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Case 33 month old with misshapen skull

Craniosynostosis

Premature fusion of one or more cranial sutures

Results in abnormal head shape by restricting growth of cranium in direction perpendicular to affected suture

.04-.1% incidence in U.S.

2-8% have primary craniosynostosis

Primary Craniosynostosis

Sagittal suture, 50-58% (most common), scaphocephaly, dolicocephaly

Coronal suture, 20-29%, brachycephaly or anterior plagiocephaly

Metopic, 4-10%, trigonocephaly

Lambdoid, 2-4%, brachycephaly or posterior plagiocephaly

Fusion of all sutures, oxycephaly (turrencephaly), or Kleebattchadel/cloverleaf skull if squamosal and metopic sutures not involved

Increased intracranial pressure, esp with multiple sutural fusions

Secondary craniosynostosis

Growth of brain keeps sutures open

If brain is small, not growing, sutures close, microcephalic

More frequent than primary type

Normal intracranial pressure

Craniosynostosis

Can be associated with sporadic craniofacial syndromes: Crouzon, Apert, Chotzen, Pfeiffer, Carpenter

Important to distinguish posterior plagiocephaly caused by positional molding from unilateral lambdoid synostosis (<2%)

Surgery for cosmetic reasons or increased ICP (usu. 3-6 months)

Endocrine abnormalities frequently associated with secondary craniosynostosis