Optic neuropathy after COVID-19 vaccination: a report of two cases.

PURPOSE: We report two cases of optic nerve pathology after the administration of the Pfizer-BioNTech and AstraZeneca-Oxford COVID-19 vaccines, respectively, and describe the implications for management of post-vaccination central nervous system (CNS) inflammation. CASE REPORTS: A 69-year-old woman presented with bilateral optic nerve head oedema, 16 days after the second dose of the Pfizer-BioNTech vaccine. She was diagnosed with post-vaccination CNS inflammatory syndrome and was treated for five days with intravenous methylprednisolone at a dose of 1 gram per day. Her optic disc swelling improved, and her vision stabilised. A 32-year-old woman presented six days after her first dose of the AstraZeneca-Oxford vaccine with two days of sudden onset of progressive blurring of vision in her left eye. Posterior segment examination revealed left optic disc swelling, and an MRI of the brain, orbit, and cervical spine was significant for left optic nerve enhancement. The patient was diagnosed with a unilateral post-vaccination optic neuritis. She was treated with a three-day course of intravenous methylprednisolone followed by oral prednisone. Her optic disc swelling and visual field improved, and she recovered 6/6 vision. CONCLUSIONS: Clinicians and patients should be aware of the potential for post-vaccination CNS inflammatory syndromes associated with COVID-19 vaccine administration. Neuroimaging and cerebrospinal fluid analysis may aid in the diagnosis of the cause of vision loss. Further studies are needed to evaluate the spectrum and frequency of optic nerve involvement associated with COVID-19 vaccination.

Gyrate Atrophy of the Choroid and Retina: A Review.

Gyrate atrophy (GA) of the choroid and retina is a rare autosomal recessive genetic condition characterized by elevation of the plasma level of the amino acid ornithine due to deficiency of the enzyme ornithine ketoacid aminotransferase. Accumulation of ornithine occurs in various body tissues but leads primarily to characteristic ophthalmic manifestations including myopia, cataract, progressive chorioretinal atrophy, and macular changes. Patients usually present with night blindness that starts in the first decade of life followed by visual field constriction and eventually diminution of the central visual acuity and blindness. The condition has been reported worldwide and its differential diagnosis is broad and includes choroideremia and retinitis pigmentosa. Treatment currently depends on life-long dietary modifications including restriction of the amino acid arginine in diet. This article describes in detail the pathogenesis, clinical features, multimodal imaging findings, and treatment options for GA of the choroid and retina and its complications.

Methotrexate monotherapy for unilateral moderately active thyroid-related eye disease.

A type 1 diabetic patient with unilateral active thyroid-related eye disease was intolerant to systemic steroid therapy due to uncontrollable blood sugar levels. She was treated with low-dose methotrexate monotherapy, which resulted in a marked improvement of her condition with no adverse events.

Optical Coherence Tomography Angiography of Macular Perfusion Changes after Anti-VEGF Therapy for Diabetic Macular Edema: A Systematic Review.

BACKGROUND: Diabetic macular edema (DME) is a major cause of vision loss in diabetics that is currently mainly treated by antivascular endothelial growth factor (VEGF) agents. The effect of these agents on macular perfusion (MP) is a current concern. Optical coherence tomography angiography (OCTA) is an imaging modality that allows noninvasive high-resolution retinal microvasculature imaging. Several recent studies evaluated the effect of anti-VEGF agents on the MP of DME patients using OCTA. Our aim is to provide a systematic review of these studies. METHODS: Multiple databases were searched including PubMed, Ovid Medline, EMBASE, and Google Scholar for relevant studies published between January 2016 and November 2020 which were included in this review. Studies were compared regarding their design, the number of included patients, the machine and scanning protocol used, the inclusion and exclusion criteria, the number of injections given, the type of anti-VEGF agent used, the outcome measures assessed, and the effect of injections on different MP parameters. RESULTS: A total of 16 studies were included. The studies assessed various OCTA parameters that define MP including the foveal avascular zone area and superficial and deep vascular density and yielded conflicting results. Seven studies showed stable or improved MP following treatment, while 7 studies showed worsening MP following treatment, and 2 studies showed inconclusive results. This could have been due to differences in study design, inclusion criteria, type of anti-VEGF agents used, treatment duration, and methods of image analysis and vascular density quantification. All identified studies were noncomparative case series, and 14 of them (87.5%) used the RTVue XR Avanti OCTA machine. Only one study compared OCTA to fluorescein angiography findings. CONCLUSION: Analysis of MP changes following VEGF inhibition for DME could benefit from a unified scanning protocol and analysis approach that uses similar study designs to eliminate potential sources of bias. This may provide more definitive conclusions regarding the effect of treatment on MP.

Optic Nerve Sheath Fenestration for Papilledema Due to Cerebral Venous Sinus Thrombosis Associated with Antiphospholipid Syndrome: A Case Report.

BACKGROUND Cerebral venous sinus thrombosis (CVST) is a serious life- and vision-threatening condition that can have a variable presentation according to the site of venous occlusion, including mimicking idiopathic intracranial hypertension. We report on a patient with primary antiphospholipid antibody syndrome (APS) who presented with papilledema due to CVST that was refractory to medical treatment but responded to optic nerve sheath fenestration (ONSF). CASE REPORT A 21-year-old man presented with blurred vision of gradual onset and a progressive course for 1 month, accompanied by fever, headache, and confusion. He had a history of lower-limb deep vein thrombosis. Examination revealed decreased vision with bilateral grade IV papilledema. Magnetic resonance venography showed evidence of CVST and laboratory investigations revealed lupus anticoagulant antibodies, antinuclear antibodies, and anti-double stranded DNA antibodies, with hyperhomocysteinemia. The patient did not meet the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus (SLE) nor the new European League Against Rheumatism and American College of Rheumatology SLE classification criteria. He was diagnosed with CVST secondary to APS and hyperhomocysteinemia and treated with acetazolamide, systemic anticoagulation, and vitamins for 1 month, but with no improvement in his ophthalmic condition. He subsequently underwent unilateral ONSF, which resulted in improvement in vision bilaterally that continued throughout a 6-month follow-up period. CONCLUSIONS Papilledema associated with CVST can be the first presentation of APS. When performed in a timely manner, ONSF can save useful vision and lead to improvement in vision in patients with papilledema due to CVST that is refractory to medical treatment.

Management of Idiopathic Intracranial Hypertension During the COVID-19 Pandemic.

BACKGROUND: In the current coronavirus disease 2019 (COVID-19) pandemic, health systems are struggling to prioritize care for affected patients; however, physicians globally are also attempting to maintain care for other less-threatening medical conditions that may lead to permanent disabilities if untreated. Idiopathic intracranial hypertension (IIH) is a relatively common condition affecting young females that could lead to permanent blindness if not properly treated. In this article, we provide some insight and recommendations regarding the management of IIH during the pandemic. METHODS: The diagnosis, follow-up, and treatment methods of IIH during the COVID-19 pandemic period are reviewed. COVID-19, as a mimic of IIH, is also discussed. RESULTS: Diagnosis and follow-up of papilledema due to IIH during the COVID-19 pandemic can be facilitated by nonmydriatic fundus photography and optical coherence tomography. COVID-19 may mimic IIH by presenting as cerebral venous sinus thrombosis, papillophlebitis, or meningoencephalitis, so a high index of suspicion is required in these cases. When surgical treatment is indicated, optic nerve sheath fenestration may be the primary procedure of choice during the pandemic period. CONCLUSION: IIH is a serious vision-threatening condition that could lead to permanent blindness and disability at a relatively young age if left untreated. It could be the first presentation of a COVID-19 infection. Certain precautions during the diagnosis and management of this condition could be taken that may allow appropriate care to be delivered to these patients while minimizing the risk of coronavirus infection.

Peripapillary microperimetry for the diagnosis and follow-up of papilledema in cases treated for idiopathic intracranial hypertension.

OBJECTIVES: To evaluate the use of peripapillary microperimetry in the diagnosis and follow-up of medically and surgically treated cases of papilledema due to idiopathic intracranial hypertension (IIH). METHODS: This study was a prospective non-comparative observational case series of patients with IIH. All patients underwent full ophthalmological examination, radiological imaging, and lumbar puncture, as well as microperimetric measurements around the optic nerve head. Patients were classified into a medical group, treated by weight reduction and acetazolamide, and a surgical group, treated by optic nerve sheath fenestration. Peripapillary microperimetric examinations were done at presentation and after one-month of treatment. Findings were compared to values obtained from an age-matched healthy control group. RESULTS: The study included 78 eyes of 39 patients diagnosed with IIH and 32 eyes of 16 healthy control subjects. The average of the sum of the 12 peripapillary microperimetric thresholds for both eyes of IIH cases before treatment was 53.4±59.9 dB, while for controls it was 171±14.0 dB (p<0.001). After 1 month of treatment, it improved to 86.7±56.1 dB (p<0.001) in IIH cases. Analyzed separately, the medical group improved from 68.9±43.4 dB to 105.2±76.5 dB (p<0.001), while the surgical group improved from 13.5±21.9 dB to 54.9±35.5 dB (p<0.001). Several potential roles for peripapillary microperimetry use in IIH management were identified including confirming papilledema diagnosis, follow up of treatment, and selection of cases for surgery. CONCLUSION: Microperimetry can accurately and quantitatively monitor changes in peripapillary retinal sensitivity in patients undergoing treatment for papilledema due to IIH.

Review on Recent Trials Evaluating the Effect of Intravitreal Injections of Anti-VEGF Agents on the Macular Perfusion of Diabetic Patients with Diabetic Macular Edema.

BACKGROUND: Diabetic macular edema (DME) is a major cause of vision loss in diabetics worldwide. Anti-vascular endothelial growth factor (anti-VEGF) agents have become the mainstay of treatment of vision loss due to DME. Long-term effects of these agents on the macular perfusion (MP) are a current concern. OBJECTIVE: To review recently published studies that evaluated the effect of intravitreal injection of anti-VEGF agents on the MP of diabetics with DME. METHODS: Different databases were searched including PubMed, Medline, Ovid, Science Direct, and Google Scholar for relevant studies published between 2010 and 2019. All studies found were compared regarding methodology and results and included in this review. Some studies relating to retinal perfusion in general and not strictly MP were also included for comprehensiveness. RESULTS: Several studies utilizing different anti-VEGF agents were identified. All the large randomized controlled clinical trials identified utilized primarily fluorescein angiography (FA) and human graders and found generally no worsening of MP associated with anti-VEGF agents use in diabetic patients with DME. Some of these studies, however, depended on post-hoc analysis. Several more recent, but smaller case series, have utilized the relatively new and non-invasive optical coherence tomography angiography (OCTA) in this evaluation and found more conflicting results. CONCLUSION: The large clinical trials recently performed depended mainly on FA in the analysis of MP changes following injections and generally found no worsening of MP. More recently, smaller case series have utilized OCTA in this analysis, yielding more conflicting results. Large randomized controlled trials using OCTA are thus needed.

REVERSIBLE NEUROTROPHIC KERATOPATHY ASSOCIATED WITH ROSUVASTATIN THERAPY: A CASE REPORT.

BACKGROUND: Rosuvastatin is a 3-hydroxy-3-methyl-glutaryl-CoA reductase enzyme inhibitor that is in wide use with few reported ocular adverse events. OBJECTIVES: To report a case of bilateral neurotrophic keratopathy associated with rosuvastatin therapy that dramatically improved following drug discontinuation. CASE PRESENTATION: A 65-year-old female presented with painless diminution of vision in both eyes of gradual onset and progressive course for 1 month. She had recently started rosuvastatin therapy for hyperlipidemia. Examination revealed bilateral stage 2 neurotrophic keratopathy with impaired corneal sensation which was previously resistant to conservative ulcer treatment. Following discontinuation of rosuvastatin therapy, there was dramatic bilateral improvement in corneal sensation, size of the corneal ulcers, and visual acuity. CONCLUSION: Rosuvastatin may result in reversible trigeminal nerve impairment and neurotrophic keratopathy.

Abdominal sarcoidosis presenting as bilateral simultaneous optic disc granulomas.

A 24-year-old male presents with diminution of vision in both eyes of acute onset with floaters. He has a history of fever of unknown origin. Examination revealed bilateral optic disc granulomas with mild vitritis. Serum angiotensin converting enzyme was found to be elevated and tuberculin skin test was negative. Computed tomography scan of the chest showed clear lung fields with no hilar lymphadenopathy but mildly enlarged pretracheal lymph nodes. Computed tomography scan of the abdomen revealed multiple enlarged abdominal lymph nodes with hepatosplenomegaly, and ultrasound-guided biopsy of one of these lymph nodes showed chronic granulomatous inflammation consistent with sarcoidosis. Immunosuppressive therapy resulted in resolution of ocular inflammation with no recurrence.

Unilateral papilledema revealing an intracanalicular optic nerve sheath meningioma.

A 43-year-old female is referred to our clinic for evaluation of an incidentally discovered unilateral papilledema in her right eye of unknown cause. She had no past ocular or medical history. Examination revealed a corrected-distance visual acuity of 20/20 in both eyes. Posterior segment examination showed an elevated right optic nerve head with blurred edges. Ultrasonography failed to reveal optic disc drusen. Visual field testing showed enlargement of the blind spot in the right eye. Magnetic resonance imaging of the brain and orbit was done and revealed enlargement of the intracanalicular and prechiasmatic part of the right optic nerve and a diagnosis of a presumed right intracanalicular optic nerve sheath meningioma was made. She was managed conservatively and several years later remains asymptomatic.

Clinical and Imaging Findings One Year Following Traumatic Chiasm Transection.

Traumatic chiasm transection is a rare condition characterized by bitemporal hemianopsia following head trauma. In a study of visual complications following head trauma, only 4.4% of patients suffered from chiasmal injuries. Complete transection of the chiasm is even more rare and best visualized using magnetic resonance imaging (MRI). We report the one-year follow-up of clinical, visual field, and MRI findings of a patient with complete transection of the optic chiasm following head trauma.

Treatment of acquired Brown syndrome in a child with a single intramuscular systemic depot steroid injection.

A 10-year-old girl presented with a complaint of diplopia and mild superomedial orbital pain in the left eye of 2 weeks' duration. She had limited elevation of the left eye, especially in adduction, moderate limitation of elevation in the primary position, mild limitation of elevation in abduction, downshoot in adduction, mild hypotropia in the primary position, and normal abduction. There was mild swelling and tenderness in the superomedial aspect of her left orbit. Fundus examination revealed intorsion of the left fundus on upgaze. She was diagnosed with acquired Brown syndrome, due presumably to a local inflammatory cause, and treated with a single intramuscular depot injection of betamethasone in her deltoid muscle. One week later, her symptoms were resolving, and there was marked improvement of elevation of the left eye in adduction, with near normal elevation in the primary position and in abduction. There was no recurrence 3 months later.

Oculomotor Nerve Palsy Associated with Duplication of Middle Cerebral Artery, Anterior Communicating Artery Aneurysm, and Parietal Meningioma.

A 61-year-old male presents with diplopia of acute onset and progressive course. He has a history of previous intracranial haemorrhage that was surgically evacuated 7 years ago and was also associated with diplopia. Examination revealed left complete oculomotor nerve paralysis with a fixed and dilated left pupil. Computed tomography (CT) revealed encephalomalacia, evidence of previous craniotomy, and an incidental left parietal convexity meningioma. CT angiography of the brain revealed a left tortuous duplicate middle cerebral artery with fenestration of its proximal part, an anterior communicating artery aneurysm, and a characteristic capillary blush of the meningioma. Possible mechanisms of oculomotor nerve involvement are discussed.

Computed Tomography Angiography of Bilateral Intracavernous Internal Carotid Artery Aneurysms.

Bilateral intracavernous internal carotid artery aneurysms are rare. They are more common in elderly females and are associated with hypertension. We present the computed tomography angiography findings of an 81-year-old female with history of hypertension who came complaining of diplopia and headache. Examination revealed bilateral sixth nerve palsy with left third nerve palsy. External ocular examination was normal. Computed tomography angiography was done and revealed bilateral intracavernous internal carotid artery aneurysms. She was referred to neurosurgery for further management.