What is Craniosynostosis ?

Cranio = Skull  Syn = Joining  Ostosis = Bone

In a baby, the skull (cranium) is made up of several plates of bone, which lie side by side, but are not actually joined. In normal circumstances, these plates expand and grow, allowing the baby’s brain to grow inside the skull. Eventually, when the brain has finished growing in early adulthood, all the plates of bone will join together (fuse) at their seams (sutures) so that the skull becomes a fixed “box” of bone, which protects the brain.

 In Craniosynostosis, one or more of the seams (sutures) has joined too early. This happens before birth, but is not caused by anything the mother did or did not do during pregnancy. The baby is born with an unusual shaped head. This is because the skull cannot grow properly in the area that has fused too early. The baby’s brain needs to grow rapidly during the first year of life, so there may be extra growth in another area of the skull to compensate for the restriction in the affected area. This may accentuate the abnormal skull shape.

The shape of the baby’s head will depend on which suture has fused early.

Some babies may have other abnormalities as well, such as webbing of fingers and toes, (syndactyly) or inability to breathe through the nose (choanal atresia).

Types of Craniosynostosis

Craniosynostosis may occur on its own in a child without any other problems and without any family history or similar conditions. This is sometimes called SINGLE SUTURE CRANIOSYNOSTOSIS.

The shape of the head usually gives a clue as to which suture is involved, each different type of head shape is given a name for description.

SCAPHOCEPHALY-a narrow ”up-turned boat” shape-the sagittal suture is fused.

TRIGONOCEPHALY-a triangular shape with pointed brow—the metopic suture is fused.

See 'Non-Syndromic Craniosynostosis' (pdf) for more information

SYNDROMIC CASES-the craniosynostosis is just one feature of the condition.  Particular patterns of abnormality are well recognised and can be grouped together into a specific syndrome which is often given the name of the person who first described it.

In APERT syndrome for example there is craniosynostosis of the sutures of the skull, the head has a rounded shape accentuated by flattening at the back and the fingers and toes have failed to develop separately (this is called syndactyly) and often needs surgery to improve them.

In other allied conditions such as PFEIFFER and SAETHRE CHOTZEN syndromes changes in the hands and feet are also seen but are often quite subtle (such as broadening of the thumbs or webbing of the fingers and toes). In the syndromic patients the bones of the face are more likely to be under developed and this may cause prominence of the eyes where the sockets are too small (such as in CROUZON syndrome), the breathing passages behind the nose are often cramped leading to snuffly noses and noisy breathing. In MUENKE syndrome changes in physical features are often minimal

See 'What causes Craniosynostosis' (pdf) for more information

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