Histopathological findings of X-linked retinoschisis with neovascular glaucoma

Background: X-linked retinoschisis (XLRS) is rarely com plicated by neovasc ular glaucoma. Only a few reports of XLRS histopathological findings with n eovascular glaucoma have been published. Methods: A 41-year-old man with XLRS complicated by neovascular glaucoma in his left eye was examined with electroretinography, B-scan, ultrasound bio microscopy and computed tomography. He was examined by ophthalmoscopy and f luorescein angiography in the other eye. An enucleation was performed in hi s left eye due to uncontrollable high intraocular pressure and persistent o cular pain. We examined the enucleated eye histopathologically. Results: Examination of the enucleated eye showed nuclear sclerosis of the lens, pigmented retrolental membrane and retinoschisis which separated the inner layer of the retina and made a large space in the vitreous cavity wit hout any apparent detachment of the outer layers of the retina. Sclerotic v essels were present histopathologically in both the inner and outer layers of the retina. There was a peripheral anterior synechia, ectropion uveae an d a fibrovascular membrane, which contained many lumina of neovascularizati on, indicating marked rubeosis iridis, Small cystic spaces were observed in both the schitic retina in the peripheral region and the foveal schisis at the outer layer of the retina. The photoreceptor cells had become markedly atrophied and multiple regions of calcification were observed. The optic n erve showed severe atrophy with gliosis, but the central retinal artery and vein were still open within the nerve. Conclusions: These histopathological findings suggest that rubeosis iridis may have developed secondarily to retinal ischemia due to occlusion of the retinal blood vessels.