Visual Outcome after Intravitreal Anti-VEGF Therapy for Macular Neovascularisation Secondary to Sorsby’s Fundus Dystrophy: A Systematic Review

Abstract

The aim of this paper is to summarise our own and to review published experience regarding the long-term outcome of intravitreal treatment for macular neovascularisation (MNV) secondary to Sorsby’s fundus dystrophy (SFD). A systematic literature search using the MeSH terms [Sorsby] and [anti-vascular endothelial growth factor (VEGF)] was conducted in NCBI/PubMed, Cochrane Central Register of Controlled Trials (CENTRAL), ScienceDirect, Google Scholar and ClinicalTrials.gov to identify publications reporting anti-VEGF treatment outcomes in SFD. Treatment outcomes were extracted for this meta-analysis from 14 publications and an own patient reporting a total of 31 cases with a mean follow-up (FU) of 54 months. Both eyes were affected in ten (32.3%) instances. Heterogenous reporting limited the comparability of the outcomes. All papers in common, however, reported satisfied to excellent responses to anti-VEGF therapy if patients were diagnosed and treated immediately after onset of symptoms. Of 20 eyes, for which visual acuity was reported before and after treatment, five worsened and seven improved by more than 1 line, whereas eight eyes maintained their function by end of the follow up, and 11 eyes (55%) maintained a driving vision (Snellen VA ≥ 0.5). Of six eyes with a VA < 0.5, VA improved in one to VA ≥ 0.5, whereas of 14 eyes with an initial VA ≥ 0.5, this dropped to <0.5 despite therapy. In MNV secondary to SFD, the delay between first symptoms and access to anti-VEGF treatment determines subretinal scar formation and thereby, functional prognosis. If treated early, this is generally favourable under regular controls and a consequent anti-VEGF treatment of MNV activity.

The Role of Vitamin A in Retinal Diseases

“…Clinically, the disease manifests with thickened Bruch's membrane, drusen-like deposits in the sub-RPE space, and choroidal neovascularization, which usually requires treatment with vascular endothelial growth factor (VEGF) inhibitors. Later, expanding areas of geographic atrophy may also develop [77,187,189,[191][192][193] (Figure 7). Indocyanine green angiography can show blockade of choroidal fluorescence by the thickened Bruch membrane [189,194] (Figure 7E).…”

Sorsby fundus dystrophy (SFD): A narrative review

“…[2,98] All the above published manuscripts have significant limitations, namely their retrospective nature and the limited number of patients/eyes treated. [2,5,6,19,24,[90][91][92][93][94][95][96][97][98][99] Some of them have also small follow-up periods, [90,92,96,97] whereas others have longer follow-up periods. [2,5,6,98,99] In addition, there is no consensus on the optimum pattern of anti-VEGF injections, that is, whether a treat and extend (T&E) protocol would be preferable to a pro-re-nata (PRN) approach.…”

“…Patients with this condition may become symptomatic during their second decade of life; however, the average onset of the disease is usually during the fourth to fifth decade of life. [ 1 , 2 ] Deposition of drusen within the posterior pole is typically observed during the initial stages of the disease. [ 4 , 5 ] Reticular drusen and pseudodrusen may also be observed on dilated fundoscopy.…”

“…[ 4 , 5 ] Reticular drusen and pseudodrusen may also be observed on dilated fundoscopy. [ 2 ] In more advanced stages of the disease, formation of a secondary choroidal neovascular membrane (CNV) or geographic atrophy can be observed. [ 4 , 5 ] Because the primary affected location of the retina is the macula, the main symptoms include distortion, reduced distant and near vision, impaired color vision, and nyctalopia.…”

Age-Related Macular Degeneration: New Insights in Diagnosis, Treatment, and Prevention

“…The earliest possible diagnosis of AMD and its neovascular complication allows for prompt treatment of the disease with intravitreal injections of anti-VEGF. Many studies have proved the efficacy and safety of bevacizumab, ranibizumab, and aflibercept in treating neovascular AMD [2,3,8] and other related diseases [9,10]. However, some differences in visual gain and anatomical outcomes exist between series, which appear to be associated with the baseline patient and disease characteristics and the treatment regimen used.…”