Acute macular neuroretinopathy following Valsalva manoeuvre: an insight into the pathophysiology.

Acute macular neuroretinopathy (AMN) affects the outer retina and is most likely induced by non-inflammatory ischaemia of the retinal deep capillary plexus and choriocapillaris. A man in his early 20s developed Valsalva retinopathy following weightlifting at the gym and presented with blurring of vision in the left eye 1 month after the initial retinal haemorrhages had resolved. A diffuse, purplish, donut-shaped, perifoveal lesion was seen on funduscopy and was well defined by an optical coherence tomography angiography (OCTA) en face image in the left eye. Outer retinal changes on optical coherence tomography (OCT) and a dense co-localised scotoma on a visual field (VF) examination confirmed the diagnosis of AMN, and the patient was started on a tapering dose of oral steroids. Improvement was seen in OCT, OCTA and VF during the 6-month follow-up visit. The use of OCTA en face imaging enabled the accurate identification of the lesion in the affected layers of the retina.

Central Retinal Artery Occlusion With Triple Cilioretinal Artery Sparing.

We report a rare case of central retinal artery occlusion (CRAO) with triple cilioretinal artery sparing in a 76-year-old male with hypertension who presented with sudden diminution of vision in the left eye (OS) for one day. Optical coherence tomography angiography (OCTA) demonstrated the presence of three cilioretinal arteries and the absence of flow signals in the rest of the macula. Primary ophthalmic treatment was instituted immediately in the form of ocular massage, and acetazolamide 500 mg per oral (PO) stat was given. Systemic investigations revealed a significant blockage in coronary circulation on coronary angiography and an atheromatous plaque at the origin of the left internal carotid artery with 50% stenosis on digital subtraction angiography. Systemic anticoagulants and lipid-lowering agents (statins) were initiated by the cardiologist. Percutaneous transluminal coronary angioplasty was subsequently performed. At the eight-week follow-up visit, best-corrected visual acuity had improved to 2/60 OS. Fundus examination of the OS revealed optic disc pallor with normal retinal background. Spectral-domain optical coherence tomography showed diffuse retinal thinning except in the area supplied by the three patent cilioretinal arteries. En face OCTA OS showed restoration of retinal flow signal in the macula. Non-invasive imaging (OCTA) is critical in establishing early diagnosis and initiating prompt treatment in this ocular emergency with underlying potentially life-threatening systemic associations.

Pediatric Lupus Retinopathy: A Rare Manifestation of a Grave Systemic Disorder.

We describe a rare case of pediatric systemic lupus erythematosus (pSLE) and its successful management. A nine-year-old female presented with bilateral diminution of vision, fever, and rash in the malar region, chest, abdomen, back, and arms for three months. Clinical examination and multimodal imaging revealed bilateral extensive retinal vasculitis with macular edema. Laboratory investigations revealed anemia, leucopenia, positive serum antinuclear antibody (ANA), and anti-extractable nuclear antigen (ENA) antibodies. A diagnosis of pediatric lupus retinopathy was made. Ocular and systemic manifestations responded well to intense systemic immunosuppression (pulse intravenous {IV} methylprednisolone, oral prednisolone and hydroxychloroquine {HCQ}, six cycles of IV cyclophosphamide, and oral azathioprine) along with topical steroids and laser photocoagulation, over the next 10 months. Though ocular manifestations are not a part of the diagnostic criteria for SLE, they may be markers of active systemic disease. Ophthalmologists and rheumatologists must treat this complex disease in tandem in order to provide optimum patient care.

Multimodal Imaging in a Case of Chronic Sympathetic Ophthalmia.

We report a case of chronic sympathetic ophthalmia (SO) in a one-eyed patient who was successfully managed with systemic immunosuppression therapy. A 77-year-old one-eyed female presented with progressive diminution of vision in the left eye (OS) for one month. She had previously undergone a right eye (OD) pars plana vitrectomy elsewhere for exogenous post-operative endophthalmitis (after manual small incision cataract surgery five months ago), following which she developed phthisis. Granulomatous panuveitis and advanced cataract were noted in the OS. Findings on multimodal imaging, including spectral domain optical coherence tomography (SD-OCT), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and B-scan ultrasonography, were consistent with those of chronic SO. Promptly, oral steroids and systemic immunosuppressants were initiated under the supervision of a rheumatologist. At the three-week follow-up, complete resolution of clinical signs was observed on multimodal imaging. Chronic SO may present with ambiguous clinical signs, leading to a diagnostic dilemma. This may cause a delay in initiating treatment, which can prove to be highly detrimental, especially in one-eyed patients. Multimodal imaging is critical in excluding differential diagnoses and proves to be indispensable in the timely management of this sight-threatening condition.

Acute-Onset Postoperative Herpetic Endophthalmitis: A Case Report.

Herpes simplex virus uveitis without corneal reactivation is more frequent than previously thought. Although herpes simplex virus has been implicated as a cause of postoperative uveitis and endophthalmitis, it has not been reported as a cause of acute postoperative endophthalmitis within the early postoperative period, specifically within one week following cataract extraction. A 55-year-old man with vascularized irregular central disc-shaped stromal corneal opacity with complicated cataracts underwent cataract surgery. Intraoperatively, there was posterior capsular rent, requiring anterior vitrectomy. On postoperative day three, the patient had an increase in inflammation in the anterior chamber (grade 4+) with marked vitreous haze (grade 4). Vitreous taps were negative for bacteria and fungi, and despite intravitreal injections of vancomycin and ceftazidime, the patient had worsening of inflammation with increasing exudates and the appearance of the fibrinous membrane in the anterior chamber. Polymerase chain reaction (PCR) of aqueous and vitreous samples at this point of time yielded positive serology for herpes viral DNA, and the patient was started on oral valacyclovir. The ocular inflammation resolved soon after switching to oral valacyclovir. Typical acute postoperative endophthalmitis starts two to seven days after surgery, and the most common isolate in vitreous biopsies is coagulase-negative staphylococci. We report a rare case of acute-onset herpetic endophthalmitis presenting within 72 hours following cataract surgery for a complicated cataract in a patient with a history of pre-existing healed viral keratitis. Our case highlights that a suspicion of viral endophthalmitis should be kept in mind as a cause of acute-onset post-cataract surgery endophthalmitis, especially in cases of surgery that fail to yield a positive result on Gram's stain, culture or PCR for bacteria and fungi.

Modified Two-Stage Approach for Management of Combined Rhegmatogenous Retinal Detachment and Choroidal Detachment With Extreme Hypotony.

Combined rhegmatogenous retinal detachment (RRD) and serous choroidal detachment (CD) present a significant challenge. No global standard of care exists for treating these complex RRDs. There is a lower failure rate when such detachments are treated with pars plana vitrectomy than with scleral buckle alone. The use of pre-operative steroids may not work in cases with moderate-to-severe CDs with severe hypotony where suprachoroidal fluid drainage is required to reduce inflammatory mediators, thus preventing proliferative vitreoretinopathy (PVR). We report a case of a 62-year-old male who had a combined RRD and severe CD with vitreous hemorrhage in the left eye (LE). There was extreme hypotony leading to a severely deformed and distorted globe with poor visualization of the fundus. The patient was started on 60 mg of oral prednisolone, and a posterior subtenon injection of 20 mg of triamcinolone acetonide was given to reduce inflammation and CD. However, despite one week of pre-operative steroids, there was severe hypotony. The patient was taken for pars plana vitrectomy with drainage of suprachoroidal fluid. Intra-operatively even after drainage of suprachoroidal fluid via inferotemporal posterior sclerotomy, hypotony persisted, and media was very hazy, precluding us from proceeding with vitrectomy in the first sitting. Oral steroids were continued, and vitrectomy was done in the second sitting, 72 hours later, with long-term silicone oil tamponade. Post-operatively patient had a well-formed globe with an attached retina and a good visual acuity. Our case thereby highlights that combined retinal and CD is a complicated diagnosis that presents with many pre-operative, intra-operative, and post-operative challenges. We could achieve good anatomical and functional success using a modified two-stage approach in our unusual case of combined RRD wth CD with extreme hypotony.

Lens Coloboma: A Rare Association of Congenital Rubella Syndrome.

Congenital rubella syndrome (CRS) may affect all ocular structures in general, either in isolation or in combination. Typical ocular complications in CRS include cataracts, microcornea, microphthalmia, glaucoma, nystagmus, and retinopathy. We report a case of a four-year-old girl who presented with bilateral total cataracts with sensory nystagmus, a poorly dilating pupil, iris hypoplasia, high axial myopia in the right eye (RE), and a retinal detachment in the left eye (LE). The systemic evaluation revealed microcephaly with an associated patent ductus arteriosus (PDA) and mild pulmonary arterial hypertension (PAH). Based on these findings, the child was diagnosed with clinically confirmed CRS. The child was taken up for right-eye cataract surgery. Intra-operatively, a lens coloboma involving the temporal equator of the lens along with a highly tessellated fundus was noted. Two weeks post-cataract surgery, the child had a best corrected visual acuity (BCVA) of 6/24. However, four weeks after the surgery, the child developed total rhegmatogenous retinal detachment in the right eye, for which pars plana vitrectomy with endolaser and silicon oil tamponade was done. Four weeks later, the child's BCVA in the right eye was 6/36, which was maintained until the last follow-up of four months. Lens coloboma may be isolated or may occur in association with chorioretinal coloboma. Ours is the first case of unilateral atypical lens coloboma associated with high myopia and bilateral cataracts in a patient with congenital rubella syndrome. Lens colobomas with high myopia have not been reported previously as ocular associations of CRS. Our case highlights that in children with CRS presenting with unilateral or bilateral congenital cataracts, the possibility of lenticular coloboma as a coexistent association should be kept in mind while taking these cases for cataract surgery.

Multifocal Serpiginoid Choroiditis Due to Mycobacterium Mageritense following Laparoscopic Hysterectomy in an Immunocompetent Host.

PURPOSE: To report nontubercular choroiditis, caused by Mycobacterium mageritense isolated using MALDI-TOF MS, following laparoscopic hysterectomy in an immunocompetent patient. CASE REPORT: A 42-year-old female presented with gradual, painless diminution of vision in the right eye for six weeks. She had undergone laparoscopic hysterectomy two months back. BCVA was 2/60 OD. Clinical presentation and imaging all pointed to a diagnosis of serpiginous-like choroiditis. Matrix-Assisted Laser Desorption Ionization-Time of Flight Mass Spectrometry (MALDI-TOF MS) had led to isolation of Mycobacterium mageritense. Patient was treated with systemic antibiotics. At 3 months follow up, BCVA improved to 6/60; fundus showed healed choroiditis and chorioretinal thinning. CONCLUSION: Atypical mycobacterial infections associated with laparoscopic surgeries can rarely lead to debilitating systemic bacteremia resulting in profound visual impairment. The importance of timely diagnosis using specific and sophisticated methods such as MALDI-TOF MS as well as maintaining aseptic precautions perioperatively during major surgeries cannot be overemphasized.

Central retinal vein occlusion post ChAdOx1 nCoV-19 vaccination - can it be explained by the two-hit hypothesis?

PURPOSE: To report a case of central retinal vein occlusion (CRVO) seven days following the first dose of ChAdOx1 nCoV-19 vaccine and propose a hypothesis for the possible underlying pathogenesis. OBSERVATION: A 31-year-old male presented with CRVO with cystoid macular edema, one week after receiving his first ChAdOx1 nCoV-19 vaccine dose. Apart from mild hyperhomocysteinemia, no major thrombophilic or systemic risk factors were found. Anti-platelet factor 4 antibodies, specific for vaccine-induced immune thrombotic thrombocytopenia, were also negative. However, he tested strongly positive (> 250 U/mL) for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) IgG spike antibodies, 2 weeks post the first dose - suggestive of a prior subclinical infection. CONCLUSION: COVID-19 is known to be associated with an altered host one-carbon metabolism resulting in hyperhomocysteinemia. We hypothesize that a prior subclinical infection with COVID-19, the first hit, may have led to hyperhomocysteinemia in our patient and vaccination must have been the second hit that triggered the thrombotic event. Further studies, including correlation of thrombotic complications with IgG antibody titres post-vaccination, are essential in order to better understand the pathogenesis of such events.

Regression of choroidal metastasis from breast carcinoma with palbociclib.

BACKGROUND: Uveal metastasis is reported to be the most common intraocular malignancy. The most common site of origin of ocular metastases in females is the breast. In some cases, uveal metastatic lesions respond to systemic chemotherapy. We report a case of a patient who developed choroidal metastasis, while on endocrine therapy with selective estrogen receptor modulator (SERM), tamoxifen, for estrogen receptor (ER) positive, progesterone receptor (PR) positive and (human epidermal growth factor receptor 2) HER2 negative primary breast carcinoma, which then regressed following systemic chemotherapy with palbociclib. CASE DESCRIPTION: An 83-year-old female, with a history of modified radical mastectomy, chemotherapy and radiation therapy for infiltrating duct carcinoma of the breast, presented with a choroidal metastatic lesion in the left eye along with liver and lung metastases, 3 years after the primary carcinoma was treated. At the time of presentation, she was on tamoxifen. The choroidal tumor showed regression after the introduction of palbociclib, a cyclin-dependent kinase 4/6 (CDK4/6) inhibitor. CONCLUSION: This report highlights the use of palbociclib, in the palliative treatment of choroidal metastasis from primary breast cancer. The use of chemotherapy for choroidal metastasis can help avoid external beam radiation therapy and its concurrent side effects. Although there are a few reports involving the use of palbociclib for metastatic breast carcinoma, all of those have been in conjunction with and/or following non-response to other treatment modalities. Ours is the first report wherein palbociclib has been used as the first-line palliative chemotherapy and helped in regression of choroidal metastasis.

Presumed bilateral diffuse uveal melanocytic proliferation - A case report and review of literature.

Purpose: To describe a case of presumed bilateral diffuse uveal melanocytic proliferation (BDUMP) associated with renal cell carcinoma (RCC) and provide an updated review of literature. Observations: A 58-year-old man, with a history of radical nephrectomy for RCC 8 years ago, presented with gradual diminution of vision. Based on multimodal imaging and detailed systemic evaluation, a diagnosis of presumed BDUMP and metastatic RCC was made. He was started on sunitinib malate as palliative chemotherapy. However, he refused plasmapheresis for BDUMP. The patient rapidly developed bilateral exudative retinal detachment. Subsequently, he progressed to bilateral neovascular glaucoma secondary to closed funnel retinal detachment. Eventually, he was lost to follow up after 13 months. Conclusions & Importance: BDUMP portends an underlying advanced systemic malignancy. Studies have not conclusively proven any definite treatment for BDUMP and survival is generally poor. Ocular side effects of palliative targeted chemotherapy for the primary malignancy, such as sunitinib, should be borne in mind.

Optical coherence tomography angiography-guided diagnosis of a traumatic choroidal rupture-associated choroidal neovasular membrane and its management with intravitreal ranibizumab.

A 25-year-old female presented with sudden onset diminution of vision in the right eye (oculus dextrus [OD]) following blunt trauma after a sports injury. Ocular examination revealed best-corrected visual acuity (BCVA) of 20/30 in OD and fundus revealed commotio retinae, localized preretinal bleed, and a large choroidal rupture (CR). She was managed conservatively at that moment. Three months following trauma, she returned with further deterioration of BCVA (20/80) in OD. Optical coherence tomography angiogram (OCTA) confirmed the presence of choroidal neovascular membrane (CNVM). She was treated with single intravitreal ranibizumab injection. Repeat OCTA after 6 weeks showed the regression of CNVM. Her BCVA improved to 20/30 at 6 months of follow-up. In the index report, we present a unique OCTA guided the diagnosis of posttraumatic CR-associated CNVM and its successful management with single intravitreal ranibizumab.

Relapsing neuromyelitis optica in an adolescent girl.

Early differentiation of neuromyelitis optica spectrum disorder (NMO-SD) from multiple sclerosis (MS) is of paramount importance as NMO-SD (especially relapsing variant) has more severe morbidity than MS. We describe a case of an adolescent girl who presented with repeated episodes of optic neuritis over a period of 4 years with normal brain MRI scans. She was treated initially as relapsing remitting MS, before showing clinical evidence of transverse myelitis (TM), and eventually being diagnosed as NMO-SD. Pulse intravenous methyl prednisolone along with immunosuppressive therapy led to remission of her disease. However, delay in diagnosis as NMO-SD led to visual disability in the left eye. Therefore, in young patients with recurrent optic neuritis and normal brain MRI, it may be prudent to get spinal MRI done to look for TM, even when asymptomatic. In addition, we should keep a low threshold for requesting aquaporin-4 antibody testing in these patients.

Memantine, Simvastatin, and Epicatechin Inhibit 7-Ketocholesterol-induced Apoptosis in Retinal Pigment Epithelial Cells But Not Neurosensory Retinal Cells In Vitro.

PURPOSE: 7-ketocholesterol (7kCh), a natural byproduct of oxidation in lipoprotein deposits is implicated in the pathogenesis of diabetic retinopathy and age-related macular degeneration (AMD). This study was performed to investigate whether several clinical drugs can inhibit 7kCh-induced caspase activation and mitigate its apoptotic effects on retinal cells in vitro. METHODS: Two populations of retinal cells, human retinal pigment epithelial cells (ARPE-19) and rat neuroretinal cells (R28) were exposed to 7kCh in the presence of the following inhibitors: Z-VAD-FMK (pan-caspase inhibitor), simvastatin, memantine, epicatechin, and Z-IETD-FMK (caspase-8 inhibitor) or Z-ATAD-FMK (caspase-12 inhibitor). Caspase-3/7, -8, and -12 activity levels were measured by fluorochrome caspase assays to quantify cell death. IncuCyte live-cell microscopic images were obtained to quantify cell counts. RESULTS: Exposure to 7kCh for 24 hours significantly increased caspase activities for both ARPE-19 and R28 cells (P < 0.05). In ARPE cells, pretreatment with various drugs had significantly lower caspase-3/7, -8, and -12 activities, reported in % change in mean signal intensity (msi): Z-VAD-FMK (48% decrease, P < 0.01), memantine (decreased 47.8% at 1 µM, P = 0.0039 and 81.9% at 1 mM, P < 0.001), simvastatin (decreased 85.3% at 0.01 µM, P < 0.001 and 84.8% at 0.05 µM, P < 0.001) or epicatechin (83.6% decrease, P < 0.05), Z-IETD-FMK (68.1% decrease, P < 0.01), and Z-ATAD-FMK (47.7% decrease, P = 0.0017). In contrast, R28 cells exposed to 7kCh continued to have elevated caspase-3/7, -8, and -12 activities (between 25.7% decrease and 17.5% increase in msi, P > 0.05) regardless of the pretreatment. CONCLUSION: Several current drugs protect ARPE-19 cells but not R28 cells from 7kCh-induced apoptosis, suggesting that a multiple-drug approach is needed to protect both cells types in various retinal diseases.

Post-fever retinitis - Newer concepts.

Post-fever retinitis (PFR) is an infectious or para-infectious uveitic entity caused by bacterial or viral agents and seen mainly in tropical countries. Systemic symptoms such as joint pain, skin rash are common during the febrile stage. On the basis of only clinical presentation, it is difficult to pin-point the exact etiology for PFR. Serological investigations, polymerase chain reaction, and knowledge of concurrent epidemics in the community may help to identify the etiological organism. Bacterial causes of PFR such as rickettsia and typhoid are treated with systemic antibiotics, with or without systemic steroid therapy, whereas PFR of viral causes such as chikungunya, dengue, West Nile virus, and Zika virus have no specific treatment and are managed with steroids. Nevertheless, many authors have advocated mere observation and the uveitis resolved with its natural course of the disease. In this article, we have discussed the clinical features, pathogenesis, investigations, and management of PFR.

Exploring choroidal angioarchitecture in health and disease using choroidal vascularity index.

The choroid is one of the most vascularized structures of the human body and plays an irreplaceable role in nourishing photoreceptors. As such, choroidal dysfunction is implicated in a multitude of ocular diseases. Studying the choroid can lead to a better understanding of disease pathogenesis, progression and discovery of novel management strategies. However, current research has produced inconsistent findings, partly due to the physical inaccessibility of the choroid and the lack of reliable biomarkers. With the advancements in optical coherence tomography technology, our group has developed a novel quantitative imaging biomarker known as the choroidal vascularity index (CVI), defined as the ratio of vascular area to the total choroidal area. CVI is a potential tool in establishing early diagnoses, monitoring disease progression and prognosticating patients. CVI has been reported in existing literature as a robust marker in numerous retinal and choroidal diseases. In this review, we will discuss the current role of CVI with reference to existing literature, and make postulations about its potential and future applications.

Takayasu's arteritis: a unique ophthalmic presentation with CRAO and BRVO.

A unique case of sequential occurrence of central retinal artery occlusion (CRAO) and superotemporal branch retinal vein occlusion (ST-BRVO) in a patient of Takayasu's arteritis is described. An 18-year-old man was diagnosed as left eye CRAO on his initial presentation and was subjected to a complete cardiovascular evaluation revealing findings diagnostic of Takayasu's arteritis. Patient was however lost to follow-up and presented 16 months later with ST-BRVO in the right eye. Multidisciplinary intervention and an appropriate ocular intervention led to complete recovery of vision in the right eye that was maintained until his last ophthalmic evaluation (2.5 years after the initial presentation). Though uncommon, small retinal vessel involvement can occur in Takayasu's arteritis as the inaugural feature. Hence, CRAO or branch retinal vein occlusion in a young patient, especially a male, mandates a thorough systemic evaluation and a high index of suspicion of Takayasu's arteritis to prevent vision threatening complications.

Alport syndrome with bilateral simultaneous anterior and posterior lenticonus with severe temporal macular thinning.

Alport syndrome (AS) is a hereditary disease with various modes of inheritance, X-linked being the the most common. Anterior lenticonus is the characteristic abnormality along with perimacular and peripheral fleck retinopathy. Our two cases of AS had simultaneous anterior and posterior lenticonus with severe temporal macular thinning on optical coherence tomography with no specific renal symptomatology and were diagnosed as AS without any invasive renal biopsy. First patient was a 19-year-old man who presented with compound myopia due to bilateral anterior and posterior lenticonus with perimacular fleck retinopathy and lozenge sign and bilateral moderate sensorineural hearing loss (SNHL). Second patient was a 24-year-old man who presented with difficulty in vision due to bilateral anterior and posterior lenticonus with bilateral severe SNHL. Our cases emphasise the crucial role of an ophthalmologist in diagnosing AS before the onset of renal symptoms and prompting further nephrological work-up in the patient or the carrier.