Surgery Day

Ethan's first major surgery to fix the Cranialfacial Microsomia was today (6/3/10). The surgery went well and there were no surprises. Total time was about 2 hours. On the docket for today:

Fix the macrostomia (wide mouth)

Fix the underlying muscles around the lip (part of the macrostomia repair)

Excise the ear tags

Fix/remove the dimple on the side of his face near the macrostomia

Clip his frenulum (to enable more range of motion for his tongue)

Clean out his ears

Examine his ears for fluid buildup/retention

Everything but the dimple removal was completed successfully. The dimple was originally thought to be the result of the abnormal musculature around the lip. It turns out it is "thin skin" as the doctor put it. So removal of the dimple would entail cutting it out and basically replacing the dimple with a scar - so the doctor decided to leave it alone.

Otherwise everything went well and as planned. Stitches at the site of the surgery where the macrostomia was will be removed next week. They will send us home with arm restraints (so he can't pull/yank on his stitches) as well as pain medications and creams/topicals to help with healing at the various surgical sites to minimize scarring.

The ENT surgeon did note that there was in fact fluid in the ears and tubes will most likely be needed. The procedure is a simple one and apparently can be done in about 15 minutes so if it is decided that we should procede along these lines, the tube insertion will most likely be appended to his eye surgery (to fix the dermoid). This surgery will probably be in the Sept./Oct. timeframe. The fluid in his ears will impact his hearing so I am fairly certain this is the course of action that will be taken.

If all goes well and according to plan (and so far it has), Ethan will be home sometime tomorrow. Dina is staying at Children's overnight with Ethan while I will stay home with Jonathan. (If anyone is wondering, Jonathan stayed with Dina's mom all day - a big thank you there!).

One interesting thing to note: it took the surgical team over 20 minutes to get the IV into Ethan because he's so chubby (baby fat). Apparently this is common: from between 9 months to 18 months, it is the hardest time to get an IV into a human being because of all the baby fat.

In the following pictures, if you notice that Ethan looks bigger overall post surgery, it is not your imagination. They pumped a lot of fluids into him during surgery to make sure he kept hydrated/electrolytes in balance.

Ethan and his Most-Absolutely-Most-Favoritest-Person-In-The-Whole-Wide-World (aka mommy) - pre surgery.

Ethan and mommy - post surgery.

Ethan post surgery. You can clearly see the stitches and surgical sites. The site on the side of his mouth appears large but is actually about right when you stop to think about it: 1/2 of that is where the mouth used to extend to, the other half accounts for where they did the repair to the muscles around the lip. And if you are wondering, the doctor gave the OK for binky immediately after surgery.

Ethan's diaper as it came back from surgery with him (they retain it to weigh it to monitor fluid intake/retention). As I've always suspected - used diapers are a dangerous form of biological warfare.

- bob

Farewell, NG Tube. We hardly knew ye...

Well, that's not *exactly* true; we got to know the NG tube *quite* well, actually. But it's gone, and as the saying goes, "na na na na, na na na na, heeeeyyy, good bye!"

We had our regular Cranialfacial clinic today and the surgical nurse looked at Ethan's G-Tube/surgical site and said it looked really good and we could start using it (we were told originally Wednesday was the first day we could use it). So we did - right then and there (as he was due for a feeding at that time).

It was actually quite appropriate that Ethan himself had yanked out the NG tube just before we left for Children's. We decided to not put it back in just in case we got the go ahead to start using the G-Tube, and, well, there you go.

The clinical visit was very encouraging. His primary cranialfacial pediatrician was very nonchalant about everything - Ethan is progressing so well that he is not concerned about *anything*.

They had originally talked about bringing the surgical schedule in substantially (macrostomia repair in March, mandibular repair within the next 2 years) but since Ethan is doing so well and since his oral feedings improved so much recently, they felt the need to do this has now abated and we stick to our original surgical plan (macrostomia repair in May-ish and mandibular repair many years off). Normally, I would be very disappointed that the surgeries are being delayed, but in this instance, the reason behind delaying them is that everything is going so well. And in Ethan's case, the later, the better - as this gives him more time to grow and develop.

The mandibular repair is now pushed out to probably around 7 years of age. Another additional reason they were originally thinking to do the first surgery at 3-5 years (and then was subsequently brought in to 1-3 years) was to address his breathing/respiratory issues. But Ethan is improving here too - enough to the point that they do not want to do/risk such an invasive surgery so early. (The original thought was that if they lengthen the jaw, this would help open up his airway more by pushing the tongue and all related structures farther out). In addition, there is the very slight possibility (in Ethan's case, the doctors said it was highly unlikely but the possibility still exists (i.e. it is a non-zero probability event)) that there may be no jaw surgeries until he is 18 years old. They will simply continue to monitor him and see how his jaw affects his eating/breathing/speech and continually evaluate/re-evaluate. Like the doctors, I do not hold out a lot of hope for having no surgeries until age 18. Most likely, it will be around age 7. This also means that the CT scan scheduled for when he is knocked out during the macrostomia repair has been cancelled (which is good - less exposure to radiation over his lifetime).

Another upshot of this is that the G-tube may come out earlier now. We thought that it may stay for 2-3 years but with the jaw surgery now pushed out several years, they said they would not leave the G-tube in that long. The criteria for taking it out is Ethan must be 100% orally fed for 2 months with good growth trajectory during those 2 months and absolutely nothing going in the G-tube. If we can get there, and it is post-macrostomia repair, it is entirely possible we could lose the G-tube within a year. If we happen to get to the 2 month criteria *before* the macrostomia repair, they want to leave it in just in case his oral feedings fall off post-surgery.

They also commented that Ethan seems to be a little ahead of the curve in terms of his cognitive and motor development. The doctors were very impressed that he can already basically sit by himself (if only for a few moments) and that he is so alert and attentive. (Recall from (much) earlier posts that Cranialfacial Microsomia brings with it a 10%-15% chance of cognitive impairment). So this was very encouraging to hear.

So to sum up, as of 1/25/10, this is where we stand:
Ethan is doing very well in his growth and development. Next major surgery to excise the ear tags, fix the macrostomia, and clip his frenulum is back to around the May time frame. Next major surgery after that to address the epibulbar dermoid still remains around the September time frame. He is off the NG tube and strictly on the G-tube. If all goes well and he continues to make progress in oral feeding, we could lose the G-tube within a year. Their appraisal is that Ethan is doing so well, our next regular clinical visit won't be for another 2-3 months.

The general prognosis, in the doctor's own words: "Ethan is in the clear now". There was originally concern for his growth trajectory (since he was such a poor oral feeder) as well as his respiratory issues. But he seems to have gotten over the hump (like achieving the necessary activation energy, to borrow an analogy from chemistry) and things are going very well now. As they put it, moving forward, there should be very few (if any) surprises and any issues that follow will be expected (he may need his tonsils and adenoids removed by the time he is a toddler, he will probably need braces, etc).

The only other thing to note: the doctor noted that Ethan's dermoid seemed to be thicker. Ophthalmology mentioned that the dermoid should not change it's characteristics in any way moving forward. We expect to hear back to see if ophthalmology wants to evaluate this. If they do, regardless of what they find (if anything) we do *not* anticipate this will change the surgery date to remove the dermoid.

In all, it was a *very* good day and we are 3 for 3 for good news. Now if only the markets would deliver such good news on a reliable basis... (for those of you who might be interested at all, yours truly is scared enough of those idiots in Washington and what they may or may not do (i.e. actually *not* reappoint Bernake) that I purchased March 100 SPY puts as protection - yes I'm that worried...).

- bob

One down...

The G-tube surgery went well and was uneventful. The procedure was scheduled for 45 minutes and by my clock, it was almost bang on. Ethan spent most of the day sleeping but he had periods of wakefulness and when he wasn't being messed around with by nurses, was actually very calm during those periods. (When he *was* being messed around with - he was pretty pissed off - but I would expect no less from my son!).

No feedings of any type (oral or NG) are allowed until tomorrow morning when they will evaluate him. Until then he is on IVs. If he's doing well, he could be discharged as early as tomorrow afternoon. We, however, are not banking on this and figure it will be Friday or Saturday before he can come home. They would prefer that he can also feed orally so that he can take his pain medications orally.

We are also scheduled for a regular Cranialfacial clinic on Monday where we will be instructed on care, cleaning, use of the G-tube and we will also meet with the plastic surgeon regarding the macrostomia repair, frenulum, and ear tag excision. By my estimation, this means we are still on track for a March surgery date.

- bob

If Bad News Occurred In An Uninhabited Forest, Could It Get Worse?

If no news is good news, what I wouldn't give to have no news for a change.

So we got back from our visit at Children's with Ophthamology and ENT. The Reader's Digest version: Ethan has severe astigmatism in his right eye due to the presence of the dermoid. Starting from around 2-3 years of age, he will need glasses and patching to correct this and prevent the onset of amblyopia (a word you do *not* want to hear). The good news is that his eyes and vision are otherwise fine. His left eye is perfectly fine. The right eye, apart from the astigmatism is fine. The ophthamologist says that the dermoid is unlikely to change moving forward and whatever damage it was going to do: it's already done. His opinion is that while this is not good news by any stretch of the imagination, it is 'typical' for children such as Ethan suffering from Cranialfacial Microsomia: it is typical for children with CFM to have dermoids of this size, it is typical for dermoids of this size to cause astigmatism, and it is typical for the astigmatism to be of this severity.

There is no need to take any corrective action until about 2-3 years as his vision is still developing and that any difficiency in visual acuity from the astigmatism is outweighed (and will be until about 2-3 years of age) by the normal difficiency in visual accuity that is due to his age (i.e. his vision won't be developed enough until age 2-3 for the astigmatism to be noticed). As a side note: if no corrective action were to be taken at all, amblyopia wouldn't set in until probably around age 5 or so, so it is not of any immediate concern (hence no action until age 2-3).

Ophthamology also believes that in light of the above, the dermoid removal is purely optional and cosmetic (if we left it in, it wouldn't make the astigmatism worse or cause any other problems, and if we remove it, it won't make the astigmatism go away). However, if we wish to have it excised, he recommends postponing it until at *least* one year of age if not later as the surgery is extremely difficult before one year of age. Moreover, it is currently impossible to judge the depth of the dermoid and as such, impossible to know how it will look after surgery: they cannot *completely* excise it - what they do is shave off the top. So this leaves some of the dermoid behind which means that there will still be some opacity in the region of where the dermoid currently resides. How opaque it will be depends on how much is left behind which depends on how thick the dermoid is (which they won't know until pre-op which is at least 8 months away currently). Regardless, he did say it would "look much better".

ENT didn't reveal much. There is some inflamation of the tissues above the vocal chords. This may be due to reflux, it may be due to the presence of the NG tube, or it may be due to a combination of both. This result is pretty much congruent with what we expected after coming out of last week's visit and recommendation of the ph probe. I suppose the good news here is that they didn't see anything out of the ordinary or abnormal.

We discussed the possibility of having his frenulum clipped to allow for more tongue movement (this has been OTPT's recommendation all along) and they (ENT) recommend to do it while under general anesthesia during his upcoming macrostomia repair (they felt that there might be some bleeding and hence couple of stitches may be required and hence it wouldn't be something to be done in clinic). They also recommended moving his (more comprehensive) hearing test up from 8 months or so to within the next 1-2 months.

We are still awaiting scheduling of the ph probe. We hope to have word on this within the next 24-48 hours. With the holidays upcoming, we expect it won't be scheduled until January.

We also have an upcoming (regular) appointment with CranialFacial in January.

-bob