Crouzon syndrome/ Craniofacial dysostosis

Crouzon syndrome, craniofacial dysostosis


  • AD – missense mutation in FGFR2 gene
  • Premature fusion of cranial sutures – increased intracranial pressure – therefore prevents skull from growing normally and affects shape of head and face


Cranial deformities:

  • Frontal bossing
  • Premature fusion of coronal suture
  • Brachycephaly
  • ‘Hammered silver’ appearance of skull

Facial malformations:

  • Maxilla hypoplasia
  • Mandible prognathism

Dental deformities:

  • High arched palate
  • Peg laterals
  • Partial anodontia

Eye deformities:

  • Exophthalmos
  • Divergent strabismus
  • Blindness
  • Neuritis
  • Hypertelorism


  • Spina bifida occulta


  • Surgical
  • Orthodontic treatment
Hammered silver appearance of skull meaning

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