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.
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 28.9 mm in the right eye and 30.0 mm in the left eye, measured by A-scan ultrasound biometry. The crystalline lens thickness measured by ultrasound biomicroscopy was 4.18 mm in the right eye and 4.12 mm in the left eye.
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.
King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia
Disclosure: The authors declare no conflict of interest.
Reprints: Abdulaziz A. Alshamrani, MD, King Khaled Eye Specialist Hospital, Orouba Street, Umm Alhamam District, P.O. Box 7191, Riyadh 11462, Saudi Arabia (e-mail: firstname.lastname@example.org).
Received January 15, 2018
Accepted March 8, 2018