Cover image: Sagittal Craniosynostosis | Craniosynostosis - Craniofacial Surgery | Sagittal Synostosis | Coronal Synostosis. (2018). CAPPS. Retrieved 17 June 2018, from https://www.cappskids.org/craniosynostosis/

Premature fusion of one or more sutures, thereby changing the growth pattern of the skull.

• Major sutures: Metopic, coronal, sagittal, and lambdoid
• Minor sutures: Frontonasal, temporosquamosal and frontosphenoidal

 

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Aetiology

• Hydrocephalus

Syndromic craniosynostosis:

• Apert syndrome
• Crouzon syndrome
• Carpenter syndrome
• Pfeiffer syndrome

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Pathophysiology

Normal closure:

• During early childhood:
  1. Metopic suture (beginning at 2 months)
  2. Sagittal, coronal and lambdoid sutures (22-26 months)
  3. Frontonasal and frontozygomatic sutures (last, at 68-72 months)

Premature closure

• Restricts the growth of the skull perpendicular to the affected suture
• Compensatory skull growth
  • Occurs parallel to the affected suture in order to accommodate the growing brain
• Resulting skull deformities:
  • Scaphocephaly
  • Plagiocephaly
  • Trigonocephaly

• Other skull deformities:
  • Cloverleaf skull deformity
    • Fusion of multiple sutures
    • Associated with hydrocephalus
  • Tower skull or acrocephaly
    • Combined sagittal, coronal and lambdoid synostosis
    • Often as part of Apert or Crouzon syndrome
  • Oxycephaly or turricephaly
    • Tall cranium resulting from delayed repair of brachycephaly
    • Often syndromic