Professor Plum~ In the Conservatory~With the Knife

As I've been researching Jayson's new diagnosis and our different treatment options and surgeons, there were many times I felt like this was like a game of Clue.  

Who would do it?  

Where would it be done?  

With which method or tool?  

There is one thing I will say definitely sets our situation apart from a game... There was no roll of the dice in this decision making process.  Our decision was very, very, very carefully made.  My husband, family, friends and I poured hours of research, tears, and time on our knees in prayer to make this decision.  I even read a Plastic Surgery medical text book and dozens of research articles!!  So pick your cards and place them in the confidential envelope.  Drum roll please....

WHO:
Dr. S (our original craniofacial surgeon) and Dr. K (a different neurosurgeon)

{furthermore known as Dr. Suit Jacket and Dr. Canuk}

WHERE:

in the Operating Room at Primary Children's Hospital in Salt Lake City

WITH WHICH METHOD:

with the Posterior Vault Distraction method (aka with the rods out the head)

and when????

to be done

THIS THURSDAY, NOVEMBER 13, 2014

Surprised?  I'm sure some are, and maybe some aren't.  This decision was definitely not made based on what Mike and I feel most comfortable with.  It was truly based on what is best for JAYSON.  I would much rather have Jayson get his surgery done and over with in the operating room, and just come home and let me snuggle him back to health.  I would rather NOT have to have rods sticking out of his head that make me vomit every time I look at them and I really don't want to have to adjust them and cause him pain.  But I will do whatever I have to for my son, including this.

Let me explain what I learned in my research in comparing the Posterior Vault Distraction (PVD) surgery and the Cranial Vault Expansion (CVE, also known as CVR) surgery and why this decision became quite easy once we looked at the facts.  

We chose PVD because

1.  PVD slowly stretches the skull and skin over the course of a couple of months leaving no or very few holes in the skull that would need to be filled later. The skull is stretched 1 mm each day for 2-3 weeks, and the skull slowly fills in with soft bone.  After the 2-3 weeks, the distraction rods are taken off and the skull is allowed to harden before the entire distraction devices are removed from his skull.  The other CVE procedure tends to leave lots of holes because the expansion is immediate instead of slowly over time.  They holes cause pain and problems and likely would need to be filled at some time.

2.  PVD provides up to an INCH of extra room, with an increase of 30-40% space in the skull. This means a small likelihood of needing another surgery to expand the skull. The other CVE procedure provides a max of 1cm per surgery, often requiring multiple CVE surgeries in the next 10 years

3.  PVD has a lower morbitidy percentage. Less deaths sounds good to me

4.  PVD has less blood loss and a quicker procedure, leading to less deaths

5.  PVD is less invasive. 

6.  PVD has more permanent results. There is little to no chance of the skull sliding back into its old place because it is stretching and filling in slowly over time. The CVE procedure has a higher percentage of sliding back into place when it is done at the rear of the head. As the child sleeps, the pressure on that part of the skull can push and slide it into its old position.

7.  The Cranio surgeon cuts open scalp from ear to ear in a zigzag pattern. Neurosurgeon cuts the skull along that same line, but straight. A little distraction device is screwed into the skull on each side. It reminds me of a door hinge, screws on each side of the divided skull. The the distraction rods are connected to the hinges and the skin is sewed up around them. Not very much exposure to the brain. The dura (covering) of the brain is not cut and the brain is not exposed.  The other CVE procedure involves the same cutting, but then removing and reshaping the skull, then creating a gap and securing it with a plate, pins or wire leaving a portion of the brain unprotected by the skull until the skull starts to grow and eventually fuse together. This fusion process often leaves holes mentioned above.  

Once we selected Posterior Vault Distraction, the doctor selection process became somewhat easy.  Distraction HAS to be done locally because Jayson will require weekly checkups and frequent x-rays with the rods in his head.  Dr. F in Dallas and Dr. S who was trained by him in Dallas (Dr. cat skinner) only do Cranial Vault Expansion surgeries.  Dr. Suit Jacket, our original craniofacial doctor, missed a lot, didn't want the CT scan, and screwed up BUT he is the best plastic surgeon at our hospital.  I wanted to give him a chance to redeem himself at the meeting we were holding today, Tuesday, with a different neurosurgeon, Dr. Canuck.  And he did.  There was no apology.  No ooops.  No explanation.  But he and the neurosurgeon were dead set on doing this and doing it well.  They seemed very comfortable with the procedure and easily answered all of my questions.  They spent over an hour with us answering my list of over 40 questions. :)  (Hey, what can I say, I'm a thorough mom especially when they cut my son's head open.)  Here are some pictures of what is going to happen and a list of my questions and the answers they gave me.  I'm sure many of our friends and family have similar questions.

The distractor that will connect to the skull on each side

Skull A is before, Skull B is after the cut and the distractor is put into place

Over 2-3 weeks the distractors will stretch the skin and separate the the two parts of the skull.  Soft bone will fill in as it slowly separates.  The distraction rods coming outside of his head will then be removed while the distraction device stays for 3-4 months while the soft bone hardens.

X-ray image of distractors

Pictures of a baby a couple of days after surgery on the left, and a few weeks out on the right.  The silver part you can see is the distraction rod.  You can see it's shorter on the right because it's further in her head stretching.  You can see the head is completely shaved, and a zig zag cut is made and stitched from ear to ear.

Questions:


DISTRACTION DEVICE

• Brand- Synthes
• Springs- No springs
• Amount of space- up to 40% more
• Internal vs. External distractors- Internal
• How many rods? 2
• Device thickness- 2
• How long will rods be in? 2-3 weeks
• When will distraction device be taken out of skull?- 3-4 months
• Likelihood of an allergic reaction- very possible with Jayson.  Plan in place.
• Rod Removal How and When- after 2-3 weeks in clinic
• Device Removal How and When- after 3-4 months, another inpatient surgery, 1-2 days in hospital
• When does adjustment begin and end- begins 3 days after placement surgery, ends 2-3 weeks after placement surgery
• Possible device complications- infection risks, device is not rigid; it's flexible. No device complications at our hospital in past 2 years
• Risks of Infection (superficial or internal)- Superficial risks, treated with hydrogen peroxide and antibiotic cream daily.  No known internal infections at our hospital in past 2 years
• O2 cannula or CPAP use at home (mask will touch incision sites and possibly distractors)- should be fine to just use O2, we will revisit this in the ICU
• Cleaning and Bathing- distractor site will be cleaned with Hydrogen Peroxide and antibiotic cream daily

SURGERY

• Suture brand and type- we will use the ones Jayson is not allergic to that we used in his Chiari wound correction surgery
• What is their experience with multiple suture cranionsynostosis- they say it is a common condition that they treat at our hospital and they have been doing distractions for two years.  They claim multiple suture cranio is not as rare as the literature says, and they see and treat it frequently
• Number of days and nights in ICU and in hospital- 1 night 2 days in PICU, another 2 days and nights in neurotrauma unit
• Length of surgery- 2.5-3 hours, he will likely be in OR 4+ hours
• Procedure for blood loss- bags of blood (Jayson's type) will be in OR ready for transfusion.  Blood transfusions are needed in 33-50% of these surgeries
• Pain and pain management- will be monitored in PICU and on the floor- I will follow up with this question more on Thursday
• Will pressure be measured before or during surgery?  Yes, an ophthalmologist will measure his intracranial pressure before they begin the surgery in the OR to have a baseline
• Chance of inpatient genetic testing- we will have a genetic consult inpatient and push for approval of whole exome sequencing during this time
• Surgery risks- blood loss, low risks because brain and dura are not being touched

Long-Term Follow Up and Care

• Follow up for rods and devices- weekly check ups, x-ray after 2 weeks
• Follow up after device removal- clinical appointments every 6-12 months
• Pain and Pain Management at home- stretching process doesn't seem to be too painful, easily managed with Tylenol and Motrin
• Changes to long term care involving docs, meds, appointments, imaging- need to follow up on this question during rounds
• Precautions for activity with rods- hard to limit toddler activity, encouraged me to get in touch with other parents to see how to keep J from touching or messing with the distractor rods
• Visitor Restrictions- none ordered, use caution

Other Questions

• Does Jayson seem to fit any of the multiple suture craniosynostosis syndromes (Crouzon, Apert, Pfeffer)- no, but he may be in that family of syndromes
• Is the scalloping or erosion to the skull problematic to the skull- no, skull is durable
• Could this craniosynostosis cause brain damage- yes, if the intracranial pressure was high for a long period of time
• Could the brain damage be reversed- if the damage wasn't actually permanent and it was temporary due to the pressure in the brain, yes it's possible
• How long until his brain moves back into the space created- pretty immediately if it's been compressed.  There will likely be extra space and that is to accommodate future growth
• How will this surgery affect his chiari- it may help and give him more space
• Does the fact he's had decompression surgery complicate this posterior surgery at all- no, it is done slightly higher in the skull from where his skull was removed

So that's it.  That's our decision and we feel good about it.  Well, about as good as one couple can feel when their baby is having his head cut open for the THIRD time.  Please pray and send positive thoughts our way---hope and pray that Jayson will do well through surgery, that his doctors will know what to do, that pain will be as minimal as possible, that we won't experience complications, and that this procedure helps Jayson as much as possible.  That truly is the thing that is keeping us going--HOPE.  Jayson is doing well now... imagine how well he will be doing when his brain is free from pressure!!!!  We are trying to stay hopeful....hopeful of many more miracles to come.  Thank you for hoping and praying with us. We truly feel your strength.

And lastly, WEAR YOUR PURPLE ON THURSDAY!  Purple is the color for epilepsy, Chiari AND Craniosynostis!  Coincidence?  Maybe.  But probably not.  :)  Be sure to tag me in your pics of purple showing your support for Little J!  Love you all.