Objective: We report the association of polypoid choroidal vasculopathy (PVC) with angioid streaks (AS) secondary to pseudoxanthoma elasticum (PXE) treated by intravitreal injection of bevacizumab.
Case report: A 50-year-old patient, followed in dermatology for a PXE, who consulted for a consulted for a decreased vision in his right eye (RE) for a month. On examination, best corrected visual acuity (BCVA) was at 1/20 P14 in the RE and at 8/10 P2 in the left eye (LE). Fundus examination revealed AS in both eyes, large patches of exudate at the posterior pole with retinal hemorrhages in the RE. Fluorescein angiography (FA) showed constant hypofluorescence by mask effect due to exudates and macular early hyperfluorescence with late diffusion associated with small hyperfluorescent lesions in the RE. We suspected CNV type 2. Macular Spectral Domain optical coherence tomography (SD-OCT) showed significant retinal infiltration with pre-epithelial hyper-reflectivity bracing the diagnosis of type 2 CNV. In view of the importance of exudates and intra-retinal infiltration, we completed by indocyanine green angiography (ICGA) which showed hypercyanecent vascular dilations grouped in clusters of grapes relevant to an associated VPC. The patient received eight monthly intravitreal (IVT) injections of bevacizumab with good anatomical evolution. At 10 months, visual acuity was 1/10 with disappearance of exudates and retinal infiltration.
Conclusion: Patients with AS should receive regular follow-up given the risk of CNV but also of VPC, especially in cases of PXE. Multimodal imaging is of great help and ICGA remains inescapable.