Right from birth I wondered why my brand new baby had a ridge on the top/back of his skull. I just assumed perhaps his parietal skull bones overlapped from the trauma of a rather fast vaginal delivery and that they would correct themselves within a week or two. I asked about this at our two week and six week post-partum appointments and was told by our doctor that it was okay and not to worry about it. As I already explained in this post, I suspected that Jonathan has sagittal craniosynostosis, and those suspicions were confirmed when he was officially diagnosed this past Monday when we saw the neurosurgeon and the neurosurgery nurse practitioner. Only 1 in 2000 babies is born with this condition, so I can understand a bit why it was missed for so long, but in all honesty I’m having a hard time wrapping my own head around it.
What is sagittal craniosynostosis?
It is a congenital birth defect of the skull — the early fusion/closure of the sagittal suture.
Sagittal craniosynostosis is the most common type of cranioysnostosis (40%-58% of all cranio cases) and affects more males than females. Early closure of the sagittal suture results in scaphocephaly. Infants will often have a protruding forehead, an elongated head, and protruding at the back of the skull.
– Cranio Care Bears
Jonathan, as I have learned, is a classic sagittal baby, and I was told at his appointment that he is a mild-moderate case. His head is definitely narrow (always has been, especially from a bird’s eye view) as demonstrated in the graphic above, not round like a normal head and he has no posterior fontanel (soft spot). His forehead has started to boss (protrude) around 8 weeks and has gotten slightly worse as the time goes on, his head looks pinched in at the temples and the sides of his head on the back are starting to flatten more and more. The only thing he doesn’t have is the protruding at the back of the skull, which I’m thankful for, because I’m hoping it won’t be as hard to correct his head shape.
How do you treat sagittal craniosynostosis?
Surgery is almost always the only option, and there are two types of surgery:
- Endoscopic surgery: “The endoscopic treatment of sagittal craniosynostosis is done via two small incisions. One is placed behind the anterior fontanel (soft spot) and the other is placed further back on the infant’s skull. The endoscopes are used to expose the areas above and below the skull, including the affected suture. Also, with endoscopic aid, the involved bone is removed, thereby freeing the baby’s brain and skull to expand and reshape normally.” – Center for Endoscopic Craniosynostosis Surgery
- Cranial Vault Remodeling (CVR) surgery: “Surgery is done by a team which includes both a Craniofacial Plastic Surgeon and a Neurosurgeon. For a CVR surgery, the head is cut in a zig-zag pattern from ear-to-ear (which allows for the scar to be more easily covered with hair in the future). The skull bone is then cut to release the fused suture. After the suture is released, the skull is then stabilized with dissolvable plates and screws.” – Cranio Angel Network
There are pros and cons to anything in life, and Jonathan is going to have the endoscopic surgery. It is about a 1.5 hour surgery and a 2 night stay in the hospital with minimal risk (about 10%) for blood transfusion. Our surgeon likes to do the endo surgery if the babies are at least 10 lbs (Jonathan was 14 lbs at his appointment) and 11-13 weeks old optimally. The CVR surgery is typically done after the baby turns 6 months old and is an OR time of at least 4 hours with double the hospital stay and almost always needing a blood transfusion.
One of the possibly-seen downsides of endoscopic surgery is the need for a molding helmet after. Jonathan will be fitted for his helmet 2 weeks after surgery and ease into wearing it 23 hours a day for about 4 months. We hope to have it off by Christmas, but of course want what’s best for him. We’ll have to drive to Edmonton (a 3+ hour drive one way) every 2-3 weeks in the beginning for helmet adjustments.
Here are some photos to demonstrate Jonathan’s sagittal craniosynostosis:
To demonstrate, via bird’s eye view, his narrow skull shape:
And you can see in this photo how he doesn’t have the protruding occiput (back of the skull) like some sagittal babies do:
He is still perfect in every single way to me, but this is a step that we need to take to give his brain proper room to grow. Please keep Jonathan and my family in your thoughts and prayers as he has surgery on Tuesday July 19. You can follow Jonathan’s journey every step of the way by liking our page on Facebook.