Bilateral Optic Disc Edema Research Articles

Combined therapy with intravitreal aflibercept and subtenon corticosteroids in eyes with severe diabetic papillopathy: two case reports

BackgroundDiabetic papillopathy is a rare diagnosis of exclusion characterized by unilateral or bilateral optic disc edema with variable degrees of visual loss. Although the visual prognosis has been generally reported as favorable, the presence of severe disc edema associated with macular edema prompts the need for treatment. We present a specific and unreported therapeutic approach consisting of intravitreal aflibercept and subtenon triamcinolone acetonide injections in two patients with evidence of diabetic papillopathy and macular edema.Case presentationIn the first case, a 60-year-old Caucasian woman affected by type II diabetes mellitus presented with fundoscopic evidence of sequential bilateral optic disc edema associated with acute severe visual loss in both eyes. The second patient, a diabetic 57-year-old Caucasian male, presented with sudden painless visual loss in his left eye. Multimodal imaging and systemic findings correlated towards an infrequent diagnosis of diabetic papillopathy. In a period of 5–7 weeks after treatment, both patients experienced almost full visual and anatomical recovery. A steady situation was observed at 12 months of follow-up.ConclusionsBoth our cases displayed a severe grade of optic disc edema, which was optimally reversed with intravitreal aflibercept and subtenon triamcinolone acetonide leading to a relatively rapid and safe improvement in visual acuity.

Diagnosis of Acute Promyelocytic Leukemia After Presentation to Neuro-Ophthalmology.

Acute promyelocytic leukemia (APML) is a medical emergency that can initially present with neuro-ophthalmologic signs. Early recognition is crucial, and immediate treatment with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) can be life-saving. The goal of this study was to describe patients who first presented to neuro-ophthalmology and were subsequently diagnosed with APML. We retrospectively reviewed consecutive patients seen at a tertiary neuro-ophthalmology centre. Patients with an unknown diagnosis of APML at presentation who subsequently went on to receive this diagnosis were included. Clinical characteristics, neuro-ophthalmologic findings, and outcome were retrieved. A total of 3 patients (2 women and 1 men) with a mean age of 30.7 (range 24-33) years were included in the study. Neuro-ophthalmologic diagnoses at presentation were severe hemorrhagic papilledema related to dural venous sinus thrombosis, hemorrhagic bilateral optic disc edema, and left homonymous hemianopia related to an occipital lobe hemorrhage. At diagnosis, the average hemoglobin was 83.7g/L (range 78-104), and the platelet count was 39.3 × 109/L (range 15-77). All patients were treated with ATRA and ATO. One patient developed papilledema and sixth nerve palsies related to this treatment, which resolved with acetazolamide. Clinical follow-up ranged from 6 to 12 months, and all patients were in clinical remission about systemic APML. Neuro-ophthalmologic symptoms may be the first manifestations of APML, and a complete blood count is an essential test in patients presenting with optic disc edema, especially if hemorrhagic.

Bilateral Optic Disc Edema in Multisystem Inflammatory Syndrome in Children Associated With COVID-19.

Bilateral Optic Disc Edema in Multisystem Inflammatory Syndrome in Children Associated With COVID-19.

Bilateral Optic Disc Edema in a Patient with Lead Poisoning

This is a Letter to the Editor and does not have an abstract. Please download the PDF or view the article HTML.

Vision impairment as the first symptom of encephalomyelitis in a paediatric patient

An 8-year-old boy was admitted to the Ophthalmology Department due to a vision impairment with an accompanying decrease in visual acuity and bilateral optic disc oedema. As a result of interdisciplinary diagnostics, acute disseminated encephalomyelitis, probably related to preceding infection, was diagnosed.

Idiopathic intracranial hypertension with initial presentation of optic disc edema

Abstract Introduction: Idiopathic intracranial hypertension (IIH) is a disorder of elevated intracranial pressure in young women of childbearing age. The typical symptom is headache, and the symptom of visual obscuration tends to be overlooked. So, the patients usually visit the department of neurology for headache management. Here, we report a patient with IIH who first presented with ophthalmic symptoms. Patient concerns: A 28-year-old overweight female complained of intermittent darkened vision in both eyes lasting for 2 months. She was diagnosed with papilledema at another hospital and received local and systemic application administration of neurotrophic drugs and glucocorticoids 1 month before. Because of the poor outcomes, she visited our ophthalmology clinic for further management. She had no other medical history. Diagnosis: On ophthalmic examination, bilateral optic disc edema with blurred boundary was observed. The cranial magnetic resonance imaging and blood examination showed no obvious abnormality. A lumbar puncture was performed by a neurologist, which demonstrated an opening pressure of 30 cm of water. The cerebrospinal fluid biochemistry and bacteriology results were normal. A diagnosis of IIH was made based on the above findings. Outcomes: Oral acetazolamide, weight loss therapy and local corticosteroid treatment were initiated. At 7-week follow-up, the darkened vision and optic disc edema was obviously alleviated. Conclusion: IIH is a neurologic disease, but patients with this disease may first present to ophthalmology department because of the visual symptoms, such as blurred or darkened vision, visual loss, and even diplopia. As ophthalmologists, we should screen for this disease in a timely manner when we encounter similar patients with bilateral optic edema.

Bilateral metastasis to optic nerve head – Interesting case report series

Most intraocular metastatic tumors occur in the uveal tract, while isolated metastasis to the optic nerve is rarely found. Breast and lung cancers are the tumor types metastasizing to the eye most frequently; however, metastases of prostate cancer, melanoma, gastrointestinal tract, and renal tumors have also been reported. We describe three rare cases of optic nerve metastasis from primary carcinoma in brain, bone, and ano-rectum, which were not found in the literature previously. Bilateral optic disc edema in a patient with primary tumor elsewhere should be evaluated completely to rule out metastasis.

Bilateral Anterior Ischaemic Optic Neuropathy as First Manifestation of Henoch-Schönlein Purpura (IgA Vasculitis).

A 46-year-old man was referred to our department complaining of a bilateral progressive decrease in his visual acuity. Fundus examination revealed bilateral optic disc oedema, indicative of anterior ischaemic neuropathy (AION), and a macular star in the right eye. Laboratory analysis showed low haematocrit and haemoglobin, elevated creatinine, and increased erythrocyte segmentation rate and C-reactive protein level. Physical examination revealed the presence of purpuric rash on the trunk and the extremities. During the investigation we performed a complete laboratory and imaging examination for autoimmune collagen diseases, vasculitides and infectious diseases, which were all negative. Histologic findings of renal biopsy were compatible with IgA glomerulonephritis and thus Henoch-Schönlein purpura (HSP) diagnosis was established. The patient was treated with methylprednisolone and cyclophosphamide. Six months later, his renal function and his visual acuity had improved, and the rash had subsided. This is a rare case of AION in a patient with HSP.

Bilateral optic disc edema with preserved visual function not related to papilledema

Bilateral optic disc edema (ODE) with preserved visual function is typically a result of papilledema, but the causes of this finding in the absence of raised intracranial pressure have not been systematically evaluated. The goal of this study was to determine the causes of bilateral ODE with preserved visual function not related to intracranial hypertension to help in developing a differential diagnosis for this finding. We retrospectively reviewed 221 consecutive patients presenting to a tertiary neuro-ophthalmology practice over a period of 2 years. Four patients met the inclusion criteria with a mean age of 53 years (range 43 to 63), 2 patients were men and 2 were women. The final diagnoses were bilateral optic perineuritis secondary to p-ANCA vasculitis, bilateral incipient non-arteritic anterior ischemic optic neuropathy (NAION), hypertensive emergency, and intermediate uveitis. The patient with NAION developed an inferior altitudinal defect 4 months after presentation, but all other patients maintained normal visual function. The ODE resolved in all cases at a mean follow-up of 4.5 months (range 3 to 6 months). Optic disc edema with preserved visual function may not be related to papilledema, and this series indicates that optic perineuritis, incipient NAION, hypertensive emergency, and intermediate uveitis be considered in the differential diagnosis of these cases.

Vogt\u2013Koyanagi\u2013Harada disease-like uveitis following nivolumab administration treated with steroid pulse therapy: a case report

BackgroundImmune checkpoint inhibitors can cause various adverse effects. Recently it has been shown that Vogt–Koyanagi–Harada (VKH) disease-like uveitis can occur in patients treated with nivolumab.Case presentationA 69-year-old man developed bilateral panuveitis after nivolumab treatment for recurrent hypopharyngeal cancer. Slit lamp examination revealed bilateral granulomatous keratic precipitates, anterior chamber cells and partial synechiae. Fundus examination revealed bilateral optic disc edema and diffuse serous retinal detachment. His human leukocyte antigen (HLA) typing showed HLA-DRB1*04:05 allele. A lumbar puncture did not demonstrate pleocytosis. Bilateral sub-tenon injections of triamcinolone acetonide were initiated. As his panuveitis did not regress completely, steroid pulse therapy was administered. That therapy led to the resolution of his serous retinal detachment and to rapid improvement in his vision. Following this, we treated him with 50 mg/day of prednisolone for 1 week and then reduced it by 5 mg every week. No bilateral uveitis relapse had occurred by his 3-month follow-up; however, he subsequently died because of his cancer.ConclusionTo our knowledge, this is the first report of a patient with NVKH who underwent a lumbar puncture. Unlike VKH, our case did not show meningismus or pleocytosis. NVKH may, therefore, have a different etiology from VKH.In cases of NVKH with posterior uveitis, steroid pulse therapy may be considered as a treatment option, as it is in VKH.

Bilateral Optic Disc Edema Associated with Pyogenic Spondylitis

Bilateral Optic Disc Edema Associated with Pyogenic Spondylitis

Bilateral optic disc edema: causes and clinical features at a tertiary center in South India

Aims: Bilateral optic disc edema: causes and clinical features at a tertiary center in South India. Bilateral Optic disc edema is one of the important fundus findings. Many times it indicates systemic pathology. The current study evaluated various causes of bilateral optic disc edema. Settings and Design: Prospective study. Methods and Material: This prospective study was conducted at a tertiary hospital in north Karnataka part of India (southern India). This study was conducted from June 2016 to November 2018. Detailed ocular examination including vision, slit-lamp examination, Visual field analysis, detailed fundus evaluation using an indirect ophthalmoscope, 78 Dioptre lens and fundus camera was done. Systemic investigations like blood examination and radiological investigations like Computerized tomography (C.T), Magnetic Resonance Imaging (MRI) were done whenever required. Results: A total of sixty-three cases with bilateral optic disc edema was recorded in this study. Thirty were females and thirty-three were males. Out of these, 36 cases had papilledema (increased intracranial pressure), 7 cases had optic neuritis, 6 cases of hypertensive retinopathy, 5 patients had Vogt Koyanagi Harada Syndrome (VKH), 3 had neuroretinitis, Cytomegalovirus (CMV) retinitis in 2 cases and in 4 cases causes could not be found. Total of 36 (57.14%) patients had papilledema, 7 (11.11%) had optic neuritis, 3 (4.8%) patient had neuroretinitis, 6 (9.52%) cases of hypertensive retinopathy, 5 (7.9%) patient had VKH, CMV retinitis in 2 (3.2%) and 4 (6.3%) idiopathic. Conclusions: Papilledema was the commonest cause followed by optic neuritis for bilateral optic disc edema.

The evaluation of patients with optic disc edema: A retrospective study.

OBJECTIVE:Optic disc edema is among major problems that neuro-ophthalmology clinics encounter. We intended to analyze patients with optic disc edema in this article.METHODS:Data related to the main complaint, associated systemic disease, visual acuity, characteristics of optic disc swelling, other ocular findings, topical or systemic drugs, treatment methods, follow-up examination, and related data of the patients were obtained retrospectively.RESULTS:There were 77 female and 23 male patients in the study. Optic disc edema was detected bilaterally in 65 patients, unilaterally in 35 patients. The duration of the symptoms until the first application was 19.82±17.18 (0–90) days. There were no systemic disorders in 74 patients but diabetes mellitus in 11 patients, hypertension in four patients, coronary artery disease in three patients, urticaria in two patients, lymphoma in one, multiple sclerosis in one patient, mastoiditis in one patient, scleroderma in one, and pregnancy in two patients were detected. While 93 patients had no additional ocular findings, 2 had uveitis, 1 had corneal dystrophy, 1 had keratoconus, 1 had cataract, 1 had previous cataract surgery, and 1 had peripheral retinal degenerations. The major etiology of the optic disc edema was idiopathic intracranial hypertension, which was detected in 44 patients. In all these patients, bilateral optic disc edema was observed and 43 patients were given oral acetazolamide and one patient oral topiramate.CONCLUSION:The presence of optic nerve edema should be absolutely evaluated in patients presenting with symptoms of vision loss and increased intracranial pressure. The early diagnosis with fundoscopic examination may increase visual acuity in these patients.

Tubulointerstitial nephritis and uveitis syndrome: An atypical case

Tubulointerstitial nephritis and uveitis syndrome is an uncommon clinical entity. Usually, renal involvement is moderate, and uveitis is acute, bilateral, anterior, and nongranulomatous (revised). We present the case of a 55-year-old female patient who went to the emergency department with complaints of asthenia, myalgia, and paresthesia. One week later, she complained of photophobia and was diagnosed with bilateral nongranulomatous acute anterior uveitis. The next day, due to hypotension and acute kidney injury, the patient was hospitalized. Renal biopsy showed tubulointerstitial nephritis. The patient was started on oral prednisolone, achieving uveitis resolution and renal function stability. After slow corticoid reduction, the patient presented uveitis recurrence complicated with bilateral optic disc (revised) edema and cystoid macular edema (CME) in the left eye. After oral prednisolone reduction, the patient suffered another relapse with CME worsening. We decided to initiate methotrexate 7.5 mg/week, achieving ocular and renal function stability.

Pediatric anti-N-methyl-D-aspartate receptor encephalitis and bilateral optic neuritis: An updated review

We present the case report of a 6-year-old girl with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis associated with bilateral optic neuritis. She initially presented with disturbing behaviors and gait impairments and was hospitalized in the context of meningoencephalitis, without agent isolation and with inconclusive laboratory tests. After discharge, she noted a loss of vision with impaired color sensation and returned to the emergency department. Her best-corrected visual acuity was 20/400 in both eyes, and fundoscopy revealed bilateral optic disc edema. She was hospitalized once again. Anti-NMDAR antibodies were detected in serum and cerebrospinal fluid. The patient was successfully treated with a pulse of methylprednisolone, followed by a tapering regimen of prednisolone. She recovered to 20/20 vision in the right eye and left eye. Our report highlights additional aspects of anti-NMDAR encephalitis in children and suggests that the disease could present atypical visual manifestations, which requires multidisciplinary approaches.

Bilateral anterior ischaemic optic neuropathy with macular star as first manifestation of Henoch‐Schönlein purpura (IgA vasculitis)

AbstractPurposeTo report a case of bilateral anterior ischaemic optic neuropathy with macular star in a patient with Henoch–Schönlein purpura (HSP).MethodsA 46‐year‐old man was referred to our clinic complaining of progressive decrease in visual acuity of the right eye (OD) since the last 10 days, and since 3 days in his left eye (OS). His visual acuity was ‘hand motion’ OD and 4/10 OS. Anterior segment examination was insignificant, while fundus examination revealed optic disc edema, and bilateral macular star. A relative afferent pupillary defect was present in his right eye with normal pupillary reflexes in the left eye. Laboratory analysis showed: Hematocrit: 22.1%, ESR: 140 mm/hr, CRP: 64.80 mg/dl, Creatinine: 4.4 mg/dl. Physical examination revealed presence of erythematous rash on the trunk and the extremities. The patient was admitted to the Nephrology department for treatment of his azotemia.ResultsDuring his hospital course we performed complete laboratory and imaging examinations for autoimmune diseases, vasculitis and infectious diseases, which were negative. Histologic findings of renal biopsy were compatible with IgA glomerulonephritis and thus HSP was diagnosed. The patient received oral methylprednisolone and intravenous cyclophosphamide therapy. Six months later his renal function had improved, the rash had subsided and his visual acuity was ‘counting fingers’ OD and 10/10 OS. Standard automated perimetry showed peripheral visual field depression bilaterally.ConclusionsHenoch–Schönlein purpura (HSP) is a small vessel vasculitis which is more common in children. Based on literature, ophthalmic manifestations of HSP usually involve the anterior segment and less frequently the posterior segment. Systemic vasculitis should be included in the differential diagnosis of bilateral optic disc edema.

Ocular pathologies emerging in pregnancy.

Objectives: To determine ocular pathologies that appear in pregnant women with no history of ocular or systemic disease prior to conception. Study Design: Cross-sectional study. Setting: Department of Ophthalmology in association with Department of Gynecology, Pak Red Crescent Medical and Dental College Dina Nath District Kasur. Period: January 2018 to April 2018. Materials and Methods: One hundred and twenty pregnant females with no history of previous ocular or systemic co morbidity were included in study. After obtaining informed consent detailed ocular and medical history was taken and complete ocular examination was done. Results: Mean age of the participant female was 24.7 years (Range 19- 40 years). Four of them (3.33%) had bilateral optic disc edema, 2 of them had central serous chorioretinopathy (CSCR) and one female had unilateral macular hole. Conclusions: Ocular pathologies emerging in pregnancy are not uncommon and should not be overlooked. Pregnant women with ocular complaints should undergo a thorough ocular and systemic examination along with necessary investigations so that the pathological eye changes and associated systemic diseases could be managed timely and properly.

The escape of retrobulbar cerebrospinal fluid in the astronaut's eye: mission impossible?

Ophthalmic abnormalities including unilateral and bilateral optic disc edema, optic nerve sheath distention, globe flattening, choroidal folds, and hyperopic shifts have been observed in astronauts during and after long-duration spaceflight. An increased understanding of factors contributing to this syndrome, termed spaceflight-associated neuro-ocular syndrome, is currently a top priority for the ESA and NASA, especially since this medical obstacle could impact the visual health of astronauts as well as the success of future missions, including continued trips to the International Space Station, a return to the moon, or a future human mission to Mars. Currently, the exact mechanisms causing this neuro-ocular syndrome are not fully understood. In the present paper, we propose a hypothetical framework by which optic disc edema in astronauts may result, at least partly, from the forcing of perioptic cerebrospinal fluid into the optic nerve and optic disc along perivascular spaces surrounding the central retinal vessels, related to long-standing microgravity fluid shifts and variations in optic nerve sheath anatomy and compliance. Although this hypothesis remains speculative at the present time, future research in this area of investigation could not only provide exciting new insights into the mechanisms underlying microgravity-induced optic disc swelling but also offer opportunities to develop countermeasure strategies.

Intracranial Hypertension in Transgender Patients

Idiopathic intracranial hypertension (IIH) is a potentially blinding condition characterized by increased intracranial pressure, papilledema, headaches, and vision loss (1). The role of exogenous sex hormones in the development of IIH is controversial (2). Five cases of IIH occurring in transgender patients undergoing female-to-male (FTM) transition with exogenous testosterone have been reported, suggestive of a causal relationship between exogenous testosterone use and IIH (3). In 3 of the 5 patients, exogenous testosterone was altered or discontinued. We report 2 additional cases in transgender patients: one in a patient undergoing FTM transition and the other in a patient undergoing male-to-female (MTF) transition. In both, the manifestations of intracranial hypertension were managed entirely without any modification of the patient's hormonal therapies, demonstrating that gender-affirming hormonal therapy can be safely continued in transgender patients with IIH. A 28-year-old obese (body mass index [BMI] 30.13 kg/m2) transgender woman was evaluated in our clinic for optic disc swelling. She had begun oral estradiol (Estrace) and spironolactone for MTF gender transition 1 year prior, and subsequently developed visual obscurations, frequent headaches, and pulsatile tinnitus. Eight months after starting hormone therapy, bilateral optic disc edema was detected and brain MRI and magnetic resonance venography (MRV) showed bilateral transverse sigmoid sinus narrowing. Two lumbar punctures (LP) revealed opening pressures of 30 cm H20 and 39 cm H20 with normal constituents. She was prescribed extended-release acetazolamide 500 mg twice daily, hormone therapy was discontinued, and she was referred to our clinic. On our examination, visual acuity was 20/15 in each eye without a relative afferent pupillary defect. There was bilateral optic disc edema. Automated visual fields (Humphrey 24-2) showed enlarged blind spots with a mean deviation of −1.36 dB, right eye, and −2.11 dB, left eye. On spectral domain optical coherence tomography (SD-OCT; Spectralis Tracking Laser Tomography; Heidelberg Engineering, Inc; Heidelberg, Germany), the retinal nerve fiber layer was thickened bilaterally, measuring 127 µm in each eye. Treatment with acetazolamide was continued. In consultation with the patient and her reproductive endocrinologist, estradiol therapy was resumed. Over the next 5 months, her headaches and papilledema resolved. Her estradiol was ultimately changed by her reproductive endocrinologist to a sublingual formulation to further increase her serum estrogen levels and suppress testosterone. She has remained in remission from IIH despite these changes to her hormonal therapy. A 31-year-old obese (BMI 56.5 kg/m2) transgender man was referred to our clinic for headaches and bilateral optic disc edema. He had previously been taking testosterone injections for FTM gender transition but had been off testosterone injections for 1 year before presentation. His visual acuity was 20/20 in both eyes, no afferent relative pupillary defect was detected, and Ishihara color plates demonstrated no deficits. Funduscopic examination revealed edematous optic discs while automated visual fields (Humphrey 24-2) revealed enlarged blind spots with a mean deviation of −3.80 dB, right eye, and −3.91 dB, left eye. With SD-OCT testing, peripapillary retinal nerve fiber thicknesses measured 385 µm, right eye, and 156 µm, left eye. MRI and MRV were unremarkable. LP revealed an opening pressure of 54 cm H2O with normal constituents. The patient was prescribed extended release acetazolamide 500 mg twice daily. He was then seen by his reproductive endocrinologist, resumed testosterone therapy, and sustained an intentional 20-pound weight loss. Over the ensuing 6 months, his papilledema resolved. There is no evidence-based consensus guiding the management of hormone therapy in transgender patients with IIH. Given the poorly understood, and possibly causative role of sex hormones in the development of IIH, clinicians may be inclined to alter gender-affirming hormone therapy once the diagnosis of IIH is made. Of the 5 previously reported cases of IIH developing in FTM patients receiving testosterone, the dose testosterone was reduced in 2 patients and changed to an alternate formulation in a third (3). Similarly, in our patient undergoing MTF transition, estradiol was stopped before our evaluation. Remission from IIH was achieved in both of our patients while continuing hormone therapy. In the patient undergoing MTF transition, hormone therapy was safely resumed and subsequently increased to suppress endogenous testosterone; she has remained in remission on acetazolamide. Likewise, in the patient undergoing FTM transition, IIH resolved with weight loss and acetazolamide, and he has remained in remission even after resumption of regular testosterone injections. Although it is uncertain if hormone therapy can precipitate IIH, we report 2 instances of IIH in transgender individuals that were successfully treated without alteration to the patient's gender-affirming hormone therapy. Patients with IIH undergoing hormone therapy for gender transition may still be responsive to conventional treatment without the need for change in hormone therapy.

Papillopathie diabétique après insulinothérapie intensive : à propos d’un cas

IntroductionDiabetic papillopathy is a rare ocular complication of diabetes. Its pathophysiology is not well known. It is a unilateral or bilateral optic disc edema with variable degrees of visual loss. ObservationA 66-year-old woman, with a twelve years old type 2 diabetes mellitus, suddenly presented a unilateral decreased vision. Her ocular examination was normal eight months earlier. Ocular examination, radiological investigations and laboratory analysis were conclusive of diabetic papillopathy. Medical history revealed a recent rapid improvement of blood glucose control after intensification of insulin therapy. A visual acuity improvement with spontaneous regression of papillary edema was observed. ConclusionThere is a risk of diabetic papillopathy associated with the rapid control of blood glucose levels after intensification of insulin therapy, even in diabetic patients without known retinopathy.