Congenital scoliosis is defined as a curvature of the spine that is the result of malformations of the vertebral elements. The fact that the spine and spinal column ever form correctly is amazing given the complexity of the process from an embryological standpoint. Most of this development happens during the third to sixth week in utero (after conception). In spite of the opportunities for error, congenital malformations are relatively rare.
The elements of the spinal column develop at the same time as several other major organ systems such as the bladder, kidneys, and heart. As a result, there is an association between congenital vertebral anomalies and other malformations. For example, there is a 20% incidence of associated genitourinary anomalies in children with congenital scoliosis. Malformations of the spinal cord are also common, and medical attention should be sought if there is any suggestion of an intraspinal problem such as dimples or hairy patches over the skin of the back, pain, or spasticity in the lower extremities.
If your child is thought to have congenital scoliosis, the evaluation should begin with a thorough physical examination in search of associated congenital anomalies. Taking x-rays of the entire spine will determine if congenital scoliosis is present. X-rays will also allow your doctor to determine the type and severity of the congenital vertebral malformations, which helps predict the long-term risk that your child's curve may get worse with growth. A renal ultrasound is usually recommended as part of the routine screening assessment if congenital scoliosis has been confirmed. An MRI of the spinal cord may be indicated if there is suspicion of a spinal cord abnormality or unexplained rapid progression of the curve.
Doctors think about congenital scoliosis in three groups: failures of formation, failures of segmentation, and combinations of these defects. The most common failure of formation is called a hemivertebra. Hemivertebra produce a growth imbalance in the spine and, therefore, result in the spine growing crooked. Failures of segmentation include block vertebra and unilateral bars, which produce a growth tether of the spine. Finally, when these occur in combination, such as a hemivertebra on one side and a bar on the other, the scoliosis can progress in very rapid manner.
When your doctor recommends a treatment plan for congenital scoliosis, it is based on the prediction of the potential abnormal growth associated with your child's particular pattern of spinal malformations. Some patterns of congenital scoliosis have a low potential for significant progression with growth, whereas others will progress aggressively at a very early age. Each pattern of malformation is unique and a treatment plan is made based on the risk of curve progression during growth.
Treatment options in congenital scoliosis include observation, bracing, or surgery. The goals of treatment are to allow the child to reach the end of growth with a reasonably straight, balanced spine, and to allow the spine to grow as much as possible. For younger children, allowing for the chest cavity to grow and develop and allowing the lungs to increase in size is becoming increasingly recognized as an important consideration in the overall treatment plan.
Observation is appropriate for small curves and balanced patterns of malformations. Some patterns of congenital scoliosis are minor enough that no treatment is needed and the long-term prognosis is good.
Bracing is rarely used in congenital scoliosis as the primary treatment. Bracing has never been shown to profoundly change the pattern of abnormal growth that results from most vertebral malformations. It is sometimes useful to brace secondary curves that result from the congenital malformation.
Surgery is used to attempt to maintain spinal balance while preserving as much trunk height as possible during growth. Procedures must be individualized, and should be attempted only by those surgeons with experience in the management of congenital spinal deformities.
This is a 13-month-old boy who presented with isolated hemi- vertebrae at the first lumbar vertebra. The secondary growth imbalance has produced a 30-degree scoliosis.
This boy presented with an eight-toed clubfoot. Further examination revealed that he had severe congenital scoliosis. The pattern of a unilateral failure of segmentation with a contralateral failure of segmentation is the most aggressive form of congenital scoliosis and requires early aggressive intervention. His pre-operative MRI was normal, but he went on to develop a symptomatic tethered spinal cord at age 4. A single horseshoe kidney required a urological procedure at an early age. This child emphasizes how multiple anomalies may be associated with congenital scoliosis.
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