I am an Orthopaedic Spine Surgeon...A carpenter of the body. I've missed work a total of 3 days in the last fifteen years of practice due to illness. In 6 years of residency and fellowship I missed only 4 days after having ACL reconstruction. I never get sick!

My stellar attendance record is about to change.


Recently, I have been diagnosed with Ameloblastoma...an uncommon, benign yet aggressive tumor of my jaw.

I am not a wordsmith, but I have been inspired by the few others out there who have blogged about their experiences with ameloblastoma. I look at it from a clinical perspective as a surgeon (While the diagnosis is foreign to me, the surgical management and treatments are very familiar to me.) and scientist, I am also the patient. A rare perspective that I thought by sharing, I could enlighten others with both clinical information, and personal experience.


Wednesday, December 19, 2012

Talking to the Man with the Plan

My appointment with my surgeon came up quickly. I was fortunate that Dr. W worked with Dr. M at Vanderbilt. My wife and I met with him and his chief resident late one Monday afternoon. A new panorex, a review of my medical history, and a quick exam led us to the meat of the discussion. My research really paid off. I was very prepared for our discussion.

Ameloblastoma is rare but aggressive. The most "conservative" treatment is actually the most invasive...surgery. In order to maximize the likelihood of eradication for good, we need to take out the tumor itself, as well as a good margin of normal bone to make sure we got it all. One centimeter is considered the standard for a good margin. That means that they needed to have 1cm of normal bone in front of, behind, and below the tumor (above it includes the teeth) to say goodbye to my little friend. I am fortunate that my tumor is small and is in the front part of my jaw. Most ameloblastomas are found near the angle of the jaw and can get quite large, necessitating taking out a large part of the jaw including the joint where it attaches to the skull. This makes recovery much longer, reconstruction more challenging, and risks go up.

Being in the front, however does put the mental nerve at risk. This is the nerve that gives sensation to my lip, and chin on the right side. The goal would be to take away bone whilst sparing the nerve if at all possible. Fortunately, the position of the tumor is such that the nerve may be able to be addressed and left intact. I will lose 3-5 teeth in the process, but since the bottom of the jaw is still ok, I won't likely have to have a segmental osteotomy where the entire jaw bone from teeth to the bottom of the jaw is removed. This leaves the jaw unstable, which requires plating to pieces together and wiring my jaws shut to splint the pieces. It also complicates the reconstruction. 


Reconstruction of the jaw, or rebuilding the bone that is removed is done by taking bone from my hip/pelvis and putting in the jaw. Eventually the body grows together there and makes my jaw sturdy again so that teeth can be put in place. Again, I am lucky here in that the defect will not be huge, nor segmental, making reconstruction easier. Dr. M may be able to graft it at the same time that the tumor is removed. It all depend on whether he can safely close the gum and soft tissue over the graft. The worst thing in the world is if the wound dehisces and comes apart. That really increases the chance of infection, and possibly losing the graft. 

Jargon Alert:
dehiscence /de·his·cence/ (de-his´ins) a splitting open. 
wound dehiscence  separation of the layers of a surgical wound

Surgery will take 5-6 hours. I'll be able to go home the next day. Nope....no tracheostomy since the surgery can be done from inside out. Unlikely will I need my jaws wired shut since the strongest part of my jaw will remain intact. The ability to graft will be decided at the time of surgery. Saving the nerve will be a big priority. Six weeks or so off work. Liquid or no-chew diet for 3-6 months depending on whether the graft can be done at the same time or needs to be delayed. Implants once the graft is healed and incorporated. All in all, about a year of work to re-build my mouth and restore my bite to allow me to chew properly.

Whew!

I consider myself REALLY fortunate. The lesion is small and in the front. Most ameloblastoma stories on the internet involve much bigger tumors, much bigger operations, and much longer recoveries. I am also fortunate to be in such a great community that we don't have to travel to an unknown city to find the experts who can manage this. Nashville if very fortunate to have such a place as Vanderbilt University Medical Center. I'm healthy. I have good friends and a fantastic wife. My office and practice are amazing. My patients will be understanding (although their best interest worries me the most, frankly).

Let's do it....

Saturday, December 1, 2012

ameloblastoma (ām'ə-lō-blā-stō'mə)

I am now about 4 weeks into the process. A week between tender teeth time and my dental exam. A couple of days for the endodontist. Another day til I saw Dr. W. Antibiotics and CT took another week. Biopsy and then follow up about 10 days later. And.....

The verdict is in. Last Friday night Dr. W called to tell me the news of my pathology report. "The good news is it's not cancer." Good. "The not so good news is that it is an ameloblastoma. It's benign but will need surgery. You will lose some teeth, but it will be okay" Yeesh

http://www.ajronline.org/content/197/3/W412/F4.small.gif
This is the picture that scared me a little
In all my reading before the biopsy, the one picture that stood out was the surgical specimen from resection of an ameloblastoma.  Teeth, jaw etc. sitting on a surgical towel waiting to go to pathology. Jeez I hope that isn't what I have, I had thought.  My surgeon brain then went into overdrive regarding how one puts it back together.  You would need some sort of bone graft...Iliac crest? Fibula? Rib? A plate to fix the bones would be needed. How long til it was healed? Would it look "normal" once all put back together?

Nahh, it couldn't be that I had thought.


Medium Power View
Low Power View
Medium Power View

 AMELOBLASTOMA!!!


Well, indeed it is ameloblastoma. This is an uncommon (although the most common odontogenic tumor seen in the jaw. Approximately 50% of odontogenic tumors), benign tumor that is known to be aggressive and will come back in 50-90% of cases if not fully removed. It actually comes from the cells from where the teeth originate from and it is what creates the enamel. Essentially normal cells gone wild. There are several types of ameloblastoma including:

  • Conventional/Solid or Multicystic (This is what I have)
    • 3rd - 5th decades of life
    • Located in the mandible in 85%, mostly posterior
    • Lesion is peri-apical (nearby to the tips of the roots of the teeth)
      • Follicular - 28% of conventional type
        • Islands of epithelial rests in stroma
      • Plexiform - 32% of conventional type
        • Strands and cords
      • Acanthomatous  - 12% of conventional type
        • Squamous metaplasia
  • Unicystic
    • 1st - 3rd decades of life
    • 5-22% of ameloblastomas 
    • Male:Female = 1.6:1
    • Often associated with impacted teeth
    • Lined with epithelium
    • Possible to treat with curettage alone in some cases
  • Desmoplastic
    • 3rd - 7th decades of life
    • 4-13% of ameloblastomas
    • Often involves the maxilla and the anterior mandible
    • Compressed islands and thin cords
  • Maxillary
    • Most feared type due to proximity to skull, orbits and intracranial structures
    • Males=Females
    • Sinus extension in men is 10.6%, in women is 5.9%
    • Radical surgery is mandatory
Here are some references and reviews about ameloblastoma types.

Okay, so I have ameloblastoma. I would have to say I was surprised, somewhat shocked and a bit anxious. What about treatment? The literature I reviewed, the case reports, and even the blogs out there all pointed to the need for resection of the tumor with a border of normal bone of about 1 cm on all sides. How much bone is removed depends especially on the location of the tumor. Those tumors growing in the back part of the jaw tended to get larger and needed bigger resections. Remember that photo (above) that scared me? That is a resection of the back part of the jaw including the part where the jaw attaches to the skull. Good, mine is both fairly small and in the front. 

Jargon Alert:
resect /re·sect/ (-sekt´) to excise part or all of an organ or other structure

Well....It's time to talk to the guy who will take care of my little friend. I am fortunate to live where I do in that there are some very good maxillofacial surgeons right here. Dr. W recommended that I see Dr. M at Vanderbilt. Not only the chairman of the department, but also his mentor in training. Having done my residency at the same time as Dr. W, I know about Dr. M and feel very comfortable with that recommendation.

Lucky me, he had an available appointment in just a couple of days. Honey, I've got myself a doctor's appointment.

Off to Vanderbilt....

Thursday, November 15, 2012

A Biopsy-ing We Will Go

Back to Dr W's office for the biopsy. Basically the plan is to drill into the bone and remove, as much as possible, the lesion and then send that material off to the pathologist to find out what it is. If it is a simple cyst, scraping it out is all that would be needed to get rid of the issue. If it is something more significant, more surgery might be needed. My vote is for the simple cyst.

I was given the choice of local anesthetic injection alone, with laughing gas, or IV sedation. I've never had a problem with dental work so I chose injection with the gas as a backup.  First, however are the photos. Dr. W is definitely old school on this. Lot's of photos both before, during and after. All photos both inside and outside looked normal for my age. No overt sign of my little friend.



Right
Left

View from the inside. The asterisk is where the lesion is.

 Next the numbing. I guess is was about 4-5 injections. Pretty quickly my entire right jaw, lip and chin were completely numb. A rubbery bumper goes in between the left molars to block my mouth open and a gauze sponge over my tongue. "All set?" Yep. Incision time...not feeling at all. Dr. W cut along the teeth at the gum line on the inside of my mouth. Cut. Suck. Cut. Suck. Peel the gum tissue off of the bone down to the floor of my mouth. I can't feel a thing.
The gum line is peeled down. This photo is reversed because he used a mirror to take it.

"So do you remember Dr. X during residency? Let me tell you a story..." Dr. W is very efficient with motion and can keep up both surgical progress and a steady flow of stories about people and experiences we both knew. It brings me back to a great time of life and keeps my mind off of what he is doing.

Now for the drill. Since the lesion is fully contained in the bone (...which is good thing. Lesions that erode through the bone into the soft tissue often require more extensive treatment.) he has to drill through the bone to get to it. From the panorex and CT scan he knows that it is centered at the roots of #28. Drill, drill, suck, suck, drill, drill. "There it is". More drilling. No pain, my nerves are ok too.

A view of the lesion. It is solid tissue, not a cyst. That needs to come out.
"It's a solid tumor rather than a cyst." The next step is to get that tissue out by picking and scraping (called currettage) in order to get as much of it out as possible. If this ends up being a minor issue, currettage will be the cure. The hole will fill in with bone fairly rapidly. If it is a more sinister problem, it will need further work. Dr. W is very careful not to scrape or pick at the roots of the teeth to protect each individual nerve.

The empty cavity
The tissue came out easily and according to Dr. W, it looked benign to the naked eye. Scrape, pick again until the cavity was empty of all the tissue he could see.

Irrigation (saline) was used to clear it out and the hole was stuffed with gelfoam (a collagen sponge that helps things clot). The gum tissue was then sutured back together. This was actually a really cool part from my surgeons eye. The suture weaved around the teeth and slings the gum back where it came from. From the outside, I couldn't tell anything had been done.


My little friend in the flesh
All back together after suturing
"The pathology will go to Louisville. They are the best at this type of stuff. They can take a while to get a result, so I'll call you with the results. See you in a week. Oh, and if you feel like it, grab a Werther's candy on the way out. They are really good."

Immediately after, I felt great...other than the big wound on my lip from biting it because it was numb. Unfortunately, the long acting local anesthetic did not last as long as expected. I was feeling great for about 3 hours, then the floor of my mouth REALLY started to hurt.

Clearly swelling from the hematoma in the soft tissues and the biopsy site was causing pain. I thought I would get by with ibuprophen, but I was glad he gave me the hydrocodone.

Jargon Alert:
hematoma /he·ma·to·ma/ (he″mah-to´mah) a localized collection of extravasated blood, usually clotted, in an organ, space, or tissue.

After a couple of days, however, my pain was well controlled with ibuprofen. The hardest part was avoiding that gum when I ate or brushed my teeth. After about 5 days, the sutures started coming apart (as they are supposed to) and I kept spitting out pieces of fishing line. Fun.

My follow-up in a week showed that my incision was healed and all looked good. The remaining sutures were removed. It's amazing how big such a small suture feels inside the mouth. True to form, the pathologists had not finished their report. "I'll call you just as soon as I know something" Dr. W says. "Grab a Werther's. Not the same family but they are really good."

And so we wait....






Research, Research, Research

Like any good doctor, I have an insatiable curiosity regarding health issues, diseases, pathology and treatments. Medicine is often like a puzzle. First find the pieces, then see where they fit. Sometimes we have to try one thing to see if it works. If it does...great. If not, we look to something else. Art based on science rather than the reverse.

Ok, I have a lytic lesion in my jaw. What could it be? Infection? Cancer? A cyst? Some sort of benign neoplasm? A space alien or retained twin? News of the weird, here I come.

Jargon Alert:
neoplasm /neo·plasm/ (ne´o-plazm) tumor; any new and abnormal growth, specifically one in which cell multiplication is uncontrolled and progressive. Neoplasms may be benign or malignant.

Orthopaedic Surgery is the study and care of maladies of the skeleton and musculoligamentous structures. The bones and the soft stuff attached to the bones is my baliwick...that is EXCEPT when it comes to bones of the skull, face or jaw. This is the realm of the Oral Surgeon, the Maxillofacial Surgeon, the Plastic Surgeon, or the Otolaryngologist. The teeth, in particular are interesting structures stemming from very specialized cells found nowhere else in the body. Special cells mean special problems. We DID NOT learn about odontogenic tumors and the like in medical school or residency. I get to learn something new.

When a doctor or scientist is looking for information about a problem, we must first narrow down the possibilities of what the issue might be because the world of medical problems is huge. When it comes to tumors/lesions etc. any clues to it's behavior is helpful. Where is it? What does it look like? How is it acting? In my case, the first step in making a diagnosis was to identify the lesion. Where it is and what it looks like are important in narrowing down the possibilities. What the lesion is doing to the bone is very important when trying to decide if it is bad (malignant) or not so bad (benign). In this case the smooth oval look on the panorex and x-rays suggest that this is likely a benign process.

Next we have to look at what possible causes of a lytic lesion in the jaw is. My job began with Google. Searching "Lytic Lesion Mandible"gave me 107,000 results. For medical conditions I first look for medical journal references, online journals and case reports. Peer reviewed journals are best for the quality of studies and reports, but may be over the head of most lay people. I stay away from (at least at first) forums, blogs, aggregators and the like. There is a lot of garbage or recycled information out there. Forums and blogs have their place, but not for collecting accurate information. Wikipedia is a good start BUT always be leery of the accuracy of the information.

Jargon Alert:
odontogenic /odon·to·gen·ic/ (-jen´ik) 1. forming teeth. 2. arising in tissues that give origin to the teeth.

Since my lesion has the appearance of a cyst (a hole in my jaw), is in the mandible/jaw, and is near my teeth, we can narrow down the focus when it comes to searching for information.

I found these two journal articles helpful in reviewing from a radiographic point of view, what the possibilities are:


These references speak more to the actual pathology of the various lesions:
 

And then this page, which is essentially a Wikipedia like site for Radiologists:


Ok, there are few malignant tumors of the jaw. Good. There are a lot of cysts, and other issues that are essentially minor problems. Excellent. There are a few bad actors both in the benign and the malignant world that we must look out for. Yep, there's always a catch.

With a good couple of steps towards understanding the lay of the land, so to speak, (The x-rays, the Panorex, the CT, and a review of the possible causes) there really is only one step left...BIOPSY. We need to get a sample of my little friend to send to the pathologist. They will cut it, stain it, and look at it under the microscope in order to determine the origin and it's identity. The pathologist is the CSI of neoplasm care.

Let's go get a biopsy.

Tuesday, November 13, 2012

Here Kitty Kitty - Time For A CAT Scan


http://lh4.ggpht.com/_yd7UkHDtQ5s/S-R-1RqSlSI/AAAAAAAAAws/EpkYR54FZA8/catscan2-thumb1.jpg
 http://www.funnytimes.com/playground/img/125852407027532.pngOk, sorry for the pun.

A CAT scan is an advanced anatomical imaging tool also known as Computerized Axial Tomography. These are 2 dimensional x-ray slices that allow us to re-create a 3 dimensional view of anatomy. CAT (or CT) is excellent for looking at the bone and skeleton. Technology these days makes CT fast and accurate (Although a single CT scan of the face exposes the patient to the same quanity of radiation as he/she gets over a 16 month period due to background radiation. Not something to ignore. Link for more information.)

The CT was NOTHING. It took literally 5 minutes. The contrast injection, however was...interesting. After the initial scan was taken, the dye was injected into my arm and another scan was done. Shortly after the injection, it felt like my butt was hot. Weird. The tech said that some people also say that it feels like they are peeing on themselves. Wish she had told me that beforehand.

Next step, back to Dr. W's office to go over the CT. Meow ;-)

So the scan confirmed that the lesion was contained in the jaw bone. No destruction of the teeth. It also showed that the lesion is on the lingual side of my jaw (the tongue side). This is important because the approach for biopsy needs to be on the side where the lesion is.

That's right....I said biopsy. Surgery to get a sample of the lesion. In many cases, the biopsy procedure actually removes the entire lesion. One and done. There may be a possibility that the biopsy shows something more serious, and more surgery would be required. Not likely, I am told.

We will do the biopsy in the office under any anesthesia that I preferred. I've never had a problem with dental procedures so I decided to start with local anesthetic (Lidocaine and Marcaine. Novocaine is no longer used, I learned) with nitrous oxide gas as a backup. (Laughing gas...common in the 1800's for recreational use, also the primary propellant in whipped cream cans. Ask me how I know!) "Any questions?" Nope. "Ok, we will see you next week."

"Oh, and grab a Werther's candy on the way out. They are really good."

A biopsy we will go...








It started innocently enough...


City House Menu
About 2 months ago, after a really good meal at City House here in Nashville, a couple of my teeth in my lower right jaw became a little sore. Not really painful...more just an ache. I thought to myself...you need to do a better job flossing. You probably have some retained meat from the awesome Pork Belly pizza the other night. Flossing, however, produced no offending porcine products, just a little blood between the two.  Are those teeth wiggly? Hmmm.
As it happened, my regularly scheduled dental appointment was the next week and when I talked to my hygienist, I mentioned that my chompers were still a bit sore. She found no sign of infection, no hidden foodstuffs, no pit or other dire finding. Whew, that would have been embarassing. We mentioned it to my dentist who decided to get some x-rays.

Something looks a little funny. More x-rays. These images showed a well circumscribed oval dark area around the roots of teeth #28 and #29. Hmmm. This was not there when they took x-rays 18 months ago. Hmmmmmmmmmmm.

"Does this ice on your tooth feel cold?" No "Well I think you might have internal resorption" (some form of internal deterioration with damage to the nerve going to the tooth.) my dentist tells me.  A sidewalk consult with one of his partners confirmed that I likely needed a root canal. Great. I've heard bad stories about those.

I was lucky enough to be able to get an appointment with a well regarded endodontist the next day. Good, I can get this over with and get on with life. Nice guy, modern office. The digital x-ray technology for dentistry is phenomenal...clear pictures and fast. Yep, there is a lytic lesion in my right jaw. "It really does not look like internal resorption, more like a bone cyst" he tells me. Cold test again clearly shows all of my teeth have normal sensation. "Good news...no root canal. Bad news...I need to send you to an oral surgeon because I dont know what that lesion is. It doesn't look bad, but we need to find out what it is." Now I was getting a little concerned.

Jargon Alert:
lytic /lyt·ic/ (lit´ik) - Having to do with destruction. It looks like a hole on x-ray.

Fortunately, he wanted to send me to Dr. W, an oral surgeon whom I know well and respect highly. We were both in residency at Vanderbilt University at the same time, me in Orthopaedic Surgery, he in Oral/Maxillofacial Surgery. Dr. W. is a very busy guy (as you would want the best guys to be) but luck had it that I could see him the next day...he had a cancellation. Thank you sir. I appreciate your insight.

Dr. W is a whirlwind of intelligence, dry sarcastic wit, and plain talk. I like him tremendously as he and I are cut from very similar cloth. First order of business is my history....2 weeks duration, not trauma, just sore...especially with biting down or pressure. Yes, they do throb with my heartbeat. No swelling, fever....etc. Next step the exam. A lot of fingers in my mouth with his scribe noting my occlusion (how my teeth fit together), my dental hygiene ("Good +"), "no masses" etc. "Does it hurt if I tap here on your jaw? Nope. How about tapping on these teeth?" Uh Huh (somewhat garbled because his fingers were still in my mouth) All the time relating stories about all the folks we trained with and under while in residency. Funny. Off to get a panorex.

Jargon Alert:
panorex - A panorex is a two-dimensional dental x-ray that displays both the upper and lower jaws and teeth, in the same film. 

Can you spot my new little friend?

There was clearly a lesion in the front half of the right side of my mandible (Jaw bone). It is well circumscribed (It has a definite outline), oval appearance without any evidence of tooth or root destruction. Good. It looked benign (meaning that it was not terribly destructive), but "it could be a problem". Okay, what now?

In order to determine the extent of the lesion, and it's specific location, we needed to get a CAT scan. This is an x-ray scan that "slices" the body up in multiple planes so that the anatomy of the bone can be more clearly and completely evaluated.

"Oh and have a Werther's candy on the way out. I'm not related, but they are really good. I'll see you next week."

And off I went...