Multimodal imaging of epiretinal neovascularization associated with type 3 macular neovascularization.

PURPOSE: To report a case of type 3 macular neovascularization presenting with an epiretinal neovascularization. METHODS: Case report. RESULTS: A 65-year-old gentleman presented with complaints of reduced vision in the left eye (LE) having a best corrected visual acuity (BCVA) of 20/200. Based on fundus examination and multimodal imaging findings he was diagnosed with type 3 macular neovascularization (MNV). An additional unusual finding was the presence of an epiretinal membrane (ERM) and epiretinal neovascularization (ERN), with the latter finding being confirmed on optical coherence tomography angiography (OCTA). He underwent three doses of intravitreal ranibizumab injection following which there was reduction in the epiretinal vascularity. CONCLUSIONS: Multimodal imaging of this case highlights the presence of epiretinal neovascularization associated with type 3 MNV, which has not been reported previously.

Natural course of non-center-involving diabetic macular edema progression in patients under initial observation.

PURPOSE: We aim to report the natural course of non-center involving diabetic macular edema (NCIDME) progression to center involving diabetic macular edema (CIDME) and associated risk factors. METHODS: This is a multicenter retrospective comparative study. Data was collected from electronic medical records from 8 centers in India covering. We included patients with type 2 diabetes above 18 years of age with treatment-naïve NCIDME on OCT and best-corrected visual acuity at baseline of 6/12 or better who were under observation for NCIDME and had 2 years follow-up data. RESULTS: Out of 72 patients with NCIDME, 26.38% patients progressed to CI DME by 2 years, and the visit wise proportion was 11.11% at 6 months, 7% at 1st year and 8.3% at 2 years. The change in CST was statistically significant at 2 years in patients who developed CIDME, the mean difference was 137.73 ± 48.56 microns p = 0.045. Duration of diabetes mellitus > 10 years was the only risk factor for conversion to CIDME. CONCLUSION: A quarter of eyes with NCIDME developed CIDME and 15% progressed from NPDR to PDR by 2 years, highlighting the disease burden in these patients with NCIDME.

Late postoperative vitreous cavity hemorrhage after vitrectomy for proliferative diabetic retinopathy-observation versus intervention.

PURPOSE: To analyze the outcome of intervention versus observation for vitreous cavity hemorrhage occurring after a 2-month period of blood-free cavity (late postoperative vitreous cavity hemorrhage-POVCH) in eyes operated by vitrectomy for complications of proliferative diabetic retinopathy (PDR). METHODS: This study was a 10-year retrospective, observational, multi-center study involving eight major vitreoretinal surgical centers across India from January 2010 to December 2019. The primary objective of the study was to assess the visual and clinical outcomes of various management approaches for late POVCH. The key secondary objective was to determine the best management option that prevented recurrence. Patients with follow-up of less than 6 months of POVCH management were excluded. RESULTS: The occurrence of late POVCH was studied in 261 eyes. The median time to occurrence was 7 months (range: 2-87) postvitrectomy/silicone oil removal. The majority (58%) experienced a single, nonrecurring POVCH event. Visual acuity outcome was independent of all management approaches (P = 0.179; mean follow-up 20.7 ± 14.1 months). With watchful observation, spontaneous resolution was noted in 83% (60/72 eyes) of eyes in 81.5 days (interquartile range, 169.75). Silicone oil injection was most effective in preventing recurrence (P < 0.001). CONCLUSION: The current treatment practice of late POVCH management in PDR suggests that watchful observation for at least 3 months could be as efficacious as any surgical intervention.

POLYPOIDAL CHOROIDAL VASCULOPATHY ASSOCIATED WITH SECTOR RETINITIS PIGMENTOSA.

PURPOSE: To report a case of polypoidal choroidal vasculopathy associated with sector retinitis pigmentosa. METHODS: Case report. RESULTS: A 63-years-old woman presented with complaints of having painless progressive reduction of vision in the left eye (LE) for the past 6 months. On examination, her best-corrected visual acuity was 20/20 in the right eye and 20/125 in the LE. Based on fundus examination and multimodal imaging findings, both eyes were diagnosed to have sector retinitis pigmentosa, and an associated active extramacular polypoidal choroidal vasculopathy was seen in the LE. Spectral-domain optical coherence tomography also revealed choroidal thinning in both eyes. Patient underwent bevacizumab injection in the LE. At 1-month posttreatment, her best-corrected visual acuity remained stable in the LE, and spectral-domain optical coherence tomography showed reduction in subretinal fluid and size of the polypoidal lesion. CONCLUSION: Polypoidal choroidal vasculopathy, a pachychoroid disease, could occur in association with retinitis pigmentosa, in the setting of thin choroids, and multimodal imaging is important to differentiate it from Type-1 macular neovascularization.

Co occurrance of Macular Telangiectasia type 2 with hemorrhagic polypoidal choroidal vasculopathy and response to anti-vascular endothelial growth factor.

We report the case of a 57-year-old female patient who presented with defective vision in the right eye due to large hemorrhagic pigment epithelial detachment with subretinal hemorrhage near the arcades with scattered retinal pigment epithelium (RPE) alteration at the macula and minimal vitreous hemorrhage inferiorly. The left eye showed right angle perifoveal dipping venule with RPE alteration temporal to the fovea, intraretinal RPE stellate plaque at the macula area. Multimodal imaging features including color fundus photo, red-free photograph, optical coherence tomography (OCT), enhanced depth imaging OCT, fundus fluorescein angiography, indocyanine green angiography, and OCT angiography were studied along with treatment response. One eye showed features of macular telangiectasia Type 2 (MacTel 2) with polypoidal choroidal vasculopathy (PCV) (hemorrhagic type) and the other eye showed features of MacTel 2 with pachychoroid pigment epitheliopathy. This report highlights the hitherto undescribed co-occurrence of MacTel 2 with bilateral pachychoroid and with unilateral hemorrhagic PCV in one eye and its favorable outcome with anti-vascular endothelial growth factor therapy.

Full thickness macular hole with subfoveal pigment epithelial detachment in chronic central serous chorioretinopathy: A therapeutic challenge.

We describe the diagnostic and therapeutic strategies employed in the management of a patient with subfoveal pigment epithelial detachments (PEDs) combined with full-thickness macular hole (FTMH) and discuss the possible pathophysiology of these diseases occurring concurrently. A 38-year-old patient with a history of central serous chorioretinopathy (CSC) presented with FTMH overlying a large subfoveal serous PED. Multimodal imaging confirmed the same and intravitreal anti-vascular endothelial growth factor (VEGF) injections and eplerenone failed to resolve the PED. Spontaneous resolution of the large PED was observed later and pars plana vitrectomy with internal limiting membrane peeling closed the macular hole successfully. However, the PED with fibrinous CSC recurred postoperatively and low fluence photodynamic therapy (PDT) was done to tackle the same. At 10 months' follow-up, the final vision was 6/9, the macular hole remained closed, and the PED had not recurred. Macular hole formation may be the result of choroidal hyperpermeability and leakage in the backdrop of CSC which gives rise to an exudative component causing mechanical stretching and disruption of the overlying sensory retina. Spontaneous uncomplicated resolution of large subfoveal PED in CSCR is rare. This case was managed with a combination of intravitreal anti-VEGF injections, surgery, and PDT. The therapeutic challenge here was the timing of surgery.

PACHYCHOROID DISEASE ASSOCIATED WITH GEOGRAPHIC ATROPHY AND SUBRETINAL DRUSENOID DEPOSITS.

PURPOSE: To report an atypical case of pachychoroid spectrum disease associated with subretinal drusenoid deposits and geographic atrophy. METHODS: Case report. RESULTS: A 67-year-old woman presented for a routine examination. Her best-corrected visual acuity was 20/20 in both eyes. Based on fundus examination and multimodal imaging findings, the presence of subretinal drusenoid deposits and geographic atrophy was seen in the right eye, which was associated with pachychoroid. The left eye revealed two active polypoidal lesions suggestive of polypoidal choroidal vasculopathy with subretinal drusenoid deposits, geographic atrophy, and a thick choroid with dilated outer choroidal vessels. She underwent an intravitreal injection of ranibizumab followed by focal laser to the polypoidal lesion in the left eye. At 3-month posttreatment, her best-corrected visual acuity was maintained at 20/20 in the left eye, with a good anatomical outcome. CONCLUSION: This case highlights a previously unreported unique association of a pachychoroid disease spectrum with geographic atrophy and with subretinal drusenoid deposits.

Healing pattern of subfoveal retinal pigment epithelium rip and aperture in central serous chorioretinopathy.

This report describes a rare case of spontaneous Grade-4 retinal pigment epithelium (RPE) rip of serous pigment epithelial detachment (PED) in central serous chorioretinopathy (CSC) and RPE aperture in the fellow eye, with favorable long-term outcomes. A 38-year-old man presented with defective vision (20/30) in the left eye (LE) due to bullous CSC associated with a large extramacular RPE rip located temporally and inferior exudative retinal detachment. Optical coherence tomography (OCT) confirmed a subfoveal serous PED with RPE aperture, subretinal fluid (SRF) and fibrinous exudation, and a large extramacular RPE rip temporally. The right eye (RE) had an asymptomatic large serous PED. The LE was treated with low-fluence photodynamic therapy, which resulted in the closure of RPE aperture and complete resolution of PED and SRF. Six-month later, the patient presented with sudden defective vision (20/120) in the RE secondary to a large fovea-involving (Grade-4) RPE rip with SRF as confirmed on OCT. Fluorescein angiography showed two extrafoveal active point leaks, which were treated with focal photocoagulation. He was also started on oral eplerenone. On subsequent serial follow-ups over 1 year, OCT showed SRF resolution and patchy reorganization of the subfoveal RPE-photoreceptor complex with good visual outcome (20/30).

Polypoidal Choroidal Vasculopathy: An Update on Diagnosis and Treatment.

Polypoidal choroidal vasculopathy (PCV) is a vascular disease of the choroid that leads to hemorrhagic and exudative macular degeneration. It may cause significant vision loss and thus affect the quality-of-life and psychological well-being. Non-invasive, non-ICGA-based OCT criteria have shown reliable results to plan adjunct photodynamic therapy (PDT) treatment, with the complete and consistent coverage of polypoidal lesions (PL) and branching neovascular network (BNN). The safety and efficacy of anti-vascular endothelial growth factor (anti-VEGF) monotherapy and its combination with verteporfin PDT have been established. However, treatment is still challenging due to frequent follow-ups, non-availability of PDT, and need for multiple anti-VEGF injection visits that increase the treatment burden and lead to patients being lost to follow-up. Effective treatments that prolong intervals between injections while maintaining vision and anatomical gains remain a critical unmet need. Longer acting molecules, like brolucizumab, have shown non-inferiority in BCVA gains and superior anatomical outcomes compared to other anti-VEGF agents. Newer therapies in the pipeline to enhance the efficacy and longevity of treatment include Faricimab and a port delivery system (PDS). This review summarizes the most recent diagnostic and treatment approaches in PCV to offer better treatment avenues.

Role of oral acetazolamide in refractory glaucomatous deep cup-related maculopathy.

A 39-year-old man, a known case of primary open-angle glaucoma on treatment, presented with defective central vision in the left eye. On examination, his intraocular pressure (IOP) was 26 mmHg in the right eye and 30 mmHg in the left eye with best-corrected visual acuity of 6/12 in each eye. Fundus examination showed glaucomatous optic neuropathy in both eyes and macular thickening in the left eye. Optical coherence tomography of the left eye showed macular detachment with peripapillary retinoschisis and a hyporeflective tract connecting schitic retina and the deep cup in the absence of an optic disc pit. A diagnosis of glaucomatous deep cup maculopathy (DCM) was made in the left eye, which persisted despite well-controlled IOP and peripapillary laser photocoagulation. The addition of oral acetazolamide (250 mg twice daily) to his regimen resulted in prompt resolution of maculopathy. Glaucomatous DCM is relatively rare, and its primary management is adequate IOP control with antiglaucoma medications. Including oral acetazolamide in the antiglaucoma regimen can help in faster resolution of maculopathy due to its additional effect on retinal pigment epithelial pump induction and stabilization of the pressure gradient.

Multimodal imaging of Unilateral Idiopathic Macular Telangiectasia Type 2 - A five-year study.

PURPOSE: To report a rare presentation of unilateral macular telangiectasia type 2 (Mac-Tel 2) followed up over 5 years. METHODS: Case report. RESULTS: A 58-year-old gentleman presented to us with complaints of painless progressive reduction of vision in the right eye for the past two years. He had no systemic illnesses. On examination his best corrected visual acuity was 20/40 in the right eye and 20/20 in the left eye. Fundus examination in the right eye revealed loss of foveal reflex along with mild temporal greying of the fovea and the left eye was within normal limits. Both eyes showed the presence of peripapillary myelinated nerve fibres. Multimodal imaging findings included spectral domain optical coherence tomography (SD-OCT), optical coherence tomography angiography (OCTA), fundus autofluorescence and fundus fluorescein angiogram which revealed findings suggestive of exudative preproliferative variant of macular telangiectasia type 2 (Mac-Tel 2) in the right eye whereas the left eye was within normal limits. After a follow up of five years SD-OCT and OCTA revealed no striking differences in both eyes, with no evidence of the disease in his left eye. CONCLUSION: This reports highlights a rare advanced unilateral presentation of Mac-Tel 2 with no evidence of even early signs of the disease in the fellow eye, over a long term follow-up.

Potpourri of retinopathies in rare eye disease - A case series.

This case series describes the ocular and retinal manifestations of rare eye diseases in systemic syndromes. This observational case series consists of five patients with varied ophthalmic manifestations and documentation of imaging in rare pediatric and adult retinopathies. Two patients had Kearns Sayre syndrome (KSS) based on the classical triad of external ophthalmoplegia, pigmentary retinopathy, and onset before 20 years of age. In one patient of KSS, the mitochondrial retinopathy was seen in an asymmetric pattern, and the second patient presented with KSS after being mis-diagnosed as myasthenia gravis elsewhere. A case of Senior Loken syndrome in pediatric age is described in this series with varied ophthalmic manifestations ranging from retinitis pigmentosa to orbital abscess. This series also enlightens features of Hallervorden Spatz syndrome presenting with bull's eye maculopathy and a case of spino-cerebellar ataxia type 7 presenting with pigmentary retinopathy.

Photodynamic therapy for peripapillary pachychoroid syndrome-a case report.

A 76-year-old man presented with reduced visual acuity in both eyes, more severe in the right eye. His previous medical history included anti-vascular endothelial growth factor treatment, with multiple intravitreal injections in both eyes, and repeated focal laser in the left eye. His best-corrected visual acuity was 20/200 in the right eye and 20/40 in the left eye. Based on fundus examination and multimodal imaging, the patient was diagnosed with pachychoroid disease with peripapillary pachychoroid syndrome in the right eye and nasal macular scarring with late cystoid degeneration in the left eye. He underwent low-fluence photodynamic therapy in both eyes. At 6 months' follow-up, best-corrected visual acuity improved to 20/120 in the right eye and was maintained at 20/40 in the left eye, with good anatomical outcome.

Multimodal imaging including optical coherence tomography angiography of benign familial fleck retina.

A 44-year-old woman presented with complaints of pain in the right eye (RE). Fundus examination revealed disc edema in the RE along with retinal flecks sparing the macula in both eyes (BE). Fundus autofluorescence demonstrated a symmetrical pattern of white flecks in BE. Spectral-domain optical coherence tomography (SD-OCT) revealed the lesions at the level of retinal pigment epithelium with impingement onto the outer retina. SD-OCT angiography through the flecks revealed hyperreflective lesions at the level of avascular retina. RE B-scan revealed a T-sign. Based on these findings, she was diagnosed with BE benign familial fleck retina (BFFR) with RE posterior scleritis. We describe the multimodal imaging features in a middle-aged patient with BFFR and provide an insight into the probable pathogenesis.

Real world outcomes of sutureless and glueless sclerally fixated intraocular lens implantation.

PURPOSE: To report the outcomes of sutureless intrascleral fixation of a 3-piece intraocular lens in the ciliary sulcus, in a large cohort of patients with aphakia of various aetiology METHODS: Retrospective, non-comparative, single centre interventional study of 250 aphakic eyes of various causes, which underwent sutureless and glueless intrascleral fixation of 3-piece intraocular lens (IOL). All patients were required to have at least 3 months of follow up post procedure to be included in the study. Anatomical and functional outcomes obtained were statistically analysed for significance. RESULTS: A total of 250 eyes of 246 patients were included in the study population. The average age was 56.5 years ± 16.4 (range 6-86 years). The mean best-corrected visual acuity (BCVA) significantly improved from 0.74 ± 0.6 logMAR (approx. Snellen equivalent 20/110) to 0.48 ± 0.36 logMAR (approx. Snellen equivalent 20/60), (p < 0.001) following surgery. Early postoperative complication (<2 weeks) included hypotony (n = 10, 4%), ocular hypertension (n = 38,15.2%) and vitreous haemorrhage (n = 50, 20%). Late complications included retinal detachment (n = 14, 5.6%%), cystoid macular oedema (n = 24, 9.6%), scleral erosion (n = 1, 0.4%), haptic extrusion to subconjunctival space (n = 3, 1.2%) and IOL subluxation or dislocation (n = 5, 2%) CONCLUSION: This cost-effective and easier technique of sutureless scleral fixated 3-piece IOL implantation provided good visual acuity outcomes in a large cohort of patients and was well tolerated.

An extended phenotype of RP1L1 maculopathy - case report.

BACKGROUND: To report the ophthalmological findings of a new phenotypical variant of RP1L1 maculopathy in an Indian patient with a homozygous variant in the RP1L1 gene. MATERIALS AND METHODS: A 39-year-old male presented with complaints of disturbance in the central field of vision in both eyes (BE) for a duration of 6 months. He underwent ophthalmic examinations and diagnostic imaging. A complete retinal degeneration panel consisting of 228 genes was evaluated for pathologic variations using next-generation sequencing (NGS), which showed a variant in the RP1L1 gene. RESULTS: On fundus examination, he was found to have ill-defined foveal mottling in BE. Spectral domain optical coherence tomography (SD-OCT) showed sub-foveal hyper-reflective deposits and outer retinal layer disruption. A provisional diagnosis of the atypical variant of adult-onset foveomacular vitelliform dystrophy (AOFVD) was made on the basis of clinical, OCT, Fundus autofluorescence (FAF) and electrophysiological features. Genetic assessment of the proband revealed the presence of a homozygous base pair deletion in exon 4 of RP1L1 gene (chr8:g.10468194_10468195del), which results in frameshift and premature truncation of the protein 24 amino acids downstream to codon 1138 (p.Lys1138SerfsTer24). This variant was confirmed in the proband's parents by Sanger sequencing. The diagnosis was revised to RP1L1 maculopathy, as the RP1L1 gene variant is most commonly associated with this entity. CONCLUSION: This report presents the multimodal imaging of a previously unreported phenotype of RP1L1 maculopathy associated with a genetic variant of RP1L1 gene, thereby expanding the spectrum associated with RP1L1 maculopathy.

Multimodal imaging of pigmented paravenous retinochoroidal atrophy.

AIM: To describe the multimodal imaging findings of pigmented paravenous retinochoroidal atrophy. METHODS: A 23-year-old female presented to us for a routine ocular examination. She had a best-corrected visual acuity of 6/6 in both eyes. Anterior segment examination was unremarkable. Fundus examination showed pigmentary changes along the retinal vasculature extending from mid periphery to post-equatorial retina suggesting a diagnosis of pigmented paravenous retinochoroidal atrophy. Swept-source optical coherence tomography of the macula showed choriocapillaris thinning at the mid periphery whereas coherence tomography angiography at the mid periphery showed a relatively normal choriocapillaris vasculature in the early stage of the disease. CONCLUSION: A relatively normal choriocapillaris structure was seen on ocular coherence tomography angiography which could have been due to a milder form of the disease in a young patient.

ATYPICAL CENTRAL SEROUS CHORIORETINOPATHY WITH CHOROIDAL DETACHMENT: A CASE REPORT.

PURPOSE: We describe long-term follow-up of a patient with atypical chronic central serous chorioretinopathy with inferior bullous retinal detachment and 360° choroidal detachment, findings on multimodal imaging, differential diagnoses, and treatment. METHODS: Case report. RESULTS: A 66-year-old male patient, known case of bilateral chronic central serous chorioretinopathy on follow-up, presented to us with sudden painless worsening of vision in the right eye. The fundus examination, optical coherence tomography, and fundus fluorescein angiography showed an atypical chronic central serous chorioretinopathy with inferior bullous retinal detachment and peripheral choroidal detachment. The patient underwent half-fluence photodynamic therapy in the right eye. At 3 months post-photodynamic therapy, there was resolution of choroidal detachment and minimal subretinal fluid was persisting in the inferior periphery, with improvement in visual acuity. CONCLUSION: Posterior choroidal loculation of fluid is a described entity in central serous chorioretinopathy. The possible explanations for anterior choroidal loculation of fluid in this case are: either a fresh excessive leakage of fluid from the posterior choroid and that fluid may have traversed to the peripheral choroid, or diffuse choroidal leakage extending up to the equatorial region, which resulted in peripheral choroidal thickening and suprachoroidal fluid accumulation.