Crouzon syndrome

Case contributed by Shervin Sharifkashani
Diagnosis almost certain

Presentation

Proptosis, blurred vision and fullness in upper eyelids temporal side and a history of facial trauma.

Patient Data

Age: 25 years
Gender: Male
ct

There is bilaterally decreased angles interposed the orbital lateral Walls and horizontal line and shallow orbital cavities, proptosis, exodeviation of the right eye, enlarged and prolapsed lacrimal glands, and remarkably narrow width optic canal in both sides. Note also soft tissue swelling in the right lower lid and adjacent cheek due to facial trauma.

Case Discussion

The case is a 25-year-old male presenting with proptosis and exodeviation of the right eye and soft tissue bulge on the temporal side of the upper eyelids during the clinical setting of facial trauma. Non-contrast orbital MDCT requested and relatively shallow orbital cavities due to relatively decreased the angle interposed orbital lateral walls and horizontal line, bilateral proptosis, right eye exodeviation, enlarged and prolapsed lacrimal glands in both sides which have led to upper eyelids temporal side soft tissue bulge and narrow width optic canal in both sides which justifies the patient's blurred vision. Findings are more compatible with crouzon syndrome.

The crouzon syndrome is a rare autosomal dominant genetic disorder and has a triad of midface hypoplasia, proptosis, and craniosynostosis the best imaging modality is skull MDCT with 3D Reconstruction. The most important complication is raised intracranial pressure and optic canal narrowing.

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