Congenital scoliosis is a curvature of the spine that results from anomalies or abnormally developed vertebrae, the building blocks of the spinal column. These anomalies occur in utero at 4-6 weeks of gestation. Specific abnormalities include hemivertebra, which is a wedge-shaped or half vertebra, unsegmented bar, which is a failure of the normal separation of the individual building blocks of the spine, and mixed abnormalities. The number of abnormal vertebra, their location, and the growth potential around these abnormal vertebrae, is what determines how severe congenital curvature will become. For very mild single vertebra anomalies, a deformity may not be readily apparent and may be picked up incidentally on a chest x-ray or other study done for another purpose. In patients in whom multiple anomalies are noted, the trunk may be severely shortened and severe spinal deformity may be noted. In these cases, the curvature will often progress, resulting in severe lung disease and/or neurological deficits if left untreated.
Patients with congenital scoliosis also have a high incidence of abnormalities in other organ systems. For example, there is a 10% incidence of cardiac abnormalities, a 25% incidence of genito-urinary abnormalities, and up to a 40% incidence of intraspinal anomalies. Therefore, the patients are carefully worked up and even patients, who are seemingly otherwise normal, are sent for testing prior to surgery. Tests performed include an echocardiogram, renal (kidney) ultrasound, and screening MRI of the entire spine. Intraspinal anomalies that can occur include lipomas or fatty benign tumors of the spinal canal, scar tissue within the spinal canal, bony or cartilaginous spicules within the spinal canal, (diastematomyelia) and various other problems. These may require separate treatment from the spinal curvature itself.
The treatment for congenital scoliosis is aggressive in that if progression is noted, even for relatively small curves, surgery is indicated. This turns out to be the most conservative approach in that early surgery often allows the patient to avoid much more extensive surgery later. It is not uncommon for patients of one to one and a half years of age to undergo surgery that is relatively limited in nature. Nonoperative treatment consists of observation at 4 to 6-month intervals and if progression is noted, surgery is indicated. Bracing may be used in only a small percentage of patients in whom compensatory curvatures adjacent to congenital anomalies may be treated to prevent them from worsening.
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