Let’s switch gears now
to diaphragmatic hernia.
This is a really unique
problem in newborns,
which is very challenging to manage.
So in this situation, a baby
is born where the intestines
have worked their way through the
diaphragm and up into the chest.
So, other things may go through so
it might not just be the intestines,
it might as well be the stomach,
the patient may have some
liver up in the chest,
or the spleen may even
get into the chest.
But in most cases it’s the intestines
that have migrated up into the chest.
There are several different types
of congenital diaphragmatic hernia
and the names of them are
sometimes found on various tests.
the Bochdalek is about 90% of
congenital diaphragmatic hernias
and it’s much more common on the left
side, as is pictured here in this x-ray,
than on the right side.
Generally, this is a posterior lateral,
in the back of the diaphragm hole,
where the intestines are squirting
up into the thoracic cavity.
This is preventing the lungs
from developing appropriately
and these children will have
respiratory distress at birth.
The Morgagni is another
type, which is much rarer,
and this is more in the front,
anterior, and just behind the sternum.
And this is about
2% to 6% of cases.
This is another type of hernia.
Lastly, rarely, patients can
have a very bad hiatal hernia,
where at the entrance of the
esophagus down to the diaphragm,
it’s simply wide open in the stomach
and other intestinal contents
can make their way
into the thoracic cavity.
In these patients,
it’s surprising, but the diagnosis
may be tricky and delayed.
These patients will often present with
intestinal sounds heard in the chest.
So, that intestinal
rumbling, the borborygmi,
if that’s heard in the chest, a chest
x-ray is absolutely indicated.
Occasionally, this can be
diagnosed much later in life.
I have a colleague who was
diagnosed at the age of 40.
Patients may have a scaphoid
abdomen, where it’s sucked in,
and you can see that ridge of
the lower ribs as that abdomen
is sucked in because the
intestinal contents have come up.
And they may have associated
Diaphragmatic hernias are more common
in some of the trisomy patients,
syndrome or trisomy 21,
or Patau syndrome or trisomy 13.
In the diaphragmatic hernia
patient, if it’s known,
these patients will generally
be intubated at birth
and they will provide positive
pressure through an ET tube
to try and help those
lungs stay open.
In severe cases, they
may have to do ECMO
oxygenation of the blood
through a machine that bypasses
the heart and lung entirely.
We do want to evaluate these patients
for pulmonary hypertension because
since they have less
they may have increased
pressures on the right side.
And when these patients are stable,
we will undergo a surgical repair
to try and fix the problem
and get the intestinal contents
back down below the diaphragm.
Lastly, let’s switch to congenital
cystic adenomatous malformations
or CCAMs for short.
The CCAM are cysts that are
found within lung parenchyma
and you can see one pictured
here on this CAT scan.
These things may be single
or they may be multiple
and these cavernous malformations prevent
the adequate growth of normal lung tissue.
They can lead to air trapping and they may
harbor infections or become abscesses.
They may grow slowly.
It’s not uncommon for one
to be reasonably small
and then gradually continue growing
through life and require resection.
They also do have some malignant potential
so these are generally excised.
And usually, we like to surgically
remove them before one year of age.
That’s a good summary of
most of the important
congenital or around birth
respiratory conditions in children.
Thanks for your attention.