We heard from the neurosurgeon’s office on Monday. Peter has cranial synostosis and we will need to attend the cranial-facial clinic in February. The CT scan results have been sent to one of the cranial-facial surgeons at MCV for his review and staff at MCV will be working on a list of proposed surgery dates.
At this point, unless Peter starts exhibiting some of the more severe symptoms of synostosis (seizures and so forth from intra-cranial pressure) he will have the surgery sometime in February or March (he turns five in March– what a way to spend a birthday.)
I’m okay, I just think it stinks and I hate the idea of putting him through all of this but he has to have the surgery. Otherwise, the condition eventually becomes life threatening.
I’ll keep posting as things progress.
This is the top of Peter’s skull from the CT Scan. As you can see there is no suture down the middle of his skull. The suture you see running from left to right is the coronal suture that runs from right in front of one ear to the other. The sagittal should run from the middle of that suture to the back of the skull but it’s not present. If it were, it would have the same zig-zagged appearance as the coronal suture.
This is a side/rear view of Peter’s skull. As you can see, about where the ear is there should be a suture but, again, there isn’t one.
Well, I’ve seen the CT scan. The doctor has received the radiologist’s report by now but he has not seen the scans (he picks them up tomorrow when he comes to Fredericksburg).
Peter has absolutely no sagittal suture. His skull looks like a shiny, smooth, round cue ball. He has the coronal suture and the lambdoid (the one on the lower back of the skull) but that’s it. I can find little evidence of the temporal sutures as well. On one side, you can kind of see where it was but the other side looks as if the suture was never there. Peter also, very, very clearly, has trigonenephaly. You can’t quite tell looking at him but his forehead is small and triangular shaped and his skull is strangely out of proportion with his face. Looking at him with his skin covering the skull, you can’t tell this but the scans tell a completely different story.
The question for us now is whether the neurosurgeon will want an ICP test done and, if so, when. We expect that we will be meeting with the surgical team in February as the neurosurgeon suggested. I wonder now just how extensive this surgery will have to be and will Peter need additional surgeries to follow-up later.
I’m also wondering if this could be related to a chromosomal abnormality. Most multiple suture synostosis cases are related to a chromosomal syndrome but Peter doesn’t display any of the other conditions related to these syndromes.
After our next meeting with the neurosurgeon, I will likely e-mail Dr. Bodurtha who is the head of genetics at MCV. She tested the boys a year ago this past summer and wanted to see them again this summer for more testing. She may have insight into what could be going on.
I’m worried that this may represent a larger, more serious issue.
I apologize that this post is a bit depressing. I try to be more upbeat but this has me worried (and rightly so) but I promise I’ll try to write of some of the funny things the boys have been doing.
Peter saw the neurosurgeon at the Medical College of Virginia on Tuesday (December 4). He confirmed that Peter has synostosis (or stenosis, depending on what source you consult). Synostosis is a medical term that means ‘fused.’ Some of the bone plates in Peter’s skull have fused early. This can cause problems with his brain growing.
The sutures or gaps between the plates are connected with fibrous tissue that is supposed to fill in over time but in children it’s supposed to remain flexible so that the skull can grow as the brain grows.
Peter has had synostosis of the metopic suture (the one that runs down the middle of the forehead) for quite some time. The doctor said this suture normally closes around 1 year of age or so and the fact the Peter has trigonencephaly (pyramid shaped forehead and probably forebrain) indicates that this sealed much earlier (this may have been why I had a devil of a time delivering him if his skull was partly fused).
The bigger concern at this time is that it seems the sagittal suture (the one that runs down the middle-top of the skull connecting the parietal plates on either side) is likely fused now as well. We go for a 3D CT scan this Monday, (December 10th) to get more information. The neurosurgeon will look over the scans and depending on how bad he thinks it is, he may order a ICP test. This test looks for intracranial pressure (ICP) that may be affecting the brain. In essence, the neurosurgeon would drill a hole in Peter’s skull, place a probe in and monitor the pressure inside for 3 days. Peter, of course, would be hospitalized this whole time.
If the neurosurgeon feels that surgery is needed (which is looking likely), we would meet with a panel of doctors in February who would sit down with us and go over the case and make recommendations. The panel would include the neurosurgeon (or two), a pediatric neurologist, speech therapist, a cranio-facial specialist, plastic surgeon, etc.
The neurosurgeon said that at this point fixing the metopic suture would be more cosmetic that anything else (basically ‘what’s done is done’) but I wonder if it isn’t the cause for Peter having a more severe case of autism. (Nathan doesn’t have any of this). The forebrain is affected by the trigonencephaly and it’s where a lot of higher reasoning takes place. Also, in synostosis cases there can also be mild hydrocephaly so this is a concern as well.
We will know more Monday and then after that, we will try to enjoy our holiday as much as possible while worrying about serious and life-altering surgery for our son.