Craniosynostosis

In normal childhood development, the cranial sutures allow skull enlargement as the brain grows. Premature fusion of one or more sutures results in restricted growth of bone alongside the suture and excessive compensatory growth at the non-united joints. The effect depends on the site and number of sutures involved. Sagittal synostosis is the most frequently occurring deformity.

Involvement of several sutures (oxycephaly) results in skull expansion towards the vertex, the line of least resistance.

PANSYNOSTOSIS (all sutures affected) results in failure of skull growth with a symmetrical abnormally small head and raised intracranial pressure. It is important to distinguish this from microcephaly due to inadequate primary brain development.

Treatment of coronal, metopic and pansynostosis involves extensive craniofacial surgery correcting both cranial and orbital deformities.

Indication for operative treatment is primarily cosmetic when only one suture is involved, but with involvement of two or more sutures operation is also aimed at prevention of visual and cerebral damage from raised ICP.

Posterior Plagiocephaly (flattening of the back of the head)

An increasing number of infants present with this condition. This is rarely due to a true lambdoid synostosis but it is thought to be an acquired 'locked suture syndrome' with secondary fusion following posterior moulding. Only those who develop a progressive skull deformity require surgical treatment. 369

CORONAL SYNOSTOSIS

Bilateral or unilateral.

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CORONAL SYNOSTOSIS

Bilateral or unilateral.

SAGITTAL SYNOSTOSIS

Lateral growth is restricted, resulting in a long narrow head with ridging sagittal suture (scaphocephaly). Treatment: by wide excision of the sagittal suture.

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SAGITTAL SYNOSTOSIS

Lateral growth is restricted, resulting in a long narrow head with ridging sagittal suture (scaphocephaly). Treatment: by wide excision of the sagittal suture.

Expansion occurs in a superior and lateral direction (brachiocephaly). This produces a short anterior fossa, shallow orbits and hypertelorism (widening of the interocular distance). Exophthalmos, elevated ICP and visual impairment from papilledema may result. Bilateral coronal synostosis commonly occurs as one of several congenital defects incorporated in Crouzon's and Apert's syndromes.

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