We report a rare case of a 11 year old boy who presented for a routine check up and was found to have moderate myopia, bilateral subluxation of lens, posterior subcapsular cataracts, lattice degeneration of retina and pigmentary glaucoma simulating Wagner syndrome. Wagner syndrome is an entity initially reported in Swiss families and has similar features to our patient. However, the association of pigmentary glaucoma is a new addition to the existing knowledge of Wagner syndrome. Glaucoma reported in Wagner syndrome till date has been open angle glaucoma due to anterior segment dysgenesis or neovascular glaucoma where the exact cause has not been elucidated. Patient was diagnosed to have pigmentary glaucoma on the basis of pigment dispersion over the corneal endothelium, increased trabecular pigmentation, midperipheral iris transillumination defects, pigment on the posterior capsule of the lens and raised intraocular pressure with disc changes.
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