Cranio Surgery: Questions and Answers

Initial Questions about Craniosynostosis:

Q: What is craniosynostosis?

It happens when one or more of the infant's cranial sutures fuses too early. The cranial sutures are the natural cracks in the skull.

An infant's skull is made up of seven bones with gaps, or cranial sutures, between them. The sutures do not normally fuse until the child is approximately 2 years old, and this allows the brain to grow and develop.

Q: Are there different types/severities?

There are four major types of craniosynostosis:

Sagittal synostosis

Coronal craniosynostosis

Metopic synostosis

Lambdoid synostosis.

It is possible to have just one type or a combination of them.

The appearance for each type of craniosynostosis can differ. More than 180 different conditions are linked to some type of craniosynostosis.

Q: What causes craniosynostosis?

The cause of craniosynostosis depends on the type. The condition can be nonsyndromic, or syndromic. Syndromic craniosynostosis occurs with other birth defects. Syndromic is when a gene mutates, the information it carries is mixed up, resulting in one of the bodily functions not working correctly. A number of genes are thought to mutate, resulting in craniosynostosis. The cause of nonsyndromic craniosynostosis has not been identified.It may be due to a cell defect in the sutures that causes them fusing too early.Sometimes, the fetus assumes an irregular position in the womb, potentially putting pressure on the head and pushing the plates of bone in the skull together. This can cause the sutures to fuse.Studies have suggested that taking valproic acid for epilepsy during pregnancy may increase the chance of the child being born with craniosynostosis. However, the link is not clear.

Q: Can craniosynostosis be prevented?

Q: Could it have been discovered in-utero?

Q: When is the skull supposed to fuse?

The skull sutures do not normally fuse until a baby is 2 years old.

Q: Why does craniosynostosis require surgery to correct?

The primary treatment method of craniosynostosis is surgery, which is best done within the first year of the child's life. The main goal of the surgery is to let the cranial vault develop normally. Without surgery, the condition can get worse and the skull deformity may increase.

Surgery normally involves a craniofacial surgeon and a neurosurgeon. A craniofacial surgeon specializes in head and facial surgery and surgery of the jaw. A neurosurgeon specializes in the brain and nervous system.

Surgery is performed under general anesthetic. The infant does not feel any pain.

The neurosurgeon makes an incision across the top of the infant's scalp and removes the areas of the skull that have become misshapen.

The craniofacial surgeon then reforms these sections of skull and places them back in the head. The opening is then sewn up using dissolvable stitches. The process can last several hours, and the child will stay in hospital for a few days after the surgery. The face is likely to swell, but this not a need for concern.

Sometimes further surgery is required to reshape the face. Sometimes the craniosynostosis reappears, also requiring further surgery. After the procedure, the child's skull development will be monitored.

Endoscopic surgery offers a less invasive approach. Two small incisions are made in the scalp and the sutures are cut with the help of an endoscope, a flexible tube that can be used to see the inside of the body during keyhole surgery. This type of surgery is quicker, and there is less bleeding and swelling, but it is only suitable in certain cases, depending on the location of the fused suture. Endoscopic surgery is recommended before the age of 3 months, because the bones are still soft. After surgery, the child may need a molding helmet to help the head to grow into a suitable shape.

Q: What age is best for surgery? Why?

*** Much of the above information was copied from Medical News

First Appointment Questions (for plastic surgeon):

Q: What is your role in the surgery?

Once the nureosurgeon has removed the skull, I reshape it by cutting slits in the bone and adding dissolvable plates. I am in charge of making the skull look as if it hadn't been fused prematurely.

Q: How many cranio surgeries have you performed?

At least one a week, but anywhere between one to three. In total, I have performed thousands.

Q: Will she need a helmet?

With metopic craniosynostosis, we overcorrect the skull shape. Over a few years it will curve back to normal without the requirement of a helmet. Other types of craniosynostosis do require a helmet after surgery.

Q: What type of incision is made?

Some surgeons do straight, curved, or zig-zag. The zig-zag shape, I find, makes the scar show less when the hair is parted on top of the head. The incision goes from ear to ear. Zig-zag a third up, then straight on top, and zig-zag on the way down.

Q: Is endoscopic surgery a possible alternative?

Although endoscopic surgery has/can been performed for metopic, the surgeon at Rady's Children's Hospital said they no longer offer it because the results were not lasting. Some patients would have endoscopic surgery and then need CVR (cranio vault remodel). He mentioned for other types of skull fusions, endoscopic is a great option. Be sure to discuss this question with your plastic surgeon.

Q: How do we keep the scar clean?

For the first two weeks, bathe daily. Use a clean container of soap and water to soak washcloth in and scrub around scar. Use the clean container to pour on and rinse incision. Pat head dry. Do NOT wash incision with bath water to prevent bacteria entering. Do NOT have shower water hit incision direction.

Q: How much hair is shaved?

Before the surgery, the plastic surgeon's assistant asked if I would prefer the whole head shaved or a strip. Since my little girls hair has started sprouting already, I opted for the strip shave and I'm glad I did! For a boy, I may have shaved the whole head but either way, it does not affect the how sterile the surgery will be.

Q: Pros and cons for doing surgery earlier at 6 months? Later at 1 year?

Earlier surgery means the skull is more malleable, but also more likely to return to original shape. Our surgeon said the more severe the fusion, the more likely it would be to show regression. Babies who are not crawling yet have an easier time with recovery being in the hospital 4 days (not able to crawl around). He also said between 6-8 months is when he prefers to do surgery, and there is not much difference between those months. After a year is not preferable but our surgeon has performed metopic on five year olds without as clean of results.

Q: What are the risks of surgery?

I signed a waver that said the risks of this surgery include: infection, eye damage, blindness, coma, and death. Our surgeon said out of the thousands of surgeries, he's never had any of these high risk situations with his cranio patients. A little comfort, but I remind myself that these are the same things I have to sign on a waver for a gymnastics bounce house. :)

Q: How likely are those risks to happen?

Infection is less that 5% and eye damage, blindness, coma, and death were less than 1%.

Q: How severe is my babies craniosynostosis?

Although, I thought it looked like a mild case, the neuro and plastics team said it was moderate. They pointed out the way the eyes were close together and pinching upwards made it moderate.

Q: Can it effect other parts of her development/organs?

Potentially. While our baby was in the NICU, doctors scanned her other organs and found everything normal. She was also given a developmental test before her surgery and was above average. The developmental physiologist said children with craniosynostosis are often slightly delayed with milestones.

Q: If teaching hospital, what is the student involvement in the surgery?

This is a question I never asked but wanted to. I was too worried they would say sometimes they take over the surgery! But I think they just observe. If your nervous though, just ask!

Q: Is it important to research family history to reactions to anesthesia?

They will ask if your family has had any diverse reactions to anesthesia. Check with your immediate family and grandparents if possible.

Q: Risks of anesthesia?

Q: Is there a place for us to sleep during recovery?

Los Angelos Children's Hospital had a couch that pulled out into a very thin bed. Like, less than a twin bed. Somehow, my husband and I were able to sardine sleep four night there but it wasn't a picnic. If you are traveling far for the surgery, check if a Ronald McDonald House room is available.

Q: What is the short-term, in hospital recovery schedule?

Immediately after surgery she will have minimal swelling. The swelling will continue to get worse until the third day. For metopic surgeries, this includes the eyes swelling shut. Having your baby in an upright position will help the blood stay down. Every 5 hour Tylenol can be given for pain. Morphine can be given if pain persists, but patient will need to be on all monitors while on Morphine. The first day or two will be spent in PICU. Here, a nurse will be assigned no more than two patients. Every 6 hours, intravenous antibiotics will be given. Our girl hated the IV flush every time. Periodically, a nurse will come in to check blood pressure and temperature vitals. For the first couple days, our girl had two hand IV's, one foot IV, a toe oxygen monitor, and three vital monitors on her chest. The fourth day swelling will go down and the eyes will reopen. This is an exciting day! Then on the fifth day the hospital will release you probably before noon.

Q: What is the long-term recovery schedule?

Bubble-gum flavored antibiotics will be given in double doses 4 times a day for the next week. Tylenol can be given often to keep pain controlled.

Q: How many follow-up appointments?

After returning home, we called to schedule a follow up 2-3 weeks post-surgery. For the next year she will have a couple more follow ups, including another developmental test. Every year for the next 10 years she will have an annual checkup.

Q: Will we meet with a geneticist?

Prior to surgery, we did not schedule an appointment with a geneticist. However, that option was available to us free of charge in the Rady's Children's NICU (we decided to opt-out) and has been offered again at L.A. Children's Hospital. It is okay to have to testing done post-surgery.

Q: Will we meet with a developmental psychologist?

Before surgery we met with a developmental psychologist who tested Chloe's milestones by asking questions and providing tasks. The tasks included picking up a cheerio, playing a peekaboo game, responding to her name, flipping pages in a board book, ringing a bell, locating a car hidden under a towel, sitting up unassisted, holding a cup by the handle, dropping blocks in the cup, obeying the word 'no', etc. After surgery, the psychologist will test Chloe again and compare developmental changes. She can also participate in a longer study that lasts into adulthood.

Q: Does she need a CAT scan?

Chloe had a CAT scan at 4 days old in the NICU and 2 days post-operation. Our surgeon needed a CAT scan before surgery but did not mind that it was from 7 months ago.

Q: What will the skull correction look like? Will the eyes be corrected (for metopic only)?

Even though I knew with metopic that the skull would be overcorrected, it was still a little shocking how flat her forehead looked after surgery. From above, the head looks very round now. Looking straight on, her forehead is very flat, the bones on the sides of her eyes have been pulled out, and her eyes do look less pinched already. The surgeon said the eyes would slowly correct themselves but I can see a difference already. There are a few soft spots on her skull that will fill in during the next 2-5 years while the flatness also rounds itself out. The only additional surgery sometimes needed is a quick 30 minutes under anesthesia for an injection of stomach fat into the sides by the eyes if they start to sink inwards.

First Appointment Questions (for neurosurgeon):

Q: What is your role in the surgery?

Remove the skull, minimize and monitor blood loss. A blood transfusion is usually needed for craniosurgery. Chloe's surgery lasted 4 hours and required a pint of blood that was donated by her father.

Q: How many cranio surgeries have you performed?

Our neurosurgeon, Dr. McComb, often works with Dr. Urata and is nearing retirement, so he has probably performed thousands with his team.

Q: What is the best way to prepare her for surgery?

One nurse assistant said to keep her well nourished. Another nurse said there's nothing extra to do. From my experience, keep them well hydrated before surgery, make sure iron intake is sufficient, and for the two weeks before surgery know that it's okay to be crazy cautious around others because if they get sick the surgery will need to be rescheduled! Also, our baby didn't take liquid Tylenol well so that was a battle during recovery. If your baby is solely breastfeeding, having him or her on a bottle before surgery would also be helpful to give momma a break!

Q: Will she need a blood transfusion?

Most likely. One donation of blood is probably enough (1 pint) but they always keep two on hand just in case. There is no difference in the safety of the blood used if it is from the blood bank or a direct donation.

Q: How much blood in need for a transfusion?

Usually 1 pint, but 2 pints are kept on hand just in case.

Q: Where can I donate blood?

Q: Does craniosynostosis effect brain development?

According to our neurosurgeon as Rady's Children's Hospital he said, "I would not try and convince you to get this surgery because of possible brain damage. That is very unlikely. This is mostly for cosmetics which is still important for the developing self-esteem of a child."

Q: Will the transfusion eliminate the immunity she has developed from breastfeeding and vaccines?

Absolutely not. (This may seem like a weird question but something I was genuinely concerned about!)

Q: What impact will the surgery have on the protection of her brain? Example: playing soccer/sports?

Our surgeon, Dr. Urata, at L.A. Children's Hospital said, "She will be able to participate in anything a child without cranio surgery could. Just don't drop her on her head on the concrete, which you wouldn't want to do with any baby..." ha!

Blood Bank Questions:

Q: What is my blood type? Q: What is my babies blood type? Q: How can I prepare to give blood? Q: How long before surgery can I donate?

Q: Is there anything I could do the week before surgery to make me ineligible to donate?

(Double check this one!! My husband went to donate and they asked him if he'd taken Advil in the past two days. This would have made him INELIGIBLE to donate!! This is because Advil is a blood thinner. Who knew?!)

Q: Can extended family donate? Q: How much blood will I donate?

Q: Can I give blood while nursing?

Pre-Operation Questions:

Q: Will she take a bottle of sleepy juice before surgery?

'Happy juice' is often given before surgery to make the separation between baby and parents easier. Our girl was asleep and did not need the juice.

Q: Does she need to come fasting?

Chloe's surgery was at 7:30AM. Her last solid could be taken at midnight. Her last breastmilk feeding was done by 3:30AM. Pedialyte was allowed 2 hours before surgery but 'bleh' have you tasted that stuff? We tried getting Chloe to take Pedialyte a week before surgery but she never warmed up to the flavor of either plain or grape.

Q: Does my baby need to take iron supplements?

Chloe is just breastfeeding so I was concerned about her iron levels. She barely eats anything else (except those little baby Puffs which she gobbles down like a turkey- but they only have 10% iron per serving). Breastmilk DOES contain iron, but my pediatrician said it's not a sufficient amount. However, her blood test came back showing she was not anemic. If you are concerned about this one, check with your pediatrician a couple months before surgery so the blood has time to absorb the iron.

Q: My baby has a _____ (stuffy nose, cough, diarrhea), will that cause the surgery to be rescheduled?

Very likely!! Be careful 2 weeks before surgery to not expose your baby to sick people! I was a crazy mom will my hand sanitizer policing.

Q: What time will her surgery be?

Younger babies have priority for earlier surgeries. 7:30AM was the earliest time at our hospital. We were there 2 hours before surgery for preparations.

Q: What results are you looking for in the morning of surgery blood test?

Our anesthesiologist needed to test her electrolytes to make sure she was hydrated and her platelet count. Our baby also had diarrhea the weekend before so they were especially concerned.

Q: What should we bring to entertain our baby after surgery?

For the first couple days, our baby simply wanted to be held and snuggled. A favorite blanket would be ideal here. Also, a toy he or she would recognize by texture. Once Chloe's eyes opened, she was so excited to see her favorite kitty Wubba Nub pacifier, and lunged at it with all her baby-might! She also loved having her squeaky Sophia La Giraffe and music box stuffed bunny. The nurses said feel free to bring ANYTHING that will make your baby feel comfortable.

Q: How will we know if something went wrong during surgery?

Our nurse assistant, Jessica, said she would be calling down from the operating room every hour to say how things were going. If there was an emergency situation and they needed us to sign up papers or update us, we had a buzzer (like the kind at restaurants) that would notify us from anywhere in the hospital. This was sooo spectacular, because then we could wait in the cafeteria or go on a walk to get out of the high-energy waiting room. There was also a progress-chart monitor with our babies operation number that we could check the minutes and status of her o

Post-Operation Questions:

Q: Is swelling on and off normal?

Yes. Especially in the first few weeks and in the mornings. Try propping up your child's head with a pillow at night to keep down the swelling. Our little girl's right eye would be swollen every morning for the first two weeks and then be down by bedtime.

Q: Who do I contact if I have a concern?

Our plastic surgeon assistant gave us his personal phone number to text with concerns which was AMAZING! We only texted him once but it was more a comfort knowing I could get easy, quick advice if I was worried.

Q: Are white bumps on the stitches normal?

Yes, white bumps are normal. If they start leaking green, yellow puss, contact your doctor.

Q: The stitches are poking out, what should I do?

You can either trim them or wait until your post-operation visit for the nurses to trim.

Q: How do I keep the incision clean?

Bacitracin gel is an anti-bacterial that can be applied once or twice a day after cleaning off the incision with a rinse of water and soap.

Q: Is liquid coming from the eye okay?

Clear liquid is normal, but if it gets thick, goopy, green, or yellow double check with your doctor/surgeon.

***Answers are not 'direct' quotes and have been paraphrased from words by our Plastic Surgeon at Rady's, Dr. Amanda Gosman, Neurosurgeon at Rady's, Dr. Meltzer, Plastic Surgeon at L.A. Children's Hospital, Dr. Mark Urata, and Nuerosurgeon at L.A.C.H, Dr. McComb. These answers are specific to Chloe's case of metopic craniosynostosis. Please refer to your specialists for specific case advice.

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