Scoliosis is a common condition related to syringomyelia in children; in
fact, some studies have shown that more than 50% of children with a
syrinx have abnormal spinal curvature. While many types of scoliosis
which develop in children and adolescents will stabilize on their own
and not cause any symptoms, scoliosis associated with a syrinx often
causes back pain and can progress rapidly.
Compounding the problem is the ineffectiveness of standard
treatment. Non-surgical treatments, such as wearing a brace, tend to
not work with syrinx related scoliosis. Surgical intervention is even
worse, with many reports of worsening neurological symptoms after
surgery. Given these facts, it is little wonder that more and more
doctors are looking to treat the underlying syrinx before turning their
attention to the spine.
In an effort to untangle this difficult situation, Dr. Ken Kontio
and a group from the Children's Hospital of Eastern Ontario, in Canada,
reviewed nine pediatric cases treated at their facility and combined
them with a thorough review of the literature. In reviewing the
published literature, studies had to detail curve magnitude, curve
progression, and the change with the treatment to be included. Overall,
the researchers ended up with a group of 98 cases from the literature,
which could be combined with the nine patients treated locally.
The group published their work in the Journal of Pedatric
Orthopaedics, in December, 2002, in a paper titled Management of
Scoliosis and Syringomyelia in Children. Because of differences in
the way the studies were conducted, the use of true statistical analysis
was not possible, however much can be learned from just looking at the
actual data (see Table 1). With the definition of curve progression as
a change in 5 degrees or more, the researchers found that among the
group of children who were treated for their syrinx (or the syrinx
stabilized on its own), 36% experienced continued curve progression, 48%
stabilized and experienced no further curve progression, and in 16% of
the cases the curve actually improved. In contrast, in the group which
did not receive syrinx treatment, 73% of the children's curves continued
to worsen, 20% stabilized, and only 7% improved. In another measure of
the difference between the two groups, in the group with syrinx
treatment, 25% eventually required surgical stabilization of the spine.
In comparison, 50% of the children who were not treated for their syrinx
required surgical stabilization of their spine.
The researchers also looked at the effect of bracing on curve
progression. Interestingly, bracing was not a very effective treatment
for either group. Among those children who received syrinx treatment
and bracing, 67% experienced further curve progression. The numbers are
even worse for the group without syrinx treatment, with 92% of children
who received bracing experiencing further curve progression. While
bracing appeared ineffective, among the nine children treated locally,
for those who needed surgery, fortunately there were no neurological
complications. Data was not presented on how many of the children whose
syrinx wasn't treated experienced neurological complications after
The exact link between syringomyelia and scoliosis is unknown.
Is scoliosis a direct result of the presence of a syrinx? While this
would seem to make sense, research - including this study - has failed
to find a statistical link between syrinx size or location, and the type
and severity of scoliosis, so perhaps syringomyelia and scoliosis are
both the result of an abnormal spinal environment. Despite the research
findings, there are several theories on how a syrinx can cause
scoliosis. One theory proposes that the formation of a syrinx damages
the motor neurons and results in an imbalance of the back muscles,
making scoliosis more likely. Another theory proposes that when a fetus
is developing, the presence of a syrinx will cause vertebra to form
abnormally and lead to scoliosis.
While there are many unknowns surrounding both syringomyelia and
scoliosis, it seems clear that the best way to treat the scoliosis is to
treat the underlying syringomyelia. Given the frequency of scoliosis in
children with syringomyelia - and the potential damage of standard
treatments - MRI should be used to identify or rule out the presence of
a syrinx in scoliosis cases where there are also neurological symptoms
or unusual progression of the curve.
common in children with a syrinx and is often diagnosed before
treatments for scoliosis associated with an active syrinx tend to not
work and surgery can worsen the neurological situation.
review of the research literature shows that treating the syrinx
benefits the scoliosis as well.
scoliosis does not stabilize, corrective surgery is safer after syrinx
link between syringomyelia and scoliosis is unknown.
an abnormal curvature of the spine. A normal spine looks straight
when seen from behind, scoliosis is a right-left curve when seen from
affects 1-2 percent of the general population.
be caused by congenital conditions, neurological conditions (like SM),
or can be of unknown origins.
scoliosis cases have an unknown cause and are labeled "idiopathic".
options include observation, bracing, and surgery.
involves wearing an external device for up to 23 hours per day.
involves fusing vertebra of the spine using rods, screws, bars, and
that exercise, drugs, electrical stimulation, or spinal manipulation
helps stop or improve curvature.
Scoliosis Research Society
Journal of Pediatric Orthopaedics 22(6), Dec 2002, pg 771-779