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Orthopaedic Surgery

Congenital Scoliosis

Congenital scoliosis is a condition that affects newborns or young infants and is characterized by the presence of an abnormal lateral curvature of the spine. Children with this condition actually begin to develop the curvature before birth, during the period of fetal growth in the womb. The curvature causes the spinal column to bend to the left or the right, in the shape of an S or a C. Abnormal development is considered the cause of the condition. It occurs when the bony spinal segments, or vertebrae, fail to form properly, seemingly creating 'extra' segments, or when normally separate vertebrae fuse together during fetal development. The defects in the spine can be minor, involving only one segment of the vertebral column, or the condition can involve nearly every level and result in a more severe deformity. Congenital scoliosis has a relatively high rate of other, concurrent spinal deformities associated with it, such as kyphosis (an abnormal forward-bending curvature) and lordosis (an abnormal backward-bending curvature), which can also occur independently. Treatment ranges from observation to surgery, depending on the appearance of the spinal curvature.

Facts about congenital scoliosis:

  • Scoliosis is technically defined by the presence of a lateral curvature in the spine of >10 degrees deviation from straight upright (described further in the 'How is congenital scoliosis diagnosed?' section).
  • The abnormal curves in congenital scoliosis tend to be more rigid than those in idiopathic scoliosis, making them more resistant to correction.
  • Curve progression in congenital scoliosis, particularly with mild curves, is somewhat unpredictable, underscoring the importance of frequent follow-ups with an orthopaedic surgeon. Around 10 percent to 25 percent of curves, usually mild, remain stable and never progress at all. Most curves, however, progress and require active treatment.
  • Sometimes the ribs of children with progressive early scoliosis become fused together, thereby reducing the volume of the rib cage. In this condition, known as thoracic insufficiency syndrome, the limited chest volume interferes with lung growth, causing problems with breathing.
  • Neonates born with scoliosis have a relatively high rate of other congenital abnormalities, such as anatomical anomalies of the genito-urinary tract (found in around 20 percent of congenital scoliosis patients) or congenital heart defects (found in around 10 percent of congenital scoliosis patients).
  • Children with congenital scoliosis usually do not experience any pain from the condition.
The normal spine

The spine, or spinal column, as it is also called, is a complex structure made up of 33 vertebral segments arranged vertically in succession from just below the skull to the tailbone. The spine has two main functions: to support the weight of the skull and upper body, and to provide a protective encasement for the spinal cord, the long, cylindrical nervous structure that sends messages to and from the brain and the rest of the body. The vertebrae are attached to other vertebrae above and below them by a number of ligaments, thick fibrous structures found throughout the musculoskeletal system that attach bones to other bones. The connection points between the vertebrae are called joints, in which small areas of smooth cartilage lined with a lubricating material, known as synovial fluid, allow the bony vertebrae to slide past each other with minimal friction, allowing movement. Unlike joints such as the knee or elbow, which allow for considerable movement, each joint of the spinal column allows for very little movement because of the number of tightly attached ligaments, thereby ensuring the strength and stability of the vital supportive column. However, the joints of the spine act collectively, with the sum of movements in all the joints allowing movements such as bending forward, back, and to the side.

The normal spine actually has a number of natural curves to it, which stems from the evolution of humans from four-legged mammalian ancestors who walked on the ground bent over, with the spine parallel to the ground. However, these normal curves are antero-posterior curves only; that is, they can only be seen when looking at the spine from the side. On the other hand, when looking from directly behind or in front of a person, the spine maintains a rigid upright structure, like a straight line. In other words, there are normally no lateral curves to the human spine.

Causes of congenital scoliosis

As mentioned above, there are two major causes of congenital scoliosis. The first is called failure of formation, in which portions of one or more vertebrae do not grow together to completion during fetal development, making the spine unstable in certain regions and creating the appearance of 'extra' spinal segments. The second major cause is referred to as an error of segmentation, in which bony regions of the vertebral column that normally grow into distinct segments actually fail to separate and end up fused together. When evidence for both causes is present in a scoliotic spine, it is called a mixed deformity. However, with either cause, the abnormalities often occur in multiple areas of the spine, and they often occur more on one side than another, at least at some level of the spinal column. This asymmetry is responsible for the development of the lateral curvature in the womb, for as the muscles and ligaments develop around the spine and apply their natural forces to the vertebra, the two different sides have unequal stability and unequal responses to those forces.

Frequently, failures of formation and errors of segmentation also occur to an unequal degree on the front and back portions of one or more vertebrae. The result is not an abnormal lateral curve, but an abnormal forward-bending or backward-bending curve, known as kyphosis and lordosis, respectively. These conditions are commonly observed concurrently in newborns with scoliosis, and require treatment as well. Another set of conditions commonly seen in congenital scoliosis is rib abnormalities. Like the vertebrae, ribs are segmented structures that extend from the spinal segments in the back to the sternum, or breastbone, in the front. Thus, they are also affected by failures of formation, which leads to missing ribs, and errors of segmentation, which leads to fused ribs. Rib abnormalities may alter the normal shape of the chest cavity and compromise a child’s ability to breathe. In such instances, correcting the problems with the ribs will be incorporated into the treatment plan.

Diagnosing congenital scoliosis

Congenital scoliosis is usually first detected in the initial newborn physical examination by a pediatrician or noticed by a child's parents soon after birth. Because of the increased incidence of other congenital abnormalities in the setting of scoliosis in the newborn, a very thorough examination must be performed on such children, including auscultation (i.e. listening with a stethoscope) for heart murmurs and an evaluation of the renal tract (i.e. kidney and urinary tract). Therefore, all patients suspected of having congenital scoliosis should receive an abdominal ultrasound to inspect the kidney and surrounding tissues. In addition, a neurologic exam of the back and extremities will be performed to insure that no other spinal conditions are present and that the spinal cord is not being affected by the presence of the abnormal curvature. The physical will be followed by a series of X-rays, which allow for a more precise measurement of the presence and severity of a curvature. As mentioned before, this requires the presence of a curvature angle of at least 10 degrees, measured with the Cobb method.

Frequently, a spinal MRI, another radiological imaging technique, will be taken to compliment the spinal X-rays. Once a clear radiological picture of the spine has been attained, a pediatric orthopaedic surgeon can often predict the chance of curve progression based on the specific appearance of the curve, and will make treatment decisions based on this prediction.

Treating congenital scoliosis

Decisions regarding the appropriate treatment for congenital scoliosis depend on the type and location of the spinal deformity and the likelihood that the curvature will worsen in the future. Some mild curves (10-25 degrees) may be treated only with observation. This entails simply that the child be seen regularly by an orthopaedic surgeon, who will monitor the possible progression of the curve with physical exams and X-rays over time. Visits must continue into adolescence, because growth spurts often trigger progression, even in a previously non-progressive curve. If progression does occur in this group, the patient may require bracing or surgical treatment.

Bracing treatment is less commonly used in congenital scoliosis than other types of scoliosis, mostly because the curves tend to be more rigid in newborns, and do not respond to the gentle forces of a brace. In some instances, however, bracing is appropriate and will be fitted individually for the patient, with a number of pads to maximize the child's comfort in the device. Except for bathing, the brace is to be worn all the time, and the progression of the curve will be monitored closely by your orthopaedic surgeon. Occasionally, the curve is completely controlled the brace, and after many years of bracing, no further treatment is needed. For curves that progress despite bracing treatment, prompt surgery is warranted.

In most instances of congenital scoliosis, a surgical operation is the most appropriate treatment. Operations may consist of removal of the 'extra' vertebrae or some type of spinal fusion, in which two or more of the vertebrae are fused together with bone bridges made of bone grafts. Surgeries may be performed through a posterior approach, in which the operation is performed with an incision in the back, or also with an anterior approach, which is a more extensive surgery that requires making an opening in the chest wall to reach the front part of the spinal column. Fusion operations may be followed by casting or bracing treatment, or may involve instrumentation, in which metal rods are attached to the spine to maintain curve correction. A device known as a "growing rod" may also be utilized, which is attached to the spine and periodically lengthened by a simple procedure, and will minimize any stunting of growth that could occur in certain cases.

For children that suffer from thoracic insufficiency syndrome, the titanium rib prosthesis is a revolutionary new type of treatment used to compliment scoliosis surgery. The expandable prosthesis is implanted in a vertical position and attached to an upper and lower rib. The device helps maintain the chest space where there are fused or missing ribs, thus allowing room for heart and lung development. As the patient grows, the prosthesis is simply expanded through an outpatient surgical procedure.

While this procedure is new and only appropriate for a small number of children with scoliosis, it offers an exciting new option to children with congenital scoliosis.

Coping with congenital scoliosis

A major spine operation during a child's neonatal or infant stage can be an emotional experience for many parents. Managing the demands of bracing or casting treatment for scoliosis has its drawbacks as well. However, the importance of such treatment steps at an appropriate, early age cannot be overemphasized for the future health of your child. Moreover, research has shown that the various treatments for congenital scoliosis are generally extremely successful. The vast majority of children grow up without major limitations to their activities and daily functioning, from simple outdoor games to competitive sports. Thus, despite some challenging hurdles very early in childhood, with treatment your child may be expected to live a normal, healthy, active life, and walk tall into adolescence and adulthood.

For more information

Naturally, you may have other questions about congenital scoliosis that are not answered in the above summary. If your child is not in the neonatal age group or may have another type of scoliosis, please refer to our descriptions of other types of scoliosis, found here.

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