Let's switch gears now and talk about
which are a little bit farther
The Arnold-Chiari Malformation
is a congenital CNS anomaly
which is a displacement of
lower than that actually proceeds through
the foramen magnum.
There are three types of
One, two and three and this have
in terms of the amount of tissue
this downward displaced
through the foramen magnum.
Type I is generally mild and may
in fact be asymptomatic
and discovered in adulthood
through imaging or found on an autopsy
much later in life when a patient
dies for some other reason.
The treatment of
if needed is generally neurosurgery.
The idea being that we want to
try and prevent both the flow of CSF
through these lesions but also
any damage to normal tissue.
Next, let's focus on cranisynostosis.
Craniosynostosis is not a problem
with the brain contents
it is a problem with the skull.
Recall that in newborns,
newborns are born with
multiple skull pieces
gradually seal together over time.
They are born for example,
a frontal fontanel
and a posterior fontanel
and these are areas where the skull
has not fused together
and actually palpating the child's brain
through the skin.
In the brain, there are some normal
sutures that you can see in children.
There is a sagittal suture,
as you can see that runs from front to back.
There is a the coronal suture,
which runs left to right across
the front of the brain.
And there's the lambdoid suture
which is in a lambda shape
which runs diagonally backwards
towards the occiput
from the top sagittal suture.
These are the normal skull sutures.
But children can have problems with
when they prematurely fuse.
So craniosynostosis is a premature fusing
of skull sutures,
which prevents the skull from growing
and that can cause substantial problems
for the brain which is trying to grow
inside and is now limited in terms of
its ability to grow.
They may have localized areas of fusion
an isolated sagittal suture,
an isolated coronal suture,
or an isolated lambdoid suture that have
sealed shot prematurely.
Or they may have generalized disease
when they have multiple suture involvement.
This results to microcephaly.
we're tracking this children's head
circumference throughout their childhood
and this is another reason
to do it.
If we see the head circumference gradually
tailing off and failing to grow,
we may well suspect a generalized
These children are going to have problems
with their brain development
and will develop developmental delay.
They may even lose milestones if
it gets very severe.
Also, patients may have syndromes.
There are several general syndromes
that are associated with craniosynostosis
I'll give you a couple of
In Apert Syndrome,
patients can have Sydactyly when
their fingers are fused together.
They also develop craniosynostosis.
Likewise in Crouzon Syndrome,
patients may have Exopthalmos
or eyes bulging outward out of
and an associated craniosynostosis.
When we see a patient with craniosynostosis,
we would often see abnormal
moulding of the head.
But everytime you see a child with
an abnormal head,
it does not mean they have
This is a very common problem
that can be mistaken for craniosynostosis.
This is plagiocephaly,
which is positional mouldling.
This may happen because parents are
laying their child preferentially on one side
or a child may have an underlying
For example, they may have an
inability to move their neck
from a congenital torticollis.
because they are not able to move
their necks so well,
they often get a plagiocephaly.
This is managed very simply,
though allowing the child to move
their head in different directions.
And encouraging the parents to help
move their head in different directions.
In very severe cases, we can
fit them with helmets.
But in most cases, simple physical therapy
can largely correct the problem
and it gradually resolves or improve
as they get older.
This is sagittal craniosynostosis.
You can see that sagittal suture
has been fused
between the coronal suture
and the lambdoid suture.
This is causing the child to have a
very long and narrow head.
This is Lambdoidal craniosynostosis.
Here you can see in the posterior
aspect of this child's head
on his or her right side,
a closed lambdoid suture.
This is causing
because of angulation,
a frontal protuberance and you can see
the affected side has a posteriorly
This is a classic head configuration
for a child with lambdoidal synostosis.
To make a diagnosis, we'd form a
CT scan of the head.
It's necessary to do a CT scan as opposed
to an MRI because CT scans are much better
at seeing bone.
Remember, CT scans
bone and blood.
So for this purpose,
we usually get CT scans and we
can do three dimensional reconstructions
from the digital images
to show to the surgeons; to help them
decide how they can best repair this child.
Surgical repair is the mainstay of therapy.
We have to fix these skulls, so that we can
expand them and allow room
for the brain to grow inside.
This surgery is a major surgery.
It is bloody and it is difficult
and it usually
requires a multidisciplinary team of
both surgeons and neurologists.
Patients and families and the doctors
and nurses who care for these patients
all have to work together because
this is a complex and painful care.
Remember that craniosynostosis
is present in several genetic syndromes.
It is important to consult
a geneticist for patients
who have craniosynostosis
to verify that there is not an underlying
Genetic conditions are important to
understand because it may relate
to future prognosis.
And it may help the parents decide
about future children
and what the risk and benefits there are.
That's the brief summary of the
structural problems in the heads of children.
Thanks for your attention.