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CRANIOSYNOSTOSIS - SINGLE SUTURE

This is the most common form of craniosynostosis where the join or the suture in the skull running along the top of the head is fused causing the head to be long and thin.

This is where the join or suture on one side of the forehead is fused causing an asymmetric appearance of the forehead and around the eyes. Sometimes it can also cause asymmetry in the lower part of the face.

This is when the join or suture running over the top of the forehead from side to side is fused. It may be associated with a syndrome and generally results in a flat broad head.

This is where the join or suture in the middle of the forehead fuses early. It can lead to a triangular shaped forehead.

This is a rarer form of single suture craniosynostosis where one of the joins or sutures at the back of the head is fused. This leads to flattening on one side and should be distinguished from positional plagiocephaly which is due to lying predominantly on one side.

CRANIOSYNOSTOSIS - SYNDROMIC

When several joins or sutures of the skull fuse too early this is known as multisutural synostosis and can be associated with a syndrome.

Crouzon syndrome was named by a French doctor and affects multiple sutures. It can also restrict facial growth, especially the midface.

Apert syndrome was described by a French doctor and involves fusion of multiple sutures in the skull. It can also affect the midface and hands.

Pfeiffer Syndrome was described by a German geneticist and affects multiple sutures in the skull. It also affects the face resulting in characteristic midface retrusion.

Saethre-Chotzen syndrome is where multiple skull sutures fuse prematurely. It results in an abormal skull shape and characteristic facial features such as a high forehead and droopy eyelids. It can also affect the hands.

ORBITAL DYSTOPIA

Vertical orbital dystopia is where the eye sockets are at different levels leading to an asymmetric appearance of the eyes.

This is where the eye sockets are in an abnormal position. They are at the same level but abnormal distances away from the midline.

ENCEPHALOCELE

This is where the lining of the brain and sometimes the brain itself protrudes through the base of the skull. They protrude into the forehead or nose.

NHS Specialist services

 

Craniosynostosis is a rare and complex condition requiring highly specialised surgical treatment and complex multidisciplinary care.

It is designated by the `Rare diseases` section of the Department of Health and NHS England, as one of the “highly specialised services” and so receives specific funding allocation.

The Department of Health and NHS England recommends management of craniosynostosis to be carried out in one of four major well-resourced designated centres.

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