Retinitis Pigmentosa Associated with Vasoproliferative Tumors and Coats-like Fundus
A 31-year-old female previously diagnosed with RP presented to our clinic complaining of decreased vision in her right eye over the past 4 days. BCVA was counting fingers at 1 meter in her right eye and counting fingers at 0.5 meter in the left one. Positive ocular findings included mild posterior subcapsular lens opacities, trace cells in the anterior chambers in both eyes, and trace blood and pigment in the anterior vitreous of the right eye. Fundus examination (A-C) revealed typical RP changes in both eyes, and macular distortion and wrinkling in the right eye. An inferotemporal exudative retinal detachment with a VPT lesion measuring 5×5×4 mm was present in the right eye and an inferotemporal VPT measuring 5×4×3mm with surrounding hard exudates was also present in the left eye. We performed FA, ultrasonography and OCT (C-G). FA revealed capillary nonperfusion with leaking aneurysmal and telangiectatic vessels not related to RP and the VPT.\nLaser photocoagulation was performed over the lesions and nonperfused areas in both eyes and after 2 weeks, cryotherapy was performed. A dense vitreous hemorrhage developed in the right eye over the following days which lasted for 3 months. As the condition was disabling, deep vitrectomy with membranectomy and ILM-peeling was performed. After 3 months, the patient showed improvement in retinal appearance (G-I). Lesion size and the associated exudates were notably decreased and vision was improved to 20/200 in the right eye but remained unchanged in the left one. There was no recurrence of the condition after 2 years of follow up.