The Full Craniosynostosis Post (with a Two Year Update)

Posted by Carly Morgan on

I missed marking the occasion but last month we celebrated the two year anniversary of Felix's successful surgery for his sagittal synostosis.

When we first learned that he had a craniosynostosis condition, I searched high and low for blog posts from other parents who had been in our position so it doesn't surprise me that the three articles I did about it at the time are now some of my most popular archived posts. I could just link to them but I thought it would be more helpful to condense the good parts into one long post with a two year update, since that's what I would have loved when we were going through it.

Bullet points for our cranio journey:

/// We didn't realize he had an issue until he was five months old and the pediatrician brought it up.

/// He had no symptoms besides a growing deformity (narrow head with a prominent forehead and back of the skull).

/// We were terrified to send him in to surgery.

/// The surgery went well.

/// We were scared of helmet therapy.

/// It was no big deal and went by so fast.

/// He's awesome and except for his scars you probably would never know he'd had an issue.

Here's a little pre-op vs. two years post op comparison:

Finding out about sagittal synostosis 

This text is pulled from an earlier blog post.

Sagittal synostosis is the most common form of craniosynostosis. Craniosynostosis is a birth defect in which one or more of the sutures in a baby's skull fuses prematurely. As the brain grows, the skull becomes misshapen (deformed) and can cause pain and neurological damage.

I guess that's the way I need to start. When you talk about it like that, it sounds totally calm and reasonable. This is a problem. This is the name of the problem. This is what happens if you don't fix the problem.

In a few days, a team of surgeons is going to take Felix, sedate him, and cut into his head to remove large pieces of his skull. But I can't think about it like that or I start to feel hysterical and that doesn't help anyone.

Unemotional list of facts:

/// The sagittal suture is the line that runs down the middle of your skull from the front to the back. It's supposed to stay open through adolescence to allow the skull to grow as a person gets older.

/// There is a large soft spot for most infants at the front of the sagittal suture where other sutures in the skull meet.

/// When the sagittal suture is fused, the skull cannot grow to accommodate the brain. It will temporarily stretch the bones of the skull to make room which forces the skull to grow long and narrow. The baby's head doesn't get any wider, but the forehead and back of the head become more pronounced. This unusual shape is usually the way a baby is first diagnosed. 

{image source}

/// Felix has a completely fused sagittal suture and his front soft spot has almost completely disappeared.

/// If untreated, the deformity of the skull becomes more pronounced and neurological problems are possible as the brain continues to grow. Pain is also possible/probable as the skull stretches against the fused suture.

/// The only way to correct sagittal synostosis is surgery. There is no alternative therapy such as only using a helmet. One approach to surgery is to do a cranial vault reconstruction in which the bones of the skull are removed and remodeled. The other approach is to do an endoscopic strip craniectomy in which surgeons remove the strip of bone containing the fused suture, along with other small pieces of bone in the skull.

Helmet therapy is used following surgery to encourage the skull to grow normally as it heals. The strip craniectomy is considered a minimally invasive surgery compared to the full cranial reconstruction as it doesn't take as long, has smaller incisions, and is less likely to result in problems with blood loss during the procedure.

/// Felix is just young enough to be eligible for the strip craniectomy. He is scheduled to have that surgery this week after he is fitted with a helmet. If everything goes according to plan, he will have the surgery, spend a few days in the hospital, come home with what could be considered a very large soft spot, and wear a helmet for the next year as his head heals. After that he shouldn't have any problems.

You know, we've had our fair share of child medical drama considering we've only been parents for six years and one month. The funny thing is that instead of spending a lot of time thinking, "Why can't we have just had healthy kids?" I spend a lot of time thinking, "It could be so much worse. At least it isn't something they can't treat. At least we have good insurance. At least we live in a big city with a great pediatric hospital."

My brain keeps doing that now and it actually does help a little. I didn't know what craniosynostosis was last week but we happen to live about ten minutes away from a team that handles this condition so often they actually run a craniosynostosis clinic that serves five states. If we'd waited an extra month or two to take him to a specialist, he wouldn't have had the option for the more minimally invasive surgery.

We're really very lucky.

And then I think about him actually having surgery. I try to imagine exactly how they're going to get him away from us to take him in for the procedure. Before we had the appointment with the specialist, I told Kyle surgery wasn't an option unless it was the only option because I'd be damned if someone was going to hurt his fuzzy head.

It's the only option.

We can't even explain it to him. He's going to wake up disoriented and bandaged and he won't even know why we took him in to have them do that to him.

I swing back and forth from feeling stupid for being all screamy about something that is considered a minimally invasive surgery and feeling like what the hell kind of mother am I that I'm going to intentionally drive him to a place where people want to cut into his head. Also, this is completely stupid, but I feel like this is my fault. It's a birth defect. I made the damn skull. I don't know how I did it, but I'm the 3D printer that gave him the skull that decided to stop growing about seventeen and a half years too early. What the hell kind of mom gives a baby the kind of skull that doesn't even grow?

You know the doctor told me at his two month appointment and at his four month appointment that she thought there was something wrong with his head. She used the word disfigured and then told me he was still kind of cute despite it so I left the appointment and called her names when I talked to my friends and started looking for a new pediatrician because sometimes heads are just funny looking and Felix is the cutest baby in the world. It wasn't until she really annoyed me by harping on his "ugly" head at a check for an ear infection that I even agreed to see a specialist just to shut her up.

I should have seen it. I spend about 22 hours a day with his head. How did I not see it?

People have asked how to help and what they can do and I don't even know what to tell them because I don't know what to do. In the days that we found out about his surgery, we went under contract for a new house because there's only so much sitting around and worrying that parents can do and at least we have something to talk about now that isn't us telling each other he's going to be fine even though neither one of us really has any more power over that than the other.

I will say that I've learned that "how are you doing" and "how are you doing today" are different questions. The first one has no other answer besides notsogood because our kid is having surgery this week, but the second one leaves space to say "I'm having a rough time and don't feel like talking" or "I'm stressed and need to vent" or "I'm excited about the house" or "I'm relieved that we have a plan in place for the baby" or "I'm at Target and they have the cutest stuff in the dollar section" and there's an understanding that whatever I just said is temporary and could easily morph into something else in the next ten minutes.

Got a care package and some $5 Starbucks e-cards, and lots of text messages. All nice, even if I haven't responded to you. Can't do visits right now and will probably screen calls because I don't like talking mid-crisis much. I like finished stories with solid endings. As in, "the baby had surgery and everything went great so they sent us home after a few days and now he's just recovering." That's a better time to chat.

I'm worried about my husband because this super stressful thing is happening with his kid but he seems a lot more even keel than I am right now. I'm hoping he's not just doing that to level me out.

How does a person have a minor procedure on their skull? Next to the brain?

The doctors seemed really smart. From what I've read, this is like a 3 or 4 out of 10 surgery for them as far as difficulty goes. Super minor.

Why on earth do the co-pays on helmets cost so much? We can make it work but only because we're blessed in many ways. Are poor people just not given the option to do the less invasive surgery if they can't afford the next 12 months of helmet therapy?

I'm kissing the top of his head so much these days that I think I'm actually rubbing the hair off. I guess that's one less thing they'll have to worry about in prep.

I'm in a couple of online support groups for kids who have craniosynostosis and so many of those kids have larger issues and other complications. We're very lucky. Apparently I get to authentically use the #craniomom hashtag just like I get to use #allergymom, #asthmamom, #eczemamom, #cerclagemom, #preemiemom, and maybe #giftedmom or #IEPmom. It's like reverse girl scout badges. First you get the label and then you go through the challenge to earn it, picking up unexpected friends in the meantime because you're bonded in the sisterhood of "this totally sucks, I didn't sign up for this, what am I even doing?"

Wow. This post is really fractured. Unlike my son's skull right now, haha.

I swear I'm holding it together, you guys. Kyle could probably use some more Starbucks though. I hear his wife is in serious danger of freaking out and running her five month old football-style straight outta the hospital as soon as they produce the teeny hospital gown. We'll see...

The Corrective Surgery 

This text is taken from an earlier post.

Welcome to the post about the endoscopic strip craniectomy surgery for Felix's sagittal synostosis. There's a sentence I didn't anticipate writing a month ago...

I wrote about the baby being diagnosed with craniosynostosis but I don't think I explained adequately how blindsided we were by this whole thing. We had a little less than a week between the pediatrician's referral to the specialist and that first appointment where we learned that he had this birth defect and then nine days after that we had the surgery. In that time I went over and over the risks/benefits in my head and for the majority of that time I was absolutely against Felix having surgery.

For one, I didn't want anyone to cut into him. Also, I didn't want anyone to cut into him. Plus, I didn't want anyone to cut into him. And finally, leave his head alone.

Seriously, though, I had a hard time wrapping my head around the idea that this perfectly lovely, happy, healthy baby had something so wrong with him that he needed a minimally invasive but still fairly major operation on his skull. I could see it, sort of, in the unusual shape of Felix's head, but I figured his head was fine by me and surgical correction was unnecessary because heads don't need to be perfect.

Of course, in my mind I was simply taking his slightly "deformed" head and making it larger for the future, missing that his head would continue to be stressed and stretched as time went on. I won't share pictures of children who don't have this condition treated because I don't have the rights to any of those photos and wouldn't want to put some poor kid up as the example for "well we certainly don't want this to happen!" but the examples I saw of this condition in later life were very sad.

Add to that pain and brain damage and my hard stance on surgery became a nonstarter. So we scheduled him for the earliest date we could to ensure that he had a shot at the endoscopic (minimally invasive) procedure.

Preparing for surgery

We didn't have control over much when it came to surgery and getting him treated but we did have some control over the prep so we spent the week before surgery doing these things:

/// keeping Felix pretty quarantined from everyone to reduce the chance of surgery-postponing viruses

/// getting the house in order to prepare for Kyle's mom to come and take care of the kids while we were in the hospital and to make life easier when we got home

/// starting a binder to keep all of his medical documents and personal info (insurance and SSN, etc.)  in one place as well as photos so it will be easier for us to see his progress

/// joining craniosynostosis support groups on Facebook to help answer some of our questions

/// connecting with Cranio Care Bears, who sent us a lovely soul-lifting care package and gave us some very helpful information prior to surgery

/// picking up some outfits that don't have to go over Felix's head to make getting dressed a little easier post-surgery

/// visiting the orthotics clinic to get him set up for the helmets he'll need during his year of recovery

/// checking in with our insurance company to make sure his surgery didn't need to be pre-approved

/// getting blood drawn the day before surgery so they could run a panel and double check his blood type * He got a tag when they did his blood at the lab and a sticker on his thigh that they used in surgery. I wish I would have looked more closely because whoever put the tag on his ankle put it on too tight and it ended up cutting into his skin overnight. It would have been worth it for me to cut it off and just take it with us. 

We also went to Build-a-Bear the day after we found out about the diagnosis and picked out a monkey and a surgical outfit to be Felix's special hospital buddy. Kyle and I took turns sleeping with the monkey and carrying it around all week so it would smell a bit like home. I don't know if it worked but Felix definitely loves Dr. Monkey.

We are profoundly blessed to currently live about ten minutes from the children's hospital including the time it takes to circle around and around in the parking garage trying to find a spot. For this reason, we didn't pack as much for the surgery as other families might. I'm also the only one who actually slept at the hospital since Kyle split his time between being with us and being home with his mom and the other two kids.

Our hospital packing list:

/// our binder with all of his papers and documents

/// my laptop and charging cord (never used)

/// a paperback book (never opened)

/// a journal and pen (hardly used)

/// a pair of leggings, long stretchy dress, sleep bra, and nonskid socks for me

/// disposable toothbrush, deodorant, hand lotion, scrunchie, and chapstick for me

/// a zip up going home outfit for Felix (to avoid clothes going over his head post-surgery)

/// kimono style snap pajama set for Felix 

/// a lunch sack of snack foods

/// breast pump and bottles (barely needed and ditched in favor of hospital's superior equipment)

/// Dr. Monkey

/// iPhone, iPad, and charging cord

/// throw blanket and pillow for me

/// Boppy pillow and soft minky blanket for Felix

At home we don't let Felix sleep with anything because we're still pretty nervous about SIDS but since he was going to be in the hospital on a million monitors we figured it was fine to crowd his crib. The Boppy in particular was nice because it gave us options when positioning him in ways that didn't irritate his stitches.

Ultimately I could have used a few more snacks and some little teether toys to keep him occupied. I underestimated how aware (and bored) he was going to be in the days after surgery so we spent a lot of time playing with his blanket and monkey friend.

Day of surgery

I fed Felix for the last time early in the morning because that was the cut off for his surgery and then gave him a bath in the kitchen sink. We were told to bathe him but not to use any lotions, creams, or powders, and to dress him in clean clothes.

We participated in a research study about craniosynostosis so we started surgery day by getting our own blood drawn at the lab. I'd had the shakes pretty bad the day before surgery because of all the stress so I was fairly tied in knots by the morning of but Kyle and I were able to joke and chat and keep each other pretty steady.

Felix had to fast starting at 7 AM for his noon procedure so he was a little fussy but he really did very well under the circumstances. After we gave our blood samples we went up to check in to surgery. For some reason, this was one of the most stressful parts of the day because we spent more time than I thought we would just sitting in the hallway waiting to be called back. By this point, Kyle had to hold the baby because he was hungry and he started to fuss whenever I got too close. Luckily, Kyle always relaxes him so he drifted off while we waited.

They finally took us back to get us ready. First we had to strip him and then wipe him off with some special cleaning towels being careful to switch towels to wipe his top and bottom half. Then we put him into his little surgery outfit which was a very well-used hospital gown that had only two working snaps and which almost put me over the edge stress wise. I can't say enough about my husband and this point. Total rock.

The neurosurgeon and plastic surgeon both came in to talk to us as well as four different nurses and a person from registration. We signed some papers and nodded a lot and everyone was very reassuring and calm. Finally, the anesthesiologist came in and explained what he was going to do (two IVs and a tube in Felix's nose) and then it was his job to actually walk us to surgery.

I feel bad for him because I'm sure that lots of parents fall apart at this point and he must get stuck in the hallway with people who completely lose it. After all, you walk down to this random line painted on the floor and then you have to physically hand your baby to this man in scrubs and he disappears around the corner with your kid while you just stand there and lean comically over the painted line to try to watch them walk away.

We did OK. I cried, but only a little, and then Kyle walked me back to the waiting area. At this point we could have just waited with all the other families for the next couple of hours but our time was broken up because I had to find a breast pump since I hadn't fed the baby in more than five hours. We were escorted to a pumping room, which was nice because it was private, and we settled and chatted and I took phone calls on unrelated things to pass the time.

When we actually did wait, there wasn't much I could do but look at the world's slowest clock. They had snacks and drinks for the families and everyone around us was chatting or knitting or reading or crying or, like the girl next to me, taking sad selfies of themselves in front of the surgery sign, fixing their hair, and taking more selfies. Hashtag prayers.

They told us that they'd give us an update about an hour and a half in but they didn't come out until two hours had passed. Two hours and six minutes to be exact because I was literally pulling my hair out by that point. At two hours and six minutes, they came out and told us that it all went well and he did great.

Twenty minutes later they took us back to recovery to see him. I don't know why, but we thought he was going to be in terrible shape. He was actually not doing too bad. He was crying a sad stridor-y cry because of the anesthesia and he was obviously out of it and sore but they let me pick him up and rock/feed him right away and his head looked almost normal except for two large suture lines on his scalp. He ate just like he would normally but his oxygen level dipped down a little since he hasn't mastered eating and breathing at the same time so they used the oxygen mask to blow air on him while he ate.

After he was done, they put him in the crib and we followed him up to the infant unit. He was asleep through all of this, even as they hooked him up and checked his vitals, and he didn't wake up for about two more hours. The first evening was rough because every time he woke up you could tell he was uncomfortable but he was able to nurse for comfort and the medications they gave him really helped.


We spent two nights in the hospital just to make sure that he was doing fine on the medications. So far, he's bounced back so much faster than we thought he would and, four days post-op, he's almost back to his usual self. He's still on medication and we're taking care of his wounds and his new enlarged (end to end) soft spot so we have to be careful with him but it hasn't been anywhere near as overwhelming as I thought it would.

It's nice to be on this side of it because now I can say things like "I'm so glad we did this" and "I would definitely recommend this" and "This was not as big of a deal as I thought it would be" even though none of that was true at this point last week. Mostly, I just love this bug. Such a trooper.

Two Months into Helmet Therapy 

This text comes from an older blog post.

It's crazy to think that it's already been two months since Felix had to have surgery on his skull for craniosynostosis. At the time it felt like the worst thing that could have happened to us but now it's faded into the background as we've moved to our new house, started summer vacation, and gotten used to living with a baby in a helmet. I actually forget that the top of his skull is missing sometimes until I'm washing his hair and there's no bone there.

It's not actually as gross as it sounds. I thought it was going to be gag-inducing that he would just be a brain with some hair on it but it really is like the soft spots babies have when they're born, if you took all the soft spots and shoved them together into one mega spot on the top. It was a little nerve wracking to have the kids be around him before we got the helmet, since head injury seemed inevitable, but we made it through the two weeks of post-op recovery until it was time to start helmet therapy.

I don't love the helmet. It's clunky and since he gets sweaty in it occasionally it has a high-school-locker-room aroma that is no bueno. We wash it out with alcohol wipes every night but the trick seems to be to occasionally wipe the whole thing out with a handful of purell, rinse it with hot water, and put it out in the sun for a half hour. That's not medical advice, mind you, since I'm pretty sure they told me not to do stuff like that, but I can't cuddle with something that smells like a gym sock even if there's a cute Felix underneath.

They estimated that he'll go through four helmets over the next year but so far he's only been in the first one. They scan him in something that looks like a big copier machine every four weeks to see how he's progressed because his job is to grow from side to side while the helmet's job is to keep him from getting any larger front to back. There was a huge shift right after surgery and the one month comparison was pretty crazy:

Since then he's been shifting so slowly that I can't really see it but the computer can pick it up on the scan. It's hard because sometimes I get paranoid that his head is actually going back to the "bad" shape since kids have relapsed and had to redo the surgery before but he still looks much different than he did. I don't know - I've always thought he's handsome.

As for the scars themselves, he's got some cradle cap right around one of the incisions that's been a little stubborn but he never had an infection or any kind of irritation following surgery. The stitches dissolved on their own so we didn't have to worry about that and the surgeon barely looked at him during follow up so I guess that isn't something they're losing sleep over.

We go back to see neuro and plastic in about a month and I'm secretly hoping he magically did all of his head shifting faster than any baby ever because those helmets cost us $500 out of pocket. To be fair, though, then he'd just be back to being a big soft spot out in the open so I guess I want the helmet to stick around for a little while. At least he rocks the helmet look!

Two Year Update

In the end, he only needed two helmets. His head popped back pretty quickly to a good shape so he was in the helmet from May until November, rather than the full year we were expecting. When he came out of the helmet, he still had a large soft spot but it was more like the normal soft spots that babies have in the first year.

The helmet therapy was reasonably painless. It got a little annoying that he did it in the summer because he did get very hot whenever we were outside and we took it off and poured cold water on his head pretty often. Also, the helmet was the number one reason I quit breastfeeding because it was clunky and uncomfortable to hold but I didn't want to take it off for feedings because he'd doze off and putting it back on woke him. We hung in there for a little while but then I gave up.

At his one year post-op visit he was cleared to not return until he turns five because he's doing so well. There's no hint of regrowth or brain damage from the time before it was corrected. He hasn't had any related issues from what we can tell.

For a while I was terrified every time he bonked his head that he'd somehow damage himself more than other kids would but one of his doctors reassured me that he was pretty much like any other toddler by one year out of surgery. So, he bonks his head and I cringe but it's never been an issue.

You can see his scars through his hair but they're no big deal. He's still stinkin' cute.