Craniosynostosis

Last revised by Dr Daniel J Bell on 16 Dec 2021

Craniosynostosis (plural: craniosynostoses) refers to the premature closure of the cranial sutures. The skull shape then undergoes characteristic changes depending on which suture(s) close early.

There is a 3:1 male predominance with an overall incidence of 1 in 2000-2500. 8% of cases are syndromic or familial.

Most occur as isolated anomalies but syndromic associations can be seen in a small proportion of cases (~10%):

Primary forms are either sporadic or familial. Secondary craniosynostosis occurs in relation to a variety of causes: 

The sagittal suture is most commonly involved (≈50%), where the lateral growth of the skull is arrested while anteroposterior growth continues, producing a narrowly elongated skull known as scaphocephaly (meaning boat-shaped) or dolichocephaly (from the ancient Greek for long, δολιχός: dolichos).

The next most common sutures in terms of involvement are:

  • coronal (~20%)
  • lambdoid (~5%)
  • metopic (~5%)

Restriction of skull growth is perpendicular to the affected suture line. Characteristic dysmorphic head shapes are associated with each type of craniosynostosis.

Ultrasound may be useful in subtle cases. General features include:

  • sutures are normally hypoechoic
    • there may be a loss of normally decreased echogenicity in the region of the fusion
  • lack of suture patency
  • ridging of the sutures

CT with 3D image reformations is the best modality used for the evaluation of sutures 5.

Treatment is often with a cranioplasty. Abnormal intracranial pressure may affect neurocognition.

Craniosynostosis was first accurately characterized by Rudolph Virchow in 1851 10.

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Cases and figures

  • Case 1: scaphocephaly
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  • Case 2: sagittal suture synostosis
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  • Case 2: sagittal suture synostosis
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  • Case 3: unilateral coronal synostosis
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  • Case 4: right lambdoid suture
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  • Case 5: plagiocephaly
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  • Case 6: trigonocephaly
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  • Case 7: trigonocephaly
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  • Case 8: dolichocephaly
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  • Case 9: frontal plagiocephaly
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  • Case 10: oxycephaly
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  • Case 11: with encephalomalacia and subdural hematoma
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  • Case 12: sagittal synostosis with scaphocephaly
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  • Case 13: progressive postnatal pansynostosis
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  • Case 14: pancraniosynostosis- cloverleaf skull
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  • Case 15: Trigonocephaly
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