Scoliosis is an abnormal curvature of the spine that affects approximately seven million people in the United States. Scoliosis is a condition where the spine curves abnormally to the right or left side and also involves a twisting or rotation of the bones of the spine. 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.
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 occurring 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 most common malformation seen in congenital scoliosis is a hemivertebra or unilateral bar. A hemivertebra (half a vertebra) refers to a condition where 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 curvature. Unilateral bar (or block vertebra) is a condition in which the bones of the spine do not properly separate from each other. 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.
Diagram showing congenital curves. Starting at the left shows a hemivertebra, in the middle is a unilateral unsegmented bar, and at the right a block vertebra. These bones have abnormal alignment and can lead to progressive curvature as the spine grows.
Surgery is needed for congenital scoliosis if there is progression of the spinal curvature. Patients with congenital scoliosis are 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 spine levels requiring treatment.
For patients with congenital scoliosis, surgery may involve removing 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 growth on both sides may be appropriate.