Congenital Scoliosis

Congenital scoliosis is a sideways curvature of the spine that is caused by a
defect that was present at birth. It occurs in only 1 in 10,000 newborns and is
much less common than the type of scoliosis that begins in adolescence.
Children with congenital scoliosis sometimes have other health issues, such as
kidney or bladder problems.

Even though congenital scoliosis is present at birth, it is sometimes impossible to
see any spine problems until a child reaches adolescence.

Types of Congenital Scoliosis

A single hemivertebra in the lower back shown in a 3-D image from a computed
tomography (CT) scan. Note the four normal rectangular vertebra below the
single triangular-shaped hemivertebra (arrow). This wedged vertebra creates the
deformity that would not have otherwise been there.


Incomplete Formation of Vertebrae

As the spine forms before birth, part of one vertebra (or more) may not form
completely. When this occurs, the abnormality is called a hemivertebra and can
produce a sharp angle in the spine. The angle can get worse as the child grows.
This abnormality can happen in just one vertebra or in many throughout the
spine. When there is more than one hemivertebra, they will sometimes balance
each other out and make the spine more stable.

Failure of Separation of Vertebrae

During fetal development, the spine forms first as a single column of tissue that
later separates into segments that become the bony vertebrae. If this separation
is not complete, the result may be a partial fusion (boney bar) joining two or
more vertebrae together.
Such a bar prevents the spine from growing on one side after a child is born.
This results in a spinal curve that increases as a child grows.


Combination of Bars and Hemivertebrae

The combination of a bar on one side of the spine and a hemivertebra on the
other causes the most severe growth problem. These cases can require surgery
at an early age to stop the increased curvature of the spine.

Compensatory Curves

In addition to scoliosis curves, a child's spine may also develop compensatory
curves in order to maintain an upright posture. This occurs when the spine tries
to make up for a scoliosis curve by creating other curves in the opposite
direction above, or below, the affected area. The vertebrae are shaped normally
in compensatory curves.

Symptoms

This 4-year-old girl's body shifts to the left. One hip looks higher than the other
and her waistline is not the same shape on the two sides.
Congenital scoliosis is often detected during the pediatrician's examination at
birth because of a slight abnormality of the back.
Scoliosis is not painful, so if the curvature is not detected at birth, it can go
undetected until there are obvious signs --which could be as late as
adolescence. A child may suspect that something is wrong when clothes do not
fit properly. Parents can discover the problem in early summer when they see
their child in a bathing suit.

The physical signs of scoliosis include:

• Tilted, uneven shoulders, with one shoulder blade protruding more than the other
• Prominence of the ribs on one side
• Uneven waistline
• One hip higher than the other
• Overall appearance of leaning to the side
• In rare cases there may be a problem with the spinal cord or nerves that
   produces weakness, numbness, or a loss of coordination.


Doctor Examination and Investigation

Physical Examination

The standard screening test for scoliosis is the forward bending test. Your child
will bend forward and your doctor will observe your child from the back, looking
for a difference in the shape of the ribs on each side. A spinal deformity will be
most noticeable when your child is in this position.
With your child standing upright, your doctor will check to see if the hips are
level, the shoulders are level, and that the position of the head is centered over
the hips. He or she will check the movement of the spine in all directions.
To rule out the presence of a spinal cord or nerve problem, your doctor may
check the strength in your child's legs and the reflexes in the abdomen and legs.

Tests

This adolescent girl's curve and the hemivertebra causing it show up clearly on
x-ray.
Although the forward bending test can detect scoliosis, it cannot detect the
presence of congenital abnormalities. Imaging tests can provide more
information.

X-rays. Images of your child's spine are taken from the back and the side. The
x-rays will show the abnormal vertebra(e) and how severe the curve is.
Once your doctor makes the diagnosis of congenital scoliosis, your child will be
referred to a pediatric orthopaedic surgeon for specialized care and further
tests.

Computed tomography (CT) scan. A CT scan can provide a detailed image of
your child's spine, showing the size, shape, and position of the vertebrae. To
see the vertebrae better, your doctor may have a 3-D image made from the CT
scan. This looks like a photograph of the bones.
This 3-D image from a CT scan shows hemivertebrae, as well as a fused, boney
block.

Ultrasound. Your doctor will do an ultrasound of your child's kidneys to detect
any problems.
Magnetic resonance imaging (MRI) scan. Because an MRI can evaluate soft tissues
better than a CT scan, an MRI will be done to check for abnormalities of the
spinal cord at least once for every patient.


Treatment
There are several treatment options for congenital scoliosis. In planning your
child's treatment, your doctor will take into account the type of vertebral
abnormality, the severity of the curve, and any other health problems your child
has.

Your doctor will determine how likely it is that your child's curve will get worse,
and then suggest treatment options to meet your child's specific needs.

Nonsurgical Treatment

Observation. A child with a small curve that seems to be unchanging will be
monitored to make sure the curve is not getting worse. Although it does not
happen in every patient, congenital scoliosis curves can get bigger as the spine
grows and the deformity of the back becomes more noticeable. It is likely that a
curve in a young child will get worse because younger children still have a lot of
growing to do.
Your doctor will follow the changes of your child's curve using x-rays taken at
6-to 12-month intervals during the growing years.
Physical activity does not increase the risk for curve progression. Children with
congenital scoliosis can participate in most sports and hobbies.
Bracing or casting. Braces or casts are not effective in treating the curvature
caused by the congenitally abnormal vertebrae, but they are sometimes used to
control compensatory curves where the vertebrae are normally shaped.


Surgical Treatment

Surgical treatment is reserved for patients who:

• have curves that have significantly worsened during the course of x-ray
  monitoring
• have severe curves
• have a large deformity of the spine or trunk
• are developing a neurological problem due to an abnormality in the spinal cord
An important goal of surgery is to allow the spine and chest to grow as much as
possible. There are several surgical options.

This x-ray was taken of the same patient after her surgery to remove the
hemivertebra and fuse the curved vertebrae with a metal implant.
Spinal fusion. In this procedure, the abnormal curved vertebrae are fused
together so that they heal into a single, solid bone. This will stop growth
completely in the abnormal segment of the spine and prevent the curve from
getting worse.

Hemivertebra removal. A single hemivertebra can be surgically removed. The
partial correction of the curve that is achieved by doing this can then be
maintained using metal implants. This procedure will only fuse two to three
vertebrae together.

Growing rod. Growing rods do not actually grow but can be lengthened with
minor surgery that is repeated every 6 to 8 months. The goal of a growing rod is
to allow continued growth while correcting the curve. One or two rods are
attached to the spine above and below the curve. Every 6 to 8 months, the child
returns to the doctor and the rod is lengthened to keep up with the child's
growth. When the child is full grown, the rod(s) are replaced and a spinal fusion
is performed.

Rehabilitation. Young children usually recover quickly from surgery and are
discharged from the hospital within 1 week. Depending on the operation, a child
may need to wear a cast or brace for 3 to 4 months.
Once they are healed, children are allowed to participate in most of the activities
that they had previously participated in.


Long-Term Outcomes

Congenital scoliosis detected at an early age is one of the most challenging
types of scoliosis to treat. The curves can be large to begin with and because
children have so much growth ahead of them, the chance of severe curve is
high.

Although fusion of vertebrae at an early age results in the spine and trunk being
shorter than they would have been, children can have outstanding results and
achieve normal, or near-normal, function.