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Veronika Vaclavik, Francis Munier, Daniel Schorderet, Viet Tran; Crystal deposits in the anterior lens cortex in Bietti crystalline corneoretinal dystrophy and first report of chroidal neovascular membrane in patient affected with Bietti. Invest. Ophthalmol. Vis. Sci. 2013;54(15):1350.
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© ARVO (1962-2015); The Authors (2016-present)
To report a previously unknown finding of glistening crystal-like deposits on the anterior lens cortex in 4 patients from 3 different kindred suffering of Bietti crystalline corneoretinal dystrophy (BCD). To describe a first case of CNV with good response to ranibizumab intravitreal injections
Four consecutive patients presenting with limbal corneal and intraretinal crystals were enrolled. One of patient developed a CNV, with metamorphopsias sudden onset of reduced vision
Mean patient age was 60.0 years (range, 40-81 years). Mean best Snellen visual acuity was 0.5 (range, light perception-1.0). All patients, except one, had homozygous or compound heterozygous mutations in CYP4V2 gene. Slit lamp biomicroscopy showed multiple crystal-like deposits in the anterior lens cortex and as well on the anterior lens capsule. Those deposits were highlighted on Scheimpflug camera. Fundus examination showed different stages of severe chorioretinal atrophy. The patient with CNV responded to intravitreal injections of Lucentis, improved her visual acuity
Crystal-like deposits might appear in advanced disease, although their exact etiology remains yet to be determined. To the best of our knowledge, this is the first time that crystal-like deposits are described on the lens cortex of patients with BCD associated with a mutation in the CYP4V2 gene. This is also a first report of a choroidal neovascular membrane complicating BCD, with favourable response to intravitreal injections of Lucentis
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