Craniosynostosis

Craniosynostosis refers to 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.

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

  • endocrine disorders
    • hyperthyroidism
    • hypophosphataemia 
    • vitamin D deficiency 
    • hypercalcaemia
  • haematological disorders causing bone marrow hyperplasia
    • sickle cell
    • thalassaemia
  • inadequate brain growth
    • microcephaly
    • shunted hydrocephalus 
Types

The sagittal suture is most commonly involved (≈50%), where the lateral growth of the skull is arrested while anteroposterior growth continues, producing a narrow 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%)
Associations

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

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

Treatment is often with a cranioplasty.

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Article information

rID: 1179
Section: Pathology
Synonyms or Alternate Spellings:
  • Craniosynostoses
  • Craniostenoses

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Cases and figures

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    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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    Craniosynostosis
    Case 4: right lambdoid suture
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    Plagiocephaly

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    Case 5: plagiocephaly
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    Trigonocephaly - ...
    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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