Intravitreal Pharmacotherapy in the Treatment of Retinitis Pigmentosa-related Cystoid Macular Oedema

Abstract

P eripheral visual field constriction and night blindness are the main features of retinitis pigmentosa (RP). However, central vision is often impaired dramatically even in the early stages of the disease when macular complications such as cystoid macular oedema (CME) occur. The pathogenesis of RP-related CME is still not well explained. Therefore, several treatment alternatives such as topical, oral and systemic pharmacotherapy; laser photocoagulation and even vitreoretinal surgery are employed by the clinicians. In this review, we summarise the clinical data on intravitreal pharmacotherapeutic agents and focus mainly on the steroids and anti-vascular endothelial growth factor agents. KeywordsAflibercept, intravitreal injection, macula, macular oedema, Ozurdex ® , ranibizumab, retinitis pigmentosa, steroid, vascular endothelial growth factor Disclosure: Oya Donmez and Ali Osman Saatci have nothing to disclose in relation to this article. No funding was received for the publication of this article. This study involves a review of the literature and did not involve any studies with human or animal subjects performed by any of the authors Authorship: All named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship for this manuscript, take responsibility for the integrity of the work as a whole and have given final approval for the version to be published.Open Access: This article is published under the Creative Commons Attribution Noncommercial License, which permits any non-commercial use, distribution, adaptation, and reproduction provided the original author(s) and source are given appropriate credit. 1,2 Night blindness together with the peripheral visual field loss is the most prominent clinical feature.1,2 Central vision is relatively spared up to the later stages of the disease process.3 However, when macular complications such as macular oedema arise, central vision is often dramatically impaired even in the early stages of the disease. The prevalence of cystoid macular oedema (CME) is reported to be 10-20% in the eyes of patients with RP. 4,5The pathogenesis of RP-related CME is still unclear. Blood-retinal barrier (BRB) impairment, retinal pigment epithelium (RPE) pump disturbance, inflammation, autoimmunity and vitreoretinal interface changes may be among the potential causes of CME. [6][7][8][9][10][11][12][13][14][15][16][17][18][19] Fishman et al. 6 examined 15 RP patients with vitreous fluorophotometry. All patients showed abnormally high concentrations of fluorescein within the vitreous which was a sign of BRB abnormality. Larsen et al. 7 demonstrated that BRB leakage was markedly increased in six RP patients with the help of ocular spectrofluorophotometry. Vinores et al. 9 investigated the immunohistochemical staining for albumin on paraffin sections of 22 normal and 29 RP eyes.Electron microscopic immunocytochemical staining for albumin was performed on additional six normal and nine RP-affected eyes. Two-thirds of the eyes with RP demonstrated extr...