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The Expert Advisor
Featured Article:
The Chiari I Abnormality in Childhood
Arnold H. Menezes, MD
The following article is a transcribed presentation from the 2004
ASAP Conference. You will find this and many more presentations in
our
2004 Conference Notebook.
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| When we examined them [see Figure 5] we
looked for oralpharyngeal abnormalities. We talked about
aspiration, difficulty swallowing, regurgitation, meaning
coming up the wrong way. Now its a very common diagnosis in
pediatrics to have what is called achalasia cardia. That
means the sphincter that closes off between the stomach and
the esophagus down about here, should close off after youre
finished eating. In 40% of children, especially males, below
the age of 2, they can regurgitate and you know that you
always put an apron or a towel or a bib around the child
after feeding them because it comes up. This is a little
different and it comes up with a big projectile movement. |
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| Vocal cord dysfunction. Your
vocal cord and mine is innervated by the tenth nerve that
keeps it moving in and out. When were breathing it opens
up, when were swallowing it closes down. If instead theres
an abnormality and its going to stay partly open, it can
allow food to go through. Second thing is that if you watch
a child and if you hear the (strider sound) that means that
things are not going right- its in a bad position. Choking
and chronic cough occur because things are going the wrong
way. Gait and motor impairment, sometimes sensory. Kids
cant tell us that theyre having sensory abnormalities but
what they do is they start rubbing their hands or they dont
want somebody to touch them.
Scoliosis. No exam is complete without scoliosis. |
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I broke this down to be very simple, [see
Figure 6] just to see that swallowing and oralpharyngeal
dysfunction is significant. 16 out of 24, as opposed to 2
out of 21. So kids come in with this problem earlier, the
older child comes in with scoliosis
and headache, theyre more able to tell us what the problem
is. And a syrinx was less common, a cavity in the spinal
column was less common, below the age of 3, then between 3
and 6. So to sum up the slide, weve got more of a problem
below the age of 3. |
In our institution when we are
considering an operative procedure on anybody with this
situation we look at the clinical presentation. We like to
see the anatomy, we do many
studies, some of them are volumetric analysis to tell us the
size of the area. We check this out with 3D CTs, MR. The
important is stability. Is this area rocking? Does the
position
change between the head tipped back / extension / flexion or
sideways? How do we assess that? With an MRI usually.Is
there hydrocephalus or is there a syrinx? And since we see a
lot of kids we talk about other things that are going on,
such as, bony abnormalities, and theyre called a skeletal
dysplasia. |
Of these children all 45 had an operation
from behind. [see Figure 7] 5 of those 45 had a problem from
in front that required attention from in front and from
behind too. Posterior fusion was needed in 3 of the 45 below
the age of 6. Now everyone whos had an anterior procedure
and the other too patients requires a posterior fixation.
Weve talked about this since 1980 and its more or less
accepted. When I first came to this meeting talking about
these Chiari problems I made a mistake that all of us do, we
forget that we are not at a medical neurosurgical convention
and we showed pictures that made
some in attendance uncomfortable. |
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