-
Journal of Clinical Medicine Sep 2022Acute idiopathic blind spot enlargement syndrome (AIBSES) is characterized by unilateral visual field loss in the blind spot area, acute onset photopsia, and...
Acute idiopathic blind spot enlargement syndrome (AIBSES) is characterized by unilateral visual field loss in the blind spot area, acute onset photopsia, and funduscopically few or no optic disc changes. AIBSES predominantly affects young adults and is often misdiagnosed as optic neuritis because of low awareness. Optical coherence tomography (OCT) has become the gold standard in diagnosing AIBSES as a disease of the outer retina. In our case series, we present three consecutive patients with AIBSES followed prospectively with and without steroid therapy. The patients, aged 25 to 27 years, presented in our neuroophthalmology department between 2020 and 2021. We report their disease course and management and discuss therapeutic options, as no well-established procedures exist. Common pitfalls and diagnostic errors are analysed. Two women and one man showed unilateral acute-onset photopsia and blind spot enlargement on perimetry without visual acuity reduction. Spectral domain OCT (Heidelberg Engineering, Heidelberg, Germany) revealed marked peripapillary changes in the ellipsoid zone and autofluorescence in all patients, corresponding to faint blurring of the optic disc margin. Characteristically, there was no P100 latency delay in the visual evoked potential in any of the patients. Two patients received weight-adapted oral prednisolone, which was gradually tapered over six to eight weeks. Two patients showed full recovery of their symptoms at six and seven months after onset, while mild defect healing was seen in one treated patient after 12 months. Follow-up OCT showed restoration of the outer retinal layers 6-12 months after disease onset. Careful history taking and an unprejudiced ophthalmological workup helps in diagnosing AIBSES in young adults with unilateral acute visual field defects. While its etiology is still unclear, accurate diagnosis of AIBSES can be made with peripapillary OCT. In our cases, the disease course of AIBSES was much better than its reputation. Early corticosteroid treatment may support outer retinal reorganisation, which can be followed with OCT in accordance with visual field restoration. This should be addressed in a prospective study.
PubMed: 36142923
DOI: 10.3390/jcm11185278 -
Retinal Cases & Brief Reports Jan 2024To describe multimodal imaging findings in a patient with a rare, symptomatic fundus lesion arising from the retinal pigment epithelium.
PURPOSE
To describe multimodal imaging findings in a patient with a rare, symptomatic fundus lesion arising from the retinal pigment epithelium.
METHODS
Case report.
RESULTS
A 36-year-old woman presented with photopsia in her left eye. Funduscopy revealed an 8-mm × 7-mm, dark brown lesion at the level of the retinal pigment epithelium inferior to the macula. The lesion had an irregular, cauliflower-like border and a light grey subretinal pseudohypopyon. On fundus autofluorescence, the lesion was markedly hypoautofluorescent with an irregular hyperautofluorescent margin. It was generally hyopofluorescent on fluorescein angiography and moderately hypofluorescent on indocyanine green angiography. Spectral-domain optical coherence tomography revealed a fine layer of subretinal fluid over the entire lesion, thinning of the outer retinal layers with loss of photoreceptors, and an irregular retinal pigment epithelium. Multiple drusen-like subretinal deposits were located along the lesion margin, and inferiorly, the pseudohypopyon appeared as a hyperreflective subretinal mass. During 3 years of follow-up, her symptoms remained unchanged and fundus photography showed minimal enlargement of the lesion.
CONCLUSION
Multimodal imaging findings of a solitary pigmented retinal pigment epithelium lesion with pseudohypopyon and subretinal fluid are shown. The lesion may represent an atypical variant of unilateral retinal pigment epithelium dysgenesis.
Topics: Female; Humans; Adult; Retinal Pigment Epithelium; Subretinal Fluid; Retina; Eye Diseases; Fluorescein Angiography; Tomography, Optical Coherence
PubMed: 35993774
DOI: 10.1097/ICB.0000000000001317 -
PloS One 2022Photopsia is a photic phenomenon that can be associated with intraocular lenses after cataract surgery. To calculate the relative light intensity of photic effects...
Photopsia is a photic phenomenon that can be associated with intraocular lenses after cataract surgery. To calculate the relative light intensity of photic effects observed after cataract surgery at the foveal region as the most sensitive region of the retina, photopsia was simulated using the ZEMAX optical design software. The simulations are based on the Liou-Brennan eye model with a pupil diameter of 4.5 mm and incorporating implanted IOLs. The hydrophilic IOLs implanted in the eye model have a power of 21 diopter (D) with an optic diameter of 6 mm and 7 mm. Four different intensity detectors are located in specific regions of the eye in this simulation. The ray-tracing analysis was carried out for variations of incident ray angle of 0° to 90° (temporally) in steps of 1°. Depending on the range of incident ray angle, the light intensity was detected at detectors located on the fovea, nasal side of the retina, or the edge surface of the IOLs. Some portion of the input light was detected at specific incident angles in the foveal region. By altering the IOLs edge design to a fully reflective or anti-reflective surface, the range over which the light intensity is detected on the fovea can be shifted. Additionally, with the absorbing edge design, no intensity was detected at the foveal region for incident ray angles larger than 5°. Therefore an absorbing edge design can make photic effects less disturbing for patients.
Topics: Cataract; Computer Simulation; Humans; Lenses, Intraocular; Light; Optics and Photonics; Prosthesis Design; Vision Disorders
PubMed: 35930598
DOI: 10.1371/journal.pone.0272705 -
International Journal of Molecular... Jul 2022Pathogenic variants in DNA-damage regulated autophagy modulator 2 gene () cause a rare autosomal recessive retinal dystrophy and its disease course is not well...
Pathogenic variants in DNA-damage regulated autophagy modulator 2 gene () cause a rare autosomal recessive retinal dystrophy and its disease course is not well understood. We present two Slovenian patients harboring a novel variant and a detailed review of all 23 other patients described to date. Whole exome and whole genome sequencing were performed in the two patients, and both underwent ophthalmological examination with a 2-year follow-up. PubMed was searched for papers with clinical descriptions of DRAM2 retinopathy. Patient 1 was homozygous for a novel variant, p.Met1?, and presented with the acute onset of photopsia and retina-wide retinopathy at the age of 35 years. The patient was first thought to have an autoimmune retinopathy and was treated with mycophenolate mofetil, which provided some symptomatic relief. Patient 2 was compound heterozygous for p.Met1? and p.Leu246Pro and presented with late-onset maculopathy at the age of 59 years. On review, patients with DRAM2 retinopathy usually present in the third decade with central visual loss, outer retinal layer loss on optical coherence tomography and a hyperautofluorescent ring on fundus autofluorescence. Either cone-rod or rod-cone dystrophy phenotype is observed on electroretinography, reflecting the importance of DRAM2 in both photoreceptor types. Non-null variants can result in milder disease.
Topics: Autoimmune Diseases; Cone-Rod Dystrophies; Electroretinography; Humans; Membrane Proteins; Mutation; Pedigree; Phenotype; Retinal Dystrophies; Tomography, Optical Coherence
PubMed: 35806404
DOI: 10.3390/ijms23137398 -
American Journal of Ophthalmology Case... Jun 2022To report a case with multiple evanescent white dot syndrome (MEWDS) following BNT162b2 mRNA COVID-19 vaccination.
PURPOSE
To report a case with multiple evanescent white dot syndrome (MEWDS) following BNT162b2 mRNA COVID-19 vaccination.
OBSERVATIONS
Case: A 67-year-old Japanese female presented with central visual field loss and photopsia in the right eye (OD) for 5 days. She was complaining blurred vision with bright spots in vision in OD, but denied any ocular symptoms in left eye (OS). She had received the second dose of BNT162b2 mRNA COVID-19 vaccine (Pfizer-BioNTech) one day before the onset of visual symptoms; flu-like symptoms such as mild fever and general fatigue also developed along with ocular symptoms such as decreased vision and hypersensitivity to light in OD following the second COVID-19 vaccine. The first dose of vaccine was administrated followed three weeks later by the second dose and was not associated with any ocular or systemic symptoms besides mild pain at the injection site. She had not been followed by any ophthalmologist before the initial visit. At the initial visit, best corrected visual acuity (BCVA) in decimal points was 0.2 in OD and 1.0 in OS. Ophthalmic examination showed multifocal white dots in the posterior retina with moderate vitritis (1+ haze and 2+ cells) in OD. Multimodal imaging in OD showed diffuse disruption of ellipsoid zone with variable punctate hyperreflective lesions at macula on optical coherence tomography, punctate hyperfluorescence in a wreath-like pattern and late staining on fluorescein angiography, and multiple hypofluorescent spots of various sizes in the late phases on indocyanine green angiography. Both multiple hypofluorescent spots and scattered hyperfluorescent spots corresponding to white dots in OD were also seen on fundus autofluorescence. Her laboratory and systemic evaluations were negative for syphilis, tuberculosis, or toxoplasma, and selected autoimmune diseases like sarcoidosis, Behcet's disease, rheumatoid arthritis, and systemic lupus erythematosus. No active intraocular inflammation or abnormality were seen in OS. One week later, the multifocal white dots disappeared in OD, and were almost invisible on fundus photography. At that time, multifocal electroretinogram showed decreased response with low amplitude density across the entire field in OD. The BCVA in OD spontaneously improved to 0.8 without any treatment. Collectively, these clinical course and findings were suggestive of a diagnosis of MEWDS after mRNA COVID-19 vaccination.
CONCLUSIONS AND IMPORTANCE
In this present case, BNT162b2 mRNA COVID-19 vaccination might have been associated with MEWDS-like entity with vision loss. It is important for physicians to monitor the ocular status carefully in patients with visual disturbance after COVID-19 vaccination.
PubMed: 35434421
DOI: 10.1016/j.ajoc.2022.101532 -
Arquivos Brasileiros de Oftalmologia 2022A 38-year-old woman presented with photopsias and progressive but painless loss of vision in her right eye. Of note, she had received the first dose of inactivated...
A 38-year-old woman presented with photopsias and progressive but painless loss of vision in her right eye. Of note, she had received the first dose of inactivated COVID-19 vaccine (Sinovac/China National Pharmaceutical Group) 2 weeks prior to the onset of symptoms. Ophthalmic evaluation revealed a wreath-like foveal pattern and multiple gray-white dots throughout the posterior pole associated with discrete vitreous inflammatory reaction. Multimodal imaging analysis confirmed a diagnosis of multiple evanescent white dot syndrome. The patient underwent treatment with corticosteroids and, over the following weeks, her visual acuity improved to standard pattern.
Topics: Adult; COVID-19; COVID-19 Vaccines; Female; Fluorescein Angiography; Humans; Retinal Diseases; Vaccination; White Dot Syndromes
PubMed: 35298582
DOI: 10.5935/0004-2749.20220070 -
American Journal of Ophthalmology Case... Jun 2022To report a patient who developed a cancer associated retinopathy (CAR) like syndrome in the setting of pembrolizumab and lenvatinib combination therapy for metastatic...
PURPOSE
To report a patient who developed a cancer associated retinopathy (CAR) like syndrome in the setting of pembrolizumab and lenvatinib combination therapy for metastatic endometrial carcinoma. Symptoms resolved with normalization of objective functional tests following cessation of medications.
OBSERVATIONS
A 52-year-old female with history of endometrial carcinoma, managed with pembrolizumab infusions and daily oral lenvatinib treatment for 18 months, presented to a tertiary eye center with complaints of nyctalopia, photosensitivity and photopsia. Further investigations revealed a reduction in b-wave amplitude on full field ERG (ffERG), a mild color vision deficit, and positive antiretinal antibodies against carbonic anhydrase II, enolase and arrestin. A preliminary diagnosis of CAR was made. One month following diagnosis, the patient discontinued both lenvatinib and pembrolizumab and subsequently reported significant improvement in her eye symptoms and vision. Repeat ffERG had normalized with a robust b-wave, with an improvement noted on repeat color vision testing. A presumed diagnosis of immunotherapy-induced retinopathy was made, with clinical findings mimicking CAR.
CONCLUSIONS AND IMPORTANCE
Pembrolizumab and lenvatinib treatment may be associated with a reversible retinopathy, with presentation very similar to CAR.
PubMed: 35265774
DOI: 10.1016/j.ajoc.2022.101449 -
American Journal of Ophthalmology Case... Jun 2022To report a case of a branch retinal vein occlusion (BRVO) following mRNA COVID-19 vaccination.
PURPOSE
To report a case of a branch retinal vein occlusion (BRVO) following mRNA COVID-19 vaccination.
OBSERVATIONS
A 34-year-old healthy male presented with blurriness in the inferior visual field, intermittent photopsia, multiple retinal hemorrhages, dilated and tortuous retinal vessels, and cotton wools spots in the right eye. The clinical examination and ancillary tests confirmed the diagnosis of a right eye BRVO. The visual symptoms started 2 days following first dose COVID-19 vaccination with the BNT162b2 (Pfizer-BioNTech) mRNA vaccine.
CONCLUSIONS AND IMPORTANCE
This is a rare case of BRVO in an otherwise healthy young man, presenting after vaccination for COVID-19 in the absence of other coagulable risk factors. As the literature on venous thrombosis after COVID-19 vaccinations remains sparse, it is critical to raise awareness that BRVO could be a vaccine-related thrombotic adverse event. We highlight that as more of the population is vaccinated, an increased incidence of BRVO may confirm the link to COVID-19 vaccination.
PubMed: 35211659
DOI: 10.1016/j.ajoc.2022.101445 -
The British Journal of Ophthalmology Jul 2023Chimeric antigen receptor T-cell (CAR T) therapy has been shown to improve the remission rate and survival for patients with refractory haematological malignancies. The...
BACKGROUND/AIMS
Chimeric antigen receptor T-cell (CAR T) therapy has been shown to improve the remission rate and survival for patients with refractory haematological malignancies. The aim of this study is to describe ocular adverse effects associated with CAR T therapy in patients with haematological malignancies.
METHODS
This is a retrospective, single-institution, case series. Patients aged 18 years or older who received standard of care CAR T therapy for relapsed/refractory large B-cell lymphoma with a documented ophthalmic evaluation were included. The primary outcome was clinician ophthalmic examination findings.
RESULTS
A total of 66 patients received CAR T-cell therapy from February 2018 to October 2019 with 11 receiving an ophthalmic examination. Eleven patients (n=22 eyes) who received CAR T-cell therapy were included in review. The median time from CAR T-cell infusion date to ocular examination was 57.5 days. The median patient age at the time of examination was 60.5 years. Ten patients had subjective symptoms prompting ophthalmic examination. Two patients reported floaters and photopsias. One patient had worsening ocular graft-versus-host disease. Two patients were identified with possible reactivation of viral infections, including herpes zoster ophthalmicus and regressing acute retinal necrosis.
CONCLUSIONS
The increasing use of CAR T therapy for malignancies underscores the importance of ophthalmologists and oncologists understanding the potential toxicities associated with its use, particularly ocular toxicities and when to refer for an ophthalmic examination.
Topics: Humans; Cell- and Tissue-Based Therapy; Hematologic Neoplasms; Immunotherapy, Adoptive; Lymphoma, Large B-Cell, Diffuse; Receptors, Chimeric Antigen; Retrospective Studies; Male; Female; Adult; Middle Aged; Aged
PubMed: 35144919
DOI: 10.1136/bjophthalmol-2021-320814 -
Cornea Sep 2022The purpose of this study was to report a case of corneal endothelial dysfunction and subsequent corneal edema in a patient with digoxin toxicity.
PURPOSE
The purpose of this study was to report a case of corneal endothelial dysfunction and subsequent corneal edema in a patient with digoxin toxicity.
METHOD
This was a case report.
RESULTS
A 77-year-old woman diagnosed with open-angle glaucoma and treated with a topical prostaglandin analog for 5 years developed blurred vision and photopsia in both eyes. Systemic medications included digoxin, furosemide, apixaban, amlodipine, enalapril, and simvastatin. Ocular examination revealed folds in Descemet membrane and corneal stromal edema in both eyes, with normal fundoscopy. Ancillary tests revealed elevated serum digoxin levels. No intervention other than discontinuation of digoxin was initiated. The corneal edema improved after that and resolved in the next 2 weeks.
CONCLUSIONS
We encountered 1 case of corneal edema secondary to corneal endothelial dysfunction in a patient with digoxin toxicity. Special care should be taken to elicit a complete history because ocular signs can be manifestations of systemic alterations with vital importance for patients.
Topics: Aged; Cornea; Corneal Edema; Descemet Stripping Endothelial Keratoplasty; Digoxin; Female; Glaucoma, Open-Angle; Humans
PubMed: 35120352
DOI: 10.1097/ICO.0000000000002981