Congenital Scoliosis

Congenital scoliosis is a condition that affects newborns and young infants and is characterized by an abnormal 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.

Spinal defects can be minor, involving only one segment of the vertebral column, or they can involve nearly every level and result in a more severe deformity.

Congenital scoliosis is often associated with other concurrent spinal deformities, 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.

Some Basic Facts About Congenital Scoliosis

• Scoliosis is technically defined by the presence of a curvature in the spine of >10 degrees.
• 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% to 25% 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 early progressive scoliosis fuse, thereby reducing the volume of the rib cage.  In this condition, known as thoracic insufficiency syndrome, the limited chest volume impairs lung growth, leading to breathing difficulties.
• Neonates born with scoliosis have a relatively high rate of other congenital abnormalities, such as genitourinary tract anomalies (found in around 20% of congenital scoliosis patients) or congenital heart defects (found in around 10% of congenital scoliosis patients).
• Children with congenital scoliosis usually do not experience any pain from the condition.

What Causes Congenital Scoliosis?

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 a segmentation error, in which bony regions of the vertebral column that normally grow into distinct segments fail to separate and end up fused.

In either case, the abnormalities often involve multiple areas of the spine and are more pronounced on one side than the other. This asymmetry is responsible for the development of curvature in the womb, as the muscles and ligaments develop around the spine and apply their natural forces to the vertebrae, the two sides have unequal stability and unequal responses to those forces.

How Is It Diagnosed?

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.  

All patients suspected of having congenital scoliosis should receive an abdominal ultrasound to inspect the kidney and surrounding tissues and a cardiac echo.

In addition, a neurologic exam of the back and extremities will be performed to rule out other spinal conditions. A spine MRI is also done to inspect the spinal cord, but that is typically done after one year of age. 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 a curvature angle of at least 10 degrees, measured with the Cobb angle.

How Is It Treated?

Decisions regarding the appropriate treatment for congenital scoliosis depend on the type and location of the spinal deformity, as well as the likelihood that the curvature will worsen in the future.

Nonsurgical Treatments

• Some mild curves (10-25 degrees) may be treated only with observation. This simply entails that the child be seen regularly by an orthopaedic surgeon, who will monitor the possible progression of the curve through 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.
• Infants with moderate and severe curves have a greater chance of progressing and often require casting and/or bracing treatment.
• Scoliosis casting may be an option, especially if the curve is flexible.
• 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 at all times, and your orthopedic surgeon will closely monitor the progression of the curve. Occasionally, the curve is completely controlled by the brace, and after many years of bracing, no further treatment is needed. 

Surgical Treatment

For curves that progress despite bracing treatment, surgery may be recommended. Operations may consist of:

• The removal of the ‘extra’ vertebrae
• Some type of spinal fusion, in which two or more of the vertebrae are fused together with bone bridges made of bone grafts.  
  • 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, thereby minimizing any growth stunting that could occur in certain cases.

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 these treatment steps at an appropriate, early age cannot be overemphasized for your child's future health.

Research has shown that treatments for congenital scoliosis are generally highly effective. The vast majority of children grow up without major limitations to their activities and daily functioning,

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.