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 with an overall incidence of 1 in 2000-2500. 8% of cases are syndromic or familial.

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 

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%)

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

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 loss of normal decreased echogenicity in region of fusion
  • lack of suture patency
  • ridging of the sutures

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

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

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Article information

rID: 1179
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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    Case 11: with encephalomalacia and subdural hematoma
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