Correlating the patterns of diabetic macular edema, optical coherence tomography biomarkers and grade of diabetic retinopathy with stage of renal disease.

PURPOSE: To correlate optical coherence tomography (OCT)-based morphological patterns of diabetic macular edema (DME), biomarkers and grade of diabetic retinopathy (DR) in patients with various stages of chronic kidney disease (CKD) secondary to diabetes. DESIGN: Multicentric retrospective cross-sectional study was conducted at seven centers across India. METHODS: Data from medical records of patients with DME and CKD were entered in a common excel sheet across all seven centers. Staging of CKD was based on estimated glomerular filtration rate (eGFR). RESULTS: The most common morphological pattern of DME was cystoid pattern (42%) followed by the mixed pattern (31%). The proportion of different morphological patterns did not significantly vary across various CKD stages (p = 0.836). The presence of external limiting membrane-ellipsoid zone (ELM-EZ) defects (p < 0.001) and foveal sub-field thickness (p = 0.024) showed a direct correlation with the stage of CKD which was statistically significant. The presence of hyperreflective dots (HRD) and disorganization of inner retinal layers (DRIL) showed no significant correlation with the stage of CKD. Sight threatening DR was found to increase from 70% in CKD stage 3 to 82% in stages 4 and 5 of CKD, and this was statistically significant (p = 0.03). CONCLUSION: Cystoid morphological pattern followed by mixed type was the most common pattern of DME on OCT found in patients suffering from stage 3 to 5 of CKD. However, the morphological patterns of DME did not significantly vary across various CKD stages. ELM-EZ defects may be considered as an important OCT biomarker for advanced stage of CKD.

Need for Vitreous Surgeries in Proliferative Diabetic Retinopathy in 10-Year Follow-Up: India Retinal Disease Study Group Report No. 2.

INTRODUCTION: To report the 10-year rate of vitrectomies and the associated factors in people with proliferative diabetic retinopathy (PDR) from a multicentric cohort of people with diabetes mellitus. METHODS: Ten centres in India with established vitreoretinal (VR) services for over 10 years were invited to provide long-term data on PDR. People with Type 1 or 2 diabetes with a clinical diagnosis of active PDR in 1 or both eyes were included. Baseline data collected included age, sex, duration of diabetes, source of referral and best-corrected visual acuity, and diabetic retinopathy status in both eyes. Available follow-up data included the numbers of panretinal photocoagulation (PRP) sessions, cataract surgery, treatment of diabetic macular oedema, use of anti-vascular endothelial growth factor (VEGF) therapy, vitrectomy with or without retinal surgeries over 10 years. RESULTS: Over 10 years, 89% needed supplemental PRP after initial complete PRP. One-third required retinal surgery, 16% needed intravitreal injection. Men (74.5%) had significant higher risk for vitreous (VR) surgery. Of the group with low-risk PDR, 56.8% did not require VR surgery, p < 0.001. Of the patients who underwent cataract surgery and had intravitreal anti-VEGF injections, 78.5 and 28.2% needed subsequent vitreous (VR) surgery, p = 0.006 and <0.0001, respectively. Independent predictors of need for vitreoretinal surgery included those who underwent cataract surgery and those with poor baseline visual acuity (logMAR). Eyes at lower risk for VR surgery included the eyes previously treated with PRP and low-risk PDR at baseline. CONCLUSION: Despite initial "complete" PRP, one-third of our study cohort needed vitrectomies over 10 years, highlighting that these patients require regular follow-up for a long period of time.

Pilomatrix carcinoma of the eyebrow.

Pilomatrix carcinoma is an uncommon malignant neoplasm originating from the hair follicle matrix. It occurs more frequently in head and neck region and rarely on the eyelid and eyebrow. It is often clinically misdiagnosed. The diagnosis is confirmed by excision biopsy and histopathological examination. A 68-year-old woman presented with a mass on her left eyebrow mimicking sebaceous cyst. An excisional biopsy with 4-mm margin was performed under frozen section. Histopathology demonstrated pilomatrix carcinoma with cystic degeneration. Periocular malignancies can have unusual origins with specific clinical features. Preoperative clinical examination is mandatory to rule out unusual features and thereby ensure appropriate management to prevent serious complications.

Deep learning-based image quality assessment for optical coherence tomography macular scans: a multicentre study.

AIMS: To develop and externally test deep learning (DL) models for assessing the image quality of three-dimensional (3D) macular scans from Cirrus and Spectralis optical coherence tomography devices. METHODS: We retrospectively collected two data sets including 2277 Cirrus 3D scans and 1557 Spectralis 3D scans, respectively, for training (70%), fine-tuning (10%) and internal validation (20%) from electronic medical and research records at The Chinese University of Hong Kong Eye Centre and the Hong Kong Eye Hospital. Scans with various eye diseases (eg, diabetic macular oedema, age-related macular degeneration, polypoidal choroidal vasculopathy and pathological myopia), and scans of normal eyes from adults and children were included. Two graders labelled each 3D scan as gradable or ungradable, according to standardised criteria. We used a 3D version of the residual network (ResNet)-18 for Cirrus 3D scans and a multiple-instance learning pipline with ResNet-18 for Spectralis 3D scans. Two deep learning (DL) models were further tested via three unseen Cirrus data sets from Singapore and five unseen Spectralis data sets from India, Australia and Hong Kong, respectively. RESULTS: In the internal validation, the models achieved the area under curves (AUCs) of 0.930 (0.885-0.976) and 0.906 (0.863-0.948) for assessing the Cirrus 3D scans and Spectralis 3D scans, respectively. In the external testing, the models showed robust performance with AUCs ranging from 0.832 (0.730-0.934) to 0.930 (0.906-0.953) and 0.891 (0.836-0.945) to 0.962 (0.918-1.000), respectively. CONCLUSIONS: Our models could be used for filtering out ungradable 3D scans and further incorporated with a disease-detection DL model, allowing a fully automated eye disease detection workflow.

Periocular dirofilariasis mimicking lacrimal sac mucocoele.

Dirofilariasis is a zoonotic infection caused by filarial nematodes belonging to the genus dirofilariae. Dirofilaria is commonly seen in dogs, cats and other carnivorous animals world wide. Mosquitoes of the genus Culex, Anopheles and Aedes are the vectors and the humans are either incidental hosts or dead-end hosts. It affects lungs, liver and other visceral organs. Ocular involvement is rarely been reported. We present a case of 51-year-old female from Kerala, the southern State of India presented with a mass mimicking lacrimal sac mucocoele whose biopsy is proved to be dirofilariasis.

Impact of online classes on eye health of children and young adults in the setting of COVID-19 pandemic: A hospital-based survey.

BACKGROUND: To analyze the impact of online classes on eye health of children and young adults during the COVID-19 pandemic. MATERIALS AND METHODS: An observational study with a written questionnaire and comprehensive ophthalmic evaluation at a tertiary eye care center in South India, during the COVID-19 pandemic. RESULTS: Of the 496 patients, most were 5-10 years old, attending online classes 1-2 h/day with majority (84.7%) having <4 h of classes. Electronic gadget use after classes was seen in 95.6% participants and 28.6% admitted to using it for more than 2 h/day. Digital eye strain (DES) was seen in 50.8% of patients of which headache or eye ache were the most common symptom (30.8%). Duration of online class was found to be the single most independent factor associated with the development of eye complaints (P = 0.001). Duration of class hours (P = 0.007) and light setting (P = 0.008) was found to be independent determinants of developing DES. CONCLUSIONS: Increased screen time, inadequate light setting, and excessive application of near vision can produce undesirable effects including the development of DES, worsening or development of new refractive errors and squint.

Post-infectious Non-paraneoplastic Autoimmune Retinopathy in Paediatric age group: A high index of suspicion, Multimodal Imaging and Early treatment can restore vision.

PURPOSE: To report a case of non-paraneoplastic Auto-immune retinopathy (npAIR) in a paediatric patient who showed excellent visual recovery with early diagnosis and prompt treatment. METHODS: Retrospective Case report. RESULTS: A five year old girl presented to us with bilateral profound vision loss of sub-acute onset following an episode of high fever, without any previous visual abnormality. A diagnosis of npAIR was made based on history, clinical findings and multimodal imaging. Intravenous methylprednisolone was started urgently followed by oral steroid. Visual acuity showed good improvement along with gradual restoration of anatomy of retinal layers in Optical Coherence Tomography (OCT) over a period of three months. CONCLUSION: Our case highlights the importance of suspecting npAIR in paediatric patients presenting with sudden bilateral painless progressive loss of vision without prior visual difficulties and the role of multimodal imaging to aid in diagnosis. The recovery of vision with restoration of photoreceptor layer also shows the nature of the disease to recover with early intervention despite a negative anti-retinal antibody test but with features highly suggestive of npAIR.

Longitudinal follow-up and outcome analysis in central serous chorioretinopathy.

OBJECTIVES: To analyse the longitudinal changes in visual acuity and risk factors for recurrence or development of choroidal neovascularisation (CNV) in eyes with acute or chronic central serous chorioretinopathy (CSCR). METHODS: This was a retrospective, multicentric, longitudinal, observational study done in patients with a diagnosis of unilateral or bilateral CSCR and having at least 4 years of follow-up between the years 1999 and 2020. Kaplan-Meier curves were used for assessing cumulative risks. Multivariate logistic, linear and cox regression models were used for risk factor analyses. The trend in visual acuity, cumulative risks of recurrence and CNV formation was analysed. RESULTS: A total of 117 out of 175 eyes (66.8%) had stable or improvement in vision at last follow-up, while 24 eyes had more than/equal to 3 line loss of vision. Four eyes (7.7%) with acute CSCR at initial presentation developed features of chronic CSCR at the final presentation. Thirty-seven eyes had recurrence during the follow-up with a 10-year cumulative recurrence rate of around 30%. On Cox proportional hazard regression analysis, history of previous treatment and male gender (p = 0.03) were associated with a lower risk of recurrence. Twenty-four developed de novo CNV by the end of follow-up and higher age (p = 0.001) and a higher number of recurrences (p = 0.05) were associated with a higher risk of early de novo CNV formation. The cumulative 10-year CNV development rate was 17.4%. CONCLUSION: A non-temporal relationship between acute and chronic CSCR was seen. Previous treatment, smoking and baseline RPE abnormality affected recurrence of SRF or CNV formation.

Acute acquired comitant esotropia precipitated by excessive near work during the COVID-19-induced home confinement.

Purpose: To evaluate the causes of acute acquired comitant esotropia (AACE) in young adults and children in the setting of COVID-19-induced home confinement. Methods: A retrospective, clinical study of all patients, who presented to the Pediatric Ophthalmology and Strabismus services of a tertiary eye care center in South India from August 2020 to January 2021 during the COVID-19 pandemic, with acute-onset, comitant esotropia. Results: 11 (73.3%) of the total 15 patients were students, above 10 years and with a mean age of 16.8 years. 12 patients (80%) had more than 8 hours of near activity a day with a mean duration of 8.6 hours per day. The most common near activity was online classes, followed by job-related work and mobile games, and 86.7% used smartphones for near work. The average esotropia was 22.73 prism diopter (PD) for distance and 18.73 PD for near. Majority (66.6%) had hyperopia with basic or divergence insufficiency esotropia, and the remaining 33.3% had myopia and fitted in to the Bielschowsky type AACE. There was no precipitating event other than sustained near work in all, except in one patient who also had fever prior to the onset of esotropia. Conclusion: The habit of long-time and sustained near work, especially on smartphones, may increase the risk of inducement of AACE.

Diagnostic ability of superficial vascular density measured by optical coherence tomography angiography to differentiate high myopic eyes from eyes with primary open angle glaucoma.

Purpose: To determine if high myopia and glaucoma can be differentiated based on the measurement of superficial vascular density in the peripapillary and macular areas by using Optical Coherence Tomography Angiography (OCTA). Methods: This prospective, observational, cross-sectional, comparative study was conducted on patients between 40 and 60 years of age diagnosed as primary open-angle glaucoma (POAG) or high myopia and compared with age-matched controls. The main outcome measures were the difference in SVD% in peripapillary and macular areas in POAG and high myopic eyes. Detailed ophthalmic examination and OCTA of the disc and peripapillary area and macula were performed. The SVD in each zone was calculated using ImageJ software and their difference were analyzed. Results: In total, 128 eyes of 70 patients were enrolled. Peripapillary SVD% in controls was 45.07 ± 3.44, 40.36 ± 8.27 in high myopia, and 31.80 ± 9.008 in POAG. The mean difference in peripapillary SVD% of POAG to control was - 13.479, of POAG to high myopia was -8.777 (both P < 0.001), and of high myopia to controls was -4.701 (P = 0.012). Macular SVD% in controls was 27.30 ± 3.438, 22.33 ± 6.011 in high myopia, and 21.21 ± 5.598 in POAG. The mean difference in macular SVD% of POAG to controls was -6.088, of high myopia to controls was -4.965 (both P < 0.001), and of POAG to high myopia was -1.122 (P = 0.984; not statistically significant). Conclusion: OCTA is a useful diagnostic tool in distinguishing glaucomatous and myopic eyes. The measurement of peripapillary SVD has a greater discriminatory ability than that of the macular area.

Updated guidelines for the management of polypoidal choroidal vasculopathy: Recommendations from the Indian Polypoidal Choroidal Vasculopathy Panel and the Vitreoretinal Society of India.

In Asians, polypoidal choroidal vasculopathy (PCV) is becoming more widely recognized as a significant cause of exudative maculopathy. The previous set of Indian guidelines on the management of PCV were published in 2018, with a literature search updated up to November 2015. As the treatment of PCV evolves, retinal physicians must constantly modify their current practice. The current guidelines are based on the most up-to-date information on PCV and are an update to the previous set of guidelines. These guidelines were developed by a panel of Indian retinal experts under the aegis of the Vitreoretinal Society of India (VRSI), based on a comprehensive search and assessment of literature up to September 2021. The final guidelines i) provide the updated nomenclature in PCV; ii) discusses the newer diagnostic imaging features of PCV, especially in the absence of indocyanine green angiography (ICGA); and iii) recommends the best possible therapeutic approach in the management of PCV, including the choice of anti-vascular endothelial growth factor (anti-VEGF) agents, treatment regimen, and the role of switching between the anti-VEGF agents. In the face of non-availability of photodynamic therapy (PDT) in India, we constructed practical recommendations on anti-VEGF monotherapy in PCV. The current updated recommendations would provide a broader framework to the treating retinal physician for the diagnosis and management of PCV for optimal therapeutic outcomes.

Carcinoma -associated Retinopathy(CAR): Role of Electroretinography(ERG) and Optical coherence Tomography(OCT) in diagnosis and predicting treatment outcome.

PURPOSE: Cancer-associated retinopathy (CAR) is a potentially blinding condition that can be stalled or,rarely, reversed if diagnosed early. This case report aims to highlight the role of Electroretinography (ERG) and Optical Coherence Tomography (OCT) in diagnosis and predicting treatment response, in the clinical scenario of unexplained vision loss in a patient with a recent diagnosis of uterine malignancy. OBSERVATIONS: A 70 year old lady, recently diagnosed with Endometrial carcinoma Stage 3, came to us with defective vision and photopsiae of both eyes of ten days duration. A high index of suspicion, flat ERG, OCT changes, absence of metastasis, and positive Antirecoverin antibody clinched the diagnosis. Early systemic steroid therapy along with surgical removal of the tumour, adjuvant radiotherapy and chemotherapy helped in visual improvement in our patient. CONCLUSIONS: Presence of intact ellipsoid zone (EZ) and external limiting membrane (ELM) in pre-treatment OCT was found to be a positive predictor of visual recovery.

Severity of diabetic retinopathy and its relationship with age at onset of diabetes mellitus in India: A multicentric study.

PURPOSE: To present clinical profile and risk factors of sight-threatening diabetic retinopathy (STDR) among people with age of onset of diabetes (AOD) <25 versus ≥25 years. METHODS: A retrospective chart analysis of consecutive patients with diabetic retinopathy (DR) n = 654) treated at 14 eye care centers across India between 2018 and 2019 was performed. Patients were divided into two groups, Group 1: AOD <25 years and Group 2: AOD ≥25 years. DR and diabetic macular edema (DME) were classified using the International Clinical Classification of DR severity scale. STDR included severe nonproliferative DR (NPDR), proliferative DR (PDR), and moderate to severe DME. A multilevel mixed-effects model was used for comparison between two groups: 1) Patients with DR and AOD <25 years and 2) Patients with DR and AOD ≥25 years. Bivariate and multivariate regression analyses were used to evaluate risk factors between the two groups. RESULTS: A total of 654 patients were included, 161 (307 eyes) in AOD <25 and 493 (927 eyes) in AOD >25 group. There was a higher prevalence of PDR with high-risk characteristics in AOD <25 group (24% vs. 12%) at baseline and 12-month follow-up (25% vs. 6%); P < 0.001. Systolic hypertension and poor glycemic control were risk factors in both groups, with no difference in these modifiable risk factors between groups. CONCLUSION: People with youth-onset DM are likely to present with severer form of STDR despite similar modifiable risk factors. Therefore, strict control of systolic blood pressure, glycemic status, and regular screening for DR are recommended to reduce the risk of STDR irrespective of the age of onset of diabetes.

Prevalence and incidence of visual impairment in patients with proliferative diabetic retinopathy in India.

To provide the real-world outcomes of people with proliferative diabetic retinopathy (PDR) in India and highlight opportunities for improvement of their disease status and to evaluate their visual acuity (VA) status. A multicenter retrospective study in which ten centers in India with established vitreoretinal services for over 10 years were invited to provide long-term data on PDR. This study population were of Indian nationality. Patients with a diagnosis of type 1 or 2 diabetes with a clinical diagnosis of active PDR in any or both eyes, who had long term follow-up for up to 10 years were included. Baseline data collected included age, sex, duration of diabetes, source of referral and best-corrected visual acuity, diabetic retinopathy status in both eyes. Available follow-up data on VA were collected at 6 months post baseline, 5 years and 10 years within a ± 3 months window. Evaluating the presenting VA of people with PDR, short-term outcomes at 6 months and the incidence of visual impairment (VI) at 5 and 10 years are the main outcome of the study. Data was available for 516, 424 and 455 patients at baseline, 5 years and 10 years respectively. Gender and duration of diabetes did not have statistically significant effect on VI outcomes. Eyes receiving treatment early in the disease course (i.e. baseline VA ≥ 6/12) had significantly better VA outcomes at 10 years versus eyes treated at a later stage (i.e. baseline VA < 6/12) (p = <0.0001). On comparing eyes with stable treated PDR and persistent PDR at end of 10 year follow up, a significantly higher percentage of eyes in the stable treated group maintained VA of ≥ 6/12 (55.1% vs. 24.2%) (p = < 0.0001), indicating persistent disease activity due to inadequate treatment results in worse VA outcomes. We found no trend in VI or blindness with increasing levels of age at both 5- and 10-year time points (p > 0.05). The age standardized incidence for VI was 11.10% (95% CI 8.1, 14.2) and for blindness was found to be 7.7% (95% CI 5.2, 10.3). Our results suggest that despite robust recent clinical trial results showing that pan retinal photocoagulation is an excellent treatment for PDR, people with diabetes in India need to be made aware of annual screening and treatment of their eyes to avoid vision impairment and blindness.

Spontaneous formation and closure of full thickness macular hole after treatment with anti-vascular endothelial growth factor therapy in polypoidal choroidal vasculopathy.

Full-thickness macular hole (FTMH) formation in Polypoidal choroidal vasculopathy (PCV) after intravitreal anti-vascular endothelial growth factor (anti-VEGF) treatment is a rare complication. Spontaneous closure of FTMH following anti-VEGF therapy has not been described in PCV till date. We present a case of Asian woman with PCV who developed a FTMH following treatment with intra-vitreal anti-VEGF injections which subsequently closed spontaneously on further course of treatment.

Focal choroidal excavation: Cause or effect?

We report the imaging characteristics of focal choroidal excavation (FCE) and associated choroidal neovascular membrane (CNVM) and interpret the probable etiopathogenesis of FCE through findings in four patients detected by spectral-domain optical coherence tomography (SD-OCT). FCE was found as an acquired entity in two of our cases subsequent to treatment of CNVM, whereas in the two other cases FCE was pre-existing. Furthermore, association of FCE with pachychoroid spectrum is reaffirmed through this case series.

Smartphone guided wide-field imaging for retinopathy of prematurity in neonatal intensive care unit - a Smart ROP (SROP) initiative.

Purpose: To suggest a low cost, non-contact smartphone-based screening system in retinopathy of prematurity (ROP), and to illustrate its potential clinical application as a potential future tool for teleophthalmology. Methods: Neonatal intensive care unit (NICU)-based bedside ROP screening done between January 2018 and May 2018. Documentation of ROP was done by using a smartphone and +40D, +28D, or +20D indirect non-contact condensing lenses. By using the coaxial light source of the phone, this system works as an indirect ophthalmoscope that creates a digital image of the fundus. With smartphone-based camera we extracted high-quality still images extracted from the video clip. Results: Total of 228 eyes of 114 infants screened for ROP between January 2018 and May 2018. Incidence of total ROP was 23.68%, out of which incidence of type 1 ROP was 8.77%. After initial screening with indirect ophthalmoscope, we uesd smartphone imaging to document ROP in 28 eyes out of 55 eyes having ROP. Image quality was good in 89.28% eyes. Field of view vary from 46°, 53°, and 90° with +20D, +28D, and +40D indirect condensing lenses, respectively, which gives excellent images for bedside ROP documentation. Conclusion: The described technique of smartphone fundus photography is a light weight, cost-effective, user friendly, high-quality wide-field fundus photographs for bedside documentation of ROP in NICUs using readily available instruments that are handy and portable with simple power sources. Smartphones has the potential to be operated with only one hand. It can also be used as a future telescreening device.

Long-term efficacy and safety of verteporfin photodynamic therapy in combination with anti-vascular endothelial growth factor for polypoidal choroidal vasculopathy.

Purpose: The aim of the study was to analyze the outcomes of photodynamic therapy (PDT) with intravitreal anti-vascular endothelial growth factor (anti-VEGF) for patients with polypoidal choroidal vasculopathy (PCV) having visual acuity (VA) better than 20/60 in a real-world scenario in India. Methods: Retrospective review of 42 eyes of 40 patients (mean age 64.3 years) with best-corrected VA (BCVA) 20/60 or better and mean follow-up of 40 months (median 38 months; range 12-71 months) treated with PDT and anti-VEGF or triamcinolone for indocyanine green angiography (ICGA)-proven subfoveal PCV. Results: : Mean BCVA improved from 0.22 logMAR at baseline to 0.15 at last visit (P < 0.001). On ICGA, polyp was observed in 42 eyes (100%) and branching vascular network (BVN) in 37 eyes (88.1%). Polyp regressed in 33 (78.6%) of 42 eyes and BVN in 26 (70.3%) of 37 eyes after combined therapy at 3 months. Mean greatest linear diameter reduced significantly (P < 0.001) from 7.22 mm to 4.11 mm. Standard-fluence PDT was performed in 35 eyes and reduced-fluence in 7 eyes. The mean number of PDT was 1.17 with mean number of injections being 6.38 at the end of follow-up. In five eyes, more than one PDT was administered. Of 42 eyes, 40 showed complete resolution of serous macular detachment (SMD) after the combined therapy at 3 months; 17 (42.5%) of the 40 eyes showed no recurrence of fluid on spectral domain optical coherence tomography till the last visit with a mean follow-up of 27 months. On long-term follow-up, SMD reoccurred in 23 eyes with a mean follow-up period of 9.64 ± 5.24 months. Of 38 eyes having a double-layer sign (DLS) on optical coherence tomography at baseline, 37 eyes were having regression of the DLS, that is, it either reduced or resolved at the final visit. At the final visit, 66.7% (P < 0.001) eyes were having fluid-free retina. No complication of subretinal hemorrhage was noted. Of the 42 eyes, only one eye had BCVA worse than 20/60 on the final visit. Conclusion: To the best of our knowledge, this is the first study to look into the long-term effect of combined PDT with anti-VEGF in PCV in eyes having good VA. Long-term effect of combined PDT appears to be a safe and effective treatment for PCV in eyes having good VA with better outcomes in real-world setting. This study further strengthens the superiority of the combined treatment modality for treatment of subfoveal PCV with no or minimal risk of complication on long-term follow-up.

"Double-layer sign" on spectral domain optical coherence tomography in pachychoroid spectrum disease.

PURPOSE: The "double-layer sign (DLS)" describes the shallow and irregular elevation of the retinal pigment epithelium from the underlying intact Bruch's membrane visualized on the spectral domain optical coherence tomography. In this study, we evaluated the frequency, characteristics of the space within the double layer and other features in the pachychoroid spectrum to aid the clinical diagnosis of these variants. METHODS: This retrospective study evaluated the features of the DLS on multimodal imaging in consecutive patients with a clinical diagnosis of one of the four variants of pachychoroid: pachychoroid pigment epitheliopathy (PPE), pachychoroid neovasculopathy (PCN), chronic central serous chorioretinopathy (CCSCR), and polypoidal choroidal vasculopathy (PCV). The features of the DLS were graded by two masked graders. RESULTS: Overall, 102 eyes of 79 consecutive patients with pachychoroid spectrum were identified for grading. Sixteen eyes with PPE did not show any evidence of DLS. The DLS was identified in 15/16 (93.75%) eyes with PCN, 11/35 (31.43%) with CCSCR, and 32/35 (91.43%) with PCV (P < 0.001). The space within the DLS showed moderate hyperreflectivity in all eyes with PCV and PCN, while the space in the DLS in CCSCR showed uniform hyporeflectivity in 10/11 (%) eyes. CONCLUSION: The DLS sign was most frequent in polypoidal vasculopathy and PCN. A hyporeflective gap within the DLS favored the diagnosis of CCSCR.

Rosai-Dorfman disease with isolated lacrimal gland enlargement.

Rosai-Dorfman Disease also called as Sinus Histiocytosis with Massive Lymphadenopathy is a rare, benign, idiopathic histiocytic proliferative disorder that occurs predominantly in children and young adults. Orbital involvement can occur in 11% of cases. Isolated lacrimal gland involvement without any local or systemic recurrence is very rare. To the best of our knowledge, only seven cases have been reported in the literature till date. Histopathological and immunohistochemical confirmation is essential in establishing the diagnosis. A benign course is usual, but in some cases, blindness or even fatality may result. We report a case of 30-year-old female with isolated lacrimal gland involvement and 19 months' follow-up.