Surgery Notes

I'm nearly flat on my back typing this--my lower back went out on me yesterday. I don't even know what to say about that. Sheesh! Thank heaven for chiropractors, ibuprofen, friends that come cook and garden, and mostly for devoted husbands....

Last Monday was a marathon of doctors' consultations. As noted before, our over educated friends Susanne and Amy were with us to take notes and to think of questions we were too stunned to ask.

Because I'm just too daunted at this point to try and turn the notes into a narrative, below is a large portion of Susanne's notes as she wrote them. Any of my additions or comments to her notes will be in purple. Any thing in brown will be additional points I've added from Amy's notes.

Team Zoe Meets with the Craniofacial Team
at Children’s Hospital

Monday, 9/8/2008

Impromptu mission statement:
Team Zoe is here to support her happy, healthy, long life!

Michael Cunninham, M.D., the primary craniofacial physician, meeting

• Cunningham is the lead physician and go to person for Zoe’s craniofacial concerns

• He reports that the test results are not back yet, but expects it to be here by Friday. The blood tests have come back, but there is still some investigating to be done. What's clear is that she does have osteopetrosis. What's still not clear is its treatability via bone marrow transplant (Cunningham is fairly certain it can be treated) and the exact genetic causes of her osteopetrosis. He wants to get more data from the lab so that we can know how likely this would show up again were Jeff and I to try for another child. There is a strong likelihood that we would have a 1 in 4 chance of this happening again as it looks like we both have a recessive gene for the condition. Ouch.
  

• Dr. Key in S. Carolina, who works exclusively with osteopetrosis, did not respond via e-mail, so Dr. C. will be calling him this week.

• Dr. C. recommends the cranial expansion surgery to relieve the pressure on Zoe’s brain.

• The surgery will be performed by both the neurosurgeon, Dr. Avelino, and the craniofacial surgeon, Dr. Hopper.
  

• The surgery does not go below the dura mater (the strong tissue that wraps the brain) so that the cerebrum (part of the brain) is not exposed.

• The optic canal (the holes that the optic nerves go through) enlargement is the most complicated part of the surgery since they have to work around the optic nerve.
  

• Dr. C. asks that they sign up for a “scales survey” (x-rays of the rest of the body) before leaving today. These x-rays were done this morning. Zoe was great--relaxed and happy. She continues to generate fans wherever she goes.
  

Then came the scheduled meeting with the neurosurgeon. First his "fellows" came in to review Zoe's chart:

• The growth of the brain usually directs the growth of the skull, with the sutures (the hairline cracks in our skulls) moving with the growth.

• Zoe’s CT scan shows early closure of ½ of the lambdoid (in the back of the skull) suture & both sides of the coronal (top/side) sutures, as well as significant opening of the anterior fontanelle (soft spot on top of head where she is bulging).
  

• Zoe’s skull bones appear much thicker and denser than they should be. The frontal (forehead) bone is 3/4 inches thick on Zoe. The standard thickness is 1/8th of an inch.
  

• The role of the neurosurgeon in the craniosynestosis surgery is to make the cuts and mobilize the bones so as to stay superficial to the duramater and to protect the brain.

• Because of the amount that bone bleeds, she will most likely require a blood transfusion during the surgery. The doctor said there is 100% chance that she will need a transfusion. I looked into being the one to donate blood to her. The nurse said this is certainly possible, but that it would make it impossible for me to be an organ or marrow donor for her as she builds antibodies to the blood given to her in transfusion. Interesting. In the off chance she needs one of my kidneys, I suppose I will let the blood bank do its job.
  

Meeting with neurosurgeon, Anthony Avelino, M.D.

• The anterior fontanelle usually closes around 18 months, and the posterior fontanelle around 3 months. Zoe’s fontanelles are too open at this stage.

• Dr. A. recommends expanding the skull and optic canal.

• He has performed the optic expansion 2x in his career; the last time was 8 years ago.

• Benefit of surgery: relieves pressure on the brain and optic nerve.

• Risks of surgery: general risks are infection and death. Specific risks are loss of blood (this is the highest risk) and neurologic damage. Three words: E Ticket Ride.
  

Meeting with nurse practitioner, Marsha

• One of her jobs is to find a surgery date. We are trying to get her scheduled in the next 2-4 weeks. We are having to bump some other children and make sure all the docs/surgeons can be here on the same day. Currently the surgery date is scheduled for October 13th. If they can swap that day with a child that is scheduled sooner--a day that has Hopper and Avelino working together, then they will do it. It is difficult when moving surgery up means postponing another kid's corrective surgery. They know that Zoe's case is more urgent than many, but other cases are pretty needy as well.
  

• She inquired about getting a coagulation screen (this was already done).

• Zoe will have a pre-operative appointment the Monday prior to the surgery

• After the surgery, Zoe will be taking pain medications every 4 hours for ~1 week (e.g., baby Tylenol).

• Zoe will stay at the hospital for 3-5 days post-op.

• She will be in the ICU for the 1st day or 2 (ratio of 2 patients to 1 nurse)

• Only one parent can stay overnight in the ICE

• She will later be transferred to the surgical unit – both parents can stay overnight (ratio of 4 patients to 1 nurse)

• Immediately after the surgery, water will be given to Zoe (in the event she were to aspirate into her lungs, water is much safer).

• Post-operatively, another CT scan will be taken.

• Zoe will not be her usual self the 1st 24 hours after the surgery.

Meeting with Dr. Hopper, craniofacial surgeon (& 3 fellow entourage)

• After Dr. A. takes the bone off, Dr. Hopper will reshape the bone and put it back on.

• After looking at the CT scan, he explained that the frontal lobe has the greatest pressure on it despite the enlarged appearance, since the frontal bone is actually 3/4” thick.

• The surgery involves removal of the “visor” – the lower frontal bone, the temporal bone, the sphenoid & zygomatic that shape the brow. For those of you unfamiliar with the skull bones, go here
  

• The surgery will have 3 phases

1. Bony reduction: this involves the removal of bone from the frontal bone (3/4” excess).
2. Bone repositioning: this is the reshaping and expanding of the bone, that will utilize some of the reduced bone to fill in any gaps or “soft spots” that result.
3. Optic canal expansion: since they’ll be going in through the front, they will be close to the optic canals so it would be best to have it done now to avoid revision later. There is no guarantee, however, that this will improve Zoe’s sight.
   

• The skull is usually designed to protect the eyes at the brow; Zoe’s skull, however, is narrow along the temple region and flat at the brow, which is part of the reason for the reshaping. Surgery will increase her brow, she may have a Schwarzenegger brow just post-surgery, but it will soften very quickly.
  

• The anterior fontanelle will flatten out as a result of the reshaping.

• A diagnosis of osteopetrosis would not change how or why they would do the surgery, since the mechanics are similar to craniosynestosis. Amy writes Hopper's response to my question of: How does Zoe having Osteopetrosis change how you approach this surgery? Overall it will be the same, but I will treat her bone more like that of a 5 year old- it will be stiffer. I have not done this surgery on kids with osteopetrosis. It is a very rare diagnosis. I have worked on kids with fibrous dysplasia which is also a genetic disorder with increased bone growth- actually more bone growth than osteopetrosis.
  

• The brain growth usually stops around the 1st year of life and the head growth usually stops around 7 or 8 years.

• Dr. H. has not worked on with osteopetrosis, although he has been involved in several surgeries that enlarged the optic canals. He says the surgical procedure for the optic canals is not unique.

1. We have decided that it may be the best time to do this aspect of the surgery as we don’t know if we will every be in this area of Zoe’s skull again. And in doing the Bone reshaping we will already be ¾ of the way into this surgery, so we see it best time to access this area.
2. Also called this decompressing the optic canal by “burring down” the bone
3. How do you protect the nerve in this surgery? The only way to do that is to go slowly using microscope and small tools.
4. Has assisted in 2 of these surgeries in his career. Both with the other Neurological surgeon at Children’s. one was 2 yr ago and one was 1 year ago. It is an uncommon surgery.
5. Kellie’s concern about Dr. Avalino having done them 8 years prior…. Dr. Hopper’s opinion: Dr. Avalino does a similar technique quite often in more dangerous/tricky areas of the body. I can understand why you are asking and I feel very confident in Dr. Avalino’s abilities.
   
   

• Zoe is at a good age to do this surgery. It’s his hope to avoid having to do a 2nd surgery.

• The surgery will take at least 8 hours.

So, that's most of what we know about the surgery. The last conversation I had with Dr. Cunningham made the issue of whether the surgeons were indeed going to enlarge the optic canals still a question. As of last Friday, when I had the last conversation with him, Cunningham planned to have a conversation with Dr. Weiss (the chief ophthalmologist) about whether there was any hope for the nerves to regenerate.

Cunningham said, I know Weiss. He will likely first say that the nerves are gone. But then, I will press him and say --- 'How about the plasticity of a baby's physiology?' I'm betting that he will then say ' Weeeelllll, it's possible...' If Weiss has that response, then absolutely I think we should go in there.

Tomorrow we'll bug Cunningham for the latest.