Crouzon syndrome and the eye: An overview

Indian J Ophthalmol. 2022 Jul;70(7):2346-2354. doi: 10.4103/ijo.IJO_3207_21.


The current literature review aims to evaluate the ocular findings and associated ophthalmic features in Crouzon syndrome. Craniosynostoses are syndromes characterized by premature fusion of sutures of the skull and Crouzon syndrome is the most common of the craniosynostosis syndromes. Early fusion of sutures results in craniofacial anomalies, including abnormalities of the orbits. To prepare this review of the ophthalmic findings in this disorder, an organized search on online databases such as PubMed, Scopus, Cochrane Library, and Ovid was carried out. The key terms searched were "Crouzon", "craniosynostosis", "eye" and "ophthalmic", and 51 research items were found. A total of 17 articles were included after scrutiny of the databases and a further 25 articles were added after augmented search. A detailed review was performed from the final 42 articles. A comprehensive description of associated anomalies is given along with the author's own technique of surgical management in cases with Crouzon syndrome having bilateral luxation bulbi with exposure keratopathy. However, for optimum management of cranial and oculo-facial dysmorphisms, a multidisciplinary team of specialists is required.

Keywords: Craniofacial dysmorphism; Crouzon; craniosynostosis; exorbitism.

Publication types

  • Review

MeSH terms

  • Craniofacial Dysostosis* / diagnosis
  • Craniofacial Dysostosis* / surgery
  • Craniosynostoses* / surgery
  • Eye
  • Face
  • Humans
  • Syndrome