SIMPLE NONSYNDROMIC CRANIOSYNOSTOSIS (ENG)

Simple Craniosynostosis occurs in approximately 1 in 2000 live births. It is not a disease which is often talked about.  However, if the simple craniosynostosis is diagnosed early and operated on, the child and his/her parents can forget about it and live a ‘normal’ life.

In this summary, only some of the nonsyndromic craniosynostosis will be described. They involve a single or double suture, they occur sporadically and are not associated with any other physical abnormality.

You can read about the syndromic craniosynostosis linked to syndromes like Apert, Pfeiffer, Crouzon on this page, for now only in French.

What is craniosynostosis ?

At birth, the skull is made of different bones that will progressively harden, but are still soft in order to allow the delivery. These bones are connected at birth by cartilage fibrous joints called sutures. They allow the skull to harmoniously grow along with the child’s brain.  The brain growth (hence the skull’s growth) is substantial in the first years of life (70-80% of the final volume is reached within the first 3 years). The sutures start to fuse at around 5 or 6 years of age but they normally should stay flexible until the growth has come to its end (18 years).Craniostenoses ENG

Children who have craniosynostosis suffer from the premature fusion of one or several sutures. The anormal fusion already starts during the pregnancy; before birth.  The skull is abnormally growing bone where the sutures are, which makes the latter ones ineffective and theoretically hinders the brain growth.  It can also cause an unsightly deformity of the skull.

Craniosynostosis is a cranial malformation caused by the premature fusion of one or several sutures. This does not apply to the fontanelles, whose premature disappearance is not a sign of craniostenosis, as this will not lead to any consequence on the brain growth if the sutures stay open. Measuring the cranial perimeter (CP) is not always helpful to detect a craniosynostosis, as for example the CP will be either normal or above the normal in a scaphocephaly.

 

MALFORMITY is not DEFORMITY.

Deformity is a consequence of an external pressure and will give in, should the pressure stop. For example, if the baby always sleeps on the same side, his or her skull can get flat on that side. If the sleeping position changes, the flat side should disappear after a while. The skull’s volume remains normal, allowing a correct growth of the brain.

You may find here the positioning advice (in French) of the Services de Neurochirurgie et de Pédiatrie du CHU in Rennes, in order to alleviate positional deformities.  The malformities we are describing here will not alleviate on their own, on the contrary, they will probably tend to worsen with growth of the child’s brain.

Below are examples of some of the more usual types of craniostenosis,

Symptoms of craniosynostosis :

 

Nonsyndromic Craniosynostosis are rare diseases, which are not well known in the general pediatric medicine. This is why they are difficult to diagnose if there is not a greatly malformed face or skull.  The malformations will increase as the child grows.

 

 

Treatment of craniosynostosis

 

If the cosmetic impact of the craniosynostosis is not socially hindering, and if the brain can grow normally, there are solutions other than surgery.

However, surgery is the primary treatment for craniosynostosis.

Depending on the age of the patient and on the type of craniosynostosis, there are different types of surgeries. The multidisciplinary team (neurosurgeon, anaesthesiologist etc) will choose which one is best for your child and explain it to you in detail. The aim of the surgery is to create a normal head shape, reduce the intracranial pressure and improve the child’s appearance. Generally a one time operation is enough, but in more complex cases a second surgery may be needed.

Never hesitate to ask all the questions that come to your mind of your surgeon/doctor, as it will set your mind at rest.

After the surgery ?

Children get back on their feet pretty quickly after the surgery. They spend 4 to 6 days in hospital (if no complications occur)

The scar needs some monitoring, but there is not a specific treatment to follow.

Normally the child does not need to wear a helmet. S/He can live like any other child.

An annual follow up will probably be offered once the post operative control has been validated.

Sources:

http://www.wikimedecine.fr/Craniosténoses

http://www.malformation-cranio-faciale.fr/wp-content/uploads/2013/04/document-de-référence-Lille.pdf

Craniosténoses non syndromiques, Pr Riffaud, Service Neurochirurgie Pédiatrique, CHU Rennes