Outer retinal tubulation formation and clinical course of advanced age-related macular degeneration

Outer retinal tubulations (ORT) are a relatively new finding characterizing outer retinal atrophy. The main aim of the present study was to describe ORT development in advanced age-related macular degeneration (AMD) and to assess its relationship with disease’s severity. Patients with advanced AMD characterized either by macular neovascularization or geographic atrophy, showing signs of outer retinal disruption or retinal pigment epithelium atrophy on structural optical coherence tomography (OCT) at the inclusion examination were prospectively recruited. All the patients underwent complete ophthalmologic evaluation, structural OCT scans and fundus autofluorescence imaging. The planned follow-up was of 3-years. Main outcome measures were ORT prevalence, mechanism of ORT formation, mean time needed for complete ORT formation, best-corrected visual acuity (BCVA), definitely decreased autofluorescence (DDAF) area, questionably decreased autofluorescence (QDAF) area, retinal layer thickness, foveal sparing, number of intravitreal injections. We also assessed the possible role of external limiting membrane (ELM) and Müller cells in ORT pathogenesis. Seventy eyes (70 patients) were included; 43 showed dry AMD evolving to geographic atrophy, while 27 displayed the features of wet AMD. Baseline BCVA was 0.5 ± 0.5 LogMAR, decreasing to 0.9 ± 0.5 LogMAR at the 3-year follow-up (p < 0.01). We detected completely formed ORT in 26/70 eyes (37%), subdivided as follows: 20 eyes (77%) wet AMD and 6 eyes (23%) dry AMD (p < 0.01). ORT took 18 ± 8 months (range 3–35 months) to develop fully. We described the steps leading to ORT development, characterized by progressive involvement of, and damage to the photoreceptors, the ELM and the RPE. Eyes displaying ORT were associated with a smaller QDAF area, less retinal layers damage and lower rate of foveal sparing than eyes free of ORT (p < 0.01). We also described pigment accumulations simulating ORT, which were detected in 16/70 eyes (23%), associated with a greater loss of foveal sparing, increased DDAF area and smaller QDAF area at the 3-year follow-up (p < 0.01). In conclusion, this study provided a description of the steps leading to ORT development in AMD. ELM and Müller cells showed a role in ORT pathogenesis. Furthermore, we described a subtype of pigment hypertrophy mimicking ORT, evaluating its clinical utility.

Foveal-Sparing ILM Peeling in a Case with Vitreomacular Traction and Foveal Detachment

A pseudophakic female patient, 80 years of age, presented with a vitreomacular traction and foveal detachment at her right eye. To avoid development of a full-thickness macular hole during surgery, foveal-sparing ILM peeling was performed. After surgery, distance-corrected visual acuity increased from 0.3 to 0.6 (Snellen) 3 months after surgery and fovea was re-attached again with restoration of the retinal layers.

Photodynamic therapy for macular choroidal osteoma

Purpose: To report a case of macular choroidal osteoma treated with photodynamic therapy. Observations: A 34-years old woman with decreased visual acuity in her left eye came to our observation for assessment of an amelanotic choroidal tumor in the left eye. On the basis of ophthalmoscopic and echographic features the tumor was diagnosed as choroidal osteoma. Imaging examination revealed subretinal fluid involving the foveal area associated with alterations of outer neuroepithelial layers and photoreceptors without evidence of choroidal neovascularization. Foveal sparing standard fluence rate photodynamic therapy was performed. After treatment, subretinal fluid reabsorption and visual acuity recovery was noted with progressive restoration of foveal architecture. Due to the relapse of fluid and visual impairment, 1 year after treatment, a second PDT session was made using the same parameters and protocol of treatment. Despite a complete subretinal fluid reabsorption and visual acuity recovery the second treatment was complicated by the development of subretinal fibrosis. Conclusions: PDT is effective to induce subretinal fluid reabsorption and visual recovery in choroidal osteoma located in the macular area. However, the risk of possible complications related to the treatment have to be considered.

Heads-up Digitally Assisted Surgical Viewing with Intraoperative Optical Coherence Tomography for Myopic Schisis Repair

Purpose: To describe the surgical approach with a screen-based heads-up, threedimensional (3-D) digital viewing with intraoperative optical coherence tomography (IOCT) for the successful repair of a myopic macular schisis (MMS) case. Case Report: A 62-year-old woman with vision loss in the left eye was scheduled for pars plana vitrectomy (PPV) and MMS repair. Surgery was performed using the NGENUITY® system for surgical viewing, and foveal-sparing internal limiting membrane (fs-ILM) peeling was performed without gas tamponade, after confirming the absence of iatrogenic macular hole with I-OCT. There were no intraoperative or postoperative complications. Visual acuity improved to 20/40 and the subfoveal macular thickness improved from 706 μm (preoperative) to 221 μm after seven months of follow-up. Conclusion: Heads-up digitally assisted viewing technology with I-OCT may be useful or preferred for patients requiring vitreoretinal surgery in the setting of MMS.

Multimodal imaging for the assessment of geographic atrophy in patients with ‘foveal’ and ‘no foveal’ sparing.

INTRODUCTION To evaluate the applicability of optical coherence tomography-angiography (OCT-A) for measuring geographic atrophy (GA) areas in age-related macular degeneration (AMD) patients with ‘foveal’ and ‘no foveal’ sparing disease and compare it to other imaging modalities. METHODS A multimodal imaging protocol was applied, using infrared imaging (IR), fundus autofluorescence (FAF), OCT-A and en-face OCT in 35 eyes of 23 AMD patients with GA. Patients were classified in two groups, with and without foveal sparing disease. GA area measurements for all imaging modalities were compared for each group separately. RESULTS The measured GA area was estimated 6.68 ± 3.18 mm2using IR; 6.99 ± 3.09 mm2 using FAF; 6.56 ± 3.11 mm2 using OCT-A and 6.65 ± 3.14 mm2using en-face OCT. There was no statistically significant difference in GA area between different modalities (p=0.977). When separate analysis was conducted for patients with ‘foveal’ and ‘no foveal’ sparing disease, although GA measurements in FAF imaging displayed higher numerical values compared to the other modalities, especially in patients with foveal sparing, no statistically significant difference in GA area was found between the different imaging modalities in either group (p=0.816 for foveal sparing; p=0.992 for no foveal sparing group). CONCLUSIONS OCT-A can be reliably used in the assessment of GA in AMD patients with and without foveal sparing disease. For both groups, measurements are comparable to IR, en-face OCT and FAF, despite the fact that the latter recorded larger area of GA, mainly in the foveal sparing cases.

Methoxsalen-induced advanced bull’s eye maculopathy

A number of systemic medications are known to cause macular toxicity, and bull’s eye maculopathy is caused by some of them like hydroxychloroquine and clofazimine. A 55-year-old female, known case of vitiligo with history of undergoing methoxsalen–ultraviolet A therapy, presented with painless defective vision in both eyes. Fundus examination and autofluorescence showed macular degeneration with bull’s eye configuration. Optical coherence tomography showed perifoveal loss of photoreceptors and outer retinal thinning with foveal sparing appearing as ‘flying saucer’. Multifocal electroretinogram showed pan-macular suppression of waveforms. Patient was diagnosed as case of methoxsalen-induced advanced macular toxicity. This is the first reported case of methoxsalen-induced advanced bull’s eye maculopathy.