Congenital Scoliosis
Scoliosis is an abnormal curvature of the spine that affects approximately seven million people in the United States. For patients with scoliosis, the parent or the patient are usually the first to notice the scoliosis but sometimes it is not discovered until a school screening or a doctor's visit. Most patients with scoliosis do not have many symptoms, but larger curves may cause outwardly noticeable deformity (such as a rib hump) or pain. In severe cases the curve may even begin affecting the lungs or heart function.
Scoliosis is a condition where the spine curves abnormally to the right or left side [Figure 1]. Scoliosis is "three dimensional" when the spine curves from side to side as well as twisting. As the spine rotates this may cause clinical changes in the appearance of the patient's back and may also lead to painful degeneration of the spine or alter organ function [Figure 2].
Figure 1: For patients with scoliosis, the spine develops a curvature as seen in the drawing on the right.
Figure 2: A photograph of a patient with scoliosis taken from behind. The curvature is seen between the shoulder blades (thoracic spine).
The term congenital means "present at birth." Congenital scoliosis refers to a curvature of the spine that is the result of a malformation of the spine occuring before the child is born. Congenital scoliosis occurs in only one of every 1,000 births. Congenital scoliosis may be associated with defects in other parts of the body, so it is important for patients with congenital scoliosis to undergo screening for these other conditions. For patients with congenital scoliosis there is a 10% incidence of associated cardiac abnormalities, 40% incidence of other spinal anomalies, and 25% incidence of genital or urinary abnormalities. The doctors at the Virginia Spine Institute may recommend testing on the the genital and urinary systems, heart, and other parts of the spine for patients with congenital spinal conditions.
The most common malformation seen in congenital scoliosis is a hemivertebra or unilateral bar. A hemivertebra (half a vertebra) refers to a condition when the bone of the spine does not grow properly. This can cause one side of the spine to grow faster than the other side, ultimately resulting in a spinal curvature. Unilateral bar (or block vertebra) is a condition in which the bones of the spine do not properly separate from each other [Figure 3] [Figure 4]. Depending on the location and type of malformation, different curvatures of the spine may develop. Many patients with small congenital malformations of the spine have no significant symptoms or any curvature that develops.
Figure 3: Diagram showing hemivertebra (right) and unilateral bar (left). These bones have abnormal alignment and can lead to progressive curvature as the spine grows.
Figure 4: A 3-Dimensional CT scan reconstruction demonstrating a hemivertebra (red arrow). This patient has developed an angular scoliosis because the hemivertebra is only growing on one side of the spine.
OPERATIVE TREATMENT OPTIONS:
Surgery is needed for congenital scoliosis if progression of the spinal curvature is noted. Patients with congenital scoliosis are usually observed closely to determine if progression is occurring. Bracing is only used in a small percentage of patients who develop compensatory curve above or below the congenital anomaly. Surgical intervention is best performed early to minimize the curvature and number of levels of the spine requiring treatment.
For patients with congenital scoliosis, surgery may involve removal of a portion of the abnormal bone. Resection of a hemivertebra may allow the spine to straighten and prevent further abnormal growth curvature. For patients who are developing curvature because one side of the spine is growing faster than the other, surgery to fuse the spine in that area to equalize the growth of the spine on both sides may be appropriate [Figure 5].
Figure 5: This is an eight year old boy with a congenital hemivertebra. He underwent removal of the hemivertebra and posterior fusion with pedicle screws. X-rays before and after show removal of the hemivertebra and improvement in spinal alignment.
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