Intravitreal avastin for choroidal neovascularization associated with stargardt-like retinal abnormalities in pseudoxanthoma elasticum

Abstract

The aim of the study was to describe a patient with pseudoxanthoma elasticum (PXE), showing Stargardt-like retinal abnormalities, who underwent treatment with intravitreal bevacizumab for subfoveal choroidal neovascularization (CNV) of the right eye (RE). A 57-year-old woman with diagnosis of angioid streaks, retinal flecks, and chorioretinal Stargardt-like atrophy due to PXE was referred to our department for sudden decreased vision in her RE (20/160). Upon a complete ophthalmologic examination, including fluorescein angiography (FA), and optical coherence tomography (OCT), the patient was diagnosed with subfoveal CNV of the RE. Owing to the subfoveal localization of the CNV, the patient was submitted to intravitreal bevacizumab injection. At the 1-month follow-up, visual acuity (VA) improved (20/40), and FA and OCT revealed the CNV closure. Twelve months after the treatment, the patient's VA remained stable with no recurrence of active CNV. On the basis of our findings, a single intravitreal bevacizumab injection seems to induce total regression of CNV complicating PXE, in a patient showing Stargardt-like retinal abnormalities. Further investigations are required to confirm our results.

Keywords: Avastin; Intravitreal Bevacizumab; Pseudoxanthoma Elasticum; Stargardt’s Disease.

Figure 1

Fundus autofluorescence shows retinal flecks and areas of welldefined retinal atrophy (A). Late phase fluorescein angiography of the RE shows macular leakage form subfoveal choroidal eovascularization (CNV), associated with cystoid macular edema (CME), within a large area of well-defined atrophy (B). The hypofluorescence of the retinal flecks, is still discernable despite the hyperfluorescent background due to retinal pigment epithelium changes. Optical coherence tomography scan demonstrates moderately reflective mass corresponding to the subfoveal CNV, associated with shallow neurosensory retina elevation and CME in the macular area (C)

Figure 2

Fundus autofluorescence shows retinal flecks and areas of well-defined retinal atrophy 1 month after intravitreal bevacizumab injection (A). Fluorescein angiography late frame of the right eye, one month after intravitreal bevacizumab injection, shows moderate heterogeneous hyperfluorescence and absence of leakage due to the choroidal neovascularization (CNV) closure (B). Optical coherence tomography scan demonstrates high reflective mass corresponding to the subfoveal fibrotic CNV, and absence of both neurosensory retina elevation and cystoid macular edema in the macular area (C)