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Indian Journal of Ophthalmology Jul 2024Acute Zonal Occult Outer Retinopathy (AZOOR) is characterized by sudden visual impairment, often presenting with scotoma and photopsia in young to middle-aged adults,... (Review)
Review
Acute Zonal Occult Outer Retinopathy (AZOOR) is characterized by sudden visual impairment, often presenting with scotoma and photopsia in young to middle-aged adults, showing a female predominance. This condition, distinct from genetic disorders like retinitis pigmentosa, lacks a hereditary basis and exhibits unique fundus changes and imaging features indicative of outer retinal dysfunction. Recent advancements have broadened our understanding, identifying variants like Acute Annular Outer Retinopathy (AAOR) and Multizonal outer retinopathy and retinal pigment epitheliopathy (MORR), each with specific clinical presentations and imaging characteristics. The diagnosis of AZOOR and its variants primarily relies on excluding other conditions through comprehensive evaluation, including imaging and serological testing. Treatment approaches, including the use of corticosteroids and immunosuppressives, remain debated, with some evidence suggesting benefits in the early stages. The prognosis of AZOOR varies, with most patients experiencing stabilization, although complications like choroidal neovascularization may occur, requiring targeted therapy. This manuscript elucidates the complexity of AZOOR, emphasizing the necessity of high clinical suspicion and the role of advanced imaging in diagnosis and management.
Topics: Humans; White Dot Syndromes; Scotoma; Tomography, Optical Coherence; Fluorescein Angiography; Fundus Oculi; Visual Acuity; Visual Fields; Electroretinography
PubMed: 38454854
DOI: 10.4103/IJO.IJO_3228_23 -
JAMA Neurology Sep 2023
Topics: Humans; Evoked Potentials, Visual; Migraine Disorders; Vision Disorders; Retina
PubMed: 37358862
DOI: 10.1001/jamaneurol.2023.1889 -
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 -
Indian Journal of Ophthalmology Nov 2020
Topics: Humans; Lung Neoplasms; Retinal Diseases; Vision Disorders
PubMed: 33120614
DOI: 10.4103/ijo.IJO_58_20 -
Journal of Vitreoretinal Diseases 2022This work presents a case of syphilitic outer retinopathy with findings similar to those of acute zonal occult outer retinopathy (AZOOR). We also discuss the clinical... (Review)
Review
PURPOSE
This work presents a case of syphilitic outer retinopathy with findings similar to those of acute zonal occult outer retinopathy (AZOOR). We also discuss the clinical characteristics, treatment, and prognosis of this entity.
METHODS
A case report and systematic literature review are presented.
RESULTS
A 56-year-old woman presented with acute vision loss, localized photopsia, a central scotoma, and retinal findings that were all consistent with AZOOR. A further workup led to a diagnosis of syphilis. Oral prednisone and intravenous penicillin resulted in the resolution of the posterior uveitis and the restoration of visual acuity. However, the central scotoma remained at the 3-year follow-up visit.
CONCLUSIONS
Syphilitic outer retinopathy is a distinct entity characterized by the disruption of the ellipsoid zone visible on optical coherence tomography and a corresponding increase in fundus hyperautofluorescence in the affected areas. Although some patients may present with a demarcation line, as is seen with AZOOR, the fundus is oftentimes unremarkable or may show only subtle retinal pigment epithelium changes. Uveitis resolution and visual acuity restoration may be expected following treatment; however, visual field disturbances may persist.
PubMed: 37007722
DOI: 10.1177/24741264211018300 -
JNMA; Journal of the Nepal Medical... Feb 2021Idiopathic Intracranial Hypertension is a rare occurrence in young, physically fit male and a diagnosis of exclusion among most patients presenting with signs and...
Idiopathic Intracranial Hypertension is a rare occurrence in young, physically fit male and a diagnosis of exclusion among most patients presenting with signs and symptoms of raised intracranial pressure. Here we describe a case of a young male in the ideal weight range with no previous exposure to offending chemicals presented with a history of headache, obscuration of vision, and photopsia. On examination, there were no positive neurological findings. Increased opening pressure was found on the lumbar puncture. Ophthalmological examination revealed bilateral papilledema. Humphrey’s Visual field test showed peripheral field loss. Magnetic resonance imaging scan of the brain and orbits were normal. The patient was diagnosed and managed in primary care setting after neurosurgical consultation. Though rare, we should suspect idiopathic intracranial hypertension in ideal body weighted male if the headache is persistent after other causes of headache have been ruled out.
Topics: Headache; Humans; Intracranial Hypertension; Male; Papilledema; Pseudotumor Cerebri; Vision Disorders
PubMed: 34506475
DOI: 10.31729/jnma.5176 -
Retina (Philadelphia, Pa.) Nov 2023To describe specific clinical, multimodal imaging, and natural history features of an unusual variant of acute zonal occult outer retinopathy. (Observational Study)
Observational Study
PURPOSE
To describe specific clinical, multimodal imaging, and natural history features of an unusual variant of acute zonal occult outer retinopathy.
METHODS
Retrospective, observational, longitudinal, multicenter case series. Patients exhibiting this unusual clinical condition among cases previously diagnosed with acute zonal occult outer retinopathy were included. Multimodal imaging, laboratory evaluations, and genetic testing for inherited retinal diseases were reviewed.
RESULTS
Twenty eyes from 10 patients (8 females and 2 males) with a mean age of 54.1 ± 13.3 years (range, 38-71 years) were included. The mean follow-up duration was 13.1 ± 5.3 years (range, 8-23 years). Presenting symptoms were bilateral in 7 patients (85% of eyes) and included scotomata and photopsia. All patients had bilateral lesions at presentation involving the peripapillary and far peripheral retina. Baseline optical coherence tomography showed alteration of the retinal pigment epithelium and photoreceptor layers corresponding to zonal areas of fundus autofluorescence abnormalities. Centrifugal and centripetal progression of the peripapillary and far-peripheral lesions, respectively, occurred over the follow-up, resulting in areas of complete outer retinal and retinal pigment epithelium atrophy.
CONCLUSION
Initial alteration of photoreceptors and retinal pigment epithelium and a stereotypical natural course that includes involvement of the far retinal periphery, characterize this unusual condition. It may represent a variant of acute zonal occult outer retinopathy or may be a new entity. We suggest to call it multizonal outer retinopathy and retinal pigment epitheliopathy .
Topics: Adult; Aged; Female; Humans; Male; Middle Aged; Fluorescein Angiography; Retinal Diseases; Retinal Pigments; Retrospective Studies; Scotoma; Tomography, Optical Coherence; Visual Fields
PubMed: 37748093
DOI: 10.1097/IAE.0000000000003927 -
American Journal of Obstetrics &... Jul 2023The retina is potentially associated with several physiological, hormonal, and metabolic changes during pregnancy. The few available epidemiologic studies of ocular...
BACKGROUND
The retina is potentially associated with several physiological, hormonal, and metabolic changes during pregnancy. The few available epidemiologic studies of ocular changes in pregnancy have mainly concerned retinopathies. Pregnancy-induced hypertension, which leads to ocular manifestations including blurred vision, photopsia, scotoma, and diplopia, might induce reactive changes in the retinal vessels. Although several studies have suggested the existence of pregnancy-induced hypertension-related retinal ocular disease, there are few large cohort studies on this topic.
OBJECTIVE
This study aimed to investigate the risk of major retinal diseases including central serous chorioretinopathy, diabetic retinopathy, retinal vein occlusion, retinal artery occlusion, and hypertensive retinopathy in the long-term postpartum stage according to the presence of previous pregnancy-induced hypertension in a large cohort based on the Korean National Health Insurance Database.
STUDY DESIGN
On the basis of Korean health data, 909,520 patients who delivered from 2012 to 2013 were analyzed. Among them, patients who had previous ocular diseases or hypertension and multiple births were excluded. Finally, 858,057 mothers were assessed for central serous chorioretinopathy (ICD-10: H35.70), diabetic retinopathy (ICD-10: H36.0, E10.31, E10.32, E11.31, E11.32, E12.31, E13.31, E13.32, E14.31, E14.32), retinal vein occlusion (ICD-10: H34.8), retinal artery occlusion (ICD-10: H34.2), and hypertensive retinopathy (ICD-10: H35.02) for 9 years after delivery. Enrolled patients were divided into 2 groups: 10,808 patients with and 847,249 without pregnancy-induced hypertension. The primary outcomes were the incidence of central serous chorioretinopathy, diabetic retinopathy, retinal vein occlusion, retinal artery occlusion, and hypertensive retinopathy 9 years after delivery. Clinical variables were age, parity, cesarean delivery, gestational diabetes mellitus, and postpartum hemorrhage. In addition, pregestational diabetes mellitus, kidney diseases, cerebrovascular diseases, and cardiovascular diseases were adjusted.
RESULTS
Postpartum retinal disease during the 9 years after delivery and total retinal diseases showed higher rates in patients with pregnancy-induced hypertension. In detail, the rates of central serous chorioretinopathy (0.3% vs 0.1%), diabetic retinopathy (1.79% vs 0.5%), retinal vein occlusion (0.19% vs 0.1%), and hypertensive retinopathy (0.62% vs 0.05%) were higher than those found in patients without pregnancy-induced hypertension. After adjusting for confounding factors, pregnancy-induced hypertension was associated with development of postpartum retinopathy, with a >2-fold increase (hazard ratio, 2.845; 95% confidence interval, 2.54-3.188). Furthermore, pregnancy-induced hypertension affected the development of central serous chorioretinopathy (hazard ratio, 3.681; 95% confidence interval, 2.667-5.082), diabetic retinopathy (hazard ratio, 2.326; 95% confidence interval, 2.013-2.688), retinal vein occlusion (hazard ratio, 2.241; 95% confidence interval, 1.491-3.368), and hypertensive retinopathy (hazard ratio, 11.392; 95% confidence interval, 8.771-14.796) after delivery.
CONCLUSION
A history of pregnancy-induced hypertension increases the risk of central serous chorioretinopathy, diabetic retinopathy, retinal vein occlusion, and hypertensive retinopathy according to 9-year long-term ophthalmologic follow-up.
Topics: Pregnancy; Humans; Female; Hypertension, Pregnancy-Induced; Retinal Vein Occlusion; Central Serous Chorioretinopathy; Cohort Studies; Diabetic Retinopathy; Follow-Up Studies; Retinal Artery Occlusion; Hypertensive Retinopathy
PubMed: 37119970
DOI: 10.1016/j.ajogmf.2023.100985 -
IDCases 2020A healthy 47-year-old immunocompetent man from Northern Canada presented for ophthalmologic assessment after experiencing one month of right-sided photopsias, floaters,...
A healthy 47-year-old immunocompetent man from Northern Canada presented for ophthalmologic assessment after experiencing one month of right-sided photopsias, floaters, and a right lower nasal quadrant visual field defect. Optic disc swelling, vitritis, chorioretinitis, peripheral retinal infiltrates and hemorrhages were noted in the right eye. A broad right inferior arcuate and nasal visual field defect were also present. Fluorescein angiography of the right retina showed dilated disc vessels and staining of the optic disc. Treponemal antibody testing, using chemiluminescent microparticle immunoassay, was highly positive; this was followed by a Venereal Disease Research Laboratory (VDRL) test with a titre of 1:32 and confirmed by Treponema pallidum particle agglutination (TP-PA) test. Testing did not demonstrate any co-infections. Cerebrospinal fluid (CSF) analysis revealed strong reactivity (4+) to the Treponemal antibody by immunofluorescence antibody absorbed (FTA-ABS) test and non-reactivity by CSF VDRL test. Syphilis PCR of CSF was negative. A diagnosis of neurosyphilis was made. He was treated with ceftriaxone 2 grams IV q24h for 14 days. The vitritis gradually improved. Familiarity with syphilis diagnostics is becoming increasingly important, especially given its recent resurgence amongst several at risk groups. This patient's case highlights that non-reactive CSF VDRL is not a reliable test in the context of positive serum results and a compatible clinical picture. CSF Treponemal tests such as TP-PA and FTA-ABS offer higher sensitivity than non-treponemal tests such as VDRL in the context of CNS involvement and ocular syphilis.
PubMed: 32528847
DOI: 10.1016/j.idcr.2020.e00840