Scoliosis is a spinal deformity in which the spine has an abnormal sideways curve (a lateral curve). The spine may curve to the right or to the left, and it may be a single curve (known as a c-curve) or a double curve (known as an s-curve).
Congenital scoliosis means that as the patient’s body developed before birth, one or more spinal vertebrae were not properly formed. There is no known cause for this abnormality, which usually occurs within the first six weeks of pregnancy. The patient may be diagnosed as an infant or young child, but it often takes until adolescence for the condition to be discovered.
Types of Spinal Abnormalities Resulting in Congenital Scoliosis
One common anomaly that results in congenital scoliosis is called a failure of segmentation. In a typical spine, each vertebra is a separate building block for the spinal column. However, in some cases two or more vertebrae may be partially or fully joined together (fused). This fusion of two or more vertebrae can cause significant problems, including a slower rate of growth for one side of the spine. As the other side continues to develop at a normal rate, the spine will curve over into a deformity.
Another problem that may occur is known as a failure of formation. This means that proper segmentation has taken place, but one or more vertebrae is abnormally shaped. A vertebra should be rectangular, but if it has not formed properly, it may be triangular or wedge-shaped. This is known as a hemivertebra, or a half-vertebra.
Several vertebrae may be affected by these abnormalities. A patient may also have a combination of anomalies in his or her spine. Some vertebrae may be unsegmented while others may not be fully formed. The curvature of the spine may be fairly small if only one vertebra is affected. On the other hand, if multiple abnormalities exist, the curve may be very serious. The patient may even have a shortened torso as a result of these congenital abnormalities.
The complications caused by congenital scoliosis can range from relatively mild to serious. If the patient has a severe scoliosis curve due to the malformation of his or her spinal column, or if the curve is progressive, serious complications may occur. Congenital scoliosis can cause one shoulder or hip to appear more prominent than the other, and it may result in an uneven waist, leg lengths may appear unequal, and one side of the rib cage may be more prominent than the other. The patient is often unbalanced as the torso shifts to one side.
In serious cases, a curved spine can push the rib cage into a strange position, causing chest pain. The ribs may also press against the heart or lungs, resulting in cardiopulmonary problems, one of the most common being shortness of breath. The vertebrae may also pinch the nerves around the spinal column, which can cause pain and neurological damage.
Though not caused by scoliosis, other anomalies may also be present along with congenital scoliosis. This is because the patient may have experienced other developmental problems in utero at the same time that the spine was forming. Other organs that might have been affected are the heart and the kidneys. Therefore, a patient with congenital scoliosis may also need treatment for other problems, and diagnosis of scoliosis may prompt several other tests to rule out further deformations within the body.
Diagnosis of Congenital Scoliosis
A physical examination will often reveal the external signs of scoliosis. A severe curve will be easily visible from behind as the patient stands normally. The torso will list to one side and a visible bend may be present. The shoulders and hips may be uneven. A forward bending test may reveal one prominent shoulder blade that juts higher than the other.
After doing a visual test, the doctor will order x-rays and possibly an MRI. The x-rays, taken both from front to back and from the side, will reveal if the spine curves. A separate x-ray of just the neck will also be taken. In order to determine the shape of each individual vertebra, an MRI will be taken to show a three-dimensional picture of the spine. This will allow the doctor to take note of unsegmented sections of the spine or any existing hemivertebrae.
Treatment Options for Congenital Scoliosis
While other forms of scoliosis usually do not require surgery, and physicians will more often use bracing or a “watch and see” approach, congenital scoliosis is a different matter. Even for small curves, any signs of progression will usually lead a doctor to recommend surgical treatment. Because this problem is present at birth, even small children may require surgery in order to prevent further complications and further development of the curve.
One treatment that may be recommended is a surgery to remove a hemivertebra. This hemivertebra excision allows the spinal curve to be corrected, and if it is performed soon enough, the remaining, adjacent sections of the spine will be able to continue developing normally.
More complex surgeries may require treatment of multiple levels of the spine. Spinal fusions may be necessary in both the front and back of the spine in order to correct the curve and stabilize the spine. Rods, hooks and screws may be used to stabilize the spine.
Since the patients are often young children, caution must be taken to avoid holding back normal growth. In order to promote further development of the spine, the patient may need to have a growing rod procedure. Instead of fusing together the bone, which deters growth, special implants may be used in a way that supports the spine while allowing it to develop. This is usually done for patients under eight years of age. At a later date, when normal growth has stopped, a fusion may be recommended to keep the spine in place.
Because of the risk of lung disease and neurological disorders if congenital scoliosis is left untreated, early diagnosis and intervention is important. If a young child undergoes surgery to correct this curvature of the spine, he or she is less likely to need spinal surgery as an adult patient. Patients are often able to resume their normal physical activities with no restrictions after several months.
The treatment of congenital scoliosis has come a long way in the last 25 years, with specially trained surgeons, like Dr. Rahman, better equipped than ever to manage the challenges of this spinal problem.