PNA: Cushing's at 16

Copyright Robin and Pituitary Network Association, 2015.

I am thankful to read a Cushing's article in the latest Pituitary Network Association's newsletter. Anything we do to put the word out about Cushing's is good.  I also want to thank high school senior Robin for sharing her story in the article Cushing's at 16.  Every story puts a face to this horrible disease. The photos featured really let you see how Cushing's can change a person's physical appearance.

"Cushing's definitely made me the person I am today, and even though the experience was terrible, I am proud of who I am now." ~ Robin, 18 years old

We all can learn a thing or two about strength from Robin!

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I do wish that the PNA would have featured a story of someone who required multiple surgeries before getting better.  Highlighting patients who had one pituitary surgery and got their cure is not representative of the patients who experience Cushing's. I know far more people who required multiple surgeries than those who were cured by one.  In my post entitled Cushie Warriors, I begged those with a Cushie in their life to support them, as many of them required multiple surgeries and treatments and still may not have a cure. It seems odd when so much information is out there about how pituitary surgery cures a Cushie, and it was important to let the few that remain part of the Cushie's support system to know that their Cushie-who-can't-get-well is not the exception but the rule. When I collected that patient treatment information and posted over four years ago in April 2011, there were already 50 people on the list. Kinda high for a rare disease, right?  I have an updated list of Cushie Warriors that tops over 125. That sure is a lot of Cushies out there who are trying to get people to understand that we are really sick and we have to go through a lot of testing to be rediagnosed in order to have multiple surgeries. It would further understanding for the complexity of Cushing's treatment if a national organization like PNA could help us get those stories out.

After gaining 120 pounds in 1 year, rare diagnosis saves man's life

Cushing's was featured this morning on the Today Show's Medical Mysteries three-part series. 

Hooray for national exposure for Cushing's!

Donelle Trotman, a father in his 30s, suddenly and unexpectedly gained 100 pounds in a year as well as experiencing strange symptoms.

Donelle shares our desire to spread the word and increase patient awareness about his diagnosis. Read the story and watch the video to see yet another example of how devastating Cushing's can be.

We thank you, Donelle, in joining us as a Cushing's Crusader. We wish you all the best in your healing process. Please contact me if I can help you in any way. 

"There was no time to lose: Untreated, Cushing's is a fatal disease."

“The tumor was no bigger than the size of the tip of my pen," Boockvar said. "And that something so small can cause a man to grow to 350 pounds and absolutely destroy his life is rather remarkable."

http://m.today.com/health/after-massive-weight-gain-cushings-disease-diagnosis-saves-mans-life-2D80527683

Game Time: Anatomy of the pituitary region

If you ask a Cushie, they will tell you that radiologists have an awfully difficult time finding tumors on MRIs of the pituitary. In their defense, these pituitary tumors are very small, often 3-8 millimeters, or a quarter to a half of an inch. These tumors sometimes fall between the 3 mm 'slices' an imaging machine makes, and the patient ends up with images with the 'slices' touching the front and back bumpers of the pituitary tumor but never see the tumor.  Many Cushing's patients have MRIs that radiologists read as clear of tumors. Uneducated primary care doctors and endocrinologists thus rule out Cushing's,  thus extending the time that the patient wanders around undiagnosed, unhelped, and unwell. 

Patients should seek out second opinions from a well-vetted neurosurgeon specializing in pituitary tumors. These doctors understand that tumor detection is more nuanced, and as such, they can usually locate these tumors by examining the structures surrounding the pituitary gland for compression, indentions, or asymmetry. In addition, these doctors are in the unique position to read the MRI before surgery and compare it to what is seen/ found during surgery, thus honing their MRI reading skills.  A radiologist has no such environment to do so. 

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ENDGAMES

Anatomy Quiz

Anatomy of the pituitary region

BMJ 2012; 345 doi: http://dx.doi.org/10.1136/bmj.e6963

(Published 24 October 2012)

1. Correspondence to: O Shaw at olga.shaw@doctors.org.uk

Identify the structures labelled A-F in this magnetic resonance T1 weighted unenhanced sagittal image of the pituitary region of the brain⇓.

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Answers

• A: Optic chiasm
• B: Hypothalamus
• C: Pituitary stalk
• D: Posterior lobe of pituitary gland
• E: Anterior lobe of pituitary gland
• F: Suprasellar cistern

Notes

Cite this as: BMJ 2012;345:e6963

http://www.bmj.com/content/345/bmj.e6963

My friend Lori faces 5th pituitary surgery

Please keep Lori in your thoughts. Her cortisol levels dramatically improved/ dropped following Monday's operation in which the neurosurgeon removed two separate tumors. However, the lab work indicates there is still tumor tissue present. She will be having another pituitary surgery -- her 5th in an area the size of a pea-- tomorrow at 7 am central time. The neurosurgeon will remove her entire pituitary gland and the bone surrounding it. This is a much more invasive surgery.

Lori has been fighting for too long to give up now. This surgery is her only option. She first went to the National Institute of Health, long recognized as the most knowledgeable about this disease, when she was only 13. Twenty years later, she is still fighting Cushing's. For many of us, Cushing's won't turn loose. This time, it will.

http://cushingsmoxie.blogspot.com/2012/09/a-case-of-ectopic-pituitary-adenoma.html?m=1

My Friend Lori, a Cushie Warrior, Faces 4th Pituitary Surgery

A friend with persistent Cushing's had her fourth pituitary surgery this past Monday. The neurosurgeon found an 8-9 mm tumor in her sinus cavity that was definitely a Cushing's tumor. This trouble spot had a tail of tissue trailing back to her pituitary to a small spot of abnormal cells.

The neurosurgeon believes that just a few microscopic cells fell during her first pituitary surgery and started a growth of abnormal ACTH cells that caused her Cushing's. The patient had this abnormality in her sinus MRI and no doctor related it to her prior history of transsphenoidal pituitary resection. In fact, pervious pituitary surgeries, while done transsphenoidally (across the sphenoid sinus), were done sublabial (under the lip). This surgery was done endoscopically or through the nose, and this offered the surgeon visual access to the tumor that may have been hiding there since her first pituitary surgery 20 years prior.

Several other Cushies with persistent Cushing's  have commented since Monday that they too have a "cyst" in their sinus cavity. Many will now ask their neurosurgeons to examine these suspicious areas more closely as possible source of abnormal ACTH cells which cause Cushing's.

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A case of ectopic pituitary adenoma occurring in the sphenoid sinus

Neurological surgery (2011)

Volume: 39, Issue: 6, Pages: 601-605
Click for full article full article.

Abstract

Ectopic pituitary adenomas are relatively rare tumors. We present a case of ectopic pituitary adenoma occurring in the sphenoid sinus. A 63-year-old woman was referred to our hospital complaining of headache. She had no endocrinological abnormalities. Magnetic resonance imaging showed a tumor in the sphenoid sinus, adjacent to the sellar floor and appearing as a low-signal on T1-weighted image and a high signal on T2-weighted image. No connection between the normal pituitary gland and tumor was observed. Using an endonasal-transsphenoidal approach assisted with neuro-endoscopy, we performed total removal of the tumor. No connection between the normal pituitary gland and the tumor was found. Histopathological analysis showed a pituitary adenoma. As demonstrated by our case report, differential diagnosis of a tumor occurring in the sphenoid sinus must include consideration of the existence of an ectopic pituitary adenoma.

A Cushie's Job: Trudging through the Medical Literature

If you have spent any amount of time on this blog, you will know that I am patient advocate who encourages you to be the most informed and self-aware patient you can be.  For us Cushies, especially us cyclical Cushing's patients, it is imperative to be up-to-date on the medical literature.

You also know how difficult that task can be as we fight to keep our energy up for basic daily tasks and chores.  You also remember how Cushing's causes cognitive impairments that just don't give us the right state of mind -- clarity or positivity -- to take on a project this big.

So, I decided to just post stuff here, as I find it.  It may be a repeat (sorry, I probably forgot), or it may be new. Regardless, it will be stuff I am stumbling through as I relearn everything I have to know about Cushing's in order to make my decision.

I am reading through the medical literature for articles like Long-term remission rates after pituitary surgery for Cushing’s disease: the need for long-term surveillance posted by our dear Cushie friend Robin at Survive The Journey.  I am also trying to wade a list of articles my friend Susan recommended about cyclical Cushing's posted on PubMed, the government's database.  These free articles include research done with your tax dollars at the National Institute of Health (NIH). The NIH is considered an authority on Cushing's and even cyclical Cushing's. Based on what I have seen in my five years, the NIH seem to specialize in pediatric Cushing's cases more than adults, i.e. I see more pediatric patients than adult patients accepted for testing and treatment at the NIH.

So, I have to make my way through the medical literature about:

• quality of life for patients after several pituitary surgeries vs. quality of life for those who chose BLA
• remission rates for each type of treatment (3rd pituitary surgery vs BLA)
• enzymes and other hormones produced in the pituitary that may vanish after repeated pituitary surgery
• patient success stories for each

I mean, all this and a bag of chips. EVERYTHING!  I read a lot of this stuff in the beginning. It was "this could happen to me" reading, so the shock resonated longer than the information. Meaning, the scariness stuck when the facts didn't. 

Now, I find my mind so hazy that I just can't read through these medically-rigorous articles anymore. I really struggle.
I can't remember what I read already.
I can't find something I already found before.
I can't even think of good keywords to search.
I've already made a million laps around the internet.

It's a mess. I AM A MESS!

So, being that it is my blog, this is what I'm gonna do.  I plan to "store" articles here that I am reviewing for my own benefit. If you see something that you have never seen before, well, you benefit, too.

HEY!  THEN MAYBE YOU CAN MAKE MY DECISION FOR ME!

I Hate You, Cushing's Disease

Hi everyone. Sorry for such a long absence. In this past, I take time off from writing when I myself am too sick to talk about Cushing's.  When I am exhausted all the days, riddled with headaches, body aches, dizziness, and muscle spasms, I don't feel like the best public speaker for Cushing's. Nor do I have the energy to queue up posts to show up in my absence. Nope. I was too sick for all that.

This absence, like other times, is caused by my own testing, diagnosis, and clearance for third pituitary surgery. That's right. You read that right.

I still have Cushing's disease. 

I still have high cortisol at diagnostic levels on four different diagnostic tests. 

I have a third tumor showing on my pituitary that did not show itself last year at surgery.

I am still fighting Cushing's.  

I hate Cushing's.

When will it ever end?!

High cortisol and high ACTH.
GO AWAY.

Things get really complicated when you are this far into a Cushing's diagnosis. The likelihood of a positive outcome decreases with each stage of treatment. There aren't a lot of people in this world "like you," and even fewer that you may have access to. Nope, for sure, these tiny details make the next decision even more difficult on the patient.


And frankly, when can I cry UNCLE?
When can I say that I've had enough of this beast called Cushing's?
When will this be over?!


I am considering next steps for treatment:  third pituitary surgery vs. bilateral adrenalectomy with risk of developing Nelson's syndrome from an untreated pituitary tumor vs. medical therapy such as Korlym.   Each has its risks and benefits.  Which risk is greatest? Which benefit is greatest? Well, it seems everyone has a different opinion on that.

I fear that the effort to make this decision will exceed the energy I have in a day.  I spent the first 72 hours after learning of the presence of a third pituitary tumor in a tailspin. First, notify the family. Second, notify my Cushies, my besties. Third, reach out to neurosurgeons for second, third, and fourth opinions.  Fourth, reach out to Cushies who have been *here,* who have made this decision, and see if they can tell me something I don't know, something I've forgotten, something I just can't know until I've lived it. Fifth, look around the rest of my life, and do some things that need completing. Things that are super important to me, like completing the redecorating of my preschooler's room. Watch out world, a Cushie's got things to do!

My main concern now is wondering how I will make the right decision. How will I know what to do? Somehow the right path for me will present itself. Right? 


I am keeping my eyes open for all the signs, asking for help from everyone I can, including friends I rely on and can trust. 


I participate in my own healthcare. I am an advocate because this is my life, my body. No one knows it better than me, and I know me best. I've never left me behind.  I've never failed to show up.  I haven't missed any important events.  I have, however, been too busy to listen to myself.  I have placed other opinions before my own.  I have muted my instincts to appease someone else.  


In this situation, after all that has happened to me, after all I have faced, I just can't let that happen. I will know the right answer when it feels right *to me.*   So, isn't that my answer?   If I can keep my aim right there, right in that small spot of people to please, I hope to hit the bulls eye. As long as I can live with my decision, I'll know I made the right one.  When that happens or what it will be, I do not know. Stay tuned as I share information I'm sorting through as I make my decision.

Day 4: Around the Cushie World in 30 days: Ashley's Pituitary Surgery

Today, I bring to you a profile of my friend Ashley R. She does not blog, but she should.  Ashley and I have been writing each for many many months online.  I have used everything in my arsenal of knowledge to guide her, and she has been an excellent student.  I take my role as Cushie advisor very seriously, and Ashley took my advice and made her own journey shorter as a result. I am very proud of her diligence and persistence, despite being very ill.

I finally met Ashley R. in Los Angeles this past January 2012.  When she saw our shared doctor, Dr. Friedman, I drove her to the appointment.  

Beyond what I have taught her, Ashley R. has been a wonderful teacher to me.  She has become one of many Cushing's patients for whom I have deep admiration.  Most importantly, Ashley R. and I have had many discussions, including how to enhance our outlook in order to create our new destiny without Cushing's and despite Cushing's. We are both committed to moving on to powerful and healthy lives that we have only because of the Cushing's.  For this and many other reasons, I am proud to call Ashley R. my friend.  I find her so compelling that I asked her permission to share her story here for you.  

Ashley R. is having surgery in Houston at the MD Anderson Cancer Center on Friday, May 4 at 8 am.  Please keep her in your thoughts and prayers.

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"You have a brain tumor? What the heck is Cushing's Disease?"

by Ashley R. on Thursday, May 3, 2012 at 4:20am ·

I've spent much of the last year being quiet about the changes in my body and mind. Yes, there was chronic illness and debilitating/disfiguring symptoms, but I never really wanted to take ownership of what was going on - it always seemed like an invader to my body. I certainly didn't want to worry anyone unnecessarily and I definitely didn't want to jeopardize any professional contacts by littering my Facebook profile with too much medical information. For the most part, I tried to keep as normal of a schedule as possible.

... but I knew things were changed. I knew that I couldn't keep up appearances for long... so I (as gracefully as I could), withdrew from everything but what it took to survive. If you were a part of my life that didn't make the cut, you likely saw my involvement dwindle. I promise, I wasn't trying to hide. I was trying to survive. I am fighting so I can come back to full involvement. I promise, I'm almost there.

So here I am, about a year and a half out from the initial start of this mess, and I am ready to put pen to paper and explain what happened. Before I start, I need to be laser clear on a few things:

1. I am so grateful for the healing that this journey has brought to my life. This process flipped my world upside down... and in the process, forced me to confront things that I had avoided. 

2. I am confident that this is already cured and I am already healed ... my body is not broken, and this was no mistake. I don't write this for sympathy -- I've already grieved for what I thought I had lost. I write this story today from a place of gratitude... gratitude for my friends and family who have supported me through this and gratitude that I was trusted with this story. My prayer is that I say what needs to be said and that there be no mistaking that Jesus has been in control of the outcome from the beginning. I am at peace with what happened. 

Understanding that, let's start at the present.

Today, I lay in bed in a Houston hotel awaiting my turn to see one of the top neurosurgeons in the country. Tomorrow, I will undergo brain surgery to remove a tumor from my pituitary gland. Today, I pray, is the last day Cushing's Disease is allowed to be in my body.

What is Cushing's Disease?

Ever heard of the stress hormone, cortisol? Cushing's Disease is simply (ha!) an overproduction of cortisol in the body, usually caused by a tumor that overproduces the "give us more cortisol" hormone (ACTH).

Picture for a moment a time in your life when you experienced EXTREME STRESS. Perhaps you went through a trauma of some kind...perhaps you had a point in time where you had too much "critical" stuff to do and not enough time. Picture how your body felt - your muscles twitching between super strong and super goo, your heart beating fast, your head going from clear to mush... your appetite dropping only to hours later leave you ravenous... your body switching to survival mode with only one objective: run away from the tiger before it eats you.

Got it?

Now picture never being able to shut that off.

That, is the only way I can give you a glimpse of what this disease process looks like.

Take a few moments and watch this video - it explains the technical side of things quite well. It is worth the 6 minutes.

How did it happen to me?

I'm not really sure... but as I looked over the patterns in my life, it is entirely possible this is something I've been cycling with since I was a young girl. We started to get suspicious January 2011 when an overwhelming amount of fatigue took over my body and I gained about 30 pounds in 30 days - while dieting, walking 3 miles a day, and regularly practicing EFT to work through the emotional side of things. Something was wrong, something was very, very wrong.

As I scrambled for answers (surely, I had to be doing something wrong), a friend mentioned Cushing's. I scoffed, "now why would I want that? That's not fun."

A few weeks later, Samuel picked me up in San Luis Obispo and drove me to Seattle to be seen and have initial tests run. When the tests came back showing that we were on the right path, the choice was made immediately to move me back to Seattle permanently.

I didn't struggle through doctor after doctor who told me I was crazy. I didn't have the energy for that... I went straight to one of the leading experts on Cushing's Disease. For 8 months, I tested - blood draws, 24 hour urine collections, and saliva tests... Over and over again. Trip after trip to the lab. It was an exhausting and humiliating experience. Scratch that... it was an exhausting and humbling experience.

I tried to stop the process. I tried hard to fight it. When I stopped fighting it, there was a bit of a lull to the process. It made no sense. Throw everything you know about your body out the window. I was gaining weight eating a clean paleo diet and could get the weight gain to stop if I ate crap junk food. It made no sense. None. It still doesn't.

By September, most of the changes in my body had already occurred. I had gained about 150 pounds in a little over 7 months. My waist circumference doubled. My hair fell out. Reddish purple lines striped my arms, shoulders, and chest. Pads of fat seemed to grow both at the back of my neck AND on top of my collarbone. My face COMPLETELY changed. My moods altered and I fought against rage in my body. My skin turned both bright red and orange. Anxiety and paranoia were present daily. 

While I've avoided posting pictures over the last year, I feel like none of these words can quite show the change like this photo can. This my friends, is what the diagnostic process cost me physically. 

end of December 2010   -------------------------     April 2012

Dramatic, eh?  It was only 16 months.

I've tried (sometimes unsuccessfully) to rationally think through these changes. I've tried to keep perspective - that most of these things I felt were caused by the hormones NOT by real threats. In short: I did everything I could to not BLAME this disease process for any of my shortcomings. There were definitely limitations, but I refuse to give in completely and let this disease process take more than it needed to.

We found the tumor in October via MRI. You'd think, with an ever growing mountain of evidence supporting a diagnosis AND a visible tumor, that the doctors would be clamoring to take that puppy out, right? That just wasn't the case.

Midway through November 2011, I learned that my doctor was retiring his practice and moving in to drug research. I never managed an official diagnosis out of him -- the day he left his practice, he had spent 8 months being "highly suspicious" but never committal. Is this a doctor thing or a man thing? Who knows.

my buffalo hump

In January 2012, I flew to California to see another Cushing's specialist. There were several of you who prayed for and helped fund this trip. I am eternally grateful for your generosity.

When I met one of the doctors the first thing he said to me was, "Why hasn't anyone helped you before now? Why did they let you get this sick?" I do not have an answer to that question.

I spent most of February and the early part of March doing one final blitz of testing. In truth, the doctor likely had enough to make the final call, but needed tests that he had ordered to come back positive to do the whole CYA thing. 

Mid March 2012, almost a year to the day of my first appointment to discuss Cushing's Disease, I had a diagnosis: Cyclical Pituitary Cushing's Disease. 

Over the year and a half of progressively getting sicker, I was asked many times why I fought so hard for a particular diagnosis. After all, most people are traumatized when they hear they have a tumor and a life threatening illness. I, on the other hand was relieved.

I have a short answer to this: I needed 100% written proof, verified by a professional, that I did not intentionally do this to myself. I needed that proof for me... and for anyone else that would raise an eyebrow to me.

Sound strange? Sound selfish? Sound a bit ridiculous? Probably.

You see, I grew up an overweight child. I was blamed, from the age of...oh...8 or 9... for my weight problems. I've had so many labels put on me by doctors that refused to look for something other than a sad child who liked chocolate. I needed the diagnosis because I spent decades hating those responsible for my physical and emotional well being. I couldn't put my finger on it... but I never bought what they were saying.

The Cushing's diagnosis gave me the courage to forgive.

The process to get the diagnosis gave me the ability to appreciate and love my body in a way I can't quite explain - though I am going to try later this week. 

Anyway, here's what's next:

I'll have drive by brain surgery through my nose on Friday... then we pray that it is a cure. The surgery is really easy guys - I promise I am going to be ok... I am at one of the best facilities in the world and have one of the top surgeons in the world. He knows this disease inside and out and is going to take good care of me. Yes, please pray for me and for him for the actual surgery... but also keep my family in your prayers... as they are the ones who have to anxiously await the good news. Dad, Kelly, and Sam are here with me... and my sister is back up in WA (worrying no doubt).

Once I return home, I get a new fight on my hands: the "drying out" process (allowing the cortisol soaked tissues to get rid of the excess stored cortisol) and the "waking up" process (where my body starts producing hormones on its own again). They say the cortisol withdrawal is akin to a heroin addict going through withdrawals. Please pray for me. Please come check on me. This process scares me more than anything else.

Then, we rebuild from a place of gratitude. We learn from the process... and we move forward.

I am overwhelmed by the support, prayers, and generosity of those around me. I am overwhelmed at the humility my husband has shown through the whole process (I love you!). I am overwhelmed by the support of my bosses and coworkers - I've never felt more cared about by any other group of women. I am overwhelmed that women I didn't know came to visit me when I first moved back to Seattle and was lonely because no one was coming to see me. I am overwhelmed by the knowledge I've had the privilege of acquiring. I'm overwhelmed by the courage of the women who have gone before me in this fight - the women who took their time to navigate this process and support me every step of the way. I am overwhelmed that you took the time to read this... and mostly, I am overwhelmed that Jesus has been there through it all, going ahead of me, carrying me, and providing what I needed to come out on the other side of this in a much better place than when I went in.

Thank you so much for everything you've done and for the love you've shown to me.

All my love,

Ashley R.

Day 24: Pituitary Surgery and The Evolution from Open Transcranial to Fully Endoscopic Transnasal Surgery, and Beyond

I have several Cushie friends who recently had pituitary surgery with others headed there within the next few weeks.  For the Cushing's community, we celebrate these surgeries for the milestones that they are.  We rejoice!  After years of misdiagnosis and ill treatment from the medical community and even our loved ones, these are the days a sick person celebrates.

After having two unsuccessful pituitary surgeries myself, I just can't read enough about this process.  Please indulge me as I share yet another post about pituitary surgery.  If you, like me, want more, try this.

Today's Moment of Gratitude:  I'm glad I found out about my Cushing's after they stopped doing craniotomomies!

http://neurosurgery.stanford.edu/pituitary/review.html

Pituitary Surgery: The Evolution from Open Transcranial to Fully Endoscopic Transnasal Surgery, and Beyond

By Mohamed S. Kabil, M.D. and Hrayr K. Shahinian, M.D.

Click here for the Skull Base Institute's research.

Historical background 

The development of pituitary surgery over the past century is largely credited to the pioneering work of Harvey Cushing in the early 1900s.1,2 Cushing accumulated extensive experience with various operative techniques including transcranial and transseptal-transsphenoidal approaches to the pituitary gland, but ultimately came to favor the transcranial procedure.3, 4 Schloffer in 1907 performed the first transsphenoidal pituitary tumor resection.5 For decades since the introduction of these techniques surgeons have debated which procedure provides the most complete resection of the pituitary lesion with the least risk of complications. Then in the 1960s Jules Hardy introduced intraoperative fluoroscopy and microscopy to transseptal-transsphenoidal pituitary surgery.1,6-8 The improved exposure afforded by these technologies allowed for complete removal of larger pituitary tumors, obviating the need for a complex transcranial operation in most patients. 

As a result, the transseptal-transsphenoidal approach came to be the procedure of choice for the surgical management of most pituitary lesions. Transcranial techniques were reserved for use in the resection of large tumors with extensive parasellar and suprasellar invasion. 

Recently, however, discussions regarding the most effective and least invasive way to perform pituitary surgery have been renewed. Developments in the field of endoscopic surgery have prompted surgeons to attempt endoscope-assisted surgery of the pituitary gland via the traditional transseptal approach.9-20 Recently, some surgeons have developed methods to perform transnasal-transsphenoidal pituitary surgery using only the endoscope for exposure.21-26 This procedure is proving to be equally if not more effective than the microscope as the primary imaging modality in pituitary surgery. 

For large pituitary tumors extending outside of the sella turcica, the transcranial approach offers the greatest chance of complete tumor removal. Often, a transcranial resection is reserved for the second stage of a two-stage operation.27,28 The first operation is performed via microscopic or endoscopic exposure of the pituitary tumor, during which time the bulk of the tumor is removed transseptally or transnasally. Any tumor extending outside the area visualized by the microscope/endoscope, or that is inaccessible during the first operation is then resected in a subsequent transcranial exploration. 

There are two major variations of the transcranial technique that are currently used for the resection of pituitary tumors: Midline Subfrontal Approach and the Oblique Subfrontal Approach. The operation is performed by first making an incision through the scalp, down to the skull on the side of greatest tumor extension. A craniotomy is then performed and the underlying dura incised; the frontal lobes of the brain are exposed and retracted to gain exposure to the tumor. Retraction must be kept to a minimum to avoid post-operative brain edema.27,29,30 Damage to the olfactory nerve can also occur during this operation, resulting in a decreased sense of smell.28,31,32 

Recently, minimally invasive techniques have also been extended to the transcranial approach. These are performed via an endoscopic transglabellar or supraorbital approach. The use of endoscopy allowed thorough visualization of all critical structures at the paramedian skull base without the need for a bicoronal scalp flap, bifrontal osteotomies, or brain retraction.33,34 

Microscopically assisted sublabial-transseptal-transsphenoidal pituitary surgery:

• Indications: This procedure is indicated in the surgical management of pituitary tumors causing hypo- (not enough) or hyperfunctioning (too much) of the gland that have not adequately responded to medical treatment, or tumors growing into adjacent structures, evidenced by visual or other neurologic changes. Micro- and macroadenomas are amenable to resection by this method, including those with mild supra- and parasellar extension.1,35-39Large parasellar extensions of pituitary tumors, however, are more difficult to manage, as the lateral margins of the microscopic field of view are limited and blind removal of tumor extending beyond the sella is hazardous. Patients with extensive extrasellar extension of their pituitary tumors must be considered for two-stage procedures as described above.28,40,41,42
• Surgical technique: While an assistant retracts the upper lip, a sublabial incision is made. Intranasal dissection is carried out separating the nasal tissue and removing a section of the central partition of the nose, forming a large "tunnel" through which the remainder of the procedure is conducted. An adjustable retractor is placed into this tunnel; its blades are advanced to the sphenoid sinus.
  
  The operating microscope is utilized for the remainder of the case, providing magnification and improved illumination of the limited operating field. The anterior wall of the sphenoid sinus is cautiously resected as it boarders the carotid arteries, cavernous sinuses, and optic chiasm. 43-45Once the anterior wall is removed, its mucosal lining is resected, exposing the posterior wall of the sinus (floor of the sella turcica). Benign adenomas usually do not invade the floor of the sella; however, expanding tumors may erode through the floor. If intact, the floor of the sella (posterior wall of the sinus) is removed. Dissection of this region determines the operative exposure, and is crucial for adequate visualization and thorough tumor removal. Deep to the floor of the sella is the dura. A sharp hook or blade is used to incise the dura, and through this incision specialized dissecting instruments are used to remove the tumor. Resection of tumor is carried out until all visually identifiable tumor is removed. As the sella is emptied of tumor, the boundaries of normal pituitary gland are identified.

The hole in the floor of the sella is then plugged using a fat graft from the abdomen, or muscle and fascia graft from the lateral thigh. This plug obliterates the space of the sphenoid sinus and helps prevent post-operative cerebrospinal fluid leakage.46 There are several models of graft design, in most cases a simple fat graft suffices.38,39,47-51 With the fat graft in place, the retractor is removed, the central bridge of the nose and flaps of tissue are repositioned, and the gum line incision is reapproximated using absorbable sutures. Both nostrils are packed with Vaseline-impregnated gauze strips. This packing remains in place for up to 48 hours postoperatively, absorbing any draining fluid and providing structural support to the nose as it heals internally. 

Fully endoscopic pituitary surgery 

With the advent of modern endoscopic equipment, momentum in the field of endoscopic pituitary surgery has stemmed from studies, which show endoscopes provide more comprehensive images of the pituitary gland and its surrounding structures than does the operating microscope.38,52 This in turn should allow for a more thorough tumor resection and fewer associated surgical complications. 

The clinical implications of these findings have been reflected in two separate studies of patients who underwent endoscope-assisted microscopic resections of pituitary tumors.15,47,52,53 These patients underwent a traditional microscopic transseptal-transsphenoidal approach to their pituitary gland tumor. Then, following what the surgeon believed to be complete tumor resection using the microscope, endoscopes were introduced into the pituitary region looking for residual tumor. In both series, an average of 40% of patients were found to have tumor left behind that was only discovered and resected during the endoscopic surveys. In other words, the microscope alone allowed for complete tumor removal in only 60% of patients. 

Improved tumor resection, elimination of intraoral and transseptal dissection along with reductions in operating time, recovery time and complications, have ushered in the completely endoscopic transnasal approach to the pituitary gland as the most recent phase in the evolution of pituitary surgery. 

• Indications: Indications for fully endoscopic pituitary surgery are identical to those for the traditional transseptal-transsphenoidal microscopic approach.16,23-26
• Surgical technique: The first step in the endoscopic procedure is to choose the appropriate endoscope. Preoperative physical examination of the nasal passages provides the surgeon with an idea of which endoscopes will be most appropriate. The surgeon must have scopes of varying diameters available, and must improvise intra-operatively depending upon the intranasal and skull base anatomy of the patient.
  
  Furthermore, every endoscope must be fitted with an irrigation sheath to clear the lens of blood or debris during dissection. This avoids the redundant removal and replacement of endoscopes for cleaning, which is both tedious to the surgeon and hazardous to the patient. The endoscope is attached to the grasping end of the holding arm, advanced into the right nostril, and used to conduct a brief survey of the anterior nasal passageway. The ultimate target of the endoscope is the sphenoid sinus. Therefore, the goal of the intranasal portion of the procedure is to create a passage to the sinus that is wide enough to accommodate the endoscope and accompanying instruments. This goal can be achieved rapidly, but should be meticulously and atraumatically performed, as bleeding from traumatized mucosa anteriorly can obscure visualization posteriorly.

The endoscope is advanced to the anteroinferior border of the middle turbinate. An elevator (dissecting instrument) is used to displace the nasal septum medially and middle turbinate laterally. A long straight suction device may also be introduced to clear the naris of any blood or mucoid secretions. As the nasal passage is widened, the holding arm is released and the endoscope advanced further posteriorly. Ultimately, the anterior wall of the sphenoid sinus is exposed, marking the extent of the intranasal dissection. The mucosal lining of the anterior wall of the sphenoid sinus is dissected away from the bone with a combination suction-cautery device and then lifted from the surface of the bone. Resection of the anterior wall of the sphenoid, the mucosal lining of the sinus, and the floor of the sella (back wall of the sinus) proceeds under endoscopic visualization. The surgical instruments are passed through the nostril, below the shaft of the endoscope, and into the surgical field to gain access to the sphenoid sinus and sella turcica. The same principles of awareness for the limits of dissection apply.16 Injuries to the cavernous sinuses, carotid arteries, optic nerves and chiasm are still possible if caution is not exercised while working within the sinus or sella. 

With the endoscope fixed in the sphenoid sinus, incision of the dura and removal of tumor proceeds as previously described. All imaging up until this point is provided by the 0º endoscope, which provides near complete visualization of the sella turcica, but only a limited view of the suprasellar structures. Therefore, once tumor resection is deemed complete, the 0o scope is replaced with a 30º endoscope. The 30º endoscope is advanced into the sella turcica and rotated clockwise and counterclockwise, thoroughly visualizing the supra- and parasellar regions. Such a comprehensive survey of these regions is not possible with the operating microscope.16,23-26 Tumor remnants in these areas are then removed, thereby eliminating sources of potential tumor recurrence. Resection is considered complete only after examination with the angled endoscope. A fat or muscle graft is then used to reconstruct the floor of the sella as previously described. No postoperative nasal packing is necessary. A small gauze sponge loosely taped beneath the nose collects any fluid that may drain from the nostril. Patients are discharged from the hospital within 24 to 36 hours of surgery. 

Endoscopic transcranial pituitary surgery: 

To minimize the deleterious effects of frontal lobe retraction and to avoid the use of facial incisions, approaches to these tumors have become progressively less invasive. Strategic placement of "keyholes" eliminates the need for excessive surgical manipulation without sacrificing exposure or outcome. 

The introduction of Endoscopic skull base surgery has allowed the resection of these tumors thru two minimally invasive approaches that involve placing a small (2cm) incision either within the skin crease in the bridge of the nose or within the hair of the eyebrow, depending on the exact location of the tumor.34 

• Indications: For large pituitary tumors extending outside of the sella turcica, the transcranial approach offers the greatest chance of complete tumor removal. Often, a transcranial resection is reserved for the second stage of a two-stage operation.27,28

Endoscopic transglabellar approach to the pituitary gland 

The adaptation of rigid endoscopy to the transglabellar approach broadens the available surgical exposure without the introduction of additional dissection or retraction. Endoscopes of varying angles of view provide a panoramic perspective of the relevant surgical anatomy and allow for thorough evaluation of the extent of intracranial and extracranial disease. The maneuverability of the endoscope allows the surgeon to position it directly at the level of dissection, effectively reducing the viewing and operating distances. Endoscopic imaging thereby facilitates complete tumor resection via a minimally invasive technique. This technique obviates the need for a bicoronal scalp flap or an extensive bifrontal craniotomy without diminishing visualization of the paramedian skull base. 

• Surgical technique: The patient is placed supine on the operating room table and the head of the bed is slightly raised. Following the induction of general anesthesia, the patient's neck is extended approximately 15° and the head is fixed in place using a three-pin clamp. Thus positioned, the frontal lobes will fall away from the floor of the anterior once cerebrospinal fluid (csf) is drained. The frontal and nasal areas are cleansed with an aqueous antiseptic solution and then draped.
  
  The base of a pneumatically powered endoscope holding arm is fastened to the operating room table opposite the surgeon; the arm extends over the patient. A 4.0 mm 0° rigid endoscope is attached to the holding arm.
  
  A 4 cm incision is made between the medial ends of the eyebrows, crossing the nasion in a skin crease. The skin flap is developed in a subcutaneous plane and retracted superiorly. The glabellar periosteum is elevated separately and retracted inferiorly for further use as a pedicled pericranial flap in reconstruction of the skull base. A small burr hole is placed in the frontal bone and the outer table of the frontal sinus is osteotomized. Once the sinus cavity is exposed, its mucosa is resected and the nasofrontal ducts are obliterated. A burr hole is then placed in the posterior wall of the sinus and a second bone flap is removed, revealing the underlying dura. The craniotomy can be extended laterally over the orbital roofs as dictated by the surgical anatomy of the tumor. An incision is made in the dura and csf is liberally drained. With relaxation of the frontal lobes, the endoscope is advanced intracranially along the floor of the anterior fossa between the olfactory tracts. Endoscopic survey reveals the degree of intracranial tumor spread. Extrasellar extensions of tumor are then exposed and removed from this superior approach.
  
  Prosthetic dural graft material can be used to ensure a watertight seal in dural repair. The pedicled periosteal flap is interposed between the dura and the paranasal sinuses to reconstruct the base of the skull when appropriate. The nasoglabellar bone flap is repositioned using absorbable microplates and screws. The skin incision is closed with careful attention to the aesthetic repair. The patient is monitored in the intensive care unit until neurologically stable and thereafter transferred to the ward until discharge from the hospital.33

Endoscopic supraorbital (eyebrow) approach to the pituitary gland 

Involves placing an incision within the hair of the eyebrow, performing a 1-1.5cm keyhole supraorbital opening and advancing the endoscope along the floor of the anterior cranial fossa underneath the frontal lobe. This approach allows a panoramic visualization of the ipsilateral anterior fossa and a partial visualization of the contralateral anterior fossa. 

• Surgical technique: Subsequent to the skin and soft tissue incision, a 1.5cm craniectomy is performed. The dura is incised and cerebrospinal fluid drained. The endoscope is introduced thru the keyhole and advanced between the frontal lobe and the floor of the anterior skull base all the way to the tumor. A panoramic view of the tumor is displayed on a flat screen. Using a combination of a custom designed bipolar electrocoagulation system and a micro cavitron ultrasonic aspirator the tumor is gradually resected. This allows a complete and total resection of virtually most anterior skull base tumors through minimally invasive techniques with minimal or no brain retraction. More than 90% of all patients undergoing these procedures are discharged from the hospital within 48 hours.

Conclusion: 

The evolution of pituitary surgery over the past decade has been characterized by a progressive trend toward less invasive surgical approaches to the gland. Innovations in medical technology have, in part been responsible for these advances. The transcranial method was abandoned for the transseptal technique when surgeons documented their ability to achieve equal surgical results without the need for removal of the skull or retraction of the brain. The endoscopic transnasal approach offers even less invasive access to the pituitary gland and surrounding area, in addition to providing better intraoperative imaging of the region. Evidence suggests that complication rates and surgical outcomes of endoscopic pituitary surgery compare favorably to those that have been reported in large series of patients who have undergone microscopic transseptal pituitary surgery.18-21 More extensive data over longer periods of follow-up will further substantiate these trends. It is clear that transnasal endoscopic pituitary surgery represents significant progress in the surgical management of pituitary disease. Furthermore, the endoscopic transcranial approach whether transglabellar or supraorbital to the pituitary gland is an effective adjunct and a far less invasive alternative to the traditional transcranial approaches. 


Glossary of Terms

• Transcranial: Surgical exposure for pituitary surgery by way of opening the skull (Craniotomy), and retracting the front of the brain to access the pituitary gland
• Transseptal: Surgical resection of a portion of the central partition of the nose to access the sphenoid sinus. (exposed either transnasally or through sublabial incision)
• Transglabellar: Surgical exposure for pituitary surgery by way of opening the skull through an opening made at the glabella (the forehead)
• Supraorbital: Above the orbit (eye socket)
• Sphenoid sinus: Sinus (cavity lined with mucosa) that lies directly behind the nose and in front of the pituitary gland: the back wall of which makes up the anterior wall of the sella tursica.
• Transsphenoidal: Surgical dissection through the sphenoid sinus to access the pituitary gland.
• Transnasal: Surgery through the nostril providing access to the sphenoid sinus and pituitary gland.
• Sublabial: Surgical incision below the lip, above the front teeth in the gum line, providing access to the nostril, or followed by splitting the palate (bone) to eventually access the sphenoid sinus.
• Sella Tursica: Bony structure at the base of the skull in which the pituitary gland rests.
• Intrasellar: Within the sella tursica.
• Subsellar: Extending below the sella tursica.
• Suprasellar: Extending above the sella tursica.
• Parasellar: Extending into the area surrounding the sella tursica: (beside, behind or in front of)
• Adenoma - Benign tumor - referring to a non-functioning mass in the pituitary gland (this paper)
• Microadenoma: Small adenoma: Less than 10mm in size.
• Macroadenoma: Large adenoma: Greater than 10mm in size.
• Anterior: Pertaining to being in front of another structure - toward the front of the body
• Posterior: Pertaining to being behind another structure - toward the back of the body
• Medial: Toward the midline of the body
• Lateral: Toward the periphery of the body (away from the midline)
• Carotid arteries: Arteries which carry blood up through the neck, eventually supplying the brain with its major blood supply: these vessels are also found within the cavernous sinus
• Cavernous sinus: Area adjacent to the sphenoid sinus containing several vital nerves and blood vessels
• Dura: Thin membrane surrounding the brain, which acts as a bag to contain the cerebrospinal fluid.
• Cerebral Spinal Fluid/Leak: Clear fluid which surrounds the brain/a hole in the dura which allows the leakage of this fluid (the dura must be opened to access the pituitary gland)
• Endoscopy/Endoscopic Surgery: Surgery performed using small illuminated lenses on long rods connected to cameras to magnify/better visualize an operating field.
• Fluoroscopy: Video x-rays
• Frontal Lobes/edema: The region of the brain in the front of the head which lies above and in front of the pituitary gland/a swelling of some tissue due to injury and accumulation of fluid
• Indication: Scientific/medical reason for performing a certain procedure
• Microscopy: Using an operating microscope to better visualize a small operating field or perform delicate surgery
• Olfactory Nerves: Nerves which connect to the nostrils to provide one's sense of smell
• Optic Chiasm: Area where optic nerves cross over, located just above the pituitary gland, which can be compressed by pituitary tumors
• Optic Nerves: Nerves which connect to the eyes providing one's sense of sight
• Resected: Cut out or removed surgically
• Turbinate (superior, middle, inferior): Bony prominences in the nasal passage way

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