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PURPOSE: To report a case of severe pigmentary glaucoma (PG) in a 13-year-old boy of a family affected by pigment dispersion syndrome (PDS). PATIENTS AND METHODS: A 13-year-old child was referred to our hospital for severe bilateral glaucoma. A complete ophthalmologic evaluation including refraction, intraocular pressure, central corneal thickness, slit-lamp biomicroscopy, gonioscopy, fundus examination, and ultrasound biomicroscopy was performed. Family members were also examined and a family pedigree was obtained. RESULTS: Ophthalmologic examination revealed a severe bilateral PG with Krukenberg spindle and a widely open heavily pigmented iridocorneal angle. Ultrasound biomicroscopy showed a deep anterior chamber with pronounced iris concavity in both eyes. Within his family, his 15-year-old sister and 7-year-old brother were both affected by PDS diagnosed on gonioscopy findings. CONCLUSIONS: We report for the first time a severe case of pediatric PG with a family history of PDS. This case demonstrates that accurate screening is necessary in cases of familial PDS and PG, even in the pediatric population.
*Department of Ophthalmology III, Quinze-Vingts National Ophthalmology Hospital, DHU Sight Restore †Center for Clinical Investigations, Quinze-Vingts National Ophthalmology Hospital, INSERM-DHOS CIC 1423 ‡INSERM, U968 §Sorbonne Universités, UPMC University Paris 06, UMR_S 968, Institut de la Vision ∥CNRS, UMR_7210, Paris ¶Department of Ophthalmology, Ambroise Paré Hospital, APHP, University of Versailles Saint-Quentin-en-Yvelines, Versailles, France.
Full article9.4.3.1 Pigmentary glaucoma (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders > 9.4.3 Glaucomas associated with disorders of the iris and ciliary body)
6.12 Ultrasonography and ultrasound biomicroscopy (Part of: 6 Clinical examination methods)
2.8 Iris (Part of: 2 Anatomical structures in glaucoma)
9.1.2 Juvenile glaucoma (Part of: 9 Clinical forms of glaucomas > 9.1 Developmental glaucomas)