Retinitis Pigmentosa Associated with Vasoproliferative Tumors and Coats-like Fundus
A 30-year-old female and a known case of RP was referred with complaints of recently decreased vision in her right eye. On examination, best corrected visual acuity (BCVA) was 20/200 and slit lamp examination was unremarkable except for trace cells in the anterior vitreous of both eyes. Fundus examination showed typical bilateral bone-spicule pigmentation and macular edema which was later confirmed by optical coherence tomography (OCT) (A). Two VPTs were present in the inferotemporal quadrant of the right eye measuring 8×6×5.5 mm and 4×3.5×2mm with extensive subretinal hard exudates. Fluorescein angiography (FA) showed some areas of capillary nonperfusion in the peripheral retina with telangiectatic and aneurysmal vascular changes around a leaking VPT lesion (). These changes resembled FA changes seen in eyes with Coats disease.\nLaser photocoagulation was applied on the VPT lesions in order to decrease their thickness and improve adjacent areas of nonperfusion. After 2 weeks, complementary cryotherapy of the VPTs was performed and the patient received an intravitreal injection of bevacizumab 2 weeks later. After 2 months, complete regression of the VPTs was noted together with persistent but stable subretinal exudates (D). The macular edema had resolved and visual acuity was improved to 20/60 at the last visit. There was no recurrence of the condition after 2 years of follow up.