Background:Werner syndrome, a typical form of progeria, develops after puberty and is characterized by early occurrence of senile signs, such as hair changes(e. g., gray hair and baldness)and skin changes(e. g., atrophy and sclerosis). Binocular cataract is a well-known complication;however, to the best of our knowledge, there are almost no reports of retinal changes. Herein, we report two cases of Werner syndrome, in which optical coherence tomography(OCT)revealed decreases in the thicknesses of the circumpapillary retinal nerve fiber layer(cpRNFL)and ganglion cell complex(GCC), and Humphrey perimetry results led to a diagnosis of glaucoma.
Case 1:The patient was a 44-year-old man whose corrected visual acuity at the initial visit was 1.2/1.2. An intraocular lens was inserted into the anterior segment of both eyes. Funduscopic findings included enlargement of optic nerve cupping in both eyes. The cpRNFL and GCC thicknesses were decreased on the inferior side. Humphrey perimetry revealed an arcuate scotoma in the left eye and glaucoma.
Case 2:The patient was a 62-year-old woman whose corrected visual acuity at the initial visit was 1.0/0.5. An intraocular lens was inserted into the anterior segment of both eyes. Funduscopic findings included enlargement of optic nerve cupping in both eyes. The cpRNFL and GCC thicknesses were decreased. Humphrey perimetry revealed visual field defects predominantly in the superior portion of the right eye and glaucoma.
Conclusions:We reported two cases of Werner syndrome complicated by glaucoma. When young patients with cataract of unidentified cause are suspected to have Werner syndrome, OCT and visual field examinations should be performed to check for concomitant glaucoma, in addition to anterior segment examinations, to confirm early-onset cataract.
Nippon Ganka Gakkai Zasshi(J Jpn Ophthalmol Soc)126:36-42, 2022.