Craniosynostosis

When we talk about cranial deformities, it is very important to differentiate positional craniofacial deformities from craniosynostosis, because the treatment and possible sequelae are very different.

Craniosynostoses are due to the premature closure of some of the most important cranial sutures that allow the baby’s skull to grow as his brain grows.

Compare the shape of your baby’s skull to the shape of a normal skull. See the different types of sutures and fontanelles:

craniosynostosis cranial sutures
Infant cranial sutures and fontanelles

When a suture closes and the skull bones join together prematurely, the head will stop growing only on that part of the skull. Where the sutures have not come together, the baby’s head continues to grow. When this happens, the skull will be abnormally shaped, even though the brain inside has reached its normal size.

But, sometimes there is more than one suture that closes prematurely. In these cases, the brain may not have enough space to reach its normal size. This can cause a buildup of pressure inside the skull, which can cause problems such as blindness, seizures, or brain damage.

There are basically 4 types of craniosynostosis , based on the suture that closes prematurely:

Craniosynostosis - type: scaphocephaly(I)
Craniosynostosis - type: scaphocephaly(II)

The most frequent craniosynostoses in infants are:

Consequences

Non-syndromic craniosynostoses present a high percentage of functional delays greater than 50%.

We invite you to read also this page about the consequences of untreated cranial deformities.

Diagnostic

To confirm craniosynostosis, expert clinical diagnosis is necessary. Only in case of doubt (especially for the closed lambdoid suture) do we resort to skull X-rays.

To plan the surgical intervention, the practice of a three-dimensional helical scan (3D CT; 3D CT-scan) is useful. We reserve the use of Magnetic Resonance Imaging (NMR or MRI) for brain mass studies.

Before surgery we propose an ophthalmological review, neurological maturation test, dysmorphological pediatric study. Only in extreme cases is intracranial pressure (ICP) taken.

Depending on each case, a genetic study is requested, especially in complex or syndromic craniosynostosis (Crouzon, Apert, Saethre-Chotzen, etc).

Treatment for craniosynostosis

For Oxycephaly, Trigonocephaly and certain types of scaphocephaly

The solution, if it does not occur spontaneously, requires surgery to avoid the problems that the brain malformation can cause in the normal development of the baby’s brain, with its possible physical and mental consequences.

Most common types of Craniosynostosis


Scaphocephaly

craniosionstosis
Scaphocephaly - frontal view
craniosionstosis
Scaphocephaly - lateral view


Anterior Plagiocephaly

Anterior plagiocephaly (I)
Anterior plagiocephaly (II)


Posterior Plagiocephaly

Posterior plagiocephalai - rear view
Posterior plagiocephalai - top view


Bilateral Brachycephaly

Bilateral brachycephaly - frontal view. Before treatment - 20 months of age
Bilateral brachycephaly - After treatment - 3 years of age


Trigonocephaly

Trigonocephaly


Oxycephaly

Oxycephaly - frontal view

Unoperated craniosynostosis

Cranial deformities should be treated as soon as possible (before 6 months of age). If they are not treated properly in time, they can trigger irreparable damage to the physical and mental development of the baby.

According to D. Marchac, on his article “Unoperated craniosynostosis patients: correction in adulthood“, “unoperated craniosynostosis creates a significant to severe disfigurement because of the frontal deformation. Therefore, only in mild cases can one propose a limited operation or an implant, avoiding an intracranial approach. In most cases, a radical intracranial approach is the only effective operation….

trigonocephaly
Scaphocephaly (lateral view)
unoperated scaphocephaly
Scaphocephaly (top view)