Co occurrance of Macular Telangiectasia type 2 with hemorrhagic polypoidal choroidal vasculopathy and response to anti-vascular endothelial growth factor

Abstract

We report the case of a 57-year-old female patient who presented with defective vision in the right eye due to large hemorrhagic pigment epithelial detachment with subretinal hemorrhage near the arcades with scattered retinal pigment epithelium (RPE) alteration at the macula and minimal vitreous hemorrhage inferiorly. The left eye showed right angle perifoveal dipping venule with RPE alteration temporal to the fovea, intraretinal RPE stellate plaque at the macula area. Multimodal imaging features including color fundus photo, red-free photograph, optical coherence tomography (OCT), enhanced depth imaging OCT, fundus fluorescein angiography, indocyanine green angiography, and OCT angiography were studied along with treatment response. One eye showed features of macular telangiectasia Type 2 (MacTel 2) with polypoidal choroidal vasculopathy (PCV) (hemorrhagic type) and the other eye showed features of MacTel 2 with pachychoroid pigment epitheliopathy. This report highlights the hitherto undescribed co-occurrence of MacTel 2 with bilateral pachychoroid and with unilateral hemorrhagic PCV in one eye and its favorable outcome with anti-vascular endothelial growth factor therapy.

Keywords

• hemorrhagic polypoidal choroidal vasculopathy
• macular telangiectasia type 2
• pachychoroid pigment epitheliopathy
• pachychoroid spectrum