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).

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.

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.

Pediatric choroidal neovascularization: Etiology and treatment outcomes with anti-vascular endothelial growth factors.

PURPOSE: To describe the etiology and treatment outcomes of choroidal neovascularization (CNV) in a pediatric population with intravitreal anti-vascular endothelial growth factors (VEGF). METHODS: Retrospective single center interventional case series. A total of 26 eyes of 23 consecutive pediatric patients with CNV of various etiologies were treated with intravitreal injection of anti-VEGF agents. RESULTS: There were 15 males (65.2%) and eight females (34.8%), diagnosed with CNV during the study period. The mean age at presentation with CNV was 11.7 ± 3.3 years, (range 4-16 years) and the mean follow was 28.1 ± 18 months, (range 8-72 months). Inflammatory CNV was the most common etiology. The mean best corrected visual acuity (BCVA) and mean central macular thickness (CMT) at presentation, were logMAR 0.8 ± 0.3 and 367.6 ± 134.8 µm respectively. At the final visit, CNV in all eyes remained regressed with significant improvement in mean BCVA to logMAR 0.4 ± 0.4 (p < 0.0001) and mean CMT to 242.5 ± 82.4 µm (p < 0.0001). A mean of two intravitreal injections per eye was required for CNV regression. CONCLUSION: Intravitreal anti-VEGF therapy for pediatric CNV is an effective treatment in majority of affected eyes.

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.

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.

Posterior choroidal fluid loculation in central serous chorioretinopathy presenting as a choroidal elevation: A rare Pachychoroid phenotype.

PURPOSE: To describe an unusual variant of central serous chorioretinopathy (CSC) presenting as a choroidal elevation with subretinal fluid and its response to accepted treatment modalities of CSC. DESIGN: A retrospective case series. SUBJECTS: Six eyes of three patients, two males and one female, with a mean age of 55.3 years, were included in the study. All of them had Pachychoroid features, solitary choroidal elevation and subretinal fluid on optical coherence tomography (OCT); none of the patients had a previous episode of CSC. METHODS: Patients were studied with biomicroscopy, ultrasonography, fluorescein angiography and indocyanine green angiography. Systemic evaluation to rule out inflammatory and infective pathologies, and an oncologist referral also was done. Patients were managed in the line of central serous chorioretinopathy, using conservative treatment, focal laser and oral eplerenone. MAIN OUTCOME MEASURES: Resolution of subretinal fluid and choroidal elevation. RESULTS: Complete resolution of choroidal elevation and subretinal fluid was noted in all the three patients. There was no recurrence till the end of follow-up. CONCLUSION: Pachychoroid disease may present as solitary choroidal elevation with subretinal fluid due to exaggerated posterior choroidal fluid loculation and has a favourable outcome. However, it should be considered after exclusion of other differential diagnoses.

Coincidental ocular findings during retinopathy of prematurity telescreening of 9105 neonates.

PURPOSE: To describe the magnitude and spectrum of abnormal ocular findings other than retinopathy of prematurity (ROP) detected during an ROP telescreening programme. METHODS: Retrospective review of the records of all premature babies screened for ROP from August 2015 to December 2019. RESULTS: A total 9105 babies were screened during the study period with total 17,075 imaging sessions. Non-ROP ocular abnormalities were seen in 566 eyes of 383 babies (4.2%) including 207 male (54%) and 176 female babies (46%). Posterior segment abnormalities were seen in 88.2% babies (338/383) with haemorrhage being the most common (77%, 295/383). Anterior segment pathologies were seen in 12% babies (46/383) with congenital cataract being the most common pathology requiring surgical intervention. Other findings included globe abnormalities in 0.8% babies (3/383), adnexal disorders in 0.5% babies (2/383) and squint in 0.3% babies (1/383). Rare life-threatening conditions such as retinoblastoma and lipaemia retinalis were also detected. Eighty-seven babies (22.7%) were diagnosed with referral-warranted non-ROP pathologies, with an overall prevalence of 1% (87/9105). Prompt surgical intervention was advised for 7.3% babies (28/383), 3.4% babies (13/383) were treated medically and 4.2% babies (16/383) needed visual rehabilitation for non-amenable pathologies. CONCLUSION: This is the largest study describing the magnitude and spectrum of neonatal ocular pathologies other than ROP in a cohort of premature babies. Digital imaging has a significant impact on early detection and timely intervention for various vision as well as life-threatening non-ROP pathologies, which would have been missed or delayed otherwise.

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.

Sequential multimodal imaging of isolated necrotic full-thickness macular hole secondary to toxoplasma retinochoroiditis.

PURPOSE: To describe the sequential multimodal imaging features of an isolated necrotic macular hole secondary to Toxoplasma retinochoroiditis. OBSERVATION: A 23-year-old male was referred for surgical management of an idiopathic macular hole following sudden decrease in vision in his right eye. Right eye examination showed best-corrected visual acuity of 20/200, mild anterior segment inflammation, and a full thickness non operculated macular hole (MH) with ill-defined ragged margins and surrounding strip of pallid edema. Further multimodal imaging including optical coherence tomography (OCT), fundus autofluorescence (FAF), fluorescein angiography (FFA), and OCT-angiography confirmed the atypical configuration and inflammatory nature of MH. Serological tests showed elevated level of Toxoplasma gondii-specific antibodies. A diagnosis of necrotic isolated full-thickness MH secondary to toxoplasma retinochoroiditis was made. Patient was treated medically with anti-toxoplasma medication for 6 months. Sequential multimodal imaging highlighted the healing process of necrotic MH with vision improving to 20/80 at 6 months after presentation. CONCLUSION AND IMPORTANCE: A high level of suspicion and multimodal imaging plays an important role in accurate etiological diagnosis and management of atypical macular hole as in our case. Sequential multimodal imaging may provide an insight into the pathogenesis and healing pattern of such lesion.

Case report of central retinal artery occlusion following bee sting injury: A possible link?

We report the case of a 54-year-old male who presented with complaints of decreased vision in the left eye (LE). He gave a history of multiple bee stings following which he had an episode of allergic anaphylaxis to the face and neck region for which he was admitted and treated with steroids. On examination, he was found to have LE central retinal artery occlusion (CRAO) which was the cause of his reduced vision. This is the first report of a bee sting venom as a cause for CRAO.

Sub-internal limiting membrane hemorrhage as an unusual presentation of polypoidal choroidal vasculopathy.

We report the two cases who presented with sudden decreased vision and sub-internal limiting membrane (ILM) hemorrhage without a history of Valsalva maneuver, trauma, or hematological disorders. Multimodal imaging revealed the features suggestive of polypoidal choroidal vasculopathy (PCV) in addition to the sub-ILM hemorrhage. A provisional diagnosis of sub-ILM hemorrhage secondary to PCV was made and was treated with intravitreal Bevacizumab injection. Treatment resulted in the improved visual acuity along with the resolution of the sub-ILM hemorrhage. These cases highlight the possibility of isolated sub-ILM hemorrhage as a presenting fundus finding in PCV, which is previously unreported. This report also highlights the importance of multimodal imaging in diagnosing chorioretinal disorders with unusual presentation.

Optic Disc Pit Maculopathy: One-Year Outcomes of Pars Plana Vitrectomy with Foveal Sparing Inverted Internal Limiting Membrane Flap.

Purpose To evaluate the anatomical and visual outcomes in optic disc pit maculopathy following pars plana vitrectomy (PPV) with inverted internal limiting membrane (ILM) flap Methods Retrospective interventional case series of 10 patients diagnosed with serous macular detachment secondary to optic disc pit who underwent PPV with inverted ILM flap and were followed up for a year. Results A p-value of less than 0.05 was defined as statistically significant. The mean age of patients was 27.2 ± 10.6 years, preoperatively the mean best-corrected visual acuity of the logarithm of the minimum angle of resolution was 0.91 ± 0.42 (approximate Snellen equivalent 20/162), which improved to the logarithm of the minimum angle of resolution of 0.58 ± 0.29 (approximate Snellen equivalent 20/76) at end of one year, (p=0.008). The mean central macular thickness was 804.9 ± 294.1 m which improved to 273.4 ± 102.54 m, (p=0.002). After surgery, at end of one year, 60% of patients (6/10) had 15- or more-than-15-letter improvement of vision on Early Treatment Diabetic Retinopathy Study (ETDRS) visual acuity testing, 20% (2/10) gained a 10-letter improvement and 20% (2/10) retained the same vision. Conclusion PPV with inverted ILM flap can be considered as a good approach for the management of serous macular detachment secondary to optic disc pit and produce good anatomical and visual results at one year with stabilization of the disease.

OCT angiography for the diagnosis and management of choroidal neovascularization secondary to choroideremia.

PURPOSE: To describe the multimodal imaging findings and treatment outcomes in choroidal neovascularization secondary to Choroideremia. OBSERVATIONS: A 13-year-old male presented with reduced visual acuity in the left eye. He gave a history of nyctalopia. His best-corrected visual acuity (BCVA) was 20/20 in the right eye and 20/40 in the left eye. Based on multimodal imaging, the diagnosis of choroideremia in both eyes with a subfoveal choroidal neovascularization in the left eye was made. He underwent five intravitreal anti-vascular endothelial growth factor (VEGF) injections of Ranibizumab over a period of 3 years, with the final injection given due to recurrence of neovascularization. Post-treatment, his BCVA improved to 20/20 in the left eye with regression of the neovascular network. CONCLUSIONS AND IMPORTANCE: This case highlights the role of OCTA in diagnosis of choroidal neovascularization in choroideremia as well as its successful management with anti-VEGF injections with long term follow up.

Long-term outcomes of half-fluence photodynamic therapy and eplerenone in chronic central serous chorioretinopathy: A comparative study.

AIMS: To compare the long-term outcomes in chronic central serous chorioretinopathy (cCSC) following half-fluence photodynamic therapy (HF-PDT) and oral eplerenone treatment. METHODS: This retrospective comparative study included consecutive patients of cCSC treated with either HF-PDT or eplerenone. The treatment outcomes of the two groups were analyzed at 3-month, 6-month, and 12-month post-treatment. RESULTS: This study included 20 eyes (20 patients) in HF-PDT group, and 18 eyes (18 patients) in eplerenone group. All baseline parameters in HF-PDT and eplerenone groups were comparable including neurosensory detachment height (217.05 ± 140.25 µm vs 178.05 ± 164.24 µm respectively, p = 0.09), best-corrected visual acuity (BCVA) (logMAR 0.24 ± 0.13 vs logMAR 0.46 ± 0.37 respectively, p = 0.1), and subfoveal choroidal thickness (SFCT) [427.4 ± 117.4 µm vs 456.38 ± 119.25 µm respectively, p = 0.45]. HF-PDT resulted in complete resolution of neurosensory detachment in higher proportion of eyes compared to eplerenone at each follow-up visits (3 months: 90% vs 27.7%, p = 0; 6 months: 100% vs 61.1%, p = 0.003; and 12 months: 100% vs 70%, p = 0.03) with a significantly shorter duration to resolution (3.3 ± 0.9 months vs 5.8 ± 3.3 months respectively, p = 0.02). Intergroup comparison showed no significant difference between mean BCVA (p = 0.38 at 3 months, p = 0.14 at 6 months, and p = 0.19 at 12 months). Mean SFCT at 12 months of the two groups differed significantly (p = 0.003) due to increased choroidal thickness of eplerenone group. CONCLUSION: HF-PDT has a superior efficacy to achieve faster, greater and long-lasting resolution of subretinal fluid in cCSC compared to eplerenone therapy.

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.