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International Ophthalmology Oct 2018To report a case of presumed autoimmune retinopathy in a patient who had been diagnosed with chickenpox immediately prior to symptom onset.
PURPOSE
To report a case of presumed autoimmune retinopathy in a patient who had been diagnosed with chickenpox immediately prior to symptom onset.
METHOD
This is a retrospective case report with fundus autofluorescence and spectral-domain optical coherence tomography.
RESULTS
A 29-year-old immunocompetent man presented with a two-month history of photopsia and a bluish light predominantly in the left eye with onset immediately following a diagnosis of chickenpox. He subsequently developed blurring of vision in his left eye with spectral-domain optical coherence tomography showing disruption and loss of ellipsoid layer in a perivascular pattern in both eyes with fundus autofluorescence demonstrating an abnormal increase in autofluorescence predominately around the vascular arcades. Autoimmune retinopathy was suspected on the basis of the clinical presentation, electrophysiology, absence of fundus lesions, retinal degenerations or dystrophies, or intraocular inflammation and temporal association with varicella infection. Antiretinal antibody testing with Western blot and immunohistochemistry were reported to be negative, however, given the consistent evidence from all other aspects of workup and investigation the patient was commenced on a trial of prednisone. This resulted in marked reduction in photopsia and expansion of visual fields.
CONCLUSION
The diagnosis of autoimmune retinopathy is ultimately clinical in nature. Despite the absence of positive retinal autoantibodies, a diagnosis of autoimmune retinopathy was made based upon consistent evidence from all other aspects of workup and investigation. This allowed prompt treatment with immunosuppressive agents with subsequent stabilization of this patient's visual function.
Topics: Adult; Autoimmune Diseases; Chickenpox; Electroretinography; Fluorescein Angiography; Fundus Oculi; Humans; Male; Retina; Retinal Degeneration; Tomography, Optical Coherence; Visual Acuity; Visual Fields
PubMed: 28825165
DOI: 10.1007/s10792-017-0687-z -
Retinal Cases & Brief Reports 2019To describe the multimodal imaging findings of transient subretinal deposits occurring in multiple evanescent white dot syndrome (MEWDS).
PURPOSE
To describe the multimodal imaging findings of transient subretinal deposits occurring in multiple evanescent white dot syndrome (MEWDS).
METHODS
The multimodal imaging characteristics of transient subretinal deposits occurring in MEWDS were investigated with ultra-widefield color and fundus autofluorescence, cross-sectional and en-face optical coherence tomography (OCT), en face OCT-angiography, and quantitative autofluorescence.
RESULTS
A 28-year-old woman presented with photopsia and temporal visual field loss in her right eye. Her best-corrected visual acuity was 20/20 in her right eye and 20/25 in her left eye. Funduscopic examination showed characteristic peripapillary hyperautofluorescent white dots of MEWDS corresponding to ellipsoid zone disruption on OCT. These lesions became confluent throughout the posterior fundus over the next 4 weeks. As the patient's symptoms were resolving, a second type of transient hyperautofluorescent lesion was noted which corresponded to hyperreflective subretinal deposits on cross-sectional and en face structural OCT. These subretinal deposits were most evident at 10-week follow-up and had nearly resolved at 14-week follow-up. Quantitative autofluorescence showed that, unlike the acute MEWDS lesions, the hyperautoflurescence of the subretinal deposits persisted after photobleaching. At multiple time points over 14 weeks of follow-up, OCT angiography showed no evidence of retinal or choroidal flow abnormalities.
CONCLUSION
Transient subretinal deposits may develop during MEWDS in areas of previous diffuse outer retinal disruption. As these deposits remain hyperautoflurescent on quantitative autofluorescence after photobleaching, they may represent accumulations of debris originating from damaged photoreceptor outer segments.
Topics: Adult; Disease Progression; Female; Fluorescein Angiography; Follow-Up Studies; Fundus Oculi; Humans; Multimodal Imaging; Ophthalmoscopy; Retina; Retinal Diseases; Tomography, Optical Coherence; White Dot Syndromes
PubMed: 28614139
DOI: 10.1097/ICB.0000000000000602 -
European Journal of Ophthalmology Jul 2019To describe the diagnostic complexities of a patient with acute zonal occult outer retinopathy.
PURPOSE
To describe the diagnostic complexities of a patient with acute zonal occult outer retinopathy.
CASE REPORT
A healthy female presented with acute bilateral visual field loss and photopsia. On initial examination, her visual acuity, colour vision, fundus examination and fluorescein angiography were normal. Fundus autofluorescence, however, revealed scattered hyperautofluorescence in both eyes and electrodiagnostic tests were abnormal. A differential diagnosis including acute zonal occult outer retinopathy was postulated and serology requested. The patient's past medical history included both breast and endometrial cancer and raised the possibility of cancer-associated retinopathy. A normal full-body positron emission tomography scan, negative antibodies for cancer-associated retinopathy and abnormal electroretinogram led to a diagnosis of acute zonal occult outer retinopathy.
CONCLUSION
It has been more than 20 years since Gass first described the syndrome of acute zonal occult outer retinopathy. Typically affecting young, healthy females, acute zonal occult outer retinopathy is characterised by photopsia, minimal fundoscopic changes and electroretinographic abnormalities. Visual field loss is permanent and often associated with slow-progressing degeneration of the retinal pigment epithelial cells. Retinal photoreceptor dysfunction is an uncommon and unrecognised cause of acute visual loss. Acute zonal occult outer retinopathy should be considered as a differential diagnosis in those patients with deceptively normal fundal examinations, abnormal electroretinograms and visual field loss.
Topics: Diagnosis, Differential; Electroretinography; Female; Fluorescein Angiography; Glucocorticoids; Humans; Immunoglobulins, Intravenous; Middle Aged; Prednisolone; Retina; Scotoma; Tomography, Optical Coherence; Vision Disorders; Visual Acuity; Visual Field Tests; Visual Fields; White Dot Syndromes
PubMed: 31282209
DOI: 10.1177/1120672119853143 -
Medicine Jan 2020Tranexamic acid (TA) has been demonstrated to reduce blood loss and the incidences of postpartum hemorrhage (PPH) during caesarean sections. We compared the clinical... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Tranexamic acid (TA) has been demonstrated to reduce blood loss and the incidences of postpartum hemorrhage (PPH) during caesarean sections. We compared the clinical efficacy of TA administration on vaginal deliveries with recently published papers.
METHODS
Electronic databases of PubMed, Cochrane Library, Embase and Chinese CNKI (Chinese database) and Wanfang were searched through November 2019.The randomized controlled trials were selected between TA and control groups. The relevant studies included four trials with a total of 4579 patients.
RESULTS
Patients treated with TA had a reduction in total blood loss (P = .009), lower postoperative blood loss (P < .00001), a reduced number of PPH (P = .02). However, the occurrence of nausea or/and vomiting is higher in the TA group (the incidence of nausea or vomiting [P < .00001], nausea [P < .00001] and vomiting [P < .00001]).
CONCLUSION
TA resulted in fewer occurrence rates of PPH, and no significant increase in occurrences of dizziness or photopsia, but higher incidence of vomiting and nausea.
Topics: Antifibrinolytic Agents; Delivery, Obstetric; Female; Humans; Postpartum Hemorrhage; Randomized Controlled Trials as Topic; Tranexamic Acid
PubMed: 32011478
DOI: 10.1097/MD.0000000000018792 -
American Journal of Ophthalmology Case... Jun 2019To report a case of presumed choroidal metastasis from soft tissue myoepithelial carcinoma and highlight challenges in its diagnosis.
PURPOSE
To report a case of presumed choroidal metastasis from soft tissue myoepithelial carcinoma and highlight challenges in its diagnosis.
OBSERVATIONS
A 52-year-old man was referred with a two-week history of photopsia in his left eye. His background medical history included known soft tissue myoepithelial carcinoma metastatic to his bone, lung, liver and chest wall. A large, raised, yellow choroidal lesion was identified nasal to and abutting the optic disc. This lesion demonstrated growth 1 month after presentation. The patient died with widespread metastatic disease 5 months after initial presentation.
CONCLUSION AND IMPORTANCE
Soft tissue myoepithelial carcinoma can rarely metastasise to the choroid and present as a rapidly-growing, yellow, echodense tumour with serous retinal detachment. MRI brain can assist in tumour evaluation and monitoring progression, while immunoperoxidase stains and molecular testing can assist with diagnosis. The condition has an aggressive natural history and poor prognosis.
PubMed: 30886937
DOI: 10.1016/j.ajoc.2019.02.009 -
Retinal Cases & Brief ReportsBirt-Hogg-Dubé Syndrome (BHDS) is a rare autosomal dominant condition that can predispose patients to numerous cutaneous fibrofolliculomas and other cutaneous lesions,... (Observational Study)
Observational Study
PURPOSE
Birt-Hogg-Dubé Syndrome (BHDS) is a rare autosomal dominant condition that can predispose patients to numerous cutaneous fibrofolliculomas and other cutaneous lesions, pulmonary cysts with spontaneous pneumothorax, and multifocal renal tumors and cancer. The genetic mutations responsible for BHDS are related to tumor suppression and the mammalian target of rapamycin (mTOR) pathway. Previous reports of the ocular findings in BHDS include eyelid fibrofolliculomas, "flecked chorioretinopathy," and one report of choroidal melanoma. We report a patient with BHDS who presented with choroidal melanoma, sector melanocytosis, and retinal pigment epithelial microdetachments.
METHODS
Observational case report.
RESULTS
A 38-year-old man with BHDS manifesting with facial fibrofolliculomas/tricodiscomas and pulmonary cysts with previous pneumothorax of both lungs was noted to have a large choroidal nevus, managed with observation elsewhere. On referral 1 year later, photopsia and visual field defect were noted, and the patient was discovered to have subtle patchy sector ocular melanocytosis of the iris and choroid with choroidal melanoma and dependent subretinal fluid. The melanoma measured 14 mm in basal dimension and 6.8 mm in thickness. In addition, pinpoint retinal pigment epithelial microdetachments were observed in both eyes at the equator and confirmed on optical coherence tomography. Custom-fit plaque radiotherapy was provided for tumor control.
CONCLUSION
The BHDS can be associated with tumors of the skin and kidney. In this case, we noted ocular melanocytosis, malignant choroidal melanoma, and bilateral pinpoint retinal pigment epithelial detachments.
Topics: Adult; Birt-Hogg-Dube Syndrome; Choroid Neoplasms; Humans; Male; Melanocytes; Retinal Detachment; Retinal Pigment Epithelium
PubMed: 28541267
DOI: 10.1097/ICB.0000000000000595 -
Retina (Philadelphia, Pa.) Mar 2019To review and describe in detail the demographics, functional and anatomical characteristics, and clinical course of pigmented paravenous chorioretinal atrophy in a...
PURPOSE
To review and describe in detail the demographics, functional and anatomical characteristics, and clinical course of pigmented paravenous chorioretinal atrophy in a large cohort of adults and children.
METHODS
This is a retrospective case series of consecutive patients diagnosed with pigmented paravenous chorioretinal atrophy at a single U.K. referral center from 1974 to 2016. Clinical records, retinal imaging (color fundus photography, fundus autofluorescence, and optical coherence tomography), and electrophysiological assessments were reviewed.
RESULTS
Twenty-three patients were identified (13 males and 10 females). The mean age at presentation was 35 years (range 10-67 years). Mean follow-up was 6.7 years (range 0-30 years). There was no family history of similar retinal disease. Thirteen (57%) patients were asymptomatic. Symptoms included photopsia (n = 1.4%), blurred vision (n = 4.17%), peripheral visual field loss (n = 3.13%), and nyctalopia (n = 2.8%). One patient had previous intermediate uveitis. Twenty-one (91%) patients had ≥6/12 in the better seeing eye at final follow-up; visual acuity loss over time was recorded in 2 patients. Color vision was normal in all 14 patients assessed. Paravenous hypoautofluorescence with surrounding increased fundus autofluorescence was characteristically observed. Optical coherence tomography over the retinal changes demonstrated choroidal, retinal pigment epithelium, and outer retinal layer thinning. Peripapillary atrophic changes on fundus photography were evident in 20 (87%) patients. Interocular asymmetry of fundus and electroretinography findings was common. The electroretinography findings showed a similar degree of generalized rod and cone photoreceptor dysfunction in most cases.
CONCLUSION
Overall, most patients with pigmented paravenous chorioretinal atrophy maintained stable vision. The lack of other affected family members, slow or absent progression, and interocular asymmetry of the retinal features is suggestive of an acquired rather than inherited retinal disorder, which is generally nonprogressive. We identify that patients commonly have marked interocular asymmetry both on structural and functional assessment.
Topics: Adolescent; Adult; Aged; Child; Choroid; Electroretinography; Eye Diseases, Hereditary; Female; Humans; Male; Middle Aged; Retinal Degeneration; Retinal Pigment Epithelium; Retrospective Studies; Vision Disorders; Visual Acuity; Visual Fields; Young Adult
PubMed: 29300249
DOI: 10.1097/IAE.0000000000001950 -
Klinische Monatsblatter Fur... Jun 2019Pharmacological vitreolysis with ocriplasmin is an effective treatment option for eyes with vitreomacular traction. Pre-marketing and post-marketing clinical studies... (Review)
Review
Pharmacological vitreolysis with ocriplasmin is an effective treatment option for eyes with vitreomacular traction. Pre-marketing and post-marketing clinical studies revealed an improvement of visual function in ocriplasmin treated eyes and showed a release of traction in up to 78% of cases. Treatment success is related to patient selection based on positive predictive factors. Adverse events, such as visual acuity loss, dyschromatopsia or photopsia are known to be self-limited in the majority of eyes. Structural outer retinal layer changes, such as ellipsoid zone disturbances or subretinal fluid accumulation on SD-OCT analysis, as well as ERG abnormalities, are transient and correlated to VMT release. Surgical outcomes in patients with a prior history of ocriplasmin injection have been shown to be comparable with patients who proceeded directly to surgery without ocriplasmin treatment.
Topics: Fibrinolysin; Fibrinolytic Agents; Humans; Intravitreal Injections; Peptide Fragments; Vitreous Detachment
PubMed: 28599332
DOI: 10.1055/s-0043-106855 -
European Journal of Ophthalmology Jan 2022The purpose of this study is to report a case of venous-air embolism during a vitrectomy for endoresection of choroidal melanoma.
INTRODUCTION
The purpose of this study is to report a case of venous-air embolism during a vitrectomy for endoresection of choroidal melanoma.
CASE DESCRIPTION
A 31-year-old man went to the clinic because of photopsias and vision loss in his right eye. On fundoscopy of the right eye, a choroidal mass with an associated retinal detachment was found near the inferotemporal vascular arcade. Multimodal imaging was performed and diagnosis of choroidal melanoma was made. Metastatic workup ruled out systemic extension. The patient underwent pars plana vitrectomy for endoresection of the lesion. During the application of laser under air, he started complaining of chest pain and dyspnea. He presented signs of supraventricular tachycardia, tachypnea, hypotension and oxygen desaturation. He was managed with orotracheal intubation, bronchodilators and vasopressor support, and stabilization was achieved. He was discharged 2 days after with no sequalae. After 1-year of follow-up, the patient has a visual acuity of counting fingers and no signs of tumor recurrence or systemic extension.
CONCLUSIONS
Although rare, vitreoretinal surgeons should be aware of this potentially fatal complication and take steps to prevent it.
Topics: Adult; Choroid Neoplasms; Embolism, Air; Humans; Male; Melanoma; Neoplasm Recurrence, Local; Retinal Detachment; Vitrectomy
PubMed: 33626942
DOI: 10.1177/1120672121995101 -
European Journal of Neurology Oct 2020Visual snow manifests as a pan-field, dynamic visual disturbance described as continuous television static-like tiny flickering dots. Current diagnostic criteria further... (Comparative Study)
Comparative Study
BACKGROUND AND PURPOSE
Visual snow manifests as a pan-field, dynamic visual disturbance described as continuous television static-like tiny flickering dots. Current diagnostic criteria further require at least two additional symptoms for visual snow syndrome (VSS) from: palinopsia (afterimages and trailing); entoptic phenomena (floaters, blue field entoptic phenomenon, photopsia, self-light of the eye); photophobia and nyctalopia. Our objective was to compare the phenotype of VSS in an Italian and British population.
METHODS
Patients with VSS were characterized clinically using the current criteria. An online survey was prepared in collaboration with the patient group Eye-on-Vision. Patients were directed to the site if they contacted us by email asking to be involved in research. After data collection, we compared the phenotypic characteristics of a subgroup of British versus Italian patients taking part in the survey. As we expected more responses from the UK, we matched 100 UK patients for gender and age with our Italian cohort.
RESULTS
Patients were enrolled from the UK (n = 100) and Italy (n = 100). The populations had similar demography. After multiple correction testing there were no differences in VSS features between the two groups. The same was true for the prevalence of migraine and previous use of recreational drugs.
CONCLUSION
This is the first study comparing the phenotype of VSS between two distinct populations. Our findings suggest that the visual snow phenotype, as well as migraine comorbidity, is similar across the two groups.
Topics: Comorbidity; Humans; Italy; Migraine Disorders; Prevalence; Vision Disorders
PubMed: 32478974
DOI: 10.1111/ene.14369