Congenital Scoliosis (Pediatric)

What is congenital scoliosis?

Congenital scoliosis is the presence of an abnormal curvature of the spine and affects newborns or young infants. Children with this condition begin to develop the curvature before birth while in the mother's womb. The curvature causes the spinal column to bend left or right in the shape of an S or C.

What causes congenital scoliosis?

Abnormal development causes the condition. It occurs when 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 high rate of concurrent spinal deformities associated with it, such as kyphosis (an abnormal forward-bending curvature) and lordosis (an abnormal backward-bending curvature), which also occurs independently.

Basic facts about congenital scoliosis are:

• Scoliosis is defined by the presence of a curvature in the spine of more than 10 degrees.
• 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 10-25 percent of curves are mild, remain stable, and never progress at all. Most curves, however, become worse and require treatment.
• Sometimes the ribs of children with progressive early scoliosis fuse together, reducing volume of the rib cage. In this condition, known as thoracic insufficiency syndrome, the limited chest volume interferes with lung growth and causes 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.

What causes congenital scoliosis?

There are two main causes of congenital scoliosis:

• Failure of formation is when 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.
• Error of segmentation is when bony regions of the vertebral column that normally grow into distinct segments fail to separate and end up fused together.

With either cause, the abnormalities often occur in multiple areas of the spine and more on one side than another. This asymmetry is responsible for the development of 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 responses to these forces.

How is congenital scoliosis diagnosed?

Congenital scoliosis is often first detected in an initial newborn physical examination by a pediatrician or noticed by parents of children soon after birth.

Due to increased incidence of other congenital abnormalities in the setting of scoliosis in newborns, a thorough examination must be performed. We use an abdominal ultrasound to inspect the kidneys and surrounding tissues and also do a cardiac echo.

In addition, a neurological examination of the back and extremities is performed to ensure no other spinal conditions are present. A spinal MRI may be done after one year of age. A physical is followed by a series of X-rays, which allow for a more precise measurement of the presence and severity of a curvature.

What is the treatment for congenital scoliosis?

Treatment depends on the type and location of the spinal deformity and the likelihood of the curvature worsening in the future.

Some mild curves (10-25 degrees) may be treated only with observation. This simply entails regular visits to one of our orthopaedic surgeons, who monitor the possible progression of the curve with physical exams and X-rays. Visits must continue into adolescence because growth spurts often trigger progression, even in a previously non-progressive curve. If progression occurs, bracing or surgery are treatment options.

Infants with moderate and severe curves have a greater chance of progression and often require casting and/or bracing treatment. Examples are:

Scoliosis Bracing

Bracing treatment is less commonly used in congenital scoliosis than other types of scoliosis 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. Braces are fit to patients using pads for comfort. Braces can be worn most of the time (except for bathing), and our orthopaedic surgeons monitor progression of the curve. Sometimes the curve is completely controlled by the brace, and after many years of bracing, no further treatment is needed.

Scoliosis Surgery

For curves that progress despite bracing treatment, surgery may be recommended. In most instances of congenital scoliosis, a surgical operation is the most appropriate treatment. Operations may consist of removing 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 grafts. Fusion operations may be followed by casting or bracing treatment or involve instrumentation, in which metal rods are attached to the spine to maintain curve correction. Another device used is a “growing rod,” which attaches to the spine and is periodically lengthened by a simple procedure too minimize any stunting of growth.

A spinal 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, research has shown that various treatments for congenital scoliosis are successful. The vast majority of children grow up to live healthy lives without major limitations to their activities and daily functioning.