Asymmetric functional evolution of bilateral giant angioid streaks after 14 years of follow-up: case report

• Bilateral giant subfoveal angioid streaks with pseudoxanthoma elasticum • High risk of choroidal neovascularization and severe hemorrhagic complications • 14-year follow-up showing asymmetric functional evolution between both eyes • Earlier switch to treat-and-extend regimen was associated with preserved vision Angioid streaks are rare, dark red, radiating fundus lesions surrounding the optic nerve head caused by breaks in Bruch's membrane. They predispose to choroidal neovascularization and recurrent hemorrhages requiring proactive management. We report a 44-year-old pseudoxanthoma elasticum patient with bilateral, atypical macular angioid streaks and a 14-year follow-up. In 2011, fundus examination revealed horizontal angioid streaks traversing the macula bilaterally without evidence of choroidal neovascularization; best-corrected visual acuity was 10/10 Parinaud 2 both eyes. The left eye subsequently developed neovascularization complicated by macular hemorrhages. Within two years, bilateral neovascularization with recurrent macular hemorrhages developed, prompting transition from a PRN to a treat-and-extend regimen. After 11 years of intravitreal ranibizumab therapy, the right eye maintained baseline visual acuity, while the left eye progressively declined to 1/10 (Parinaud 16) due to fibrotic sequelae. This case illustrates an interesting angioid streak pattern, characterized by giant horizontal streaks crossing the fovea, an anatomical configuration associated with particularly high risk of choroidal neovascularization and submacular hemorrhages. The 14-year follow up demonstrates the limitations of a pro re nata regimen in these high-risk presentations, as delayed transition to treat-and-extend failed to prevent subfoveal fibrosis and severe visual loss in the left eye. Conversely, earlier treatment initiation and earlier transition to treat-and-extend protocol was associated with preserved vision in the right eye. This case could suggest that a proactive approach may optimize visual outcomes as in age related macular degeneration. There is a lack of guidelines and studies on this therapeutic strategy. Close monitoring, assessment with optical coherence tomography angiography, and preventive education are essential, particularly regarding the risk of neovascularization and hemorrhage in patients with foveal-spanning angioid streaks. A proactive regimen may represent a more suitable and effective option for these patients when complications appear.