Evolving criteria for group E retinoblastoma (2): Impact on outcomes.

OBJECTIVE: To assess the impact of evolving criteria for group E retinoblastoma on ocular survival outcomes. DESIGN: A retrospective observational study. METHODS: Single-institution consecutive case series of patients with advanced intraocular retinoblastoma (groups D and E) were classified based on International Intraocular Retinoblastoma Classification (IIRC) and International Classification of Retinoblastoma (ICRB) criteria. The main outcomes measured were ocular survival, frequency of histopathologic risk factors (HRF), and the need for adjuvant therapy. RESULTS: A total of 332 eyes of 298 patients were classified into group D (150, 45%) and E eyes (182, 55%) based on IIRC criteria. ICRB classification resulted in upstaging of 57 group D eyes (17%) to group E. Eyes that were upstaged to group E from D in the ICRB classification (E1) differed significantly, with a greater proportion undergoing primary enucleation (17 of 57, 30%) than those that were not (10 of 93, 11%) (p = 0.003). Similar significant differences were observed between group E2 and E3 eyes (p < 0.0001). Ocular survival according to Kaplan-Meier estimates at 12 months of 79%, 59%, 49%, and 1% differed significantly between all groups (ICRB D, E1, E2, and E3, respectively). CONCLUSION: Proposed new subgrouping of group E eyes into E1, E2, and E3 based on clinical criteria is based upon natural history of tumor progression and is predictive of ocular survival. Preservation of the existing lower boundaries for group E by ICRB and IIRC offers the possibility of reanalyzing existing published data.

Iris freckle: a distinct entity.

PURPOSE: To report distinctive clinical and imaging features of iris freckles to differentiate them from iris nevi. DESIGN: Retrospective observational study. SUBJECTS: 53 patients (277 freckles) with incidental iris freckles and 102 patients (104 nevi) with iris nevi that are either clinically stable or pathologically confirmed. METHODS: Patient data were collected from the Department of Ophthalmic Oncology, Cleveland Clinic, Cole Eye Institute database (2012-2023). Lesion characteristics were recorded from slit-lamp examination descriptions and review of colour photographs. Ancillary imaging features observed using anterior segment optical coherence tomography (AS-OCT) and ultrasound biomicroscopy (UBM) were assessed in patients (where available). MAIN OUTCOME MEASURES: Comparison of clinical and imaging features of iris freckles and iris nevi. RESULTS: A total of 277 iris freckles and 104 iris nevi were analysed. Iris freckles were more frequently bilateral (17%; nevi 0%) and multiple (69%; nevi 2%) and located centrally (89%; nevi 17%) compared with iris nevi (p<0.001). The median freckle largest basal diameter and thickness were 0.8 mm (nevi; 2.1 mm, p<0.001) and 0.04 mm (nevi 1.0 mm, p<0.001), respectively. All iris freckles had irregular margins without any secondary effects compared with iris nevi. Iris freckles appeared flat without effacement of iris folds compared with iris nevi on AS-OCT (p<0.001). Iris freckles were not detectable by UBM. Heat map revealed that freckles demonstrated several features with uniform or near uniform values, whereas nevi demonstrated more variability in values across features. CONCLUSIONS: Iris freckles exhibit specific clinical and imaging features reflective of their characteristic histological composition that support their classification as a distinct entity within the spectrum of iris pigmented lesions.

Prevalence of Neurological Symptoms and Imaging Findings in Von Hippel-Lindau Patients Referred to Rasool Akram Hospital, 2018-2021.

This study aimed to assess the prevalence of neurological symptoms and related imaging findings in patients with von Hippel-Lindau (VHL) at Rasool Akram Hospital from September 2018 to September 2021. This analytical observational study examined eligible patients over the period from September 2018 to September 2021. We collected demographic information (age, gender) along with imaging findings and results of neurological and eye examinations. Comparison between qualitative variables was also done using the Chi-square test or Fisher's exact test. Also, an independent t-test was used to compare quantitative variables between the two groups. SPSS version 22 software was used for statistical analysis of data. A significant level was considered less than or equal to 0.05. Of the 54 examined patients (48.1% were male and 51.9% were female) with an average age of 36.42 ± 13.37 years. A significant majority (87.0%) reported a positive family history of the disease. The most common type of disease was Type 1 observed in 94.4% of cases and Type 2A was the next most frequent (3.7%). The most common pattern of retinal pathological lesions seen in the examination was related to bilateral lesions (79.6%). The most common pathological finding was related to the presence of a mass in cerebellar magnetic resonance imaging (48.1%). Considering the findings of the present study, which highlight a significant frequency of bilateral retinal lesions as well as masses in the central nervous system and endocrine system, it is evident that patients require careful follow-up and various interventions after being diagnosed with the disease. This approach is essential to manage and potentially mitigate the complications associated with these conditions.

Advanced intraocular retinoblastoma: evolving criteria for group E disease.

With evolving criteria for classifying intraocular retinoblastoma (RB) from International Intraocular Retinoblastoma Classification (IIRC, 2005) to International Classification of Retinoblastoma (ICRB, 2006), there have been changes in the inclusion criteria for groups E eyes that have not been widely recognized. This brief review highlights issues with the current classification systems (ICRB and IIRC) and layouts a theoretical framework arguing for merging of 2 existing classifications into 1 and subdividing the expanded group E RB, thereby preserving the possibility to reanalyze existing published data.

Double Retina Sign: A New Optical Coherence Tomography Finding in Combined Hamartoma of the Retina and Retinal Pigment Epithelium.

Purpose: To describe a case of combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) with peculiar optical coherence tomography (OCT) findings. Methods: Case report. Results: A 7-year-old girl with a history of decreased visual acuity in the left eye since early childhood presented with pigmented epiretinal membrane in favor of CHRRPE based on clinical and paraclinical findings. In OCT images, an area of retinal defect was noted, and the retina doubled up on itself near the defect (double retina sign). Conclusion: Careful examination of OCT images in patients with CHRRPE can reveal new findings.

Semi-supervised segmentation of retinoblastoma tumors in fundus images.

Retinoblastoma is a rare form of cancer that predominantly affects young children as the primary intraocular malignancy. Studies conducted in developed and some developing countries have revealed that early detection can successfully cure over 90% of children with retinoblastoma. An unusual white reflection in the pupil is the most common presenting symptom. Depending on the tumor size, shape, and location, medical experts may opt for different approaches and treatments, with the results varying significantly due to the high reliance on prior knowledge and experience. This study aims to present a model based on semi-supervised machine learning that will yield segmentation results comparable to those achieved by medical experts. First, the Gaussian mixture model is utilized to detect abnormalities in approximately 4200 fundus images. Due to the high computational cost of this process, the results of this approach are then used to train a cost-effective model for the same purpose. The proposed model demonstrated promising results in extracting highly detailed boundaries in fundus images. Using the Sørensen-Dice coefficient as the comparison metric for segmentation tasks, an average accuracy of 93% on evaluation data was achieved.

Investigation of germline VHL variants in Iranian patients with retinal capillary hemangioblastoma and genotype-phenotype analysis.

BACKGROUND: Retinal capillary hemangioblastoma (RCH), while sporadic in some cases, is the most common and earliest manifestation of von Hippel-Lindau disease (VHL). This is the first report on different types of VHL variants and genotype-phenotype correlations in Iranian families with RCH. MATERIALS AND METHODS: In this prospective observational case series study, 17 families with RCH were included. PCR was performed to amplify 3 exons of VHL gene. Afterward, Sanger sequencing was performed on all PCR products. For the detection of VHL copy number variations, MLPA was used. RESULTS: Our study identified 10 different types of VHL variants. Missense mutations were the most common variants found and affected the structure of α domain of the VHL protein (pVHL). The majority of mutations (72.7%) in the patients with RCH and central nervous system hemangioblastoma (CNS-HB) were located on α domain. CONCLUSION: α domain of VHL may play a potential role in the pathogenesis of RCH. Our findings suggest that genotype-phenotype characteristics in those variants in α- domain may predispose patients to RCH with CNS-HB.

Targeted chemotherapy in retinoblastoma: A step forward from patient survival to globe survival.

BACKGROUND/ OBJECTIVES: Despite being the most common intraocular malignancy in childhood, there is a substantial disparity between developing and developed countries in terms of patient and globe survival in retinoblastoma (Rb). The current study intends to determine patient and globe survival before and after the introduction of the new targeted treatment modalities in a developing country. METHODS: Medical records of 350 patients (516 eyes) with retinoblastoma referred to a tertiary referral center for Rb in Tehran, Iran, were reviewed. In order to compare patient and globe survival before and after the availability of the new treatment modalities, including intra-arterial and intravitreal chemotherapy, the patients were divided into group 1 (2001-2007) and group 2 (2008-2018) based on the calendar period of diagnosis. RESULTS: Two-hundred-twenty-three eyes of 149 patients and 293 eyes of 201 patients were categorized into groups 1 and 2, respectively. The 5-year patient survival was 97% across the current survey, and the overall survival rate was 96% in group 1 and 99% in group 2 (P = 0.08). Overall, 50% of eyes with retinoblastoma underwent enucleation, which was the primary in 63% (116/184) of the unilateral and 30% (99/322) of the bilateral cases. Primary enucleation was significantly lower in group 2 (35%) in contrast to group 1 (50%) (P < 0.001). In addition, globe survival improved significantly in the International Classification of Retinoblastoma Groups D (17% in group 1 vs. 66% in group 2, P < 0.001) and E (1% in group 1 vs. 23% in group 2 P < 0.001) during the two timelines. In enucleated eyes, despite the increased rate of prelaminar involvement in group 2 (13% vs. 2% in group 1, P = 0.003), the rate of high-risk histopathologic findings was similar between the two groups. CONCLUSION: Similar to developed countries, the application of new targeted treatment modalities, including intra-arterial and intravitreal chemotherapy, has been associated with significantly improved globe survival in Rb patients. However, it should be noted that even with the availability of these novel treatment options, the decision for on-time enucleation should not be deferred.

Analysis of the outer retinal thickness pixel maps for the screening of hydroxychloroquine retinopathy.

PURPOSE: To describe the findings and new interpretation methods of retinal thickness maps in the setting of hydroxychloroquine (HCQ) therapy. METHODS: A case series of 27 patients with a history of HCQ intake of more than 5 years and 21 normal subjects as the control group were studied. Patients were tested using swept-source optical coherence tomography (OCT). Custom-made 10 × 10 retinal thickness pixel maps of the 6 mm⨯6 mm area centered on the fovea were created for the full, inner, and outer retina of each eye and normal values obtained from the control group were used to calculate the statistical significance of each pixel and highlight suspicious pixels. A pixel was shown with light or dark gray color if its p value was ≤ 0.05 or ≤ 0.01, respectively. Cross-sectional OCT images and visual fields were examined as well and used to confirm the diagnosis of HCQ retinopathy. RESULTS: The pixel outer retinal thickness maps appeared accurate in detecting lesions even in eyes with unremarkable full retina thickness maps. The size and location of atrophic retinal defects were shown on pixel maps. Outer retinal thickness maps were able in differentiating outer retinal thinning from inner retinal thinning as the hallmark of HCQ retinopathy. CONCLUSION: Outer retinal thickness pixel maps can be used for the screening of HCQ retinopathy.

Spotlight on Targeted Chemotherapy in Retinoblastoma: Safety, Efficacy, and Patient Outcomes.

As the most common primary intraocular malignancy of childhood, retinoblastoma (RB) has had a complex journey in its management, following a course from enucleation as the first life-saving treatment to numerous globe-salvaging therapies during the last century. Currently, this potentially lethal disease has achieved high survival rates owing to multidisciplinary management and the introduction of neoadjuvant and multimodal chemotherapy. Therefore, the goal of treatment is shifting toward conserving the globe and vision as much as possible. Up until recently, many advanced cases of RB were enucleated primarily; however, targeted chemotherapy via the ophthalmic artery and management of intraocular seeding by local administration of chemotherapeutic agents have revolutionized the globe-conserving therapies. The added benefit of avoiding systemic complications of cytotoxic drugs resulted in these methods gaining popularity, and they are becoming a main part of care in many referral centers. Initially, there were some safety concerns regarding these approaches; however, increasing experience has shown that these modalities are relatively safe procedures and many complications can be averted by changing the choice of the drug and using some prophylactic measures. It is hoped that, in the near future, with advances in early diagnosis and patient-targeted molecular therapies, as well as gene-editing techniques, the patient's vision can be saved even in advanced RB.

Retinotomy and Retinectomy in the Management of Rhegmatogenous Retinal Detachment Associated with Advanced Proliferative Vitreoretinopathy.

Purpose: To report the anatomical and functional outcomes of retinotomy and/or retinectomy for the management of rhegmatogenous retinal detachment (RRD) complicated by advanced proliferative vitreoretinopathy (PVR). Methods: In this retrospective study, the charts of patients who underwent pars plana vitrectomy with retinotomy and/or retinectomy for the management of RRD complicated by PVR were reviewed. Primary outcome measures were final best-corrected visual acuity (BCVA) and anatomical reattachment rate. Results: Sixty-one eyes of 61 patients with a mean age of 48.56 ± 15.92 were studied. The mean follow-up time was 21.38 ± 23.08 months. The mean angle of the retinotomy was 171.31° ± 79.15°. Thirty-two (52.5%) of them needed extensive (≥180°) retinotomy. In addition, simultaneous retinectomy was performed in 36.2% of the cases. The BCVA was 2.18 ± 0.63 and 1.85 ± 0.71 logMAR before the surgery and at the last visit, respectively (P = 0.001). The initial anatomical success was achieved in 45 eyes (73.8%) after retinotomy surgery. Sixteen eyes (26.2%) had recurrent RD and needed reoperation, which was performed 5.60 ± 4.01 months after the initial retinotomy surgery. At the last examination, the retina was attached in all patients. Conclusion: Retinotomy with/without retinectomy is an effective procedure in the majority of patients with RRD associated with advanced PVR; however, additional surgeries are needed in a significant number of eyes to achieve final anatomical success.

Intravitreal recombinant tPA before vitrectomy for diabetic tractional retinal detachment: A randomized controlled trial.

PURPOSE: To investigate the effect of intravitreal recombinant tissue plasminogen activator (rt-PA) injection before vitrectomy on surgical facility and outcome in diabetic tractional retinal detachments (TRD). METHODS: Prospective, randomized, interventional clinical trial. A total of 38 eyes with diabetic TRD were candidates for vitrectomy. Patients were randomized 1:1 to receive intravitreal rt-PA, five to seven days before vitrectomy or no injection. Intraoperative surgical facility and anatomic success at month 3 after surgery were assessed. RESULTS: Mean patient age was 54.2 ± 9.4 years. There was no statistically significant difference between rt-PA and no injection groups with regard to anatomic success (89% versus 95%, respectively, P = 0.547) and best corrected visual acuity at 3 months (2.0 versus 2.1 logMAR, respectively, P = 0.840). However, surgical facility score was statistically significantly lower in rt-PA injection group compared to no injection group (4.1 ± 1.7, 5.8 ± 2.0, respectively, P = 0.007). CONCLUSIONS: Vitrectomy for TRD was easier after preoperative rt-PA injection, without affecting the anatomic and visual outcomes.

Ruthenium-106 plaque radiotherapy for uveal melanoma: analysis of tumor dimension and location on anatomical and functional results.

BACKGROUND: To report the long-term outcomes of Ru-106 plaque radiotherapy in eyes with uveal melanoma (UM) and to assess the effect of tumor thickness and location on final outcomes. METHODS: Medical records of 234 patients undergoing Ru-106 plaque radiotherapy for UM were reviewed, and the visual outcome, globe preservation, and patient survival were evaluated. The results of 2 groups were compared: 1. between thin (small and medium-sized, thickness < 7 mm, 148 eyes [63.2%]) and thick (thickness ≥ 7 mm, 86 eyes [36.8%]) tumors, and 2. between large (largest basal diameter [LBD] > 12 mm, 109 eyes [46.6%]) and medium/small (LBD ≤ 12 mm, 125 eyes [53.4%]). In addition, a comparison of the juxtapapillary location in 46 eyes (19.7%) versus tumors arising elsewhere and between tumors with and without ciliary involvement in 48 eyes (21.5%) were done. RESULTS: The patients were followed for a median of 54.2 months (range: 6-194.5 months). After adjusting for baseline visual acuity (VA), there was no significant association between final VA and different dimension and tumor location groups. Final globe preservation was 91.9%, and there was no significant difference between different dimension- and ciliary body involvement groups regarding anatomical success rate. The juxtapapillary tumors had lower globe preservation (80.4% vs .94.7%, p = 0.002). The hazard ratio (HR) for enucleation in juxtapapillary tumors was HR = 6.58 (95-CI: 3.84 to 11.21). The overall metastasis rate was 6.8%, with no significant difference in juxtapapillary tumors (4.3% vs.7.4%, p = 0.455). CONCLUSIONS: Ru-106 plaque radiotherapy is an effective treatment for thick and large UM. With this type of treatment, the globe preservation rate is lower in juxtapapillary tumors, but there is no significant difference in the metastasis rate.

"Inverted Snowing-Cloud" Sign in Endogenous Candida Endophthalmitis.

Candida spp. is the most common cause of endogenous fungal endophthalmitis. The diagnosis of this rare disease is based on clinical findings supported by positive blood culture. Recently, it has been shown that optical coherence tomography (OCT) characteristic findings are beneficial in making a correct diagnosis of fungal infection in cases with endogenous endophthalmitis. The current photo-essay aims to highlight the role of OCT in diagnosis of Candida endogenous endophthalmitis where OCT imaging of one of the retinal lesions disclosed a pre-retinal hyper reflective lesion with overlying punctate vitreous opacities. We propose "inverted snowing-cloud" sign for this OCT pattern considering the resemblance of the vitreous opacities to snowflakes.

Trans-Scleral Cyclophotocoagulation in Refractory Pigment Dispersion-Like Glaucoma Secondary to Ciliary Body Melanoma.

Purpose: To report a case of ciliary body melanoma with acute high intraocular pressure (IOP) due to pigment dispersion, treated by limited trans-scleral cyclophotocoagulation (TSCPC) and plaque radiotherapy. Methods: A 33-year-old woman was referred to clinic with acute ocular pain and decreased visual acuity from 1 week before presentation. The IOP was 55 mmHg accompanied by red eye, perilimbal injection, mild corneal edema (stromal and epithelial), scattered pigment dust on central corneal endothelium, 4+ anterior chamber pigments, and pigmented cells. Gonioscopy revealed a bulging mass posterior to the iris root, about 2 o'clock width alongside a heavy dark brown pigmentation of all angle structures. Ultrasound biomicroscopy confirmed a ciliary body mass of about 4.5 mm × 4 mm × 3.3 mm in the superior ciliary region, in favor of melanoma. Due to no response to maximal antiglaucoma therapy, a limited TSCPC in the inferior hemisclera was done. After control of the IOP, plaque radiotherapy with Ru-106 was done. Results: Three days after the cyclophotocoagulation, IOP decreased to 18 mmHg. Visual acuity reached to 20/25 and IOP remained 18 mmHg, with timolol/dorzolamide drop twice a day. Anterior chamber pigments gradually decreased, and antiglaucoma and steroid drops were tapered during 1 month, thereafter. The IOP was 14-16 mmHg with timolol/dorzolamide bid at months 3 and 6 of follow-up and 21-22 mmHg without any drop at months 12 and 18, with no sign of glaucomatous optic neuropathy. Conclusion: Limited cyclophotocoagulation may be a good choice for controlling the high refractory IOP in cases of intraocular neoplasms such as ciliary body melanoma, which are planned for salvage therapy such as plaque radiotherapy.

Comparison of retinal vascular arcade trajectory between eyes with an idiopathic macular hole and the healthy fellow eye.

PURPOSE: We aimed to present a method to evaluate the retinal arcade vascular trajectory by measuring the distance between the retinal veins in eyes with idiopathic macular hole (IMH) and comparing this measurement with healthy fellow eyes. METHODS: In this cross-sectional study 18 patients with unilateral IMH were enrolled. We used standard fundus photographs, which were obtained from each eye with a digital fundus camera using a 55-degree lens. The calculation of the retinal arcade vascular trajectories was done by drawing and measuring five vertical lines within the macular area. RESULTS: The mean age of the patients was 65 ± 9 years. The between-groups differences of each vertical line were not statistically significant, except for the differences between the most temporal line, which was greater in the diseased group (9388 vs. 8322 µm; P = 0.034). The ratio between the fifth (most temporal) and the third (fovea-center) vertical lines was greater than 1 (V-shape) in 72% of eyes with a macular hole, whereas it was less than one (U-shape) in 78% of control eyes (P = 0.003). CONCLUSIONS: We demonstrated that in eyes with an IMH the vascular arcade has more tendency to diverge on its path temporal to the fovea.

Association of RB1 rs9568036 and CDKN1A rs1801270 Polymorphisms with Retinoblastoma Susceptibility.

Purpose: To investigate the association of polymorphisms (rs9568036 and rs1801270) in the RB1 and P21 genes with susceptibility to retinoblastoma (RB). Methods: This case-control study was designed with 50 patients with RB and 50 controls. Polymerase chain reaction was performed to amplify the intron 17 of RB1 rs9568036 and exon 2 of P21 rs1801270. Then, all the amplified fragments were subjected to directional sequencing, and finally, the association between genotypes and the development of RB risk and invasion was studied. Results: A statistically significant difference in genotypic or allele frequencies of single-nucleotide polymorphisms (SNPs) (rs1801270 and rs9568036) was found between Iranian RB patients and the controls (P > 0.05). However, the frequency of genotype RB1 rs9568036 observed a statically significant difference in the RB patients compared to the control group, and the nonwild-type allele A increased the chance of susceptibility to developing RB by 2.92 times. Conclusion: The rs9568036 SNP in the RB1 gene may increase susceptibility to the development of RB in the affected patients. In spite of that, this polymorphism does not influence RB patient's invasion. Further investigation with a large enough sample size is recommended to validate this hypothesis.

Short-term changes of cornea and tear film after ruthenium-106 plaque therapy for intraocular tumors.

PURPOSE: Plaque therapy is a well-recognized treatment for intraocular tumors. In current study, we aimed to prospectively investigate the short-term effects of ruthenium 106 (Ru-106) plaque therapy on the cornea and ocular surface parameters. METHODS: Twenty-five patients diagnosed with choroidal melanoma which undergone Ru-106 plaque therapy from 2016 to 2018 were included. Tear osmolarity, tear film break-up time, Schirmer test I, fluorescein dye staining based on Oxford staining method; Ocular Surface Disease Index (OSDI) questionnaire and corneal specular microscopy were performed. These tests were assessed preoperatively and then 3 months postoperatively. RESULTS: The mean (±SD) age of subjects was 48.52 ± 15.18 years. The patients were followed for a mean(±SD) period of 3.64 ± 2.40 months. Total mean (+SD) delivered radiation dose to the tumor apex and total received radiation by the sclera was 83.20 ± 26.31 and 640.65 ± 472.69 Gray (Gy), respectively. In longitudinal analysis, OSDI score and Oxford staining score increased significantly (P = 0.002 for both variables) and the prevalence of dry eye disease (DED) increased from 20% preoperatively to 72% at 3 months postoperatively (P = 0.001).The changes in the all specular microscopy parameters were statistically nonsignificant (all P values > 0.05). CONCLUSION: There is a considerable increase in the rate of DED following plaque therapy for the treatment of choroidal melanoma in short-term follow-up. The OSDI questionnaire and fluorescein staining test are valuable tools for early detection of DED postoperatively.

Evaluating Reading Performance in Different Preferred Retinal Loci in Persian-Speaking Patients with Age-Related Macular Degeneration.

PURPOSE: To evaluate reading performance in different preferred retinal loci (PRLs) using a Persian version of a Minnesota Low Vision Reading (MNREAD) chart in Persian-speaking patients with age-related macular degeneration (AMD). METHODS: In this cross-sectional study, 35 patients with AMD were assessed. The reading performance was investigated by the MNREAD chart without using low vision aids. The location of PRL was determined monocularly using an MP1 microperimeter (Nidek Technologies, Padua, Italy). The anatomical location of the fovea was determined using optical coherence tomography (OCT). Images were taken with the MP1 microperimeter, and Spectralis HRA-OCT device was processed using graphic software to determine the location of the PRL on the retina. RESULTS: Thirty-five patients (51 eyes) with a mean age of 73.8 ± 7.7 years (range, 54-88 years) were assessed. Mean best corrected distance visual acuity (logMAR) was 0.65 ± 0.35 (range, 0.2-1.3). Mean levels of reading acuity (RA) (P = 0.009) and critical print size (CPS) (P = 0.015) were significantly different in different locations of PRL. Average scores of maximum reading speed (MRS) (P = 0.058) and reading accessibility index (ACC) (P = 0.058) were not statistically significant in different locations of PRL. There was a positive correlation between PRL-fovea distance and RA (P < 0.001, r = 0.591) and CPS (P < 0.001, r = 0.614). Significant negative correlations were observed between PRL-fovea distance and MRS (P < 0.001, r = -0.519) and ACC (P < 0.001, r = -0.545). CONCLUSIONS: This study provides evidence for differences in the reading performance of Persian-speaking patients with AMD in different PRL locations. The average scores of all reading indices obtained in the right-field PRL are lower than those in other areas and are highly correlated with the PRL-fovea distance.