00:00
Hello. This is the lecture on craniosynostosis.
00:05
We're going to start by going over the definition and
physiology,
we'll discuss the causes of it, the manifestations
of it and what each type looks like,
and then we'll discuss the basic treatments for it.
Let's start with definition.
00:21
Craniosynostosis is a premature closure
of a suture or sutures of the skull.
00:28
The sutures of the skull are basically the
gaps between the regions of the skull.
00:34
Typically in the normal newborn head, they end up
fusing together in a certain predictable order.
00:41
In craniosynostosis, something fuses prematurely.
Again, it may be one suture or it may be more than 1.
00:50
These are the terms that represent that various sutures or
borders or junctions in between the regions of our skull.
00:58
You may not see this very often, these terms,
you'll usually see them more in radiology reports,
but it's helpful to see them here so
you can understand the condition.
01:08
In craniosynostosis, the skull is forced to grow in a way it
typically does not grow in order to accommodate brain
expansion.
01:16
This is actually a fairly common condition. It
occurs in about 1 to every 2000 to 2500 births.
01:26
This is the normal closure of the
suture lines between the skull bones.
01:32
Generally by about 2 months of age or 8 weeks, the posterior
fontanelle has closed, but the anterior fontanelle remains
open.
01:43
There is a fibrous material that exist in the junctions
in these sutures lines and also in the fontanelle.
01:49
Eventually it becomes bone through
a process called ossification,
but that happens gradually as the
infant's brain needs to grow.
02:00
At about 6 months, there is this fibrous union so basically
the
regions are starting to come together in the posterior area.
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Then around 18 months or by 18 months,
the anterior fontanelle is closing.
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You may not feel it any
more at this point.
02:21
An astounding almost 70% of the adult brain
weight is achieved by about 18 months of age.
02:28
This may be why many toddlers especially
seem very top heavy and very clumsy,
they fall a lot and they
often hit their heads.
02:36
and that's really just because their heads are
so heavy in proportion to the rest of their body.
02:44
By the age of about 12 years, the
sutures or the borders in between
the regions of the skull are not separable
by increased intracranial pressure.
02:55
And what this means is the skull is not going to be as
forgiving to
small increases in pressure as a result of, for example,
brain injury.
03:06
Craniosynostosis occurs if the bone somewhere in the
head fuse together before the brain is fully developed.
03:14
This is animation that's going to show you in real time
what could happen if one of the sutures closes early.
03:21
We're going to watch the suture at the top
near that ear at the top close prematurely
and you'll see how the brain shifts in the direction
to how it grows and it can also affect the head shape.
03:34
Basically it displaces it
away from diffuse suture.
03:39
Premature suture closure inhibits the normal growth and
development of the brain and causes it to shift in this way.
03:46
So that's the way it might look
if that suture close prematurely.
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This is the way it might
look on the outside.
03:54
We're going to look at each type of
craniosynostosis a little more closely in a moment.
04:02
These are the types of craniosynostosis. I'm
going to show you how each one presents.
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The sagittal suture runs along the top
of the head across the fontanelle.
04:13
Sagittal craniosynostosis is actually the most common type.
It's also known as scaphocephaly.
04:21
When this happens, when the
sagittal suture closes prematurely,
it forces the head to grow kind of long and
narrow just like the picture on the far left.
04:32
The next one, unicoronal means one of
the coronal sutures close prematurely.
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It's also known as
anterior plagiocephaly.
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The coronal sutures run from each ear to the
sagittal suture at the top of the head.
04:49
This type, the unicoronal craniosynostosis, basically
occurs when one coronal suture closes prematurely
and the infant may, as a result, have a flattened
forehead on the side that closed early.
05:05
Sometimes, both coronal sutures
will close prematurely.
05:10
When this happens, the head can grow broad and short
and this is likely to result in increased ICP,
which makes sense because when you have more than
1 area of the skull that's closing prematurely,
there's even less room for growth of the brain so
it's more likely that pressure is going to build up.
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This one is also known
as brachiocephaly.
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Then there are 2 types that are fairly rare.
One is metopic craniosynostosis.
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So the metopic suture typically runs from the nose
to the sagittal suture at the top of the head.
05:47
Closing early may result in a
triangular looking head like this.
05:51
It's also called trigonocephaly which can be kind of easier
to
remember if you think of the triangular shape that it
results in.
06:01
This is one of the rarest types and
often requires surgical correction.
06:06
One of the other rare types of
craniosynostosis is lambdoid craniosynostosis.
06:12
It's also known as
posterior plagiocephaly.
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The lambdoid suture typically runs
along the back side of the head.
06:19
If it closes early, the head can
be flattened on the back side.
06:25
Alright, let's review the
causes of craniosynostosis.
06:29
The causes and risk factors for
this condition are largely unknown.
06:33
There are genetic and environmental
components that are both suspected,
and sometimes this condition can be part
of a syndrome or a cluster of defects.
06:44
Maternal thyroid disease and some fertility medications may
also contribute to the rest of craniosynostosis in the baby.
06:51
Alright, let's go over the diagnostic findings
and the manifestations of this condition.
06:57
The manifestation basically depends on
which sutures closed, how early they closed,
and whether or not the brain
has had a room to grow
whether there has been a brain
damage as a result of it.
07:10
Some signs that can lead to the diagnosis of
craniosynostosis
are a firm raised edge where sutures are supposed to be
flat,
so where they close too early; the absence of a soft
spot or fontanelle where one is supposed to be present;
and/or slow or no growth in
the head size over time.
07:35
Usually this condition is
detected during routine check-ups
because the newborn and the infant are all
having head circumference measurements
and had assessments
done at every check-up.
07:47
A CT scan can confirm the
diagnosis once it's suspected.
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Let's go over the treatment.
07:56
The treatment basically depends on
the timing and when it's caught
and then what the goals are so the goals will
be set according to the signs and symptoms
and the effects it's
had on the child.
08:09
Usually, surgery is
indicated or needed
and the timing of that surgery depends
on which sutures were closed.
08:17
The goals of the surgery include
relieving any pressure on the brain,
correcting the actual condition,
this craniosynostosis,
and then allowing for proper growth of
the brain and the head after that point.
08:31
Cranial vault remodelling is the
surgery done to treat this condition.
08:37
Most infants who have it require
this particular surgery.
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Usually only one surgery is required
when the child is about 4-6 months old.
08:47
About 10% of the children may need
a second surgery at some point
to correct some kind of minor deformity
or a possible relapse of the condition,
but most of them have pretty good outcomes
as long as the condition is caught early
before there is too much pressure
or any damage to the brain.
09:08
Occasionally, you may see a child wearing a
helmet to help treat this condition as well.
09:14
This special helmet may also be
used to help reshape the skull.
09:19
This is the NCSBN Clinical
Judgment Measurement model.
09:23
It's a framework being used now for many NCLEX
next generation questions and case studies.
09:29
You may hear about it in nursing school and you may
have some tests questions that use this framework
so we're going to connect a
little bit of this content
from the lecture to this measurement model
specifically the first 2 steps of it,
recognizing cues and
analyzing cues.
09:45
In order to be able to accurately recognize cues and
analyze cues in an infant who may have craniosynostosis,
you have to first be familiar what
a normal newborn head looks like.
09:57
We have a whole lecture
dedicated to that
and I'll tell you all about it and show
you how to measure a head circumference.
10:04
Then, you also have to be able to assess
for signs and symptoms of craniosynostosis
and be able to understand what they
are and when you might see them.
10:14
For example, if you don't feel or see a
fontanelle on an infant that should have one,
that might be a sign that
they have craniosynostosis.
10:25
If you feel a raise from edge at where you think
is a suture line between 2 regions of the skull,
that might be another indicator.
10:33
And if you see a misshapen head,
if you know the misshapen head,
there should be no misshaping of the head
after the initial couple of days after birth.
10:42
The head should be round and
should be even and symmetrical.
10:45
That might be another cue.