Rahela Nasir.
Crouzon syndrome with ophthalmological complications.
J Rawal Med Coll Jan ;16(1):80-1.

Crouzon Syndrome is characterized by premature craniosynostosis. It has an autosomal dominant inheritance but represents fresh mutation also. Other craniofacial abnormalities include ocular proptosis caused by shallow orbits with or without divergent strabismus. There may be increased intracranial pressure for which surgical morcellation procedures are indicated. A case of craniosynostosis is reported which is diagnosed as Crouzon Syndrome with ocular complications on clinical grounds. Split craniotomy was performed by a neurosurgeon to relieve raised intracranial pressure and to enhance brain growth. Crouzon Syndrome was originally described in 1912 by Crouzon in a mother and her daughter. It is an autosomal dominant inherited disorder but represents fresh mutation also. Crouzon syndrome is characterized by premature craniosynostosis which is quite variable but the coronal suture is nearly always bilaterally involved. Craniofacial abnormalities include brachycephaly, shallow orbits and maxillary hypoplasia. Other facial features include prominent nose, frontal bossing, ocular proptosis with or without divergent strabismus and hypertelorism, although age related modifications have been reported. Here we report a case of 6-year-old boy who presented with visual problems and was diagnosed clinically as a case of Crouzon Syndrome with ocular complications.

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