Sudden Painless Loss Of Vision Research Articles

Atypical central retinal artery occlusion as the first presentation of POEMS syndrome: a case report

BackgroundPOEMS syndrome is a plasma cell disorder, which clinically manifests from paraneoplastic syndrome: polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. The most common ocular manifestation is optic disc swelling, whereas other ocular manifestations; cystoid macular edema, serous macular detachment, venous sinus thrombosis, infiltrative orbitopathy, uveitis, neovascularization of the disc, peripapillary choroidal neovascularization and optic disc drusen, had also been reported.Case presentationA 52-year-old Thai man presented with 5-day sudden painless visual loss in the left eye. Ocular examination revealed visual acuity of 20/20 and no light perception in the right and left eye, respectively. Right fundoscopic examination was significant for hyperemic generalized optic disc swelling. Left fundoscopic examination revealed opaque and edematous entire retina giving the appearance of central retinal artery occlusion (CRAO) along with pallid “chalky white” optic disc swelling. Fluorescein angiography showed profound leakage of bilateral optic nerve heads and arteriolar filling defect in macular area along with leakage of small retinal arterioles in the left eye. Indocyanine green angiography demonstrated choroidal filling defect in the left eye only. Neuroimaging showed enhancement and luminal narrowing of left internal carotid artery, early subacute watershed infarctions in the left cerebral hemisphere and pachymeningeal enhancement. Cerebrospinal fluid analysis revealed high protein level with normal opening pressure. Intravenous methylprednisolone was initially started without any benefit. After extensive investigations, diagnosis of “POEMS syndrome” was made based on polyneuropathy, elevated lambda light chain level, elevated plasma vascular endothelial growth factor (VEGF), hepatomegaly, spinal sclerotic bone lesions, and thrombocytosis. Furthermore, sural nerve biopsy demonstrated neuropathy and positive VEGF staining. He was treated with eight cycles of bortezomib, cyclophosphamide and dexamethasone (BorCyDex). Polyneuropathy and thrombocytosis had remarkably improved after 2nd cycle, whereas, visual impairment had shown no recovery. Hepatomegaly was significantly reduced after the completion of BorCyDex. Our case eventually received autologous hematopoietic stem cell transplantation with high dose melphalan.ConclusionsTo our knowledge, we illustrated the first patient given CRAO as the first presentation and ocular finding ever reported in POEMS syndrome. Both cerebral and ocular infarctions were presumably the result of VEGF-induced cranial vasculopathy as evidenced by neuroimaging.

Ophthalmic artery occlusion following neuro-embolization of the external carotid artery, a case report

BackgroundEmbozene® is a new neuroembolizing microsphere used to reduce intraoperative bleeding for head and neck tumours. We report a case of iatrogenic ophthalmic artery occlusion after Embozene® embolization of the external carotid artery (ECA).Case presentationA 22-year-old African gentleman presented with left nasal obstruction and epistaxis for 2 years and was diagnosed with nasopharyngeal carcinoma. He subsequently underwent embolization of the maxillary branch of the left ECA using Embozene® Microspheres - 250 μm in size before endoscopic tumour excision to reduce intra-operative bleeding. He complained of sudden painless profound visual loss in the left eye (LE) two hours after embolization. Visual acuity in LE was no light perception. Fundus examination showed pale retina with no cherry red spot. Arterial narrowing and segmentation were seen in all quadrants. A diagnosis of left ophthalmic artery occlusion was made. Despite immediate management including ocular massage and lowering of intraocular pressure, the visual loss remained. Retrospective review of digital subtraction angiogram showed an anastomosis between the left ophthalmic artery and anterior deep temporal artery as a potential route for microspheres migration.ConclusionPre-operative angio-architecture understanding and diligent selection of embolic material are helpful in preventing this adverse event. The use of newer agents for embolotherapy may cause migration of embolic material from the external to the internal carotid system leading to ophthalmic artery occlusion and blindness.

Bilateral retinitis following typhoid fever

BackgroundPost typhoid fever immune related reactions affecting the eye is a rare finding which can have various presentations in which typhoid retinopathy is not a well recognized sequelae.Case presentationHere we present a case of 59 year old male who presented with right eye sudden painless loss of vision 4 weeks after typhoid fever which was diagnosed and treated successfully. His BCVA was 2/60 in right eye and 6/6 in left eye. Fundus examination showed retinitis along with macular serous detachment in right eye and retinitis in left eye. Significant improvement in BCVA in right eye was observed after treatment with oral steroid with resolving retinitis lesions. Diagnosis of post typhoid immune mediated retinitis was made with good resolution following treatment.ConclusionsImmune mediated retinitis is a rare sequelae to typhoid infection which can be successfully treated with systemic steroids with good resolution of the lesions.

Bilateral Simultaneous Nonarteritic Anterior Ischemic Optic Neuropathy Associated with The Use of Sildenafil: A Case Report

Abstract Introduction: Nonarteritic anterior ischemic optic neuropathy (NAION) is a rare condition but one of the most important ocular side effects of sildenafil. Case Presentation:We presented a 65-year- old man with the history of sudden painless loss of vision in both eyes following taking sildenafil. Conclusions: Fundus examination revealed blurred margin and elevated disc in both eyes. Visual field defects were bilateral inferior altitudinal defects, and optical coherence tomography (OCT) displayed a prominent nerve fiber layer edema in both eyes. We asserted that use of sildenafil may lead to reduce blood pressure and interfere with microcirculation in the optic nerve head. So, the potential risks of unregulated over-the-counter sexual enhancement supplements such as sildenafil must be educated to the patients using these supplements.

Branch retinal artery occlusion in a patient with one eye. Laser Nd:YAG approach

Clinical caseAn 82-year-old man was admitted to the emergency department complaining of a sudden painless visual loss in his left eye (OS). He was diagnosed with branch retinal artery occlusion (BRAO) with a visible embolus. In 2012, he had a central artery occlusion (CRAO) in his right eye (OD). An embolysis with Nd:YAG laser was attempted, the retinal arterial blood flow was restored completely and the visual field was improved, with no secondary complications. ConclusionsNd:YAG laser embolysis is a treatment to be considered in patients with BRAO with a visible embolus. The risks and benefits of the procedure should be evaluated, comparing it with possible permanent loss of visual acuity and other vascular complications caused by BRAO.

Oclusión de rama arterial retiniana en un paciente con ojo único. Abordaje mediante láser Nd:YAG

Clinical caseAn 82-year-old man was admitted to the emergency department complaining of a sudden painless visual loss in his left eye (OS). He was diagnosed with branch retinal artery occlusion (BRAO) with a visible embolus. In 2012, he had a central artery occlusion (CRAO) in his right eye (OD). An embolysis with Nd:YAG laser was attempted, the retinal arterial blood flow was restored completely and the visual field was improved, with no secondary complications. ConclusionsNd:YAG laser embolysis is a treatment to be considered in patients with BRAO with a visible embolus. The risks and benefits of the procedure should be evaluated, comparing it with possible permanent loss of visual acuity and other vascular complications caused by BRAO.

A case of sudden painless loss of vision with partial spontaneous recovery.

A case of sudden painless loss of vision with partial spontaneous recovery.

Optic Disk Pit with Sudden Central Visual Field Scotoma.

Purpose. To describe a case of optic disk pit (ODP) with sudden central visual field scotoma. Methods. A 49-year-old woman presented, reporting sudden painless central visual field loss 3 months prior to presentation. Neuroophthalmologic, systematic, and laboratory evaluation and full imaging processes were performed. Results. Fundoscopy and color photography demonstrated an optic disk pit inferotemporally. Perimetry identified central visual field horizontal scotoma. OCT revealed absence of serous retinal detachment, but disclosed inner retina thinning corresponding to the area of the visual field loss. Fluorescein angiography demonstrated delay in the cilioretinal arteries and also disclosed a relative delay in the perfusion of an arterial branch off the inferior retinal arcade. Clinical and laboratory evaluations were negative for any related pathology. Conclusion. Sudden central visual field scotoma in patients with ODP may be associated with delayed vascular filling of CRA and retinal arterioles within the optic disc anomaly region.

Idiopathic vitritis in the setting of Boston keratoprosthesis.

The aim of this study was to revisit the clinical paradigm attributed to Boston keratoprosthesis recipients presenting with idiopathic vitreous inflammation. A retrospective chart review was performed of keratoprosthesis recipients at Massachusetts Eye and Ear Infirmary, from January 2000 to August 2013, for demographic data, indication(s) for surgery, timing and presentation of vitreous inflammation, and best-corrected visual acuity at baseline, on presentation, and after resolution of vitritis. Twenty-three (23 eyes) of 346 patients developed idiopathic vitreous inflammation after keratoprosthesis implantation. Six of 23 patients presented with signs and symptoms similar to infectious endophthalmitis but were culture negative. The proportion of patients who fit the previous paradigm of sudden painless loss of vision without external signs of infection ("sterile vitritis") at their first presentation with vitritis was only 4 of 23. Vision decline was variable (median, 9 lines on Snellen chart; range, 0-24), as was time to recovery of best vision (median, 8.9 weeks; range, 0.9-36.7). Nine eyes had repeat bouts (43 episodes in 23 patients). Ten of 43 episodes did not recover to baseline vision. Seventeen of 23 eyes with idiopathic vitritis after keratoprosthesis later developed other complications. The current paradigm for idiopathic vitritis after keratoprosthesis implantation includes sudden painless loss of vision with full recovery of vision on treatment with periocular corticosteroids. However, idiopathic vitritis after keratoprosthesis can also mimic infectious endophthalmitis with pain and external signs of inflammation. Visual loss can be gradual. Vision may not recover to baseline despite treatment. Vitritis may be a part of a common pathway of chronic inflammation after keratoprosthesis.

Visual Field Defect after Cardiac Surgery: The Striking Role of Interdisciplinary Collaboration.

Perioperative visual loss (POVL) is a potentially devastating complication that can occur following ocular or nonocular surgery. The leading causes of this disease are retinal vascular occlusions, ischemic optic neuropathies, and cortical blindness. POVL pathogenesis is strictly influenced by surgery, anesthesia, and patients' comorbidities. We report of a 55-year-old caucasian man who presented with complaints of sudden painless loss of vision and unilateral campimetric deficit. We recorded a preserved visual acuity but at fundus examination a bilateral ischemic optic neuropathy (ION) was suspected. Our hypothesis was supported by uncommon and peculiar visual field defects and a history of cardiovascular surgery shortly before was a striking data. When we examined his medical records we found strong accordance with what is reported in literature to be risk factors for postoperative ION development. He presented intraoperative hypotension, anemia, and hypothermia, he was older than 50 years, and surgery lasted for more than five hours. We are currently monitoring his visual acuity and visual fields which remain unchanged. As there is no proved therapy for such severe adverse events, we recommend intraoperative check of blood pressure, blood loss, and body temperature, associated with repeated eye checks and patients' interview.

Single intravitreal aflibercept injection for unilateral acute nonarteritic ischemic optic neuropathy.

Acute nonarteritic ischemic optic neuropathy (ANAION) is the most common optic neuropathy in the elderly population without a well-established treatment. A 67-year-old man with a sudden painless visual loss in his left eye of one-day duration was diagnosed to have left ANAION. Next day, 2 mg aflibercept injection was injected intravitreally in OS. Visual acuity improved to 7/10 from 1/10 a week after the injection. Mean retinal nerve fiber layer thickness (RNFLT) was reduced to 159,7 μm from 182,4 μm at the first week. Visual fields improved dramatically during the follow-up of three months. The aim of this study is to present a case having ANAION treated with a single intravitreal aflibercept injection and discuss the place of intravitreal anti-VEGF injections in the treatment of armamentarium of ANAION.

Ocular metastasis from a rare thyroid carcinoma

Objective: To report a case of primary thyroid carcinoma with ocular metastasis.Methods: This is a case report of a 39-year-old male, who initially presented with a sudden painless loss of vision in the left eye and an anterior neck mass. He was initially diagnosed to have retinal detachment with vitreous hemorrhage based on ocular ultrasound. Fine Needle Aspiration Biopsy of the neck mass initially revealed medullary thyroid carcinoma. Computed topography of the chest and abdominal ultrasound revealed lung and liver metastasis. Whole body bone scintigraphy also revealed bone metastasis.Results: Patient underwent total thyroidectomy with bilateral modified radical neck dissection of the neck mass and vitreous biopsy of the left eye. Histopathology report revealed malignant carcinoma of the thyroid, medullary type while vitreous showed malignant cells from the thyroid. Patient was scheduled to undergo chemotherapy for the widespread metastasis.Conclusions: Ocular malignancy can present in many ways, thus it is necessary to consider it as a differential for cases of retinal detachment and vitreous hemorrhage. Choroidal metastasis from malignant thyroid carcinoma is rare, more so the medullary type.

Embolus characterization in branch retinal artery occlusion by optical coherence tomography.

T his retrospective study analysed cases of branch retinal artery occlusion (BRAO) who had been examined by spectral-domain optical coherence tomography (SD-OCT) (Willerslev et al. 2014) to assess the characteristics of the intraluminal content of retinal arteries at the site of occlusion in BRAO. This case series included a selected group of 11 patients with BRAO seen at the Department of Ophthalmology at Glostrup Hospital between January 2010 and June 2013 and Copenhagen University Hospital Roskilde between September 2012 and August 2013, who presented with sudden painless visual loss and a visible retinal arterial plaque/embolus. All BRAOs were isolated (only one per eye) and located in a branch of a temporal vascular arcade artery and had an ophthalmoscopically visible plaque. All patients had undergone infrared 30 degree fundus photography and SD-OCT (Spectralis, Heidelberg Engineering, Heidelberg, Germany) at variable times during work-up. Scans are shown with brighter areas representing higher signal intensity at the conventional 2:1 height:width ratio (Sander et al. 2005). Fluorescein angiography, carotid artery Doppler ultrasonography and echocardiography were available in selected patients. In all cases, SD-OCT demonstrated endoluminal material at the site of arterial occlusion, the structure of which was divided into two distinctly different categories (exemplified in Fig. 1). A flake of highly reflective, yet transparent material, oriented with its edge towards the direction of flow and positioned in the middle of the artery was seen in three cases (Fig. 1). The reflectivity profile of the blood above and below the flake on SD-OCT and the fluorescein angiograms were normal, indicating that these emboli had little or no effect on blood flow. Amorphous or gritty endoluminal material was seen in 8 cases, all of which showed perfusion centrally, in what may have been a recanalized portion of the embolus, the remaining embolus material giving the appearance of a grossly thickened artery wall. All but one patient had risk factors for thromboembolic disease such as arterial hypertension and/or hyperlipidemia and their medication included antiplatelet drugs and/or statin. Antiplatelet drugs and statin were initiated in one patient after diagnosis of BRAO. None of the patients had known carotid atherosclerosis or stenosis before the incidence of BRAO. After clinical work-up, 6 of 11 cases were diagnosed with carotid atherosclerosis or stenosis but none with need for carotid artery surgical intervention. The cases presented here demonstrate that SD-OCT can visualize retinal artery emboli and demonstrate perfusion characteristics and embolus structure, from totally occluded artery segments over recanalized emboli to crystalline emboli. Only two case reports have previously been published on this subject (Shah et al. 2010; Oster et al. 2009). The non-invasive nature of SD-OCT and its ability to image most patients without pupil dilation offers advantages over fluorescein angiography when time is of the essence. Emboli with crystalline characteristics (flat, angular outline, highly reflective, transparent) may be taken to indicate that these were cholesterol flakes dislodged from an atheromatous arterial lesion. The other emboli appeared to take the shape of the arterial lumen, indicating that they were soft. Theoretically such emboli could have originated as thrombi from the atria of patients with atrial fibrillation or from peripheral veins in patients with a patent foramen ovale (Hayreh 2011). Six of 11 patients had carotid artery atherosclerosis which could be a potential source of emboli to the retinal circulation. In the presence of a retinal artery occlusion such a potential source of emboli should mandate consideration of therapeutic options aimed at preventing new embolism (Chang et al. 2012), such as the administration of statins and antiplatelet therapy. Recanalization seemed to be present in all 8 amorphus emboli. The intravascular reflectivity profile depends on the rate of blood flow (Willerslev et al. 2014) and could therefore be turbulent or abnormally high at a vascular stenosis produced by a retinal embolus. However, given the small diameter of the lumen of the observed stenoses, such details could not be resolved. An

Central retinal vein occlusion in a patient with retinal vasculitis and Crohn's disease.

The authors report a rare case of a 47-year-old woman with Crohn's disease (CD) who presented with retinal vasculitis and central retinal vein occlusion (CRVO) during remission. The patient complained of sudden painless visual loss in her left eye (OS). Ophthalmologic evaluation revealed a best corrected visual acuity (BCVA) of 20/20 in the right eye and hand movements in OS. Ophthalmoscopy and fluorescein angiography of OS showed signs of nonischemic CRVO and extensive vasculitis. She was treated with oral prednisolone, mercaptopurine, and intravitreal bevacizumab in OS. After 1 month of treatment, VA of OS improved to 5/10 and after 1 year it was 10/10 with complete resolution of retinal vasculitis and nonischemic CRVO.

Research advances in the risk factors of nonarteritic anterior ischemic optic neuropathy

As the most common acute optic neuropathy among individuals who are over 50-year old,non arteritic anterior ischemic optic neuropathy ( NAION) is presumed result from a circulatory insufficiency,or infarct, within the lamina or retrolaminar portion of the optic nerve head that is supplied by the short posterior ciliary arteries. It usually presents with sudden painless unilateral visual loss, characterized visual field defects, relative afferent papillary defect and pallid optic disk edema. The exact etiology of NAION remains elusive. Although numerous risk factors have been proposed, most of them are controversial. We summarized all these risk factors in this review.

Unusual presentation of ocular tuberculosis: multiple chorioretinitis, retinal vasculitis and ischaemic central retinal vein occlusion

A 54‐year‐old male patient presented with a sudden painless visual loss in his left eye. Ophthalmic examinations revealed panuveitis, ischaemic central retinal vein occlusion, multiple chorioretinitis and retinal vasculitis. The diagnosis of tuberculosis was confirmed with anterior tap analysis and QuantiFERON‐TB test. Anti‐tuberculosis treatment and intravitreal anti‐vasculature endothelial growth factor therapy were performed.

English

AIM: To report a case of Central Retinal Artery Occlusion (CRAO) in young males in its initial manifestation as Primary Antiphospholipid Syndrome. METHODS: 32 year healthy male, with abrupt sudden painless loss of vision in right eye since 48 hours, with Grade 2 Relative afferent pupillary defect, visual acuity of hand movements in OD and 6/18 in OS. Fundoscopy disclosed signs compatible of central retinal artery occlusion confirmed with FFA. Carotid Doppler imaging and echocardiography was done to determine the source. RESULTS: Antiphospholipid antibody cofactor, beta2-glycoprotein 1 antibodies, IgM, was positive with titre of more than 94 un its/ml on two occasions, 12 weeks apart, with normal range being less than 20 units/ml for each isotope (IgG, IgM, or IgA).According to the 2006 revised Sapporo criteria Antiphospholipid syndrome was diagnosed. Thorough examination excluded other system involvement. Immunological studies excluded other systemic disorders. CONCLUSIONS: In literature, prevalence of CRAO is 0.85% for every 100000 and prevalence of Antiphospholipid Syndrome in patients showing a major retinal vascular obstruction is 5% - 33%. Antiphospholipid syndrome should be ruled out in every young patient who presents with Central retinal artery occlusion. Association must be considered, as Central retinal artery occlusion could be the initial manifestation of antiphospholipid syndrome with high risk of recurrence.

Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage

Bilateral anterior ischemic optic neuropathy is a rare complication of massive haemorrhage and related hypotension and anaemia in young individuals. We report a 34-year-old woman with bilateral non-arteritic ischemic optic neuropathy (NAION) after a massive spontaneous abortion-related haemorrhage who presented with sudden painless visual loss in her left eye. Visual acuity was 20/20 in the right eye with only hand motion discernible in the left eye. There was a left relative afferent papillary defect (RAPD). Fundus examination revealed bilateral swollen, hyperaemic optic discs and nerve fiber layer haemorrhages. Brain MRI and magnetic resonance venography were normal. The diagnosis of bilateral NAION was made and intravenous pulse corticosteroid therapy (1000mg/day) was administered for three days. On the sixth day, optic disc oedema regressed bilaterally and on the third week, the visual acuity improved to 20/80 in the left eye. The visual field showed only a small spared area in the nasal region, and persistent RAPD was present. After two months, fundus examination showed a small and crowded optic disc on the right and a pale optic disc on the left. Severe acute haemorrhage is an important risk factor for NAION in healthy young individuals. In addition to correction of hypotension and anaemia, intravenous high dose corticosteroid might be beneficial for treatment.

Acute Optic Neuropathy Associated With an Intracranial Mass in a Patient With POEMS syndrome

A 43-year-old man with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes), including long-standing optic disc swelling, had sudden painless vision loss in the left eye. Brain MRI revealed an intracranial mass adjacent to the left optic nerve and enhancement of the optic nerve. The mass decreased in size following chemotherapy for myeloma with some recovery of vision. This represents a unique case of optic neuropathy due to presumed plasmacytoma in osteosclerotic IgA myeloma and POEMS syndrome.

A Woman with Unilateral Sudden Painless Vision Loss

A 46-year-old woman presented to the emergency department with a sudden onset of blurry vision in her left eye that started 1 week ago and remained persistent. At evaluation, her visual acuity was 20/40 in the left eye and 20/20 in the right eye. A bedside ophthalmoscopic examination was performed and she was referred to ophthalmology for fluorescein angiography and further care.