRI). This uses powerful magnets to produce a more detailed image of the spine, including the bones and the spinal cord and nerves. It may be used when a child has neurologic symptoms — such as difficulty walking or slow reflexes — or to prepare for surgery.
- Computed tomography (CT) scan. This uses x-rays and a computer to produce more detailed images of the body than x-rays alone. It can also detect problems with the ribcage.
- Other medical tests. These may be needed to diagnose problems with the heart, genitals or urinary system, including the kidneys.
If congenital scoliosis is diagnosed in infancy, treatment may be started as early as six months of age. The goals of congenital scoliosis treatment are:
- Stop the curve from worsening
- Preserve the function of the spinal cord and nerves, including the ability to walk and move
- Treat or prevent conditions of the heart and lungs
- Correct deformities of the spine
Watch and wait. For less severe curves, a doctor may recommend regular monitoring to see if the curve worsens.
Spine bracing. Bracing helps straighten the spine and can reduce pressure on the child’s lower back. This treatment doesn’t work as well for congenital scoliosis as it does for idiopathic scoliosis, an abnormal curve of the spine that develops without defects of the vertebrae.
The most common types of congenital scoliosis surgery are growing rods and spinal fusion. If a child has other health issues—such as heart, lung, or kidney problems — they may need separate treatments for those.
Growing rods. During this surgery, one or two rods are attached to the spine under the skin. The rods are attached above and below the curve to help the spine grow straight. The rods need to be lengthened every few months as the child grows.
Spinal fusion. This surgery involves straightening and fusing two or more vertebrae with bone grafts and metal implants. This stabilizes the spine and reduces the curve. It may be used after the child has stopped growing, or in place of growing rods if those are not a good treatment option for the child.
Hemivertebra treatment. If a misshapen vertebra is causing the abnormal curve, a surgeon may remove it in order to straighten the spine. The vertebrae above and below are then fused to stabilize the spine at that location.
Vertical expandable prosthetic titanium ribs (VEPTR) treatment. This surgical treatment is used if the congenital scoliosis affects the development of a child’s ribs and lungs. This condition can cause difficulty breathing and can sometimes be fatal without treatment. VEPTR stabilizes the spine and allows the child’s spine, ribs and lungs to grow.
Exposure of the embryo to certain drugs and hormones during early pregnancy increase the chance that a child will be born with congenital scoliosis. Women who are pregnant or intending on getting pregnant should check with their doctor before starting any medications or hormones.
However, other factors may also cause congenital scoliosis, including a lack of oxygen in the blood going to the embryo. It may not be possible to prevent these.
For many children with congenital scoliosis, the curve will worsen over time, either slowly or more rapidly. How fast and much a curve worsens depends upon the child’s age, the location of the curve, and the type of spinal defect.
Curves are more likely to get worse when children are growing rapidly — during the first few years of life and during the pre-teen and early teen years. If the defect is severe, the curve may worsen even after they have stopped growing.
Children whose congenital scoliosis is detected early have the best outlook. This allows them to undergo treatments that can restore all or most of their normal function.
Congenital scoliosis is a potentially serious condition. Early diagnosis and treatment offers the best outlook for the child, for both the defects of the spine and related medical conditions such as problems with the heart and lungs. Parents who are concerned about their child’s spine or health should contact their doctor.