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Acta Medica Medianae
Vol. 48, No2 , April, 2009
UDK 61
YU ISSN 0365-4478

 

Correspondence to:
Gordana Stanković-Babić

Ophthalmology Clinic, Clinical  Center

48 Dr Zoran Djindjic Blvd.

18000 Nis,

E-mail : gordanasb@nadlanu.com

 

 

 

 

 

 

Case reports
UDC:617.7-007-07

 

Ophthalmological and radiological picture of Crouzon Syndrome – Case Report

 

Gordana Stankovic-Babic and  Rade R. Babic

  

Ophthalmology Clinic, Clinical  Center Nis1

Institute of Radiology, Clinical Center Nis2

 

Crouzon syndrome (CS) accounts for about 4.8% of all cases of craniosynostosis. Crouzon syndrome occurs in approximately 1 per 25.000 births worldwide. It may be transmitted as an autosomal dominant genetic condition, but 25% of cases represent a fresh mutation. There is no race or sex predilection. The appearance of an infant with CS can vary in severity from mild presentation with subtle midface characteristics to severe forms with multiple cranial sutures, fused and marked midface and eye problems.

A ten-year-old girl suspected to have congenital glaucoma was sent to a consultative examination to the Ophthalmology Clinic, CC Nis. The very appearance of the girl indicated the diagnosis – Crouzon syndrome, which was confirmed by ophthalmological and radiological examinations. The patient was referred to the ophthalmologist in charge for further attendance and was recommended to undergo a surgical treatment by a maxillofacial surgeon.

Exophtalmus, hypertelorism, divergent strabismus, hypermetropy, head, orbit and jaw deformities, extremely illustrative, mostly rarely seen in everday practice, are the elements of the clinical picture of Crouzon syndrome. It deserves our attention, taking into consideration ocular complications and multifactorial purblindness which it causes.  Acta Medica Medianae 2009;48(2):37-40.

 

Key words: Crouzon syndrome, craniosynostosis, strabismus, purblindness