Gradual Loss Of Vision Research Articles

Persistent syphilitic ocular manifestations despite treatment: a case series

BackgroundPenicillin has remained the most effective treatment for syphilis for several decades. Syphilitic retinal manifestations may persist following treatment and cause visual problems. In this case series, we describe three syphilis patients with persistent posterior segment manifestations due to chronic inflammation, incomplete treatment, and reinfection.Case seriesRecommended initial treatment for all patients was 14 days of intravenous penicillin. Oral prednisone was added 48 h after initiation of penicillin therapy. Case 1: A 48-year-old female presented with gradual vision loss for two months. Fundus imaging revealed syphilitic outer retinopathy (SOR), papillitis, and acute syphilitic posterior placoid chorioretinopathy (ASPPC). After treatment, she had persistent cystoid macular edema (CME) and was treated with intravitreal triamcinolone injections and ketorolac drops. Case 2. A 24-year-old male presented with sudden vision loss for two days. On imaging, he had ASPPC, papillitis, and SOR. IV penicillin treatment was given for 10 days only. He had persistent SOR and was retreated with doxycycline and prednisone. Case 3: A 52-year-old male presented with eye pain and visual loss for one week. There was evidence of ASPPC and papillitis on imaging. One month after treatment, he had persistent papillitis and was restarted on oral prednisone. One year later, he was found to have recurrent ASPPC and was confirmed to be reinfected with syphilis, for which he was retreated.ConclusionWhen treating persistent syphilitic ocular manifestations, we recommend checking that the penicillin treatment was complete and the RPR titers are declining. If both hold true, then the affected eye should be treated with anti-inflammatory therapy. Other factors that contribute to poor visual prognosis include treatment delay, poor initial visual acuity, macular edema, and HIV coinfection.

Retinal Proteome Profiling of Inherited Retinal Degeneration Across Three Different Mouse Models Suggests Common Drug Targets in Retinitis Pigmentosa

Inherited retinal degenerations (IRDs) are a leading cause of blindness among the population of young people in the developed world. Approximately half of IRDs initially manifest as gradual loss of night vision and visual fields, characteristic of retinitis pigmentosa (RP). Due to challenges in genetic testing, and the large heterogeneity of mutations underlying RP, targeted gene therapies are an impractical largescale solution in the foreseeable future. For this reason, identifying key pathophysiological pathways in IRDs that could be targets for mutation-agnostic and disease-modifying therapies (DMTs) is warranted. In this study, we investigated the retinal proteome of three distinct IRD mouse models, in comparison to sex- and age-matched wild-type mice. Specifically, we used the Pde6βRd10 (rd10) and RhoP23H/WT (P23H) mouse models of autosomal recessive and autosomal dominant RP, respectively, as well as the Rpe65-/- mouse model of Leber´s congenital amaurosis type 2 (LCA2). The mice were housed at two distinct institutions and analyzed using LC-MS in three separate facilities/instruments following data-dependent and data-independent acquisition modes. This cross-institutional and multi-methodological approach signifies the reliability and reproducibility of the results. The largescale profiling of the retinal proteome, coupled with in vivo electroretinography recordings, provided us with a reliable basis for comparing the disease phenotypes and severity. Despite evident inflammation, cellular stress, and downscaled phototransduction observed consistently across all three models, the underlying pathologies of RP and LCA2 displayed many differences, sharing only four general KEGG pathways. The opposite is true for the two RP models in which we identify remarkable convergence in proteomic phenotype even though the mechanism of primary rod death in rd10 and P23H mice is different. Our data highlights the cAMP and cGMP second-messenger signaling pathways as potential targets for therapeutic intervention. The proteomic data is curated and made publicly available, facilitating the discovery of universal therapeutic targets for RP.

Takayasu Arteritis Presented with Unilateral Vision Loss : A Case Report

Background: Takayasu Arteritis (TA) is a rare chronic granulomatous inflammation of the aorta or its branches and is prevalent all around the world. It causes stenosis of large arteries and ischaemic damage to target organs. There is usually a delay in recognising TA because of the rarity and unfamiliarity with the disease, unspecific early symptoms and lack of diagnostic equipment for early diagnosis. The purpose of the study to reminding readers of this issue and emphasize the necessity for further research on how to deal with in the best way. Case Report: We report a case of 30 years old male presented on 22nd August 2023 at Institute of Applied Health Sciences (IAHS) Chattogram with gradual loss of vision of left eye over a month accompanied by headache and later on diagnosed as a case of Takayasu’s Arteritis. Conclusion: Visual loss is the most common ocular symptom in Takayasu arteritis. Since a variety of causes may result in reduced vision in Takayasu arteritis. A careful history is very important to ascertain the cause of visual loss in Takayasu arteritis patients to reduce unnessary investigations and referrals. IAHS Medical Journal Volume 6(2) December 2023; 90-92

Overview of latent tuberculosis-related central serous chorioretinopathy: Review and case report

We present a case of multifocal central serous chorioretinopathy (CSCR) in a 40-year-old male who presented with gradual loss of vision in both of his eyes during the previous 3 months. The anterior segment examination was normal; the dilated fundus examination revealed severe retinal elevation in the macular area in both eyes. Optical coherence tomography confirmed the presence of subretinal fluid with a central macular thickness of 353 μm OD and 356 μm OS. On the basis of ocular findings, a diagnosis of multifocal CSCR was made. One month later, the patient complained of further vision loss. His erythrocyte sedimentation rate was elevated, and the Mantoux test (22 mm × 22 mm), the QuantiFERON TB gold (5 IU/ml), and the cytomegalovirus immunoglobulin G (2277.92 AU/ml) yielded positive results. A diagnosis of latent tuberculosis infection was made. After 2 months of starting antitubercular treatment, the patient’s visual acuity improved.

Bi-allelic variants in COQ8B, a gene involved in the biosynthesis of coenzyme Q10, lead to non-syndromic retinitis pigmentosa

Retinitis pigmentosa (RP) is a Mendelian disease characterized by gradual loss of vision, due to the progressive degeneration of retinal cells. Genetically, it is highly heterogeneous, with pathogenic variants identified in more than 100 genes so far. Following a large-scale sequencing screening, we identified five individuals (four families) with recessive and non-syndromic RP, carrying as well bi-allelic DNA changes in COQ8B, a gene involved in the biosynthesis of coenzyme Q10. Specifically, we detected compound heterozygous assortments of five disease-causing variants (c.187C>T [p.Arg63Trp], c.566G>A [p.Trp189Ter], c.1156G>A [p.Asp386Asn], c.1324G>A [p.Val442Met], and c.1560G>A [p.Trp520Ter]), all segregating with disease according to a recessive pattern of inheritance. Cell-based analysis of recombinant proteins deriving from these genotypes, performed by target engagement assays, showed in all cases a significant decrease in ligand-protein interaction compared to the wild type. Our results indicate that variants in COQ8B lead to recessive non-syndromic RP, possibly by impairing the biosynthesis of coenzyme Q10, a key component of oxidative phosphorylation in the mitochondria.

The immunobiology of corneal HSV-1 infection and herpetic stromal keratitis.

SUMMARYHuman alphaherpesvirus 1 (HSV-1) is a highly successful neurotropic pathogen that primarily infects the epithelial cells lining the orofacial mucosa. After primary lytic replication in the oral, ocular, and nasal mucosal epithelial cells, HSV-1 establishes life-long latency in neurons within the trigeminal ganglion. Patients with compromised immune systems experience frequent reactivation of HSV-1 from latency, leading to virus entry in the sensory neurons, followed by anterograde transport and lytic replication at the innervated mucosal epithelial surface. Although recurrent infection of the corneal mucosal surface is rare, it can result in a chronic immuno-inflammatory condition called herpetic stromal keratitis (HSK). HSK leads to gradual vision loss and can cause permanent blindness in severe untreated cases. Currently, there is no cure or successful vaccine to prevent latent or recurrent HSV-1 infections, posing a significant clinical challenge to managing HSK and preventing vision loss. The conventional clinical management of HSK primarily relies on anti-virals to suppress HSV-1 replication, anti-inflammatory drugs (such as corticosteroids) to provide symptomatic relief from pain and inflammation, and surgical interventions in more severe cases to replace damaged cornea. However, each clinical treatment strategy has limitations, such as local and systemic drug toxicities and the emergence of anti-viral-resistant HSV-1 strains. In this review, we summarize the factors and immune cells involved in HSK pathogenesis and highlight alternate therapeutic strategies for successful clinical management of HSK. We also discuss the therapeutic potential of immunoregulatory cytokines and immunometabolism modulators as promising HSK therapies against emerging anti-viral-resistant HSV-1 strains.

Bardet-Biedl Syndrome: A Case Report

Bardet-Biedl syndrome (BBS) is a rare autosomal recessive multisystem involvement genetic disorder with a broad spectrum of clinical presentations. It is characterized by rod-cone dystrophy, polydactyly, obesity, learning disabilities, hypogonadism in males or genital abnormalities in females, and renal impairment. We present a case of a 12-year-old boy who complained of gradual loss of vision (particularly noticeable at night), obesity, hypogenitalism, and polydactyly. In addition, he had postaxial polydactyly in all limbs, obesity, retinitis pigmentosa, learning disabilities, hypogenitalism, speech delay, developmental delay, astigmatism, and acanthosis nigricans. Multidisciplinary approaches need to manage this disorder and need regular followup. Genetic counselling is essential. J Dhaka Med Coll. 2022; 31(2) : 264-267

Genotypic and Phenotypic Characterization of a Cohort of Patients Affected by Rod Cyclic Nucleotide Channel-Associated Retinitis Pigmentosa.

Retinitis pigmentosa (RP), a heterogeneous inherited retinal disorder causing gradual vision loss, affects over 1 million people worldwide. Pathogenic variants in CNGA1 and CNGB1 genes, respectively, accounting for 1% and 4% of cases, impact the cyclic nucleotide-gated channel in rod photoreceptor cells. The aim of this study was to describe and compare genotypic and clinical characteristics of a cohort of patients with CNGA1- or CNGB1-related RP and to explore potential genotype-phenotype correlations. The following data from patients with CNGA1- or CNGB1-related RP, followed in five Italian inherited retinal degenerations services, were retrospectively collected: genetic variants in CNGA1 and CNGB1, best-corrected visual acuity (BCVA), ellipsoid zone (EZ) width, fundus photographs, and short-wavelength fundus autofluorescence (SW-AF) images. Comparisons and correlation analyses were performed by first dividing the cohort in two groups according to the gene responsible for the disease (CNGA1 and CNGB1 groups). In parallel, the whole cohort of RP patients was divided into two other groups, according to the expected impact of the variants at protein level (low and high group). In total, 29 patients were recruited, 11 with CNGA1- and 18 with CNGB1-related RP. In both CNGA1 and CNGB1, 5 novel variants in CNGA1 and 5 in CNGB1 were found. BCVA was comparable between CNGA1 and CNGB1 groups, as well as between low and high groups. CNGA1 group had a larger mean EZ width compared to CNGB1 group, albeit not statistically significant, while EZ width did not differ between low and high groups A statistically significant correlation between EZ width and BCVA as well as between EZ width and age were observed in the whole cohort of RP patients. Fundus photographs of all patients in the cohort showed classic RP pattern, and in SW-AF images an hyperautofluorescent ring was observed in 14/21 patients. Rod CNG channel-associated RP was demonstrated to be a slowly progressive disease in both CNGA1- and CNGB1-related forms, making it an ideal candidate for gene augmentation therapies.

Sporadic Optic Nerve Glioma Causing Bitemporal Hemianopia

Abstract Primary tumours of the optic nerve are relatively rare. Optic nerve gliomas are usually seen in children under the age of 10 and present with gradual vision loss followed by proptosis. A 19-year-old female presented to our neuro-ophthalmology clinic with chief complaints of disturbances in vision in both eyes and headache. Ocular examination showed best corrected visual acuity (BCVA) of 6/6 in both eyes and visual field analysis revealed bitemporal hemianopia. Urgent neuroimaging was advised and her MRI brain revealed optic pathway glioma involving the intracranial segment of right optic nerve and optic chiasma. Improvement in vision was noted during post-surgical follow-up. We present this case to highlight the importance of visual field examination in a young patient with headache, even with a BCVA of 6/6.

Through Indian Eyes: A Case Series on Steroid-induced Secondary Cataract

Lichen planus, a chronic autoimmune dermatological disorder, often necessitates the use of topical corticosteroids for symptom management. However, this case series highlights the emergence of topical steroid-induced secondary cataracts in two patients, emphasizing a previously underrecognized complication. Both patients, females aged 36 and 43, presented with gradual vision loss in their left eyes, attributed to prolonged corticosteroid use for lichen planus and skin allergies, respectively. Ophthalmological examinations confirmed the diagnosis, leading to cataract surgeries. The pathogenesis involves complex cellular and molecular mechanisms, emphasizing the need for vigilant monitoring during corticosteroid therapy. Moreover, the lack of stringent reporting systems for adverse drug reactions in India underscores the necessity for comprehensive monitoring to mitigate economic and quality of life burdens. The study emphasizes the importance of judicious corticosteroid use, tapering regimens, and patient education to minimize complications while effectively managing dermatological conditions. This underscores the critical need for heightened awareness, surveillance, and patient-centred approaches to mitigate the risk of corticosteroid-induced secondary cataracts, ensuring optimal therapeutic outcomes and long-term ocular health.

Experiences of keratoconus patients attending public eye care facilities in South Africa.

Keratoconus (KC) is a progressive condition that usually affects young persons between their first and fourth decades. Myopia and irregular astigmatism are the common presentations which are proceeded by corneal steepening and thinning. Keratoconus is a progressive ectasia of the cornea that presents bilaterally, although often asymmetrical. To explore the experiences of KC patients attending public eye care facilities in Capricorn District of Limpopo province. Capricorn District, Limpopo province, South Africa. Descriptive qualitative phenomenology approach was used in the study to explore the lived experiences of patients diagnosed with KC, attending public eye care facilities. Purposive sampling was used to select 16 patients who were diagnosed with KC and referred for contact lens fitting. Data were collected through face-to-face, one-on-one interviews. Patients reported to have developed gradual vision loss which worsened when they grew older. There was lack of knowledge of KC amongst patients and this was exacerbated by limited health literacy and vocabulary barriers. Patients had difficulty performing daily activities where some had dropped out of school while others worked at a slower pace and reduced working distances. Patients with KC received inadequate information on their condition due to limited health literacy and vocabulary barriers. Programmes to promote practitioner and patient education are required to improve the perceived service level provided to KC patients.Contribution: The findings of this study will assist to improve the experiences of KC patients on perceived service quality received from public facilities.

A Mathematical Study for Promoting Disability Inclusion in Glaucoma: A Comprehensive Approach

Globally, glaucoma—a progressive optic neuropathy—is one of the leading causes of permanent blindness. This abstract provides an overview of glaucoma’s effects on visual impairments, emphasizing the physical, psychological, and social consequences affected individuals face. Glaucoma primarily damages the optic nerve, leading to gradual peripheral vision loss, often unnoticed until an advanced stage. This visual impairment significantly lowers the quality of life by making it difficult to read, drive, and recognize faces, among other daily activities. This study examines how prostaglandin analogues affect intraocular pressure (IOP) and aqueous humor outflow into Schlemm’s canal via the trabecular meshwork emphasizing how these effects relate to glaucoma disability. The essential ocular fluid known as aqueous humor travels from the posterior part behind the iris via the pupil aperture into the anterior region. It ultimately leaves the eyes through the drainage system. The eyes’ ciliary body, an essential component of the drainage system, continuously produces aqueous humor, mainly ejected through the trabecular meshwork into Schlemm’s canal. Increase in IOP, a defining feature of glaucoma disability, is brought on by a decreased aqueous humor drainage from the angle of the drainage system. This abstract underscores the importance of proactive screening, treatment, and support for individuals with glaucoma to mitigate visual disabilities and enhance their overall well-being. This study emphasizes the critical role that aggressive glaucoma screening, effective treatment, and strong support networks play in reducing the impact of visual impairments and improving the overall health of those who are affected.

Chondromyxoid Fibroma In Skull: An Unusual Site

Chondromyxoid fibroma are the rarest of the benign cartilaginous neoplasms. Mostly it involves metaphysis of long bones, however, rarely can be found in calvarium / skull base. We report a case of a 30 year-old female who was referred to the Radiology Department at Shifa International Hospital, Islamabad with history of progression and gradual loss of vision in unilateral eye. The patient had contrast enhanced MRI of brain which showed a heterogeneously enhancing mass lesion abutting the clivus, CP angle and brain stem with internal areas of necrosis. Differentials of chordoma, meningioma and chondrosarcoma were given. The patient then underwent surgery which confirmed the mass to be chondromyxoid fibroma

A review on early diagnosis of glaucoma using machine learning techniques

Glaucoma refers to the accumulated loss of retinal cells within the optic nerve or the gradual visual loss caused by optic neuropathy. It is an illness that affects vision in the eye and is considered an irreversible condition that leads to degradation of eyesight. There are often no early warning signs of glaucoma, making it difficult to notice changes in vision due to subtle effects. To date, a large number of Deep Learning (DL) models have been developed for the accurate diagnosis of glaucoma. This work proposes an architecture for deep learning-based glaucoma detection using Convolutional Neural Networks (CNNs). CNNs can distinguish between patterns associated with glaucoma and non-glaucoma conditions, providing a hierarchical structure for classification. Using the proposed method, the disease is detected based on the optic cup-to-disc ratio. The diagnosis is further enhanced by integrating an image data generator for data augmentation. The results demonstrate that the proposed model achieved 98% accuracy, outperforming many existing algorithms.

Vogt‐Koyanagi‐Harada‐like findings as the first manifestation of neuroleukaemia

Aims/Purpose: To report a well‐documented case of a 62‐year‐old woman presenting with VKH‐like findings as the first manifestation of neuroleukaemia.Methods: Case study.Results: A 52‐year‐old woman who had received a diagnosis of acute myeloid monoblastic leukaemia (AML) one month prior and was in remission after the induction phase presented with floaters and gradual visual loss in both eyes. Visual acuity was 20/200 and 20/25, respectively. Anterior segment exam was normal in both eyes. Funduscopy showed reduced foveal reflex. On spectral‐domain OCT, subretinal fluid was found in both eyes. Fluorescein angiography showed hypofluorescent areas compatible with multifocal serous retinal detachments and multiple hyperfluorescent dots surrounding the detached areas. Days after, the patient debuted with facial palsy and binocular diplopia due to bilateral VI cranial nerve (CN) palsy, left VII CN palsy and right complete III CN palsy. An MRI was performed with no signs of infiltration. A cerebrospinal fluid sample was drawn by lumbar puncture and its analysis denoted infiltration of AML cells with similar immunophenotype to that on initial bone marrow biopsy. Our patient presented with an atypical case of Vogt‐Koyanagi‐Harada (VKH) with multiple leakages along the periphery of the detached retinal area. This sparing of the macula should be considered a suspicious pattern, and combined with the finding of CN palsies was enough to suspect a relapse in the form of CSF infiltration, making the diagnosis of neuroleukaemia possible.Conclusions: Exudative retinal detachment can be a primary presentation of AML or denote a relapse. VKH is a diagnosis of exclusion, as one of the main criteria for its diagnosis is the absence of other systemic diseases. This becomes especially important in atypical presentations and older patients. So as not to miss life‐threatening underlying conditions, leukaemia and lymphoma should be considered in the differential diagnosis of VKH.

An unusual case of bilateral type 1 idiopathic macular telangiectasia

Purpose: To report an unusual case of Type 1A idiopathic macular telangiectasia (IMT).Methods: A single case presentation.Results: A 54‐year‐old female presented with gradual vision loss in both eyes. Best‐corrected visual acuity (BCVA) was limited to counting fingers in both eyes. Fundus examination showed bilateral hard macular exudates associated with small macular telangiectasia. Fluorescein angiography (FA) revealed early bilateral macular punctuated hyperfluorescence corresponding to macular telangiectasia. Macular swept‐source optical coherence tomography (SS‐OCT) showed bilateral cystoid macular oedema associated with serous retinal detachment. OCT‐angiography (OCT‐A) showed clearly some of the perifoveal aneurysmal dilations and telangiectasia. Based on these findings, the patient was diagnosed with bilateral type 1A IMT according to Gass and Blodi classification and was treated with intravitreal anti‐vascular endothelial growth factor (anti‐VEGF) injections. Three months later, SS‐OCT revealed partial regression of the exudative signs and BCVA improvement.Conclusions: We described a case of a bilateral Type 1A IMT, affecting a middle‐aged woman, without peripheral involvement. Usually, this entity is unilateral and evolving the mid periphery. Macular SS‐OCT helped to visualize the extent of retinal involvement in the presence of masking macular hard exudates. OCT‐A visualized some of the telangiectasias however, compared to FA, some vascular abnormalities were undetectable, probably due to a turbulent or low blood flow. Treatment with intravitreal Anti‐VEGF can lead to incomplete response.

Effect of Kachayapanam Kuzhambu Anjana in the Management of Kaphaja Kacha(immature Cataract)

INTRODUCTION :-Kacha is one among the Dristigata roga of Netraroga explained in Ayurvedic classics. When the dosas gets localized in the 3rd Patala of netra, the person gets the disease called Kacha, in which he sees objects present above but not below, objects seen are covered with thin cloth, area of vision assumes the colour related to dosha and gradually the vision diminishes. Immature cataract is charecterized by a variable amount of opacification, present in certain areas of lens. It is a vision impairing disease charecterised by gradual progressive loss of vision. It is one of the leading causes of preventable blindness.MATERIALS AND METHODS:- A patient with presenting symptoms of kaphaja kacha or immature cataract was selected from the Shalakya Tantra OPD of ITRA Jamnagar. Both internal as well as local treatments were given to the patient.RESULTS:- Patient showed marked improvement in the Visual Acuity both aided and unaided after 3 months of treatment.DISCUSSION:-Anjana is one among the Netra Kriyakalpas in which medicine is applied as collyrium in the eyes. It is administered only when the Amavastha is removed and the dosas are localised only on the eyes. In this case Anjana played a major role in reducing and controlling the progress of immature cataract or Kacha.

Geographic atrophy: pathophysiology and current therapeutic strategies.

Geographic atrophy (GA) is an advanced stage of age-related macular degeneration (AMD) that leads to gradual and permanent vision loss. GA is characterized by the loss of photoreceptor cells and retinal pigment epithelium (RPE), leading to distinct atrophic patches in the macula, which tends to increase with time. Patients with geographic atrophy often experience a gradual and painless loss of central vision, resulting in difficulty reading, recognizing faces, or performing activities that require detailed vision. The primary risk factor for the development of geographic atrophy is advanced age; however, other risk factors, such as family history, smoking, and certain genetic variations, are also associated with AMD. Diagnosis is usually based on a comprehensive eye examination, including imaging tests such as fundus photography, optical coherence tomography (OCT), and fluorescein angiography. Numerous clinical trials are underway, targeting identified molecular pathways associated with GA that are promising. Recent approvals of Syfovre and Izervay by the FDA for the treatment of GA provide hope to affected patients. Administration of these drugs resulted in slowing the rate of progression of the disease. Though these products provide treatment benefits to the patients, they do not offer a cure for geographic atrophy and are limited in efficacy. Considering these safety concerns and limited treatment benefits, there is still a significant need for therapeutics with improved efficacy, safety profiles, and better patient compliance. This comprehensive review discusses pathophysiology, currently approved products, their limitations, and potential future treatment strategies for GA.

Genetic and molecular biomarkers for geographic atrophy.

Geographic atrophy (GA) is characterized by atrophy of the retina, retinal pigment epithelium and choriocapillaris, causing a gradual loss of vision over time. Treatment options to prevent initiation or progression of GA are limited; two recently FDA-approved inhibitors of the complement system (pegcetacoplan, avacincaptad pegol) showed a modest decrease in GA lesion growth in phase 3 clinical trials. Exploration of genetic and molecular biomarkers in GA plays a critical role in our battle against this blinding disease to improve early disease detection, to find more effective therapies, and to provide personalized care to patients. In this review, we provide a comprehensive overview of the current literature investigating genetic and molecular biomarkers for GA. Genetic studies identified multiple genes and variants that play a role in progression to GA and GA lesion growth, involving pathways such as complement activation, extracellular matrix interaction and lipid metabolism. The number of published studies assessing molecular biomarkers for GA initiation and progression in ocular matrices is limited. Several studies evaluated molecular biomarkers in the systemic circulation, showing higher levels of complement activation and a causal role of lipid subfractions in GA. Larger, well-powered studies are needed to identify novel and validate existing biomarkers, and to investigate the potential of combining genetic and molecular markers with imaging techniques for more accurate diagnosis and monitoring of GA. The development of personalized medicine approaches based on individual genetic and molecular profiles could hold promise for more effective and targeted treatments for this devastating disease.

Abstract 116: Reversal of Acute Ocular Ischemic Syndrome Following Rescue Carotid Revascularization: A Case Report

Introduction Ocular ischemic syndrome (OIS) is a rare, vision‐threatening condition resulting from severe carotid artery disease secondary to severe atherosclerosis or arteritis. Common clinical features include acute/subacute to gradual vision loss, ocular pain, amaurosis fugax, photophobia and diplopia. We are presenting a case of acute ocular ischemic syndrome secondary to acute re‐occlusion of the right internal carotid artery (RICA) post Carotid Endarterectomy (CEA), that reversed with carotid revascularization. Methods Case Summary: 59‐year‐old male with history of T2DM, hyperlipidemia, tobacco use presented to an outside hospital with complaints of dizziness, blurry vision, left upper extremity weakness and multiple falls with head trauma. Exam revealed left lower quadrantanopia, mild left arm weakness and left hemineglect. Computed Tomography (CT) Head showed scattered right middle cerebral artery (MCA) territory infarcts. CT Angiography (CTA) revealed ulcerated plaque with a small focus of thrombus intraluminally in the RICA. Subsequently, the patient was initiated on heparin infusion and underwent CEA. On postoperative day (POD) 2, the patient experienced severe stabbing right eye pain with reduced visual acuity and worsening of left arm weakness. Imaging revealed complete occlusion of the right common carotid artery (RCCA). Perfusion imaging did not reveal a core or a penumbra. Given acute loss of vision likely secondary to ocular ischemic syndrome, decision was taken to proceed with rescue stenting. Using a transfemoral approach, an 8‐French balloon guide catheter was advanced into the proximal RCCA. A primary suction thrombectomy was performed under proximal flow arrest. After achieving a complete recanalization in one pass, we started the patient on cangrelor 30 mcg/kg intravenous bolus followed by 4 mcg/kg/min infusion. Post bolus, we placed a tandem closed‐cell carotid stent measuring 8x36mm & 10x37mm. The patient reported immediate relief of ocular pain, vision and weakness following the procedure. He transitioned from cangrelor infusion to ticagrelor 90 mg twice daily and aspirin 81 mg daily. On post‐stenting day 7, the patient experienced acute onset headache, and imaging revealed re‐occlusion of the entire carotid artery sparing only the ophthalmic segment of the RICA which now filled retrograde via anterior communicating artery (AcoA) from the contralateral carotid artery. However, his visual acuity remained unchanged and reported no ocular pain. At one month follow‐up the patient remains symptom‐free. Results Discussion: Incidence of OIS is estimated to be 7.5 cases per million every year. Carotid artery atherosclerosis is the most common etiology. Majority of OIS (∼90%) present with gradual vision loss and approximately 40% present with ocular pain. Gradual vision loss is predominantly related to chronic ocular hypoperfusion and resultant iris neovascularization and glaucoma. In this case, patient suffered acute vision loss secondary to acute ICA occlusion following CEA. Emergent carotid revascularization resulted in restoration of vision and resolution of ocular pain. Conclusion Our main goal is to emphasize the importance of rescue stenting to address symptoms of aOIS. Immediately post‐stenting, there was remarkable improvement in ocular pain and visual acuity. Although, the carotid re‐occluded within seven days post‐stenting, we hypothesize that gradual re‐occlusion while on DAPT provided enough time recruit and preserve collaterals into the right ophthalmic artery via retrograde flow into the ophthalmic segment of RICA from the contralateral carotid artery via the AcoA which was subsequently confirmed on imaging.