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Glaucoma With Crouzon Syndrome

Alshamrani, Abdulaziz, A., MD; Al-Shahwan, Sami, MD

doi: 10.1097/IJG.0000000000000946
Online Articles: Case Report/Small Case Series

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 28.9 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.

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.

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:

Received January 15, 2018

Accepted March 8, 2018

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