Treatment of Intraretinal Cystic Spaces Associated With Gyrate Atrophy of the Choroid and Retina With Intravitreal Bevacizumab

Abstract

Purpose: To evaluate the use of intravitreal bevacizumab injections for the treatment of intraretinal cystic spaces associated with gyrate atrophy of the choroid and retina. Methods: Retrospective chart review of 5 eyes of 3 patients with intraretinal cystic spaces associated with gyrate atrophy and treated with intravitreal bevacizumab injections was performed. Information obtained included history, examination findings, optical coherence tomography (OCT), OCT angiography, fluorescein angiography, and microperimetric findings before and after the injections. Results: The mean age of patients was 11 ± 4.6 years. All patients received three monthly bevacizumab injections. The mean corrected distance visual acuity was 0.27 ± 0.10 at baseline and improved to 0.36 ± 0.12 after the injections ( P = .015). The mean central macular thickness was 569 ± 127 µm at baseline and improved to 422 ± 123 µm after the injections ( P = .067). Microperimetry and OCT angiography performed in 1 patient before and after the three injections showed improved macular sensitivity and vascular density measurements following the injections. Conclusions: Intravitreal bevacizumab is safe and effective in the treatment of intraretinal cystic spaces associated with gyrate atrophy. [ J Pediatr Ophthalmol Strabismus . 2020;57(6):400–406.]

Gyrate Atrophy of the Choroid and Retina: A Review

“…56 It may also be useful during follow-up of treatment. 57 Fundus autofluorescence may help demonstrate and follow up the extent of areas of chorioretinal atrophy associated with the condition as characteristic hypoautofluorescent areas (Figure 3). 56 Optical coherence tomography (OCT) is useful in evaluating the macular area and can show variable features that include cystoid macular oedema, 43 intraretinal cystic spaces (Figure 1D), 6 foveoschisis, 26 macular hole, 44 choroidal neovascularisation, 45 foveal thinning, 26 epiretinal membranes, 56 outer retinal tubulations, 56 and choroidal atrophy.…”

“…7,54 Treatment modalities for cystoid macular oedema or intraretinal cystic spaces associated with GA includes the restriction of arginine in diet, 72 supplementation with vitamin B6, 72 administration of topical or oral carbonic anhydrase inhibitors, 73,74 topical non-steroidal antiinflammatory drops, 73,74 and intravitreal or subtenon steroid injections which, however, may increase the risk of cataract formation and elevation of the intraocular pressure. 28,30,75,76 Other treatment options also include injection of intravitreal anti-vascular endothelial growth factor agents, 6,57 which was shown to improve macular sensitivity and vascularity. 57 Treatment of ocular complications of GA includes cataract surgery and intraocular lens implantation for visually significant cataract which may also be associated with zonular compromise.…”

Optical Coherence Tomography Angiography of Macular Perfusion Changes after Anti-VEGF Therapy for Diabetic Macular Edema: A Systematic Review

“…Diabetic macular edema (DME) is the most common cause of loss of vision in diabetic patients, affecting around 21 million people globally [1]. The treatment of macular edema associated with various retinal diseases, including DME (Figure 1), is currently dependent on the repeated intravitreal injection of different anti-vascular endothelial growth factor (anti-VEGF) agents [2][3][4][5][6]. Other treatment modalities for DME include steroid injections and macular laser photocoagulation [2].…”

“…DCP: deep capillary plexus; DME: diabetic macular edema; DMI: diabetic macular ischemia; FA: fluorescein angiography; FAZ: foveal avascular zone; FD: fractal dimension; Full: full retinal thickness; MP: microperimetry; OCTA: optical coherence tomography angiography; PDR: proliferative diabetic retinopathy; SCP: superficial capillary plexus; VD: vascular density 6. Journal of Diabetes Research previously treated with anti-VEGF agents while 2 eyes were treatment-naïve.…”

Comment on: Imaging in inherited retinal disorders

“…For example, in a recently conducted and relatively large multicenter study that analyzed optical coherence tomography angiography (OCTA) findings in patients with cystoid macular edema associated with GA, there was enlargement of the superficial and deep foveal avascular zone (FAZ) areas in patients with GA compared to age matched controls which also correlated with the macular cyst area. 4 Interestingly, following the successful treatment of intraretinal cystic spaces of the macula in one of these patients using anti-VEGF injections, there was a decrease in the size of the FAZ area, which further emphasizes the relationship between cyst size and FAZ area in patients with GA. 5 Several patients with GA were also found to have areas of decreased vascular density especially in the temporal perifovea on OCTA, 3,5 which seemed to improve following anti-VEGF treatment which was also associated with improvement of macular sensitivity on microperimetry. 5 Finally, using en face OCT in a patient with GA, there was evidence of multiple hyporeflective cystic spaces with a honeycomb pattern at the level of the inner nuclear and outer plexiform layers, 3 which may potentially assist in the exclusion of foveoschisis and allow a more accurate diagnosis and management of the macular pathology associated with GA.…”