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The pandemic of COVID-19 caused by the SARS-CoV-2 virus is ongoing and a serious menace to global public health. An ocular manifestation is an initial sign of the infection. To date, a comprehensive immune profile of patients with mild COVID-19 has not been well developed. Here, we report a 53-year-old female who noticed a sudden decrease in visual acuity (VA) in both eyes on the fourth day after COVID-19 infection. At presentation (acute phase), the best-corrected VA (BCVA) on the decimal chart was 0.5 in both the right and left eyes. Color fundus photography showed perivascular chorioretinal atrophy with peripheral pigment loss, similar to the fundus appearance of pigmented paravenous chorioretinal atrophy (PPCRA) in the inferior arcade vessels of both eyes. Optical coherence tomography indicated thinning and blurred boundaries of the outer retina in the lesion sites, implying anatomical destruction. She was followed up without any systemic medications. After approximately 15 weeks (remission phase), the BCVA recovered to 0.6 in the right eye and 0.8 in the left. Systemic immune profiles were analyzed using mass cytometry. In the acute phase, monocytes and basophils were dominantly elevated, which suggested the activation of innate immune responses to SARS-CoV-2 and allergic inflammation. In the remission phase, Th2-like cells, plasmablasts, and neutrophils increased predominantly, implying the maturation of adaptive immunity and the preparedness of innate immunity to combat the infection. Our findings indicate that perivascular chorioretinal atrophy resembling PPCRA is a clinical feature of the ocular phenotype of COVID-19, caused by systemic immune responses. [ABSTRACT FROM AUTHOR]

The pandemic of COVID-19 caused by the SARS-CoV-2 virus is ongoing and a serious menace to global public health. An ocular manifestation is an initial sign of the infection. To date, a comprehensive immune profile of patients with mild COVID-19 has not been well developed. Here, we report a 53-year-old female who noticed a sudden decrease in visual acuity (VA) in both eyes on the fourth day after COVID-19 infection. At presentation (acute phase), the best-corrected VA (BCVA) on the decimal chart was 0.5 in both the right and left eyes. Color fundus photography showed perivascular chorioretinal atrophy with peripheral pigment loss, similar to the fundus appearance of pigmented paravenous chorioretinal atrophy (PPCRA) in the inferior arcade vessels of both eyes. Optical coherence tomography indicated thinning and blurred boundaries of the outer retina in the lesion sites, implying anatomical destruction. She was followed up without any systemic medications. After approximately 15 weeks (remission phase), the BCVA recovered to 0.6 in the right eye and 0.8 in the left. Systemic immune profiles were analyzed using mass cytometry. In the acute phase, monocytes and basophils were dominantly elevated, which suggested the activation of innate immune responses to SARS-CoV-2 and allergic inflammation. In the remission phase, Th2-like cells, plasmablasts, and neutrophils increased predominantly, implying the maturation of adaptive immunity and the preparedness of innate immunity to combat the infection. Our findings indicate that perivascular chorioretinal atrophy resembling PPCRA is a clinical feature of the ocular phenotype of COVID-19, caused by systemic immune responses.

Pigmented paravenous chorioretinal atrophy (PPCRA) is a rare idiopathic retinopathy. We report monozygotic twins, discordant for PPCRA. There were no signs of active uveitis and the basic uveitis workup was noncontributory. PPCRA can be an acquired disorder, but epigenetic factors, and germline disease-causing variants cannot be excluded. Purpose: To demonstrate phenotypic discordance between a monozygotic twin pair, one of whom exhibited pigmented paravenous chorioretinal atrophy (PPCRA). Methods: A patient and his identical twin brother, attending Moorfields Eye Hospital, were reviewed. Clinical assessment included visual acuity and color vision testing, fundus imaging including autofluorescence, spectral-domain optical coherence tomography, and static perimetry. In addition, the affected sibling underwent pattern and full-field electroretinography (PERG and ERG) according to ISCEV standards. Zygosity testing was performed using short tandem repeat analysis. Results: The 48-year-old proband was referred with abnormal visual fields and difficulty reading at near. Examination revealed 20/20 Snellen visual acuity bilaterally, normal color vision, and bilateral asymmetric outer retinal atrophy with intraretinal pigment migration along the course of the retinal veins, consistent with PPCRA. The visual field defects were contiguous with the blind spot and mirrored the retinal involvement in both eyes. Pattern ERG showed mild macular dysfunction and full-field ERG was within normal limits. Blood testing for common uveitic entities was noncontributory. The proband's twin brother's clinical assessment and retinal imaging showed no abnormality. Zygosity testing showed the twins to be identical for 24 short tandem repeat microsatellite markers, indicative of monozygosity. Conclusion: Some cases of PPCRA, without an obvious inflammatory etiology, do not have a clear Mendelian inheritance pattern and may represent an acquired disorder. [ABSTRACT FROM AUTHOR]

A 60-year-old male patient admitted to our clinic with complaints of decreased vision, nyctalopia, and metamorphopsia in both eyes, mainly in the left eye. The best-corrected visual acuity was determined as 20/25 on the right and 20/200 on the left eye with the Snellen chart. Anterior segment examination was normal. Fundus examination revealed chorioretinal atrophic areas and bone spicules around all vascular arcades in both eyes. Optical coherence tomography displayed normal foveal architecture in the right eye; however, retinal pigment epithelial detachment, intraretinal cysts, subretinal fluid, and subretinal hyperreflective material were detected in the left eye. Examination findings and fluorescein angiography were supported by visual field and electrophysiological tests, and the patient was diagnosed as pigmented paravenous chorioretinal atrophy (PPCRA) with Type 1 macular neovascularization. We aimed to present the co-occurrence of PPCRA and macular neovascularization to the best of our knowledge, which has not been reported previously. [ABSTRACT FROM AUTHOR]

Pigmented paravenous chorioretinal atrophy (PPCRA) is an uncommon form of chorioretinal atrophy characterized by perivenous aggregations of pigment clumps associated with peripapillary and radial zones of retinal pigment epithelial atrophy that are distributed along the retinal veins. Most patients are asymptomatic, and evidence suggest that PPCRA is slowly progressing. Unless macular involvement is present, the majority of patients usually retain a normal visual function. Our ability to diagnose PPCRA has recently improved thanks to multimodal imaging, especially with the advent of ultra-widefield (UWF) imaging. Blood tests and functional and genetic testing can help with the correct differential diagnosis of pseudo-PPCRA or other disorders with similar characteristics. Although the cause of PPCRA is unknown, it is possible that it has a genetic basis. In this review we provide a summary of the multimodal imaging characteristics of PPCRA, and discuss its possible pathogenesis, based on the genes that have been associated with this disease.

Background: Pigmented Paravenous Chorioretinal Atrophy (PPCRA) is a rare and predominantly sporadic form of chorioretinal atrophy. Ocular and systemic inflammation has been considered a possible etiology of PPCRA. In this report, we describe an unusual case of PPCRA in a child who was recently diagnosed with chronic granulomatous disease. Case description: A 4-year-old boy was referred for ophthalmic assessment after a seizure. Fundus examination revealed atrophic chorioretinal lesions typical of PPCRA. We had also referred this patient to a gastroenterologist for chronic abdominal pain and diarrhea. The patient was first diagnosed as a case of Crohn's disease, but in the setting of mesenteric lymphadenopathy, a workup for immune dysfunction was performed. Nitro-blue tetrazolium test (NBT) was negative, suggesting a chronic granulomatous disease, which was finally confirmed by genetic testing. Conclusion: The presentation of PPCRA has been sporadic in the majority of cases. Inflammatory and hereditary origins have been anecdotally cited. Our young patient showed concurrent presentation of inflammatory and hereditary origin of PPCRA. We suggest that a careful investigation of systemic inflammation should be done in children with suggestive extraocular symptoms in the setting of PPCRA. [ABSTRACT FROM AUTHOR]

Purpose: To report an atypical case of unifocal, unilateral pigmented paravenous retinochoroidal atrophy (PPRCA) characterized by thickening and cystic degeneration of the retinal nerve fiber layer (RNFL). Observations: A 79-year old Asian woman presented with a large area of atrophic, pigmented change along the inferior arcade of her right eye. She denied nyctalopia and any other visual complaints. Visual acuity was 20/40 in both eyes and visual fields were significant for a large absolute peripheral scotoma superiorly in the affected eye corresponding to the atrophic area. Spectral domain optical coherence tomography through the lesion showed loss of choroid except for largest Haller's layer vessels, significant retinal pigment epithelium atrophy with migration and pigment clumping, outer retinal layer loss and RNFL thickening with cystic degeneration. Fundus autofluorescence imaging showed a large area of hypoautofluorescence corresponding to the area of atrophy. Full field electroretinogram demonstrated normal scotopic response and reduced photopic response in the right eye. Conclusions and importance: PPRCA is typically bilateral and symmetric, affecting primarily the outer retina and choroid. However, in rare cases, this disease can present unilaterally and/or unifocally, with degeneration extending to the inner retinal layers.

Purpose: To report a case of Pigmented Paravenous Chorioretinal Atrophy (PPCRA) associated with a novel RPGRIP1 dominant variant., Methods: Case report. The patient underwent multimodal retinal imaging, including spectral-domain optical coherence tomography (OCT), OCT Angiography (OCTA), blue-light autofluorescence (BAF), and ultra-widefield pseudocolor retinography and autofluorescence. Genetic testing was performed using next-generation sequencing., Results: A 67-year-old male presented with a clinical suspicion of retinitis pigmentosa. His best-corrected visual acuity was 20/32 in the right eye and 20/200 in the left eye. On fundus examination, paravenous pigment clumping and chorioretinal atrophy were seen bilaterally, matching confluent hypoautofluorescent areas departing from the optic disc. This clinical presentation suggested a case of PPCRA. Genetic testing found a heterozygous deletion of nucleotide 631 (c.631del) in the RPGRIP1 gene, a frameshift variant that generates a premature stop codon (p.Ser211Valfs*64) and therefore results in a truncated or absent protein product. The variant was regarded as likely pathogenic (class IV)., Conclusion: In this report, we describe a case of PPCRA in association with a novel, likely pathogenic c.631del, p.Ser211Valfs*64 variant in RPGRIP1 , a gene that has been associated with Leber congenital amaurosis and cone-rod dystrophy. Our case expands the spectrum of genes associated with PPCRA and prompts further studies to ascertain the molecular etiopathogenesis of this disease.

In this retrospective case series, we studied the predisposing factors, causative organisms, clinical spectrum, and outcomes of 12 cases of culture-proven infectious scleritis. Nine of 12 patients had a history of preceding trauma (surgical or accidental). Past surgical history included small-incision cataract surgery (4), pterygium surgery (1), and trabeculectomy (1). Six patients had multifocal scleral abscesses due to Pseudomonas, Klebsiella, or Nocardia. Only 2 patients retained useful vision (>6/18). A poor visual acuity at presentation usually resulted in a worse visual outcome (P = 0.005). Four eyes developed phthisis. The addition of surgical intervention did not result in a significantly better visual outcome than medical management alone (P = 0.209), but resulted in a higher globe preservation rate (P = 0.045). Therefore, we concluded that infection must be ruled out in cases of scleritis with preceding history of trauma, and aggressive surgical intervention improves the anatomical outcome but does not change the visual outcome.

Congenital eyelid imbrication syndrome (CEIS) is an extremely rare, benign, transient, self-limiting eyelid malposition disorder. The classic triad of signs in patients with a CEIS consists of bilateral upper eyelids overriding the lower eyelids when child was in sleep, bilateral medial and lateral canthal tendon laxity and tarsal conjunctival hyperemia. We report a third case of congenital combined eyelid imbrication and floppy eyelid syndrome in healthy neonate that was resolved within a week with conservative treatment.

Amblyopia screening is often either costly or laborious. We evaluated the Canon Powershot TX1 (CPTX1) digital camera as an efficient screener for amblyogenic risk factors (ARF). We included 138 subjects: 84-amblyopes and 54-normal. With the red-eye-reduction feature off, we obtained Bruckner reflex photographs of different sized crescents which suggested anisometropia, while asymmetrical brightness indicated strabismus; symmetry implied normalcy. Eight sets of randomly arranged 138 photographs were made. After training, 8 personnel, marked each as normal or abnormal. Of the 84 amblyopes, 42 were strabismus alone (SA), 36 had anisometropia alone (AA) while six were mixed amblyopes (MA). Overall mean sensitivity for amblyopes was 0.86 (95% CI: 0.83-0.89) and specificity 0.85 (95% CI: 0.77-0.93). Sub-group analyses on SA, AA and MA returned sensitivities of 0.86, 0.89 and 0.69, while specificities were 0.85 for all three. Overall Cohen′s Kappa was 0.66 (95% CI: 0.62-0.71). The CPTX1 appears to be a feasible option to screen for ARF, although results need to be validated on appropriate age groups.

Ischemic abducens nerve palsy usually presents as isolated cranial nerve palsy in the middle aged and elderly patients with known risk factors such as diabetes mellitus, hypertension, dyslipidemia, carotid artery disease, etc., In this report, we describe four patients with isolated abducens nerve palsy who presented with an acute onset diplopia whose detailed history and examination were suggestive of an ischemic etiology. Detailed systemic and laboratory evaluation revealed hyperhomocysteinemia as the only potential risk factor. To the best of our knowledge this is the first report of association of hyperhomocysteinemia and isolated abducens nerve palsy.

We investigated a case of unilateral optic nerve hypoplasia using spectral domain optical coherence tomography (SDOCT). Optical coherence tomography was done on both eyes using 5-line Raster scan for the fovea to analyze the retinal nerve fiber layer thickness, inner retinal layer thickness, outer retinal layer thickness, and optic disc cube scan for the disc. Retinal nerve fiber layer thickness, inner retinal layer thickness, and outer retinal layer thickness were manually measured at 21-points of each five lines, and results were compared between both eyes. Retinal nerve fiber layer thickness and inner retinal layer thickness of optic nerve hypoplasia were significantly thinner than the opposite eye, but there was no significant difference in the thickness of the outer retinal layer between both eyes.

Rhinosporidium seeberi , till recently known as a fungus, has been reclassified as a protistan parasite. It infects humans and many animal species. The authors describe a rare case of oculosporidiosis with involvement of the lacrimal sac exhibiting features of idiopathic orbital inflammatory disease in a young male patient. Clinical features, pathophysiology, and management of lacrimal sac rhinosporidiosis have been discussed.

We are presenting the initial results of inverted internal limiting membrane (ILM) flap technique for large macular hole. Five eyes of five patients with large diameter macular hole (>700 μm) were selected. All patients underwent inverted ILM flap technique for macular hole. Anatomical closure and functional success were achieved in all patients. There was no loss of best-corrected visual acuity in any of the patients. Inverted ILM flap technique in macular hole surgery seems to have a better hole closure rates, especially in large diameter macular holes. Larger case series is required to assess the efficacy and safety of this technique.

Systemic imaging of patients with suspect ocular tuberculosis include chest X-rays and computed tomography (CT) scans. Reports have suggested a role for 18 fluorodeoxyglucose-positron emission tomography/CT (FDG-PET/CT) scans. We report on the clinical utility of 18 FDG PET/CT in two patients. Case 1: A 38-year-old female patient presented with recurrent anterior uveitis. A 18 FDG-PET scan revealed metabolically active supraclavicular and chest lymph nodes. An aspiration cytology of the cervical lymph node revealed caseating granulomas suggestive of tuberculosis. Case 2: A 58-year-old female patient presented with recurrent anterior uveitis. A 18 FDG-PET scan revealed metabolically active lymph nodes in the neck. A biopsy of the cervical lymph node revealed epithelioid granulomas suggestive of tuberculosis. Both patients were started on standard antitubercular therapy with a subsequent marked reduction of activity. PET/CT scans may suggest the sites of safe high-yield biopsies.

Pigmented paravenous chorioretinal atrophy (PPCRA) is an uncommon retinal disorder of unknown etiology that is neither well understood nor classified. We report an atypical case of PPCRA, associated with Coat′s like response (CLR) in a 64-year-old man of Asian origin. Both the eyes were involved, though asymmetrically.

Paravenous pigmented chorioretinal atrophy (PPRCA) is a generally multifocal, bilateral and symmetric rare entity associated with autoimmune diseases and other ocular complications. We present the clinical case of a patient with rheumatoid arthritis who attended for pain of several days. He presented decreased visual acuity of the left eye (LE), nodular scleritis and chorioretinal atrophy with pigment accumulation in bone spicules in the inferior temporal vascular arcade and lamellar macular hole (AML). The right eye shows no alterations. LE autofluorescence (AF) shows a hypoautofluorescence lesion with defined edges. Fluorescein angiography (FAG) shows hyperfluorescence consistent with retinal pigmentary epithelial degeneration and blockage in pigment areas. The visual field (VC) reveals a defect in the superior hemifield. This case describes an atypical unifocal and unilateral PPRCA. This variant must be known to make a correct differential diagnosis, as well as to provide adequate prognostic information., (Copyright © 2023 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

Unilateral extensive myelination of the peripapillary nerve fibers may be associated with anisometropic myopia, strabismus, and reduced vision. Myelination of optic nerve fibers terminate at lamina cribrosa. Yet in some patients, myelination progresses into the peripapillary retinal nerve fibers and may affect the visual acuity. In this report, we described 4 patients. All patients presented extensive peripapillary myelinated nerve fibers associated with myopic anisometropia. After routine ophthalmic and orthoptic examinations, all patients underwent treatment for amblyopia through correction with spectacles, contact lenses, and the occlusion of the good eye. Corrected visual acuity improved in 1 patient, but 3 patients had no increase in visual acuity despite treatment with full cycloplegic refraction and appropriate patching. Probably because of structural abnormalies of the macula, visual results are often disappointing with appropriate correction of the refractive error and occlusion.

Pigmented paravenous chorioretinal atrophy (PPCRA) is a rare disorder which is diagnosed primarily because of the typical fundoscopic appearance of retinal pigment epithelial (RPE) atrophy and clumping in a paravenous distribution. A mildly affected and asymptomatic 54-year-old mother and her mildly affected daughter and severely affected son presented with pigmented paravenous chorioretinal atrophy. The severely affected (proband) 28-year-old man manifested the characteristic paravenous chorioretinal atrophy with pigment clusters in both eyes with macular involvement. Besides the characteristic fundus picture, he also had chronic angle closure glaucoma. His 23-year-old sister presented with unilateral involvement. Her right eye showed focal perivenular retinal pigment epithelial hyperplasia at the 2 o'clock position and dilated, tortuous retinal veins, while her left eye had only dilated and tortuous retinal veins. Both patients were hyperopic. Their mother had an area of chorioretinal atrophy in one eye near a retinal vein. The scotopic ERG responses were markedly abnormal in the male patient, while his sister had a mild decrease in amplitude of both a and b waves in both eyes. One of the children of an unaffected family member was found to have dilated and tortuous retinal veins and hyperopia (III-12). To our knowledge, this is the fourth report of familial occurrence of pigmented paravenous chorioretinal atrophy. The present pedigree is compatible with X-linked recessive or dominant inheritance. [ABSTRACT FROM AUTHOR]

Pigmented paravenous chorioretinal atrophy (PPCRA) is an uncommon retinal disorder of unknown etiology that is neither well understood nor classified. We report an atypical case of PPCRA, associated with Coat's like response (CLR) in a 64-year-old man of Asian origin. Both the eyes were involved, though asymmetrically. [ABSTRACT FROM AUTHOR]

To report fundus autofluorescence (FAF) imaging findings of a patient with pigmented paravenous chorioretinal atrophy (PPCRA). In a patient with incidentaly detected bilateral PPCRA, FAF images were recorded with a new-generation confocal scanning laser ophthalmoscope (Heidelberg Retina Angiograph 2, Heidelberg, Germany). Atrophic areas typically appeared as hypoautofluorescent on FAF imaging, and borders of the chorioretinal atrophy areas appeared hyperautofluorescent, where RPE metabolism was presumably disturbed but chorioretinal atrophy has not yet appeared. It was remarkable that in some paravenous areas, hyperautofluorescence was seen where there were no evident abnormal findings in fundoscopy. PPCRA has a typical appearance by FAF imaging. FAF imaging may be a useful noninvasive tool in the recognition of the activity and predicting the progression of the disease. [ABSTRACT FROM AUTHOR]

The aim of this study is to investigate fundus autofluorescence (FAF) as well as fluorescein angiography (FA), indocyanine green angiography (IA), and optical coherence tomography (OCT) in a patient with pigmented paravenous chorioretinal atrophy (PPCRA). A funduscopic examination revealed chorioretinal atrophy along the paravenous area in both eyes. A marked bone spicule pigment clumping together with the atrophy was noted left eye. FA and IA showed a window defect and hypofluorescence, respectively, which exclusively corresponds to the atrophic area along the retinal vein area and the optic disc both eyes. FAF revealed geographic hypofluorescence along the paravenous and supranasal retinal areas. Hyperfluorescence was noted, which comparatively surrounded the hypofluorescence in the peripheral paravenous distribution. Hypofluorescence detected by FAF corresponded to the areas of retinal thinning and atrophy detected by OCT and FA. FAF is a useful examination in PPCRA, which can noninvasively demonstrate the distribution of deficit and dysfunction of retinal pigment epithelium.