More Info on Endoscopic Treatment

I did a bit more calling around yesterday and learned a few things.

First of all, I called the St. Louis Children's Hospital to get some more information about whether Addy could be accepted as a candidate for endoscopic surgery at their facility. The receptionist talked to the doctor there, and told me that while in the past they have corrected cases of coronal synostosis using the endoscopic procedure, they were "not excited" about the outcome. Meaning, the results didn't look as good and the head shape did not correct itself as nicely. (I asked if this meant they had problems with the sutures closing up again, and she said that did not normally happen.)

Apparently, most of the surgeries that are performed endoscopically in St. Louis deal with saggital synostosis, which is when the bones fuse together down the middle of the head. (Saggital synostosis is the most common type, making up about 50-60% of cases, while coronal is the second most common with 20-30%.) The receptionist told me that the doctor would be willing to consider performing the surgery on Addy, but we would have to go into it with the understanding that she might need further correction down the road.

With that in mind, I tried contacting the University Health System in San Antonio. In my searches for doctors who perform this surgery, this facility was the first thing that came up. I didn't try them first only because their location is significantly further away. Apparently, though, these people are the pioneers of endoscopic treatment of craniosynostosis, and the lady who answered my questions there gave me the impression that they do only endoscopic repairs. (I forgot to ask for complete clarification on that point.)

Besides these factors, I was also impressed by the fact that, even though I contacted them by email instead of by phone, the nurse there called me back within about a half an hour of my email. She was very helpful in answering my questions, and she sent me an email with more information soon after we got off the phone. It also doesn't hurt that they have a video on their website showing Addy's exact condition and explaining how they treat it, plus a whole series of before and after photos for coronal synostosis patients!

Obviously, this facility does routinely perform endoscopic surgery on coronal synostosis patients. The nurse there told me that they have had good results with these surgeries, but emphasized that they are very dependent on helmet therapy following the procedure. Apparently this would involve Addy wearing these helmets until she was 12-18 months old, getting new helmets 4-6 times within that period to adjust for her growth. The nurse also emphasized that we would have to return to San Antonio for these helmets and not get them done somewhere else, because (as she said), "other places do it wrong." That could explain why the people in St. Louis have not seen good results, if they do not do the helmet therapy correctly.

So, we are still going to meet with the surgeon at Duke on Thursday. Unless he gives us a good reason to pursue the traditional "open" treatment, however, we will most likely see if we can get Addy accepted to the place in San Antonio. We are definitely liking what we are seeing there, and although the distance is further, it sounds worth it, especially since it seems advantageous to get this issue fixed as soon as possible. I asked the nurse in San Antonio whether she could tell me the risks of waiting for the open procedure, and she said she could not tell me specifically, except that the brain would continue to grow and skew the head shape even further the longer we wait. That will be one question on which we can hopefully get more clarification on Thursday.

Please pray that we will get all the information we need to decide which method of surgery would be best for Addy. Please also pray that we would get accepted to San Antonio if that is the route we choose, and that God would work out the logistics of getting us there for the surgery and follow-ups.

Thanks!

Current Pictures

This series of pictures should show you a little more clearly what Addy's case of craniosynostosis looks like. The probable closure is the right coronal suture (going from the middle of the head down to the right ear).

Here she is! Ain't she a cutie?

Below is her "concerned" face. This look was even more exaggerated when she was first born. You'll noticed that the left side of the head (on your right in this picture) looks slightly larger than the other, the right eyebrow looks higher, and the left eye is slightly deviated from the other one. My pediatrician seems to think the eye deviation may be because there is pressure on her optic nerve, but a surgeon's assistant with whom I spoke seemed to think this was unlikely because of where the optic nerve is in the brain. I'm not sure who to believe on that matter.

Left side of the face -- looks normal and cute

Right side of the face

You may notice this side of her face looks fairly different than the other. You may also see the slight bump in the middle of her head, showing where (I believe) the front bone is slightly overlapping the back.

Here you can see that the left side of the forehead is definitely positioned further out than the right side.

Even more clearly here...

This is most obvious from the angle of looking down on her head, as in the picture below. Notice you can actually see her right eye from this angle, but you can't see the left eye. The forehead is retracted on the right side like this because the fused bone has not allowed it to expand forward as it should. (Compare this to the craniosynostosis photo in this post, and you will see why I said that picture looked like Addy.)

Back of the head -- note the left side is bigger than the right here too

She still is pretty darn cute for having a crooked head, though, isn't she? :-)

Being silly

We love you, little sweetie pie!

Our Current Plan

Our pediatrician (Dr. Y) is out of town this week. Because Addy is already getting close to the age where she will be too old for the endoscopic surgery, we really need to get the ball rolling if that is going to happen. Hence, I have been researching this matter and calling all over the place for information for myself. I guess I'm too impatient to wait for my doctor to do all that for me.

I have contacted the facilities in which endoscopic surgery is available in NY, FL, and MO. I am unsure whether any of these (or others) would accept Addy's case even if we decided we would like to go the endoscopic route. As one of the doctor's assistants told me, the endoscopic procedure "looks great on paper," but it isn't really that simple.

I today spoke with another doctor's assistant at Duke hospital, which is not far from where we live. I explained our situation and told her that we are essentially trying to weigh pros and cons at this point, but we are not exactly sure if we know what all the pros and cons are.

Although Duke does not offer endoscopic correction of Addy's condition, they are a good facility for performing the open procedure. We will likely go to them if we decide to go that route. The woman I spoke with today advised me to speak with one of the surgeons who actually does these surgeries at Duke. He will hopefully be able to confirm Addy's diagnosis and tell us whether she definitely does need surgery. He should also be able to give us some more of the pros and cons that we may not know about, in order to determine what would be the best method of surgery in Addy's case.

Our appointment is set for Thursday, the 29th. Please pray that we would be able to get our questions answered, and that God would give us the wisdom to know which method of surgery would be best for our little girl.

Thank you for your thoughts and prayers as we begin this journey.

Treatment / Surgery Options

How is craniosynostosis treated?

(Please note that this is merely my understanding of the treatments at this time. I will likely learn much more as I go along.)

There are two types of surgery available to treat this condition. The traditional "open" method involves making an incision from ear to ear across the top of the head and peeling back the scalp in order to access the fused bones. A piece of the bone is removed to reopen the suture, and the bones are repositioned to achieve a more normal shape. Customized helmets are used after surgery to further guide the bones to grow in the correct shape (in most cases, I believe).

A newer method includes the use of an endoscope in order to provide a less invasive treatment. With this approach, two small incisions (and inch or two in length) are made, and an endoscope is used to guide the separation of the bones beneath the scalp. Helmet therapy must be used after surgery to guide the growth of the bones. This surgery, however, is only available to children under 5 months of age. (Some facilities only accept patients under 3 months.) Older children require the more extensive surgery described above.

What are the pros and cons of the different surgeries?

This is the million dollar question at this point. :-) Here is what I have been able to determine so far. All of this is to the best of my knowledge, but I could very well find that I am mistaken once I learn more.

For endoscopic surgery --

Pros:

* Less blood loss -- About 10% of cases require a blood transfusion, whereas blood transfusions are very common with the traditional open surgery.

* Shorter surgery length -- These surgeries typically take about an hour, whereas the open surgery can take up to 6-8 hours (though I assume this is probably for more series cases).

* Shorter hospital stay -- Children generally are held for observation for about 1 day, versus a typical 3-7 days for the other method.

* Less scarring -- Since the incisions are much smaller, obviously the scarring will be less. Either way, though, doctors try to give a result that will not be obvious once the hair grows out.

Cons:

* Limited availability-- The endoscopic version of the surgery is only available at a handful of facilities around the country, including New York, Florida, Missouri, Texas, and California. I have found a total of 9 facilities to date that offer it according to their websites.

* Time constraint -- As mentioned above, this surgery is only available for children under 5 months, and is ideally done by 3 months. Since Addy is already just over 3 months old, she is pushing the age limit if we are not able to get this done asap.

* Longer helmet therapy -- It is my impression, though I have not heard this directly, that the helmets would have to be worn longer than with open surgery (probably until she is a year old, as opposed to maybe for 4-5 months).

* Possibility of further surgery -- I have been told that it is possible (perhaps even most likely?) that Addy's type of craniosynostosis (coronal) will still require the open surgery to be performed when Addy is older, perhaps around 2 years old. I am not sure whether this is because the suture will most likely close again and cause more problems, or whether there will simply need to be a few adjustments made in order to achieve a more symmetrical, aesthetically pleasing head and face shape.

At this point, we are trying to determine which type of surgery will be the best for Addy. More details in the next post about how we are planning to work on this determination.

What Is Craniosynostosis?

Until two weeks ago, I had never heard of this condition, much less needed to research what it was and try to pronounce it in discussions with hospitals all over the country. You can find plenty of information about the condition on the internet (which is mostly what I have done), along with pictures of extreme cases that will scare you silly. Rest assured, Addy's case is not that frightening to look at. (I plan to post a few pictures soon.) But for those who don't want to spend hours on the internet researching the condition, I will try to provide some basic information here. I just might be stealing some things verbatim from the Wikipedia article on it (among others), but let's just keep that to ourselves. Fortunately there is no penalty for plagiarism here.

What is craniosynostosis?

Craniosynostosis is a condition in which one or more of the sutures (gaps between skull plates which are held together with strong, fibrous tissues) closes prematurely in an infant's head. Normally the sutures remain flexible throughout childhood, allowing the brain to expand as needed. When the sutures close prematurely, it can cause the head to grow abnormally as the brain tries to find other directions in which to expand, and it can cause brain damage if not corrected.

Normal infant head

An example of craniosynostosis which looks almost exactly like Addy's case

(note the closed suture and asymmetric head shape)

Upon initial diagnosis, Addy apparently has a closure of the right coronal suture, as pictured above.

What causes craniosynostosis?

The cause of the majority of craniosynostosis cases is unknown. It is estimated that it occurs in 1 out of every 2000 births, so it is fairly common. It can be the result of an inherited syndrome (about 20% of cases), or it can occur sporadically, without any known link or cause. Addy's case seems to be the latter, as she is physically and developmentally normal in all other areas as far as we know.

Other important questions to be answered in the following post...

About Addy -- Initial Diagnosis

Addy was born on April 20th -- a beautiful baby with a beautiful birth. I noticed very quickly after her birth that her head had a bit of a strange shape to it, but wrote it off as being a little misshapen from the birth. I expected it would right itself within a few days or weeks.

At her 2-week check-up, the nurses thought it strange when I pointed out that her head shape had not changed much. The left side was a bit higher and bigger than the other, and the front skull plate on the right side seemed to overlap the one behind it. I was told it should correct itself in time.

When Addy was 7 weeks old, I took her to a new doctor consultation. We had not picked a pediatrician before she was born, so we were going to Dr. Y to find out a bit more about her practice. We liked what we saw and decided to make an appointment for July 8th, for Addy's 2-month check-up. Before we left, I asked Dr. Y to take a look at her head and tell me what she thought. She agreed that it would probably correct itself, but said we should keep an eye on it.

I watched her head carefully over the next month. While the height difference between the left and right side seemed to even out a bit (or at least the difference became less distinct), the overlapped plates on the right seemed to remain the same. The right side of her forehead was also retracted backward compared to the other side, making her right eye open wider and appear almost bug-eyed sometimes. In addition, I began to notice that her left eye was a bit "lazy" at times -- deviating a bit to the left and down compared to the other eye. Also, the back of her head clearly showed that the left side was bigger and wider than the other, making it difficult for Addy to hold her head up straight because of the weight difference.

At our first official appointment with Dr. Y, I expressed my concern about these things right at the outset. Dr. Y completed her usual examination, and while she found Addy to be completely healthy and normal in all other ways, she wanted to check into Addy's head issues further, particularly because it could possibly be affecting her left eye.

Dr. Y called me later that day, requesting that I take Addy to an urgent care to get her head x-rayed. She told me that Addy may have a condition called craniosynostosis, which in layman's terms means that some of the skull plates have fused together prematurely. I took Addy in for the x-ray, and sure enough, the doctor who reviewed the x-ray believed this to be the case.

Although this is not a completely "official" diagnosis, I am quite sure that Addy does indeed have craniosynostosis. I have done enough research and seen enough pictures of children with the condition to convince me. We should be finding out soon whether the initial diagnosis is correct.

If Addy does have this condition, she will most likely need surgery to separate the fused bones, allowing her brain and head to grow normally as they need. I have read some places that helmet therapy can be used in place of surgery (using custom-shaped helmets to encourage the head to grow in the correct shape), but other seemingly more official reports have stated that surgery is the only possible treatment. (My guess is that the difference may be because some children have suspected craniosynostosis, but it turns out to be a different condition which can be treated with helmet therapy.) To my knowledge, craniosynostosis is treated with helmet therapy in addition after the surgery is complete.

We are still in the information-gathering stage at this point. I will soon be posting more of what I have learned up to this point (hopefully answering some questions that family and friends may have), and will update further as we find out more and continue on this adventure.