Abstrato

Crouzon Syndrome: Variability in clinical severity at a presentation a clinico-radiological evaluation.

Ahmad Mostafa Abdallah

Background/Aims: Crouzon syndrome (CS) was first described in 1912 by a French neurologist Octave Crouzon (1874-1938), as a hereditary syndrome of craniofacial dysostosis, which included a triad of skull deformities, facial anomalies, and proptosis. Here, the authors present 5 cases of CS of different clinical severity at the time of presentation. Case Report: The demographic, ophthalmic, radiological, and systemic findings of 5 patients with CS with variable clinical severity at presentation will be discussed in detail. Conclusion: CS can present with different clinical manifestations involving the skull, orbits, and eyes. The clinical and radiological findings of this syndrome vary in severity from a mild presentation with subtle manifestations, to a severe form with marked midface, brain, orbital, eye, cardiac, and respiratory complications. Management of patients with CS requires a multidisciplinary approach, by a team of craniofacial experts. Early surgical intervention is highly recommended in many patients with CS.