Definition Of Craniosynostosis
An infant's skull is comprised of a number of free-floating bones connected together by fibrous areas called sutures. This flexible connection tissue allows the infant's head to fit through the birth canal and the skull is able to grow in response to the brain.
Craniosynostosis is a condition where the early (premature) fusion of the sutures of the bones in the skull occurs. This premature fusing of the sutures restricts and distorts the growth of the skull. The resulting cranial growth restrictions can lead to increased cranial pressure growth which can lead to vision problems and impede intellectual development. Also the abnormal head shape is associated with low self-esteem and possible behavioral problems. It is possible to correct these deformities in some cases which can relieve cranial pressure and in the long-run, improve the self-esteem of the child.
The child's head takes on a distinct shape which depends on the fused suture or sutures:
- Premature fusion of the coronal sutures forces the skull to grow wide relative to its length (also known as brachycephaly).
- Premature fusion of the sagittal suture forces the skull to grow long relative to its width (also known as scaphocephaly).
- Premature fusion of the metopic suture produces a narrow, triangular forehead with lateral pinching of the temples (metopic craniosynostosis, also know as trigonocephaly).
- Premature fusion of one of the coronal or lambdoid sutures results in asymmetrical head shape with flattening (also known as plagiocephaly).
- Premature fusion of both the coronal and sagittal sutures (oxycephaly) results in a an abnormally high conical head shape.
- Premature fusion of the coronal, lambdoid and posterior sagittal sutures results in a cloverleaf skull (also known as kleeblattschadel).
Craniosynostosis does happen in isolation but is also present in numerous syndromes including Apert, Crouzon, Pfeiffer, Saethre-Chotzen, Carpenters and Opitz-C syndrome.
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