Glaucoma With Crouzon Syndrome

Background:Crouzon syndrome is the most common form of craniosynostosis, and mutations in the fibroblast growth factor receptor 2 and 3 (FGFR2 and FGFR3) genes are implicated in its pathogenesis.Observation:A 10-year-old female patient with Crouzon syndrome and congenital glaucoma treated with trabeculectomy and ocular hypotensive medications was examined. The patient had proptosis, exposure keratopathy, megalocornea, thin central corneal thickness, a shallow anterior chamber, mild anterior subcapsular cataract, and a cup-to-disc ratio of 0.9. Ultrasound biomicroscopy revealed a shallow anterior chamber, posterior synechiae, and closed angle in the right eye, as well as a narrow angle in the left eye, despite an axial length of 28.9mm in the right eye and 30.0mm in the left eye, measured by A-scan ultrasound biometry. The crystalline lens thickness measured by ultrasound biomicroscopy was 4.18mm in the right eye and 4.12mm in the left eye.Conclusions and Importance:Despite long axial lengths, shallow anterior chambers with occluded angles are possible in Crouzon syndrome and are most likely caused by FGFR2-related anterior segment dysgenesis. To the best of our knowledge, this is the first report that describes closed angles and anterior segment dysgenesis as a secondary cause of congenital glaucoma in Crouzon syndrome.