A new type of minimally invasive decompression surgery technique at TCI

August 6, 2012

Hi everyone,

I apologize in advance for the length of this posting; but my story has

in fact been a long one (aren't they all?) I have been reading the

contributions from the group over the last few weeks and I finally feel

like I'm ready to be a contributing member. I've had Dr. Bolognese read

over my story and he said I did a good job describing the technique he

used. I really do hope that this might help someone some day.

Thank you for reading this and for being willing to share you own lives

with those of us who have felt so alone for so long.

God bless you all.

Beth

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My Chiari Story

In the last couple of decades, most of my conversations withdoctors have

left me with more questions than answers. Don't get me wrong. I

have had some good doctors that havehelped me keep going, but none that

really had any answers as to why I was suffering. That all changed back

in November of2011 when I first met Dr. Bolognese at The Chiari

Institute.

Fast forward to the end of my last meeting with Dr. B in April2012. He

asked that I share mystory with the Chiari community. Itseems that

relatively few people have had the type of Posterior Fossa Decompression

surgery that I had threemonths before in January. In fact,if I

understand correctly, at the time of my November diagnosis there were

onlytwo people in the world that did this type of surgery, Dr Bolognese

and someonein Beijing China. So, here Iam. I hope this helps someone

out therewho is considering surgery.

Flashback – Fall 1995: I vividly remember that headache. I was a

somewhat ambitious mathematics education doctoral student at

theUniversity of Georgia. I alreadyhad plans for my dissertation and

life was going well. It was the beginning of the term and I,along with

other graduate students and some faculty members (including my

advisor),were heading from our offices on the first floor to our seminar

class on the 6thfloor. Other than adult-onsetasthma that I had acquired

a couple of years earlier, I was in fairly goodshape at the time. I

decided totake the stairs. To get there faster,I decided to run up the

stairs. Bythe time I reached the 6th floor, I had a headache. It was

on the left side of my head andit pounded! I was sure it would goaway,

so I went into class. I waswrong; it got worse.

I don't remember much about that class except the pain andtrying to

keep up with the discussion. (A couple of days later, I recall telling

my advisor that I hoped that Ididn't make a fool of myself during

that afternoon's seminar. His only reply was " Well … at

least youdidn't dance on the table. " Ugh!) I definitely do

remember getting backdown to my office. Lights were toobright. Sounds

were too loud. And every step made my head feel likeit was going to

fall off. Over thenext couple of hours, I took four Advil and then a

couple of Tylenol that I hadbummed off of someone in our department.

Nothing helped. I was notable to drive myself home that evening and I

was scared.

I still had a headache the next day and I went directly tothe

University's student medical clinic. I got my first diagnosis of

migraines. After that day, headaches became a wayof life for me. The

rest ofthat year was okay. I was gettinguse to the headaches. Ilived

by keeping a bottle of Excedrin Migraine with me at all times.

At the beginning of Summer 1996, I had completed all but oneof my

comprehensive exams and was working out the details for the

dissertationstudy I hoped to start in the fall. In the meantime, I

managed to work in a couple of weeks as an OlympicVolleyball

statistician at the 1996 Atlanta Olympic Games. It was an amazing time,

but I rememberthe headaches intensified during those few weeks. I

thought it was just the pace I was living at during thosedays.

When the Olympics ended, I went back to UGA and startedworking on my

next comprehensive exam. I felt exhausted. I wassure that if I rested

enough, I would get better. Within a week, I started running a

low-grade fever, had a sorethroat, and felt excruciating pain in several

joints, especially my left shoulder. Within a few more weeks, I could

barelyget out of bed. We were veryconcerned that I may have caught some

bug during the Olympics.

It took six months of testing and a trip to a rheumatologistto rule out

everything from Lyme's Disease to AIDS. The student medical clinic

ran so many tests on me that theybegan taking spare blood taps for

future tests since my arms were beginning tolook like a heroin

addict's arms from all the needle marks. The only thing that they

could findbeyond the symptoms I was exhibiting was anemia.

By midterm, I returned to school. Pain would go from one joint to

another: hips, knees, ankles, toes, jaw, neck,... no joint was

discriminated against. The worst pain was in my shoulders, vertebrae,

wrists and fingers. I also would have periods where Iwould

functionally black out. Iwould leave my office building to walk to my

car in the graduate student parkinglot; and the next thing I knew, I was

on the other side of a four-lane streetand I was half way to my car.

Thatwas extremely scary. By the end ofthe six months and all the

testing, I had a diagnosis of Chronic FatigueSyndrome.

I managed to graduate with my doctorate and I startedteaching

mathematics at Central Michigan University in Fall 1997. In 2000, Ifound

a job closer to my family in North Carolina. I became a faculty member

in the Mathematical Sciencesdepartment at Appalachian State University

in Boone, NC. I love teaching. My students are the reason why I get

upin the morning, especially when I suffer with my symptoms.

Fatigue is something different from just being tired. It's like

someone pulls a plug in thebottom of your feet and all of your energy

leaks out onto the ground. I would lie down to rest and then allof the

sudden my heart would start racing. Then there would be days when I

would have vertigo andnausea. My ribs would slip out ofalignment

causing a great deal of pain. My hands would be so painful I

couldn't hold a pen. There were violent bouts of irritablebowel

syndrome. I couldn't hearwell out of my right ear and in the other

I had tinnitus. I had cognitive issues like not beingable to remember

how to get to a friend's house or a restaurant that I had beento

just recently. Symptoms wouldcome and go. I had a heart murmurand then

I didn't. My eyes didn'ttrack together and then they did.

Butthe headaches … they were my constant companions. And they got

worse. One lasted for 21 days and another lastedalmost a month. These

marathon headaches only stopped after large doses ofnarcotics and muscle

relaxants (sometimes multiple rounds) that would knock meout.

Through it all, I never missed a day of work. I kept going. I did

change my primary care physician about five yearsago. The new doctor

believed mewhen I said that I felt like something else was wrong with

me. My joint pain, fatigue, and headacheswere becoming almost

unbearable. I wasn't sure how much longer I could keepfunctioning at

my current level. After listening to my history, he was concerned that

I mighthave Myesthenia Gravis. So wesought out a neurologist.

Theneurologist was pretty sure I did not have MG, but he did diagnose me

againwith classic migraines. When Iwent back to my primary care

physician, I asked him what was next toexplore. He said that was it. I

asked him about the mind-numbingfatigue and the myriad other nuisance

symptoms I had. He gave me a hug and said, " Some people just have

chronicfatigue. "

In Fall 2009, my ovaries were destroyed by two masses the sizeof

baseballs. I was sent to anoncologist in Charlotte, NC. Within 3 weeks

I had a complete hysterectomy and the masses were removed. Thank God.

They weren't cancer. But I was left with a bladder that didn't

really want to work well. That got somewhat better with

physicaltherapy. Still, the next fewmonths were challenging. I

wasworking and trying to bounce back from surgery. When I wasn't at

school, I spent most of my time in bed. Then one spring morning in

2010, I wokeup with a severe pain in my neck. My next new primary care

physician thoughtI had a ruptured disc and sent me for a neck MRI

(CSPINE). That's when life got veryinteresting. The

radiologistconfirmed the ruptured disc between C6 and C7 and … he

also saw a Chiari IMalformation approximately 5 mm long.

A summer of neck traction took care of the ruptureddisc. But it took a

couple ofyears to take care of the Chiari. First of all, my neurologist

told me, " That's not a Chiari. " Evidently, according to

hisexperiences, I just had slightly herniated cerebellum tonsils. And

since I didn't have a Chiari, itwas highly unlikely that those

tonsils could be playing a role in myheadaches. He told me that I

didnot exhibit the classic Chiari headache pattern. So, we forgot about

the Chiari Malformation note on theradiologist's report and

continued to try to find something that would help mereduce the

frequency of my headaches. I was seeing my neurologist every three

months to discuss the results ofthe latest prescription or strategy and

then discussing what to do next.

By Fall 2010, I was suffering with headaches on a dailybasis. After a

glucose tolerancetest, we found out that I had reactive hypoglycemia. I

changed the way that I ate and that helped some of myheadaches. But I

picked up a newsymptom, something that I call a " brain zap " or

" zing " . It is like having an electric currentrunning through my

head. Sometimesit runs just through my head. Sometimes it runs through

my head andthen down to the tip of my fingers. That winter, another

symptom intensified. I was having tingling in my hands almost all of

thetime. Over the last decade, I hadsporadic bouts of these " pins

and needles " , but now they were much morefrequent.

I had a brain MRI in Spring 2011. Nothing new really materialized from

that test. However, I was desperate for painrelief. Part of my head

pain camefrom the occipital nerves that radiated up from the base of my

skull. I had an occipital nerve block on theleft side of my head but

that really didn't help much. The biggest source of relief came

fromtaking two Neurontin at night so that I could sleep. I couldn't

take the medication during the day because theymade me so drowsy.

Then came one of the most pivotal moments of my illness. Ihad a headache

that was so painful, I honestly was aware of nothing else aroundme. I

was going to see my niece inan end-of-summer-drama-camp production. We

were waiting outside the building forthe doors to open on a Friday

evening. I felt nauseated and the pain in my head just continued to

build. I took multiple Excedrin Migraine, butit didn't touch the

pain. I satdown on a bench while everyone else went to get my niece

some flowers for herperformance. Anything could havehappened to me that

evening while I sat there and I would not have evenregistered it. I

could have beenkidnapped, mugged, etc, and I would have been helpless to

do anything aboutit. All I was aware of was thepain.

I called and made an emergency appointment with myneurologist as soon

the office opened. He said that we had exhausted treatment options. He

recommended that I see aneurosurgeon to see if the escalating headaches

had anything to do with theruptured disc in my neck. InAugust, I saw a

neurosurgeon in Asheville NC. After an hour of neurological testing (in

which I was verysurprised to find out that I had decreased sensation in

my left arm and theleft side of my face), the doctor asked me into his

office. He pointed to an image on my brain MRIand asked me if I had

ever heard of a Chiari Malformation. I just kind of laughed and said

that Ihad. The neurosurgeon went on tosay that he was fairly sure that

my headaches were being caused by the Chiariand that I would probably

benefit from decompression surgery. He went on to explain that he

wouldremove some bone for the base of my skull and the top vertebrae to

make roomfor the cerebellum tonsils to move back up into my skull and

give room forcerebral spinal fluid (CSF) to flow. I was dumb-founded. I

toldthe surgeon that I wanted to think about it for a little while

before I madeany decisions.

After discussing the results of this visit with myneurologist, we

decided to go for a second opinion. An appointment was set up with a

neurosurgeon inWinston-Salem NC. Before I could goto that appointment,

I started losing feeling in my right arm and hand. I also had some

bizarre involuntarymovements in my left wrist. I wasgiven an emergency

appointment with at Physicians Assistant in Winston-Salem. He was very

surprised when I asked ifany of my symptoms were related to the Chiari.

He brought in the neurosurgeon on call that day. After giving me less

than five minutesto discuss my symptoms and briefly looking at my

MRI's, that neurosurgeon toldme that I most likely had carpal tunnel

in my wrists. I asked how the carpal tunnel could impact myheadaches.

He then said, " Oh thoseare probably tension headaches. You are

stressed, aren't you? " At that moment? Stressed …maybe.

Furious … absolutely. Iknow what tension headaches are and there was

no way that my horrible headacheswere tension headaches. Iwent out

into my car and cried.

I returned to work that afternoon and told one of mycolleagues, who

incidentally does have carpal tunnel, about the currentdiagnosis. I

finally told her thatthere was nothing wrong with me and that it was all

in my head. I was just going to shut up and behealthy … even if it

killed me. Myfamily could find out what was actually wrong with me when

they did theautopsy. This colleague (and verygood friend) told me that

I had 24 hours to " be healthy " and not think about myhealth

issues. But after that, Ieither had to continue my search for a doctor

who could help me find out whatwas wrong or she was going to kick my

butt. I will forever be grateful to her for that.

Twenty-four hours later, I realized that even if I went toevery

neurosurgeon in NC, I would probably end up with the dichotomy

ofopinions I had just received. Thereseems to be very few doctors who

really have a deep understanding of ChiariMalformations. So, I decided

tofind someone who specialized in Chiari. I spent a few days searching

the Internet. From what I have read, there are some good surgeons

outthere, but it seemed that The Chiari Institute (TCI) had an entire

staff ofprofessionals who could look at my case from a variety of

viewpoints and Dr.Bolognese seemed to be one of the best Chiari

specialists in the country. I filled out all of the new

patientquestionnaires posted on the website and immediately emailed them

to TCI. When I received a reply that my casewould be looked at by the

interdisciplinary group at The Chiari Institute, Ifelt like I had won

the lottery. It wasn't long until I was invited to meet with Dr

Bolognese. I breathed again.

I saw my neurologist before going to NY for my TCI appointment. He

conducted an EMG and nerve study torule out carpal tunnel. We wereboth

surprised that I couldn't feel anything when the needle went into

themuscles on my upper left arm and shoulder. My neurologist said I

seemed to have a classic Chiari " cape " of sensation loss. This

wassomewhat of a revelation for him. Allof the tests indicated that my

problems were not with the muscles or nerves inmy arms, but seemed to be

coming from my spinal cord.

Meeting with Dr Bolognese was a bit of a shock. As usual, I had a

headache, so it tooksome time to process everything. FortunatelyI had

one of my best friends with me (my roommate from college who had

beendiagnosed with a Chiari Malformation a few years earlier) to make

sense ofeverything. Dr Bolognese confirmedthat I also had a Chiari I

Malformation. It almost felt as if he was clairvoyant. He told me

things about myself that he couldn't have known(like the fact that

my headaches were one-sided) because I didn't report it toanyone at

TCI and things I didn't even know myself (like the fact that I

hadproblems swallowing and it had come on so slowly that I wasn't

even aware ofit). He said that my Chiari was " bear-hugging " my

spinal cord (blocking CSF flow) and that it was asymmetric (longeron the

left side than on the right -- hence the headaches on the left side of

my head). I felt such relief to finally have anexplanation!

Then there were those fateful words … " but that's

notall " . I had another problem. On the back side of my spinal cord

wasthe Chiari. On the opposite side,I have a retroflexed odontoid.

DrBolognese explained that this normally vertical bone supports the

skull on to aperson's neck, but mine was leaning backwards and

sometimes it wobbles and rubsagainst my spinal cord. So, thisalso was

also causing some of my problems. Dr Bolognese said that my spinal

cordwas " literally between a rock and hard place " . That little

piece of information sent me into a tailspin. I was not prepared for

this newweirdo thing that was wrong with me or for the next discussion

that we hadabout possible surgery.

This long story finally brings us to the point of mynarrative. Dr B

said that we hadtwo surgical treatment options. Thefirst option would

be to do two operations at one time: a decompression for theChiari and

craniocervical fusion (where he would support the skull on my neckwith

two metal rods, one on each side of my spine, which would be screwed

intoseveral vertebrae and into my skull). That really sounded

terrifying. Also, Dr B said that the fusion surgery would limit the

range of motionof my head by 50% in all directions. Or … our second

option would be to do just the decompression surgery, only insteadof

removing bone to make room for Chiari and improve CSF flow, he would go

inand burn the tonsils and then amputate them. Okay … to be honest

that didn't sound any better. He was going to amputate my brain!

Dr. Bolognese went on to say that a traditionaldecompression surgery

really was not an option for me. If he went in and removed the bones

tomake room for the cerebellum tonsils (as the Asheville neurosurgeon

describedto me earlier), he would have to make a fairly large incision

that woulddisrupt the neck muscles and connective tissues that were

currently keeping myskull stable on my neck (they were taking up the

slack left by the retroflexedodontoid). In that case, I wouldprobably

be back in the hospital within a couple of months because I would notbe

able to lift my head. So, hewas planning to do a relatively new type of

Posterior Fossa Decompressionsurgery that would be minimally invasive,

meaning that he would disrupt thosesupporting structures as little as

possible in getting access to the ChiariMalformation.

Here is how he described this new technique to me. He would start by

making a smallincision just long enough to get access to the bottom of

my skull and the topof my C1 vertebrae. Then he wouldshave off about a

millimeter of bone at the bottom of my skull and a millimeterof bone at

the top of C1. He thenplanned to work in that small window between the

skull and C1 to burn out theoffending Chiari and amputate the burned

tissue. (Dr B did later say that thisbrain tissue really wasn't used

for anything. Honestly! Hecould have said that 15 minutes earlier

before he ever used the term " amputate " in regard to my brain!)

Anyway,this would be quite a tedious surgery. There was a major artery

to avoid and it's a very small space to workin. It would take a

significantamount of time to do this surgery.

At the end of our conversation, (which took about an hour –I have

never had a doctor take that much time with me and that includes the

oncologistwho operated on me when we thought I might have ovarian

cancer), Dr. Bolognesesaid that he really couldn't recommend one

surgical option over the other atthis point. I had to make thedecision.

I went back home tothink. I knew I had to do something.That was a

no-brainer (no pun intended). I was becoming incapacitated by my

headaches and that is never good whenone is a mathematics teacher.

Andthe numbness in my arms was alarming. In the meantime, I had some

tests run to see if my asthma would impactsurgery and if my bones were

strong enough for the fusion. Fortunately, my asthma

waswell-controlled. But I did haveosteopenia (the beginning stages of

osteoporosis). That last fact, ruled out doing the craniocervical

fusionuntil my bones were stronger. So,Dr. B decided to do the

decompression surgery first. I started taking Fosamax to strengthen my

bones just in casewe might need to go ahead at a later date and do the

fusion.

My Posterior Fossa Decompression surgery on January 10, 2012took 7.5

hours to complete. I hadseveral blood vessels intertwined around my

Chiari that had to besystematically moved. My familysaid it was so good

to see me after the surgery, but it was hard to recognizeme with all the

swelling around my neck and face (as a result of lyingface-down all that

time and the surgical trauma around my neck area). In less than twelve

hours, I was out ofNeuro ICU. I was up walking thehalls the very next

day. On thesecond day after surgery, Dr. B had me running (well …

not really … it was morelike slowly shuffling) up and down a flight

of stairs. I spent a total of five days in thehospital and another two

at the Variety House across the street fromhospital. I was on a plane

home toNC seven days after surgery.

It is now August 6, 2012, and I feel the best that I have indecades. Dr

Bolognese was elatedat my 3-month follow-up appointment in April. The

odontoid bone had not moved much as a result of thesurgery. I had a

Grabb measurementof 8.3 mm before surgery and a 9.0 mm measurement

after. I need to point out that I do not seemto have one of the

connective tissue disorders known as Ehlers-Danlos Syndrome(EDS) that

can make the odontoid bone very unstable. I scored a 3 out of 9 on the

EDS questionnaire that TCI gaveme. Dr B said I may never have tohave

the fusion surgery and told me I could go off the Fosamax as far as he

wasconcerned. Wow!!!

I still have occasional symptoms from the retroflexedodontoid, such as

the " brain zaps " and some tingling in my fingers every nowand

then. But my CSF flow is somuch better. I still have someoccipital

nerve pain, BUT I have not had a single bad " migraine " -like

headachesince the surgery. I'm completelyamazed. I truly realized

the extent of this miracle when my brother asked me ifI had anything

that he could take for a headache. I reached for my purse and suddenly

realized that I did nothave any pain relievers in my purse. In fact, I

had stopped carrying my ever-present Excedrin Migraine bottlemonths ago.

My biggest issues these days stem from the muscle spasms Ihave around

the incision. Theyreally tighten up my scalp and I find it amusing that

most of my headachestoday are tension headaches from these spasms. I

feel like going back to the neurosurgeon in Winston-Salemand explaining

to him the difference between tension headaches and Chiariheadaches.

Sigh.

I am gaining my energy back each week. At night, I still use the

Neurontin tohelp me sleep because of the occasional occipital nerve pain

and I use Zanaflex(muscle relaxant) to help with muscle spasms. I take

Cymbalta and a couple of Motrin in the morning tohelp with any

muscle/joint pain and muscles spasms. If the neck muscles continue to

spasm during the day, I usemy TENS (TranscutaneousElectrical Nerve

Stimulation) unit. The TENS unit also seems to help combat theoccipital

nerve pain during the day. So other than that, I don't use any pain

meds. My neurologist has now lengthened the time between myappointments

to every six months and we are talking about taking me off of someof my

medications. He has beenreally amazed by my story and considers it a

learning experience for him. That makes me happy because my

healthissues may make a difference for someone else that comes to him

with headachesdown the road.

I am working on strengthening my muscles and range of motionin my neck.

I have a littlestiffness at times, but all in all I have the same range

of motion as I did beforethe surgery. Since my left arm isa little

weaker than my right arm, I'm doing 15 minutes of weight lifting

withboth arms (3 lb weights each arm). It has really helped. Also,I am

protecting my investment. Iam using a hard cervical collar (Miami J

collar) when I drive or use thetreadmill. No more roller coastersor any

activity that could cause my neck to jerk. I also use the collar

whenever I start having the " brainzaps " . It helps to keep my

neckimmobile and minimizes those zings.

So, there's my story. My advice is to search for the best doctor

around and keepquestioning. There are gooddoctors out there, but they

do not always have the experience to see thenuances of Chiari nor do

they always know about the related-issues like aretroflexed odontoid or

Ehlers-Danlos Syndrome. Also I would like to thank those patients who

camebefore me. Great surgeons like Dr.B had to learn from their

difficult experiences (like the fact that having aretroflexed odontoid

could result in the patient not being able to lift his/herhead after a

traditional decompression surgery).

I know I will always have symptoms. There is no " cure " for a

ChiariMalformation. Damage has alreadybeen done. But I am so much

betternow!!! I now know the source ofall my strange ailments; that in

itself is priceless. And here's my vanity showing … my scar is

a little over twoinches long. Yes, I'm smiling.

For years, many people have prayed for me. My father was at the top of

thatlist. My Dad passed away from complicationsrelated to Alzheimers 16

days after Dr. B diagnosed me. I don't find that a coincidence. I

am so blessed. I thank God for walking beside me everstep of the way of

this journey that led me to TCI. I also thank Dr Bolognese for giving

me a new lease on life.

Forever grateful,

Beth Searcy

Boone, North Carolina

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August 6, 2012

beth,

I am so glad dr b helped you.. Your symptoms are so familiar. This paragraph in

particular rang true.

It's like