craniosynostosis

Craniosynostosis

Craniosynostosis

Craniosynostosis (cray-nee-oh-sin-oh-sto-sis) is a rare condition found in around 3 in 10,000 live births. The condition affects how the bones in the skull grow and is translated from Greek; ‘cranio’ means head, and ‘synostosis’ means fusion. Craniosynostosis treatment is not the same as flat head syndrome treatment and requires surgical treatment from a craniofacial unit.

Craniosynostosis is the premature fusion of one or more of the joints (sutures) between the bone plates that make up your baby’s skull. These sutures are normally flexible to allow for the natural birth process, and they remain open during growth to allow the brain to grow and develop naturally within the skull casing. Over time, as brain growth completes, the sutures close naturally to form a protective bone casing around the brain. In the case of a synostosis, one or more of these sutures sticks together prematurely causing the head to develop an unusual shape as the skull is unable to grow properly.

This condition can be confused with plagiocephaly and other variants of flat head syndrome as they all result in a skull deformation. At Technology in Motion, our experience is that we see approximately 1 in 100 infants with an undiagnosed synostosis.

 

Which sutures are involved in Craniosynostosis?

There are four bones in the top of the skull and these are connected by flexible fibres known as sutures. These allow your baby’s head to model during the birth process and then remain flexible throughout childhood to allow the brain and the head to grow normally. There are 4 bones in the top of the head and these are held in place by the metopic, the coronal and the sagittal sutures. If any one of these sutures close before they should, it means that the head can’t grow as it should and the suture must be operated on to allow the head to grow without deformity.

The most common suture to be involved is the sagittal suture. If this suture is closed, the head can’t grow in the width and a long thin head shape, with an overdeveloped brow is the result. This shape is called a scaphocephaly.

The next most common type of synostosis is the metopic suture. This is at the front of the skull and if this suture is prematurely fused the head can’t grow in width at the brow and the resulting shape is called a trigoncephaly (triangle shape).

The next most common suture to prematurely fuse is the coronal suture on one side. This is called a uni-coronal synostosis and this severely affects the face. The bridge of the nose is pulled to the side of the synostosis and the eye is ‘pulled’ up and out on the same side. The ear on the affected side is also pulled forward towards the synostosis and there is usually a flattening at the front and the back of the head on the same side as the synostosis. This is most easily seen from as scan of the head shape.

These three are the most common types of synostosis. There are others and some are associated with genetic syndromes. These require complex case management and are picked up early in life.

 

Craniosynostosis Surgery

There are two types of surgery available to open each of these synostoses:

1. The more traditional type involves exposing the skull at the area of the synostosis, removing the suture and the bone area which has been deformed, remodelling the bone and reattaching it to continue to grow, now with no restriction. Surgery times can vary depending on the type and severity of the suture and the age of the baby, but 5 hours is the usual duration of traditional surgery.
This type of surgery remodels the head shape in theatre and there is usually no need to use any remodelling helmet afterwards.

2. The second type of surgery uses an endoscope, a small camera which allows sight to the operation area and endoscopic tools to release and remove the fused suture. This type of operation takes about 1 hour but the bones are not remodelled in theatre. Post operatively the baby wears a helmet up to the age of 18 months to allow the head to grow back into shape within a moulding helmet such as a TiMband.

In the UK, doctors won’t tell parents about endoscopic surgery. Parents must ask their surgeon about it. Endoscopic surgery has been available since the early 2000’s, starting in the USA and gradually being taken up by surgeons around the world as training became available. There are some UK surgeons who offer endoscopic surgery but at the time of writing, they can offer endoscopic surgery which opens the suture and allows the brain to grow normally, but the Health Service will not fund the ongoing helmet treatment, the parents must do this themselves and the NHS Trust usually authorises this NHS/Private type of service with the parents before going ahead. Information on this can be found via the NHS website and via a BMA document.

 

Craniosynostosis Post-Op Treatment

Technology in Motion offers post-operative cranial remoulding treatments for infants who have undergone surgery. Our clinical partners in the Czech Republic have extensive experience in treating infants post-operatively and we benefit greatly from this collaboration to the benefit of our patients.

 

More Information

We’ve also compiled a list of frequently asked questions which will help you understand more about Plagiocephaly and other variants of flat head syndrome. Likewise, we have a selection of case studies and testimonials that provide an insight into our treatments. Or you can read about parents’ first-hand experiences on our Facebook page.

If you have any questions, then we’d love to hear from you. Simply call us on 0330 100 1800 (local rate)

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