What is Craniosynostosis?

Before Cara’s diagnosis, I’d never heard of craniosynostosis. As we are preparing our hearts and minds for this surgery (next week!), I thought I’d share a little about the condition and Cara’s experiences for those of you who might be interested. This is one of my many compulsive projects, so it’s not a totally selfless act… (sorry, not sorry).

I love this explanation from Cranio Kids:

In an infant, the skull is not a solid piece of bone, but several boney plates separated by fibrous sutures. These sutures allow the skull to expand as the brain grows, and will eventually fuse to form a solid skull. Craniosynostosis is a condition in which one or more of these sutures fuse prematurely, causing restricted skull and brain growth. It is often sporadic with unknown causes, but can sometimes be linked to a genetic syndrome.

Although her facial features were slightly lower-set, her soft spot small, and her forehead more pronounced, Cara’s head size has measured consistently within the normal range from the beginning. It was the “I’m missing something” intuition of our pediatrician even after DNA testing came back normal that began our journey towards a diagnosis. We were recommended to a geneticist who right away suspected bicoronal synostosis, which a CT later confirmed.

There are several different types of craniosynostosis, but this is what Cranio Care Bears says about Cara’s form:

Coronal Craniosynostosis

Coronal Craniosynostosis is the second most common form of Craniosynostosis and affects mostly females.  Coronal synostosis may occur on either side or may be bilateral. Infant will often have an elevation of the eye socket, flattening of the ridge of the eye and displacement of the nose on the affected side.

Because both of Cara’s coronal sutures closed, she has no asymmetry of her face, but there is flattening of her forehead from her brow bone up and a recession of her brows.

Left untreated, Cara’s facial features would progressively look more abnormal. The biggest issue, however, is that it could force her brain to grow incorrectly (or not at all), and it could cause problems with vision or hearing as pressure increases on those nerves. We feel so grateful to report that Cara shows no signs of delayed development right now, and she has passed all vision and hearing tests she’s been given.

In order to treat the premature fusing of the sutures, and in order to prevent (further) intracranial pressure, surgery is done. Cranio Kids says this:

The most common treatment is surgery performed by a neurosurgeon and craniofacial surgeon. There are three goals in surgery; open up the fused sutures to allow room for normal skull and brain growth, relieve any pressure that may be on the brain, and give the head a more normal appearance. Some cases may require more than one surgery. The prognosis for a child with craniosynostosis is generally good when treated, but will depend on which sutures are fused, how many are fused, and whether or not a syndrome is involved.

There are actually two types of surgeries of which I am aware. There’s a less invasive version done before 3 months of age called “Endoscopic Strip Craniectomy.” The sutures are cut, but not reshaped. Babies wear a helmet for around a year after surgery to correct the head shape over time. Cara is not a candidate for that surgery.

Her surgery is called CVR (cranial vault reconstruction) with FOA (frontal-orbital advancement), done between 9 and 11 months of age. Basically, a portion of her skull will be removed, reshaped, and reattached. The brow bone will also be moved forward to better protect her eyes. We are told that the surgery will take between 10 and 12 hours including all anesthesia and post-op work. I’m going to need an anesthetic, I think.

The surgery is scary, but the prognosis is good. For the next few years, Cara will be closely monitored by the surgical team for effectiveness of the surgery and by an ophthalmologist, psychologist, etc. for developmental milestones in case early intervention is needed. Genetics will determine (later) whether to further test for the underlying cause or not. We have no family history, but with two sutures fused, the likelihood of a genetic abnormality may be increased.

Craniosynostosis is rare, but affects more than I realized, 1 in 2,000 births. You can read about some of those here.

I’m so grateful for an early diagnosis, an “on-time” surgery, and no current evidence of intracranial pressure. I’m relieved to have a confident and experienced surgical team. And, I’m thankful for a God-given support system of love, prayer, and financial support. You guys are awesome, and we feel God’s presence more pervasively than ever before.

Now, on to pack my activity bag for surgery day (another compulsive project). Any suggestions?