|
|
|
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.
Page 1
[1] [2]
[3] [4]
[5] [6] Next
>>
|
|
|
Abstract
The clinically symptomatic child with the hindbrain
herniation syndrome (Chiari I) can differ significantly
from the adult patient. A prospective analysis of these
children has brought an understanding of the various
presentations.
The primary symptoms below age 5 have been failure to
thrive secondary to repeated aspirations,
gastroesophageal reflux and headaches. Between ages 3-10
years, headaches and scoliosis were the main symptoms
together with neurological abnormalities secondary to
the cerebellar tonsillar impaction and
syringohydromyelia.
The factors affecting treatment have been clinical
symptoms/signs, posterior fossa volume, craniovertebral
junction abnormalities and instability. Taken together,
these substrates provide for successful outcome. |
|
|
|
Transcribed Presentation Notes |
Good morning. I sound like a preacher dont
I -- expecting you to say good morning back again! I thought
Id talk to you today about
something thats always fascinated me, the problems that
occur in the young child. I think most of us have come to
the conclusion
that the Chiari I malformation is not a nervous system
neural abnormality per se, but rather that which is the
result in the
decrease in the housing space in the back of the head. We
call that a mesodermal abnormality. |
 |
 |
But why should it happen in the very young,
before the skull has fully formed and developed? And is
there any way in which we can predict or help these children
who come in with different forms of
presentation? So in light of that, we reviewed-- this is a
prospective study thats been going on for many years-- we
reviewed our series and we thought wed focus on childhood. |
Ive been at the University of
Iowa since 1969 [see Figure 1] and my interest in terms of
keeping a detailed log started out fairly early. We have a
prospective data base and it looked at nearly 600 surgical
patients, because of my interest in abnormalities of the
skull base, bony abnormalities, about 2/5 of them, nearly
half of them, were patients who had problems with skull base
abnormalities. But when we focused on children below the age
of 6 years, [see Figure 2] we thought that our reason for
doing this would be, how did this defer in terms of
presentation from the adults or anybody above that age? And
why did we pick the age of 6? Because it begins to be
statistically significantly. We wanted to achieve an early
diagnosis, and more importantly, we need to educate medical
practioners, such as pediatricians, family practioners,
general practice, neurosurgeons too, and I think that that
was something we needed to achieve. Once we came to some
conclusions, we thought that our best bet was to have
special meetings, instead of going ahead and presenting this
at neurosurgical national meetings, which is what we do, but
also to educate the pediatricians. The Journal of Pediatrics
has a circulation of over 300,000 and its translated into 4
languages and goes out world wide. |
|
|
|
|
