Headaches and Pituitary Tumors

November 8, 2019

hello and thank you all for joining us
for today’s webinar the Pituitary Network association is a nonprofit organization
that relies on the support of our members and donors we offer this
webinar series to help educate patients their families and their healthcare
providers. During the webinar feel free to type in your questions at any time
please note that all questions will be saved until the end of the webinar we
have a lot of time to answer as many questions as possible any questions that
are not answered will be reserved and answered by email Today’s webinar Headaches and Pituitary
Tumors as being presented by Dr. Garni Barhoudarian. Dr. Barkhoudarianis an Assistant Professor
of Neurosurgery and Neuroscience at the John Wayne Cancer Institute at St.
John’s Health Center in Santa Monica California. Dr Barhoudarian completed
his undergraduate education at UCLA majoring in microbiology and human
genetics he attended the University of Michigan Medical School where he was the
Edgar Akon neurosurgery research fellow he completed his Neurosurgery
residency at the UCLA Medical Center then completed a fellowship in pituitary
surgery and Neuroendoscopy at Brigham and Women’s Hospital and Harvard
Medical School Dr. Barhoudarian’s clinical interests are minimally invasive skull
base surgery, pituitary disorders, benign and malignant brain tumor surgery, neuroendoscopy, trigeminal neuralgia, and hemifacial spasm and mild to severe head
injury concussion. He is the Director of the Brain Tumor Center skull base and
endoscopic microdissection laboratory Dr. Barkhoudarian is involved in a
number of clinical and translational research studies evaluating the genetic
diagnosis treatment and results of patients with malignant metastatic tumors meningiomas, chordomas and pituitary tumors. He is also
evaluating children as well as active and former NFL athletes with chronic
repetitive concussions and brain injury. He has a special interest in the
advancement of technologies for minimally invasive neurosurgical
procedures. We are now going to turn the
presentation over to Dr. Barhoudarian there may be a brief delay as we change
presenters. Okay can you see my screen? I can yes all
right it is all yours. Excellent I apologize for the delay I’m making my
picture smaller I can see what I will talk about ok can you hear me OK Yes we can hear you just fine Excellent, well first of all I’d like to thank you all
for inviting me to speak today at the Pituitary Network Association it’s truly an honor to be a
member of this organization and it really is our goal to try our best to
help our patients with all sorts of different types of pituitary tumors and
as many of you know some of them can be straightforward but others can be quite
challenging and I think one of the more challenging things that people have been
tackling with pituitary tumors is the symptom of headaches. Headaches can be
pretty obvious in some cases and it’s very clear that that’s related to the to
the tumor or to the lesion that we see But often times it’s kind of a grey zone
and I hope to spend the next 45 minutes or so to try to elucidate some light on
the issue of headaches and the symptom of these types of tumors. So without
further ado will move forward here. As many of you know the pituitary gland is the
Master Gland it’s it’s really the It’s at the nexus of the brain and it involves so many
different systemic structures and organs involving everything ranging from growth
and stress and sexual reproduction thirst regulations etc. But there’s also
other things that the pituitary gland controls along with its relationship
with the hypothalamus that’s a little bit more subtle and it’s in these subtleties
where the difficulties arise in managing some of our patients. The most interesting part from my
perspective from a surgeon’s perspective is it’s location really is a deep in the
center of the brain and it lies in the nexus of a number of really critical
structures so obviously the pituitary gland itself is a critical structure but
also the optic chiasm depicted this whitish grey band here is just
above the pituitary gland and any pressure on the chiasm can cause vision loss tunnel vision usually. It’s right on either side of the carotid arteries which supply blood to
the brain and just past the carotid arteries are these important nerves that supply
sensation to the base and also control eye movement. and of course there’s the brain
just above it and just the size of it so any sort of tumor in this region can
affect any of these structures and you can get all sorts of different types of
presentations which actually is one of the reasons why it’s so difficult to
make a good diagnosis of pituitary tumors in some cases. Here is an
example where we move from the normal anatomy in this cartoon where the optic
chiasm and the carotid arteries are well separated from the pituitary gland then you
you have a tumor that grows in this area and all of a sudden you have
pressure on these and most of the time these are not invaded by the tumor but
at least they’re being pushed on and When that occurs you get dysfunction
in various symptoms. Probably one of the more common symptoms
people get are headaches and we’re going to spend a lot more time on that but
also it’s important to know that people can sustain vision loss or blurred
vision, color loss etc tunnel vision. Many sorts of endocrine abnormalities either
due to overproduction of hormones as we see in Cushing’s disease or Acromegaly or in patients with Prolactinomas. or hormone dysfunction which is
the opposite obviously which can occur independently of these different types of
syndromes. and most commonly we see a combination of this above scenario and
usually headaches play a role in at some level and in some of our patient’s. I won’t discuss the incidental finding
the incidentalomas as we call them. Many times though people think these are incidental tumors they actually may not be we just haven’t really diagnosed the syndrome as well as we thought. There’s a cartoon that I have borrowed from a colleague of mine at
UCLA Bill Yong a pathologist who actually did a nice job characterizing the different types of
structures that surround the pituitary gland so if this blue is the pituitary gland
we have the covering of the pituitary gland the meningioma of the meninges the
bone surrounding it the cerebral spinal fluid that bathes it, the
brain, the optic nerves the chiasm and the hypothalamus and each points in this area there could be
different types of pathology that exists whether it’s a pituitary adenoma of the
most common type of pathology there a Rathke’s Cleft Cyst or more uncommon
things like craniopharyngiomas, chordomas, granulomatosis, optic gliomas, etc. So we have to
deal with all sorts of different types of diagnoses to to figure out exactly
what we’re dealing with this is an example in a patient who unfortunately did not survive but had
Cushing’s disease and had a micro adenoma very small adenoma causing for
Cushing’s disease resulting in her demise this is taken about fifty years
ago by Jules Hardy and that’s a good example of how significant even a small
tumor like this can affect a patient with that disorder. This on the other hand
is a patient who again many many years ago who had a very large pituitary adenoma and this even though it
wasn’t secreting any hormones cause. problems because it grew so large that
ultimately obstructed the normal flow of cerebral spinal fluid and developed hydrocephalus and then slipped
into a coma and so thankfully we don’t see this common but these are kind of
the extreme situations of pituitary tumors and how can present. There’s all sorts of other types of tumors a Rathke’s Cleft Cyst, Duramoids craniopharyngioma, meningioma epidermoids schwannomas and chordomas, and they all occur at different areas or regions surrounding the pituitary gland around the sella which is the home of the pituitary and each of these tend to present in somewhat of a unique way and will touch upon some of these next few slides so
just to kind of start things off I want to discuss how we define headaches
believe it or not there’s an entire society dedicated to headaches and the
International Headache Society and they have categorized every single time you
can think of including headaches that are associated with brain tumors so this
is their criteria and basically they say a headache with at least one of the
following characteristics and fulfilling C & D so C & D down here. So at least it
being progressive localized worse in the morning or aggravated by coughing or bending forward. You’d want to see an intercranial neoplasm on the imaging although it’s not always there at least it’s not always visible and then the headaches usually temporal
or spatial fashion related to the neoplasm basically meaning that the
tumor grows the headache gets worse and then here’s the catch though the last
comment states that the headache must resolve within seven days after surgery
for removal of this lesion. This kind of circular logic that yes you have a
headache related to tumor but the only way to really prove it is by taking it
out and see if it goes away well that probably would result in many
unnecessary operations in patients. So it’s our job to try to figure
out which patients headaches are actually related to the cyst or tumor in which patients
may we may be able to treat medically and not need surgery. This is a study
from a while ago looking at the different locations headaches associated with different
types of tumors this is believed in the nineteen fifties when this was published
talking about the location over to this region of the temples associate middle
meningeal artery the top of the head associated with the sagital sinus, the forehead and the orbit associated with
all these different structures and in this does include the cavernous sinus of the pituitary region. So this is a they’ve already known that fifty,sixty years
ago when this was published and we’ll talk a little more about the location of the
headaches this is a study that came out of a fifteen years ago and basically
they looked at about two hundred and eighty patients over a period of time
with any sort of brain tumor and they just wanted to see what are some
criteria that would be associated with headaches just to kind of get a sense of
what what really correlated with headaches so these are all sorts of
tumors not just pituitary tumors in all comers 60% of patients had headaches and
the patients who did have headaches 50% actually had persistent headaches after
surgery so we would categorize this a headaches that were not related to the tumor itself and that suggests that about 85% of patients who had headaches related to
the brain tumor got better after surgery. suggesting that their headaches were
related to the brain tumor directly so that was the start now they decided to
examine this a bit more carefully so they looked at patients who have
progressive headaches versus not progressive critics in his piece of the
progressive headaches those who have more aggressive type of tumor like a
metastatic brain tumor or glioblastoma were statistically significantly higher
risk of getting progressive headaches Where as people who had kind of static
headaches that they’ve always been there for many many years is non progressive headaches the most
common tumor was benign type of diagnosis of meningioma these are slow-growing
tumors so it was interesting difference between the progressive and the non progressive headaches. Also the location if the tumor was above all the
tutorials above the cerebrum of the brain these patients are typically at
lower chance of presenting with headaches as opposed to a lower down in
the skull base or the cerebellum those patients had a much higher rate of
presenting with headaches than their counterparts and
interestingly if there was a tumor in the ventricle which is the fluid filled areas of the brain these patients actually had a notable higher
rate of presenting with headaches though there is other criteria that we will
talk about down the line that could contribute to a progressive headache and the key on is the development of mass effects in addition to the tumor so say the
tumor is obstructing the normal cerebral spinal fluid flow and the patient gets
hydrocephalus well these patient definitely have a higher risk of progressive
headaches and also if there’s any midline shift so enough pressure on one
side of the brain that it shifted over to the other side which we see from time
to time even in pituitary tumors then these patients could develop progressive
headaches these are more serious types of tumors that we want to do it. So
what are the typical symptoms associated with headaches from pituitary tumors
specifically. This information was gathered by my work with Dr. Ed Laws
and his team at the Brigham and Women’s Hospital basically asking patients who
have pituitary tumors what type of headaches they had, their severity
etcetera and we found that most patients had daily headaches that are typically bi-
frontal meaning in the front forehead region or behind the eyes or they were at the vertex
at the top of the head the general lasted 1-4 hours in
duration especially at their most severe most severe stages they could be
unilaterally could be only on one side it didn’t necessarily mean that it had to be
on both sides to generate a headache and in general patients complain of these are
moderate or severe headaches they were kind of mild forget about it headaches they
were definitely interfering with their lifestyle. In a minority of patients
about 15% of patient’s they would wake up because of or with the headache and that
was actually key finding because that usually suggests that something with more
aggressive is going on in the brain and in rare cases but notoriously one could develop really sudden and severe onset headaches and when we hear that in the
setting of a pituitary tumor we have to be concerned that there is bleeding
into the tumor and a condition called the pituitary apoplexy can occur we’ll talk more about that but the key
point is in that setting you don’t want to sleep on it you don’t want to take a
bunch of aspirin and you want to go straight to the emergency department
because this is a surgical emergency until proven otherwise. So what are some of the mechanisms that
we think can contribute to the headaches and we’ll talk more about this in depth
with this slide I wanted to point out that you have a large cystic lesion here
this is an arachnoid cyst or Rathke’s Cleft Cyst and its pushing and stretching the normal
contents of surrounding the pituitary gland we think this is the number one
mechanism of headaches meaning that the dura mater is being stretched and the
nerve fibers that are on the dura mater because the brain actually doesn’t have
nerve fibers is actually in the covering of the brain and pituitary gland those
when they stretch generate headaches interestingly though larger tumors are
less likely to cause headaches because they generally tend to be slower growing
and the brain can accommodate and the nerve fibers can accommodate and they don’t
generate the pain signal that you expect cystic lesions like Rathke’s Cleft Cyst,
arachnoid cyst, cystic craniopharyngiomas or cystic adenomas tend to be more
likely to present with with headaches because we think that the cyst is
expanding at a different rate than the rest of the tumor is and of course as I mentioned if there is bleeding into the tumor, if theres hemorrhage or apoplexy then
there’s a higher rate headaches this was a breakdown of our patients who did have
headaches and you can see in these patients we look at their typical headache and
their most severe headache and in patients with macroadenomas their typical headache range about three to four most severe was about a five same with the microadenomas but patients with cystic lesions had
consistently higher average rate higher severity of headaches so again pointing that cystic tumors tend to cause more significant headaches. This is a study
that came out about ten years ago looking at pituitary volume of headaches and characterizing the volume of the size of the tumor and it’s interesting that they’ve
concluded that the size was not the only factor contributing to the headaches so they looked sixty-three patients 70% of these patients had headaches and this is their
scatterplot basically plotting the tumor volume measured in milliliters
with the headache score they had compiled and you can see there is no linear or
logarithmic correlation associated with this it’s completely random you can have
very very large tumors with very little or no headaches and you can have very
small tumors with significant headaches so there is a wide range that we’re
looking at here they did note that if a tumor was invading some of the nearby structures to
the cavernous sinus which is where the carotid artery lives and those veins and those nerves live as I was talking about earlier these tend to have a higher rate of
headaches and is the patient already prone to headaches so if there’s a family history
of headaches or if there’s a history of migraines there’s a higher rate that that patient will generate headache
related to the tumor This is the data that they published after surgery so whether it
was through the nose through the brain or just radiation they found that 23
patient’s did improve eight actually got worse and nineteen really didn’t
change much so about a bit less than 50% of patients were either were better than
only a fraction were worse. Now here’s the interesting part in a number of these
patients who had hormone secreting tumors so say Acromegaly or Cushing’s disease when they were treated for their hormone secretion alone so sandostatin, octreotide or lanreotide their headaches all got better and also in patients with prolactin secreting
tumors who were treated with cabergoline or bromocriptine their headaches mostly got better in this subpopulation so there is definitely a correlation with hormone
secretion and headaches particularly the Acromegaly and Cushin’gs subgroups and we think that there is a direct relationship with the the syndrome and
the headaches. So basically their conclusion was that 50 to 95 percent of
patients could respond to surgery at least with some improvement of the
headachesand in their series that the best improvement occurred in arachnoid
cyst patients, Rathke’s Cleft Cyst patients and patients with pituitary apoplexy and resolution of the hormone secretion. So I’d like to switch gears just a little bit and talk about some of the
mechanisms we think about the little bit more depth with regards to the development of
these headaches we talked a little bit about this already but the direct dural
stretch is one mechanism that we feel is the most likely cause of these
headaches but also their structures nearby so the trigeminal nerve with is the nerve that supplie sensations in the face of lives in the cavernous sinus they can get irritated
and you can get a syndrome called trigeminal neuralgia a different type of headache more like a facial pain
than a headache if there’s inflammation surrounding the lesion so if it’s an
infection or inflammatory process that itself can irritate the dura and cause headaches
and if there’s increased intracranial pressure either due to direct Mass
Effect from a very large tumor or due to hydrocephalus with you mentioned earlier
that is itself can cause headaches in addition to the tumor being president and
then if there’s any bleeding directly into the tumor theres just a sudden increase in this volume of the tumor that can cause headaches as well so we’ll talk
about all these so as I mentioned direct dural stretch essentially could
occur anytime you have deformation of the normal structures so the dura which is
this layer here that I’m tracing covering the pituitary gland the cavernous sinuses which is the blue blood filled areas as well as the nerves that go to the eye and to the face well when we as in you saw this and the
figure before when this gets stretched the nerves get irritated this is a diagram of the different parts of the trigeminal nerve as it innervates the head which many people are already aware of this that most of the facial sensation is
innervated by the trigeminal nerve cranial nerve but this is the inside this is
on the inside of the brain and pituitary gland lives right there right where my
arrow is, it’s called the sella turcica and the pituitary gland sits right there and this
entire area is innervated by the first branch of the trigeminal nerve so come back here you see that the first branch the V one branch of the trigeminal nerve
is innervating the eye and the forehead this is the most common location of the
headaches as we had mentioned for pituitary tumors. Also it goes up to the top of the
head to the tip of it of the nerve extension and that’s also another common location so these are all related to the location of the pituitary tumor
sometimes however the pituitary tumor won’t just stay here and we’ll
go into the posterior fossa which is this purple area back there or out sideways and you’ll get a pain that refers out to the cheek or towards the back of
the head as a result you can see the back of the head here or the cheek area
there. So here’s an example this is not obviously not the same patient but
here’s an example of a patient with a microadenoma as we have shown before that grows into a large macroadenoma as it progresses and you can see how the
dura will get stretched to the point where you would expect it to cause some headaches not all the time but sometimes. This type of lesion is actually a cyst so this is
a Rathke’s Cleft Cyst and I know that because it’s splitting the anterior pituitary gland and the posterior pituitary glands and we see here that the Rathke’s Cleft Cyst quickly get large
quickly meaning over a few months or years but still it can enlarge and
stretch into this area you can already assume that there’s going to be some
stretch of the pituitary gland these cysts can occur within the
pituitary gland or above its up along the the course of the infundibulim with the
connection of the pituitary gland in the brain This is an example of a patient who had
headaches related to a cyst above the gland and here we are in surgery and
you can see that there’s a little bit of bowing through the dura its a little bit stretched we cut into the pituitary gland which is a common way of getting into
this area and we drained this Rathke’s cleft cyst and headaches subside because
the dura which is above it stops being stretched here you see after surgery This is a patient with an arachnoid cyst
the fluid matches the cerebral spinal fluid within a system surrounding the brain and
essentially this is a CSF leak within the pituitary gland the CSF is pushing into and stretching the pituitary gland so it’s a decent amount of pressure and just by draining this we
were able to decrease the pressure So this is a similar patient this is bone that you’re seeing so the bone is
actually been thinned out by this process to the point that its eggshell thin
and we can easily enter with the soft instrument or a blunt instrument and
were able to open and expose this area as we move forward we enter them this we enter we see as soon as we enter the cavity with fluid gushes out and
we’re going to introduce the endoscope into the cavity and you’re going to see at
the top the area where there is a communication
with the brain and the pituitary fossa and you can see there’s going to be some fluid coming from just this area right up here the top left of the screen you’ll see a small amount
of fluid coming out and that’s why this patient is developing the cyst and as a result
these headaches because there is a direct communication in that area all we need to do is a obliterate that and the cyst will not reform and the headaches should should resolve over time Well trigeminal neuraligia is another condition that I treat and trigeminal neuralgia is a syndrome where the trigeminal nerve
is irritated due to various reasons and the first thing we think about when we
see a patient with trigeminal neuralgia is to make sure that there aren’t any structural
lesions causing pressure invading the trigeminal nerve that would result in
pain and so we get an MRI in many times or a number of times we identify a tumor in
the area that could be pushing on the trigeminal nerve. However numerous times we we don’t see that and we have to look at other causes the
symptoms of trigeminal neuralgia are a bit different therefore it’s basically severe stabbing
or lancinating pain so this shooting pain down the face that could be triggered by
something as simple as touching the face, chewing, brushing your teeth, or even air or wind touching the face can trigger this this intense pain pain out of proportion to
the stimulus and it’s typically responsive to drugs like a cabergoline, sorry carbamazepine which is tegretol or other anti-seizure drugs or anti-epileptic drugs however in some cases it doesn’t really respond well. If we’ve ruled out other
causes then we wonder if there’s a blood vessel that’s a sagging and pushing on
the nerve there which is one of the more common types of treatments for this
essentially what that does is it irritates the nerve and it causes a short circuit what’s called ephaptic
transmission of the trigeminal nerve and allows for any sort of sensation to be
interpreted as pain by the break this is an example of a surgery where we’re
looking down in the area you can see this white band right there in front of the
brainstem that’s the trigeminal nerve and there’s an artery right there indenting and and pushing into the
trigeminal nerve irritating it every time it pulsates and resulting in the pain of
patient is experiencing So in this type of operation we would separate the artery
from the nerve by putting in different types of substances generally
teflon to separate the trigeminal nerve from the from the surrounding
structures so here you see an endoscopic view of the trigeminal nerve and the
arteries running right along it and all we all we do is put a little bit of teflon
there we go and secure that into place the teflon will prevent the
artery from touching a nerve in the face The patient’s pain is most of the time
improved by this about 90% of the time this works when we see an artery
touching the nerve. As I mentioned before sometimes it’s not an artery touching a
nerve sometimes it’s a tumor or lesion along the nerve that could be the
culprit so this is an example of a patient with
a right-sided the images are flipped right-sided facial pain in right-sided lesion
that’s in this area where the trigeminal nerve lives called Meckel’s Cave and it’s extending towards the brain stem so this is the patient who really only has pain in the face and
we operate on this mainly to make a diagnosis and if it is something benign that should be removed then remove it completely and here we have our exposure
and you can see this is where the pituitary gland lives up in the top left
of the screen this is the carotid artery that supplies
blood to the brain and it’s actually going sideways and upwards here and this is
the covering of the nerve as it’s coming over the hump is it goes into the face
you can see that little fibers of the nerve coming through and just behind it we see this solid structure here that solid structure is the lesion we cut into it and we biopsy that and in this
patient that the biopsy proved to be I believe in this patient proves to be a
granulomatous process so it basically is an inflammation of the nerve itself and
that became a big mess and that was pushing on the nerve and just by diagnosing that were able to
get her on the appropriate treatment and her pain her lesions got smaller and her pain went away Well other things can also irritate the
nerve such as other inflammatory conditions like sarcoidosis or Wegener’s vasculitis, Temporal arteritis, or even surgical products that we put in there at the time of surgery can irritate the nerve. Well Meningitis is definitely one of these conditions and we
had a patient come in with a pretty severe headaches nausea vomiting and the
CT scan showed a large lesion but nobody really investigated that she had been having low grade fevers and they just started her on steroids and she got a little bit better but actually then turn to took a turn for the worse and came to our hospital with meningitis, the meningitis was actually the cause of her headaches she had this very large tumor and it had actually had been associated with cerebral spinal fluid fistula or leak and that was a conduit for bacteria to enter into the brain and these are her findings
and she actually had an elevated white blood cell counts of her blood quite
significant low sodium level and multiple pituitary hormones that were
affected we were able to treat her meningitis
which was somewhat profound and once we got that under control immediate diagnosis of chordoma and we went forward with surgery and this is an interesting finding well we see the tumor in this
area here we also see evidence of meningitis on the diffusion-weighted
image of the brain along surfaces which is not a common finding but it is
concerning and requires urgent management and this is her operation here and you
see that she had a large tumor in this area we go to remove much of it and they’re
just a part that’s not going into the brain stem and this is the part that we
carefully debulk and I will just speed this up in the interest of time and you can see there’s
a tumor than remains as we’re approaching in this area going towards the spine and the brain
stem and we carefully remove as much of this tumor as we can safely in her case
was somewhat stuck to the brain and we don’t want to damage the brain so we
remove as much as we could and we left a little coating behind just so that she would have normal function as she would wake up but after surgery she actually did well her
headaches got better her meningitis improved now that we have a seal preventing cerebral spinal fluid from from leaking and we’re able to get her back to her daily life.
But this is another example of inflammation of the of the dura so this
normally should be a thin white line and you see it’s much thicker you see a
large lesion here that’s quite bright in the pituitary gland region and this is a
patient with sarcoidosis so another inflammatory condition in this area. This is a patient that I took care of who had Wegener’s granulomatosis another
inflammation and this interesting situation was that not only did the pituitary gland get inflamed but the nerves to the eye the optic chiasm got inflamed that actually caused the patient to lose vision until we are able to treat her with a pretty
significant high-dose steroids and rituximab were able to get her lesion to
shrink and her vision to improve. Last, second to last is the topic of hydrocephalus hydrocephalus essentially translates to
water on the brain basically it’s a result of dysfunctional
cerebral spinal fluid circulation we have called it many things here this
is a hudrokephalon not to be confused with hydrocephalus essentially what happens is you have the normal fluid-filled cavities of the brain and they’re constantly making
fluid and allowing it to flow from this area here to this third ventricle here
down through this little aquaduct through the fourth ventricle it comes out
through these holes here three holes here and it bathes the entire brain and
spinal cord and then gets absorbed by the blood vessels at the top of the brain and this gives the brain some buoyancy keeps it from being moved around too much nourishes the brain and allows the nerves to work well that’s a very important part of part of the brain physiology however if
you have a blockage either within the system or in the absorption of the fluid
then this will all back up and get larger because as i mentioned there’s no off switch. This is called the choroid plexus because it looks like coral and water and
it is actually producing the fluid in that area nonstop this is what it looks like under
water and so anytime you have a blockage anywhere along this flow we would worry
about hydrocephalus the fluid that it made bathes about the
entire brain twenty five milliliters of which are just in the ventricles most of
it are actually bathing the brain is made continuously you make about 20
to 30 millimeters per hour as and adults and you make about six hundred milliliters
per day so the brain will make three or four times the fluid it needs per day in
this area it relies on a sodium potassium pump relying on ATP to pump it across and it utilizes the carbonic anhydrase enzyme to convert the water in the CSF side so that’s a target that we can utilize because Acetazolamide which is a diuretic drug can be used to inhibit carbonic anhydrase and decreases
CSF production although it’s just a temporary effect for most patients there’s some other drugs that can work but this is really the only drug that’s been shown to help
decrease in a little bit but not significantly so so in general to thing
to remember that there really is no off switch with the system in the brain is
not smart enough ironically to know that it needs to stop doing that and it hurts
itself in the process Just an aside years ago I went to France
with Bob Bartlett from University of Michigan and our Victor Vaughn Medical History Group and we got to visit the catacombs beneath the streets of South Paris where
we saw famous peoples skulls and bones buried there we also went to the museum and the
Pasteur Institute we saw these skeletons and these skeletons are unfortunately children that passed away because they had hydrocephalus can see their heads are quite large and
quite blown up there and it’s because the skull actually accommodated to the
excess fluid there which cannot happen in an adult but it’s eerily similar to
some of our favorite Hollywood characters here that we’ve seen. This
is probably true patient with hydrocephalus that they call as an alien in
roswell. But going back to more serious topics the way we would treat a patient
with hydrocephalus is is one of two options either we shunt the fluid so
it put a catheter into the fluid-filled areas that ventricles of the brain
connected to a valve underneath the skin and tunnel this tubing all the way
down to the belly or other structures into the body to absorb the fluid in
that generally will take care of the excess pressure that this is exerting this has
been used for fifty sixty years now. The other option is to perform what’s called
an endoscopic third ventrical ostomy which basically is kind of
like an internal shunt or a bypass connecting the inside and the outside of
the brain and this is only useful when you have a blockage in that original
system as i’ve mentioned if you have a blockage say here or here you can
create a perforation of the floor just behind the pituitary gland and the fluid
will flow through this area and will decrease the hydrocephalus in this area
Well the hydrocephalus can contribute to these headaches as we talked about
particularly with patients with Craniopharyngiomas because they tend to grow
into the third ventricle and depending on the acuity we may need to treat that
urgently first before we do the surgery for the craniopharyngioma or other offending lesion. This is a patient that we took care of who actually have been treated
another facility with a large lesion that was proven to be a craniopharyngioma they approached this with a craniotomy and they actually put in a shunt at the same time to treat her
hydrocephalus which she had at the time. So she came in and fortunately in her wound had broken
down and we had exposed hardware so we were obligated to remove the shunt and we
put in a catheter to manage her pressures then we went through the nose
and we debulked some of this tumor and we connected the fluid-filled areas of the ventricles to
the to the rest of the brain region to the subarachnoid space that actually
relieved her hydrocephalus and she didn’t even need ventricle the ventriculostomy tubing or a shunt anymore so she was able to become shunt free. So that’s a third
way of managing is to just remove the offending lesion as best as possible she
actually did well we we treated with radiation and their tumor actually
strong and well controlled And this is a good example of a patient that we saw
and I asked this to some of our students What is the most common missed lesion in the brain? and the answer is the second one the patient has a pituitary adenoma
causing pressure on the optic chiasm but also has a colloid cyst causing significant
hydrocephalus and in this patient the treatment that we opted for was to first
remove this colloid cyst because it was causing hydrocephalus before we
effectively treated the pituitary gland which is causing hypopituitarism and we
were doing that because we didn’t want to have a scenario where the
hydrocephalus resulted in excess pressure and if we had a cerebral spinal
fluid leak it wouldn’t heal well so here we are into the ventricles and you can see
this choroid or coral looking structure that’s making the fluid and
just going too fast forward through much of this and were able to remove the cyst
which is hiding behind this vein in this region here and by by opening this up we
create an opening into this cavity here which is it was called the Foramen of
Monro and allowing us to decompress the area
well Once we complete we can see into the third ventricle we have a good
decompression and the patient actually did well without any worsening of his hydrocephalus and then we went on to take out the pituitary tumor and he’s been recovering well since then. And finally I want to leave you with the concept of pituitary apoplexy and of all the types of headaches that we are discussing today this is probably the
most concerning because not only is it about headaches, but it could be life
threatening or site threatening both of which you want to address right away. So this is an example of a 43
year old man who woke with acute sudden onset left eye blurred vision and a sudden onset headache and his vision had actually gotten a little bit better since that episode but we saw him and we
recommended him to undergo urgent surgery because he had notable vision loss and
hypopituitarism and you can see here the visual fields were were affected by this
lesion but he had hypothyroidism slightly elevated prolactin level and
you can see here pituitary adenoma with hemorrhage inside
this dark area is blood that had bled into the into the tumor area and so
we were able to decompress this patient and he did well. This is a study recently
looking at the different presentations of patients with pituitary apoplexy and
I don’t know if you can see this well a number of different conditions we
think can precipitate it including high lipids or hypercholesterolemia some people think dopamine agonists can actually cause this that’s a controversial statement. Some of the presenting symptoms headaches are common, oculomotor palsy so meaning double vision because the eyes aren’t moving well it’s actually very specific to this type
of condition and then hypopituitarism. So low thyroid, low growth hormone low cortisol and in general overall could be associated with pituitary apoplexy. and long-term we find that some people
respond well to early surgery and unfortunately some people have delayed
surgery and in in those cases we end up with more some of the adverse effects of
this. So if surgery is delayed there’s a higher rate of visual loss visual field
loss which is statistically significant and we think there may be a little bit
of a higher rate of hypopituitarism although this wasn’t statistically
significant so this is that patient with pituitary apoplexy here and I’m going to speed this up as well as we enter into the covering of the pituitary gland you see that that’s the
gland and you see that is quite vascular and when we entered into this area
you’ll see that they’re some blood products that will spill out with a necrotic
tumor, much of this tumor is necrotic this area there’s some blood that spills
out for the cavity where the patient had bled and so we will forward and remove
as much of the tumor as we can safely here we are. Now we’re looking at the
pituitary gland separating from the rest of the structures here we seal
that up with fat and collagen sponge. The patient did well afterwards after surgery This is his vision, visual fields at two months follow-up that showed that his vision actually had improved despite his onset headache sudden onset symptoms. So in pituitary
apoplexy the most common symptoms are a sudden onset headache they usually say it’s the worst headache of my life it’s like a gunshot to the head. Typically associated with vision loss
usually tunnel vision or bitemporal hemianopsia hormone dysfunctions this extreme
fatigue just can’t get out of bed and your very abulic your that’s a warning
sign on it’s own and other neurological deficits of double vision facial pain numbness etcetera If any of these occur even in somebody
who we are monitoring their benign tumor we offer offered not to operate this is a
surgical emergency and needs to be dealt with right away so we don’t have permanent vision loss and permanent hormone dysfunction So to summarize I think the take home points of this talk are just be aware of headaches that occur when your waking up
those tend to be associated with more aggressive disease in general. Be aware of progressive headaches because not only could they be associated with more
aggressive symptoms more aggressive tumors but they could be associated with
hydrocephalus or cerebral adema or cerebral brain swelling in the area
Size is not everything and hormone resolution actually can help with
headache treatment. Tumors with cysts or tumors with hydrocephalus are typically
respond well to surgery As far as headaches are concerns and as I
mentioned resolution from hypersecretion can be associated with improvement of
the headaches and with that I’ll leave you and I’d be happy to answer any questions. Thank you That was excellent, thank you. Okay we do have some questions. The first one is it all headaches that resolve completely one
week after surgery? my spouse had surgery for pituitary
adenoma and the headache lasted up to three weeks after surgery she had a
spinal drain put in three weeks after surgery due to a leak? So then the definition by the
International Headache Society with seven days and it’s an arbitrary number
that is not how these headaches actually resolve I would say a typical headache
that has dumped directly associate with a pituitary lesion takes a few weeks two
or three weeks to resolve it in your case it sounds like you had a
confounding issue with having a spinal drain and spinal drains alone can cause
headaches so that may be muddying the water a little bit but I would see a few weeks
is is generally a reasonable amount of time Not Ionly really get concerned when
the headaches are still present at about two to three months in that case we want
to look at other causes make sure that the sinuses are clean there’s no sinusitis issue that the
pituitary region is stable we usually get a new MRI at that point and then obviously
rule out other causes of headaches ok What is the prognosis of relapse of an
atypical pituitary tumor with a mitotic index greater than 2? Different
topic so we’re talking about an atypical pituitary adenoma generally the recurrance rate is a bit higher than the typical pituitary adenoma A typical adenoma normal recurrence rate is about 8 percent in 10
years and atypical adenoma recurrs or progresses about depending on what study or read fifteen
to thirty percent in 10 years so it’s two or three times as likely to recur
and the other point is this was a study that came out about two years ago that
it’s not just the grade of the adenoma it’s also its invasiveness if you have a
high atypical adenoma it is it has a certain rate of
recurrence you have an invasive adenoma it has a certain rate of the kurds but
if you have an atypical invasive adenoma a lot more aggressive in these times
reporter progress and it’s very hard to achieve remission in patients who we
would recommend very close follow-up and probably adequate therapy at the radio
surgery or or even she was all about has been used in some of these cases but
those are it’s not just the pituitary grade that’s that’s cheap but also its
invasiveness and sometimes it’s hard to differentiate that on a pathology slide
because the pathologist only just the meat of the tumor so you have to look at
the imaging and see if there’s any invasion on the imaging and if you’re
lucky the pathologist will get some dinner on the covering of the region and
that will actually help diagnose invasion as well ok can hire igf-1 cause headaches yes based on that study that I showed
higher education definitely can cause headaches we mechanism for his multiple
it could be due to a TMJ it could be due to sinus obliteration because he says
with a Camaro attention of your sinuses and sinusitis sometimes or at least
allergic rhinitis and also some of the bony aspects if you have significant
neck pain that can also translate to headaches as well so there’s numerous
houses for headaches with patients and patient back regularly and they showed
you and that’s like many of these patients who were treated with with even
just somatostatin analogs improving their headache severity ok can
small micro nano must cause headaches too so in our series that we looked at a
hospital we did not find it knows they typically patients that we’ve operated
with my grandmas had another process going on I don’t they had a blocked
number two was your prior to therapy or their tuitions as these really are
correctly and in those cases as we would say reaching remission would
actually help with the headaches like to know there’s a little bit fifty-fifty
with regard to responsive headaches and then the other thing to remember is many
patients get MRI’s just because of the headaches and we see these small at
anonymous so it’s very likely that it could have been an incidental adenoma
now we we identify and this is where it’s really key to conservatives to
really characterize the headaches you get multiple images and see if that
grows and if they had a pink it all with relation to the size of the tumor or
with relation to the severity of the headaches as a tumor change and those
are those are some factors to think about because there’s definitely a Venn
diagram overlap with headaches impatient with 22 cherry tumors ok thank you
because of the gutter brain connection via Vasil vagal nerve is there any
literature suggesting a connection between post gastric bypass or lap and
and later development opportunity to Terry tumor or dysfunction causing havoc
that is a very good question I have not heard of that I have not seen that in
our literature the vagus nerve actually is a bit separated from the petit jury
system it’s more control the brainstem level so hard to create a link there but
I have not seen that in our letter ok thank Terry glenn has been removed
totally remove a starter and regular blood work is done how can anyone
determine what normal levels are in such a patient how can their levels be
compared with charge is for patients with no known allergies so we’re talking
about the hormone levels I believe so oftentimes we still go by normal levels
in patients who don’t have to turn reasons that we want to see a cortisol
levels and the igf-1 levels to be to patients who have not charity work of
course age and gender matched some of the challenges I think we see our
patients who have hypothyroidism because the general public treat hypothyroidism
by looking up the TSH thyroid-stimulating hormone level but in
patients with hypothyroidism due to the tutor really should have had both you
cannot swallow that anymore so you actually have to follow your patient’s
symptoms as well as the t3 and t4 those are the active and reactive normal
levels and those would be the reverse rape monitoring that sixty different
location does not appear to torture and as hypothyroidism where you would just
say the other hormone beckett’s a bit altered his prolactin particularly in
patients with a very very large 222 @ November we looked into this field has
been published but we we think there is a correlation with very very low levels concurrently with the ocean so a local
like novel after surgery is a bit deceiving compared to look like before
but then the question that doesn’t even really matter so really doesn’t affect
our patients along ok can enlarged periventricular space mean anything when
a person suffers autonomic dysfunction and has family history of meningioma as
pituitary tumors and interventricular sis it’s hard to say because now we’re
talking about kind of a chicken and the egg situation one would say you have
large ventricles because it’s causing pressure and is it causing pressure on
the surrounding brain structures or the other large banks closed because of rain
has atrophied because of the process like you know side Rieger syndrome or
something that would cause dysfunction and that would require an
evaluation by a neurologist who specializes in these to make sure
they’re there isn’t something else going on however there are some quite sure
that we use to see if the ventricles are out of proportion to the size of the
brain and if you read some of those specialty sale maybe the ventricles are
causing some of those problems and there’s a syndrome called normal
pressure hydrocephalus that can result in some symptoms of decline incontinence and a typical tease but
usually want to see some of those in a trial before treating those cats in
water shot the same person also commented I meant a small six million
manner while robbing space in the basal ganglia to clarify that something
different those that would be hard to say that I
have to look at the film’s to be certain enterprise the water is function but
generally bert roberts spaces are are thought to be something we wouldn’t
treat for sure and maybe sometimes not even related to the pathology being seen
so it really is evaluated by a minor all is ok and the presence of persistent
progressive localized headache in the absence of hormone abnormalities on
initial down and just enough of a reason for evaluation by an endocrinologist
versus just stand with the PCP internists so the question is a question
to be evaluated by an endocrinologist primary-care probably should discuss it
with your primary care physician sometimes there are some subtle
differences in the hormone levels and not all of their being assessed so that
may be a reason to talk to an endocrinologist who particularly
somebody who specializes in the particular a plan but I would want to
know more about the condition and also if there’s any abnormality and what are the general symptoms I was
diagnosed with a form element manner to carry my grandma the frontal headaches
you mentioned and often they are severe in nature and cause vomiting and mimic
the description of a migraine sensitive to light my endocrinologist simply
advised me to wait and be rescan a year although I have hypopituitarism and
headaches pressure on my head would you suggest a second opinion if so is there
a different type of doctor I should be seeing other than an endocrinologist
sounds like they had occurred offices is correct you know headaches with nausea with a
very small to carry my credit is probably not caused by bad mic right now
it’s too small to even cause pressure in the area if your hormones if you don’t
have hypersecretion senior on like kissing disease or automatically then I
don’t think that’s related to your headaches I would stick with your other
colleges to treat your future dysfunction and reimage in the year as
mentioned and helping my clients treated I i dont your primary care doctor or a
neurologist ok could nice to mess and one i mean a compromise of the optic
nerve from a nine-millimeter non secreting so nice time someone I earned my status
in one direction i think im assuming thats the case generally nice that
missus an issue of either the brainstem or the nerves coming out of the brain
stem that would control the issue of side of the brain stem and that it
really is that the ISO to my status is actually generated down to that level
the nerves that are coming the jury heard discussing this belated
admission they don’t generate a nice night with so I don’t think that’s generally the case we have to look down
at the brainstem in the throat physician evaluate you for your nice statements as
well ok I had a question that I received actually prior to 11 arcing and may have
discussed this a little bit but wanted to know if you encounter any cases where
pituitary tumors had caused headaches that manifested as trigeminal neuralgia
pain was diagnosed with atypical trigeminal neuralgia house at four hours
once they also began rather than in urban first of a few minutes years before my two morrow is dying that
finally diagnosed my research indicates that the nerve root for the Giants
seminal event is directly behind the pituitary so it seems to me there must
be some sort of connection also if they do know or have experience with this is
there any particular clinical significance associated with it so we
did discuss it has as you mentioned so I’m glad we talked about that but i’ll
i’ll summarize in shorts and a generally the tutor to her in the set are aligned
itself did not cause you’re trying to look around it’s usually two roads that
are over to the side that are either of the top of the sinus which is right next
to the teacher glad we’re down into records cave which is where the
absolutely exhausting and in those cases you could get right around so if you
have it invasive pituitary adenoma into the cavernous sinus are pushing on the
cover of sizes could be related I don’t know if you had surgery in with your
diagnosis is it exactly but very hard to predict clinical significance based on
that I think that if there is compression and potentially surgery
removal can help with the pain but I would really have to carefully look at
your meeting to make sure we can coordinate that well okay if a patient
has many of these systemic symptoms meaning daily debilitating headaches fatigue in addition to sudden
onset hypopituitarism vision loss in vision disturbances along with other
central nervous system sometimes but a normal MRI you say that not all these
tumor show up on an MRI at that point I would definitely discussed this with a
neurologist because if you have that kind of significant symptoms Michael and
nobody can be seen probably not because of your headaches but definitely talk to
her oldest and also want to rule out there in your arms like Cushing’s
disease but you probably typed your primary care doctor about that first
five-millimeter not secreting pituitary tumor and a typical bilateral time and
in general and all three branches I have intense pressure in both ears and AM I
had twenty-four seven of my doctors can explain why I have this pressure on
twenty seven hundred milligrams gabapentin economy from the operating
room can you repeat that question here yes sorry about that I have a
five-millimeter non secreting pituitary tumor and a typical bilateral trigeminal
neuralgia in all three branches of intense pressure in both ears and in my
head 24 7 not have my doctors can explain why I have this pressure on
twenty seven hundred milligrams gabapentin yeah I don’t think the
teacher at Norma not small would be able to cause bilateral try to unroll I think
there’s something else going on I would definitely recommend a neurologist who
specializes in trigeminal drought or facial pain if these medications are
cutting it there some other options but remove them to take it would probably
not up with your headaches ok I had surgery for acromegaly in 1997 and then
on to understand now we are 20 milligrams sense recently I was unable
to get the shop for two months and got a severe headache was a minute to the
hospital for two days and many tasks including bonds concluded that there was no reason
for the headache two days later I got this and this and that I R 29 shot my
headache went away but the lack of injection have been the cause of the
headache if so any suggestions percent to medicare so this won’t happen
again I mean it certainly makes sense based on what you said certainly fits
with your I don’t know if you like to buy it and acknowledges what type of
test they had you may want to look into that a little bit further the only other
way to prove it is to stop it again as if you had a comeback and resolve the
gas then you have a pretty convincing argument there but it did I think that
falls into line that you may have a direct link there there may be also a
correlation being on it for so long just the dopamine levels affected by the
Senate staten then all of a sudden you lose that a fact that actually make
contributors while but now that’s just not facing down and he farted hard
evidence may have pituitary tumors or meningioma and hydrocephalus been linked
to Parkinson’s no Parkinson’s is a different type of condition is it the
generative condition and we have seen parkinson’s with Parkinson White
syndrome with trauma or other structural the isn’t but not really a tumor in the
area ok I have economically surgery to remove
micro macro years ago surgery did not remove tumor I still
have a headache have tried a number of medications and kinda just accepted that
now I guess more than anything I just want to know if this is abnormal sounds
like it fits with the central I’m not sure if you’re on medication to help
treat sir but but certainly that could help get your Disease Control and we’d
like to see if your headaches with that can hide yourself less
pressure ever be relieved by a CSF leak through a fistula between maxillary
sinus where infected tooth has eroded job interesting questions that’s not
what you’d want to really fight or selfless but we certainly have seen CSF
officials as a result of elevated intracranial pressure that comes through
the sphenoid bone and I haven’t seen in the maxillary sinus but I have seen it
in the sphenoid sinus and not require surgery to fix it but it doesn’t solve
the issue of the hydrocephalus so you’d want to get that created by a
neurosurgeon and also you may want to make sure that is yes efforts of
responsibility and sleeping in that area and and before it becomes an infection
ok I UD releasing a synthetic hormone over the course of five years contribute
to the development of a pituitary Mike right now so I usually they are coated
with progesterone type of hormone and I don’t think that in itself can induce it
would be highly unusual I think that covers all of the questions we have and
we’re getting past time frame here and you’re getting calls from the are so I
think we will wrap it up this concludes our webinar presentation we did not get
a chance to answer your question will be answered them via email the pain and wishes to thank you for
participating in a webinar a brief survey at the end please help us provide
you with the information you need I did have a question about whether or
not this will be available after it has been recorded and after a little bit
added and some converting it should be available on our website tomorrow so if
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be available probably by tomorrow afternoon you have any questions you can contact
us at webinar a pituitary door or give us a call at 8:05 4999 973
you’re getting a bunch of little messages thank you thank you and so we
definitely appreciate your time market area and that’s great information
that you provided for us thank you so much this concludes our webinar and until
next month will do this again thank you thank you

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