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Objective: To report acute multifocal retinitis in association with serologically-proven Coxiella (C) Burnetii infection (Q fever) with endocarditis. Material and methods: A single case report documented with multimodal imaging. Results: A 67-year-old cattle breeder presented with a 2-week history of persistent fever, headache, and floaters in both eyes. On examination, his best-corrected visual acuity was 20/20, and there was 1+ vitreous cells in both eyes. Bilateral fundus examination showed multiple small superficial white retinal lesions scattered in the posterior pole and midperiphery associated with a few retinal hemorrhages. These retinal lesions did not stain on fluorescein angiography (FA) and showed focal hyperreflectivity and thickening primarily involving the inner retinal layers on optical coherence tomography (OCT). There also was a band-like hyper-reflective area in the middle retina consistent with paramacular acute middle maculopathy. Transthoracic echocardiogram (TTE) showed a mobile echodensity on the anterior aortic leaflet consistent with a diagnosis of endocarditis. Elisa assays performed on paired serum samples collected 2 weeks apart showed increase in antibodies against C burnetii from 60 IU/ml to 255 IU/ml. The patient was treated with doxycycline 100 mg twice a day for 18 months, with subsequent resolution of the endocarditis. Sequential ocular examinations showed gradual resolution of all acute retinal findings without visible scars. Conclusion: Acute Q fever, caused by C burnetii infection, should be considered in the differential diagnosis of acute multifocal retinitis. A systematic cardiac assessment with echocardiography is essential for early diagnosis of associated endocarditis and for prompt administration of appropriate antibiotic treatment to improve clinical outcomes. [ABSTRACT FROM AUTHOR]

A diabetic 20-year-old male patient, not previously vaccinated against SARS-CoV-2, was referred to our emergency department for rapid bilateral visual loss with left periorbital pain, proptosis, palpebral edema and swelling. 1 A 20-year old COVID-19-affected patient presented with rhino-orbitocerebral mucormycosis. Hence, Ophthalmologists should have a high index of suspicion for mucormycosis development in diabetic patients with COVID-19 illness, treated with corticosteroids. [Extracted from the article]

Objective: To report a case of abducens nerve palsy with associated retinal involvement due to rickettsia typhi infection. Material and methods: A single case report documented with multimodal imaging. Results: A 18-year-old woman with a history of high-grade fever was initially diagnosed with typhoid fever and treated with fluoroquinolone. She presented with a 5-day history of diplopia and headaches. Her best-corrected visual acuity was 20/20 in both eyes. Ocular motility examination showed left lateral gaze restriction. Lancaster test confirmed the presence of left abducens palsy. Fundus examination showed optic disc swelling in both eyes associated with superotemporal retinal hemorrhage and a small retinal infiltrate with retinal hemorrhage in the nasal periphery in the left eye. Magnetic resonance imaging (MRI) of the brain and orbits showed no abnormalities. A diagnosis of rickettsial disease was suspected and the serologic test for Richettsia Typhi was positive. The patient was treated with doxycycline (100 mg every 12 h) for 15 days with complete recovery of the left lateral rectus motility and resolution of optic disc swelling, retinal hemorrhages, and retinal infiltrate. Conclusion: Rickettsial disease should be considered in the differential diagnosis of abducens nerve palsy in any patient with unexplained fever from endemic area. Fundus examination may help establish an early diagnosis and to start an appropriate rickettsial treatment. [ABSTRACT FROM AUTHOR]

Objective: To describe a case of Behçet disease (BD) uveitis manifesting with sequential bilateral neuroretinitis associated with prepapillary inflammatory vitreous exudate (PIVE). Material and methods: A single case report documented with multimodal imaging. Results: A 37-year-old man developed neuroretinitis with associated PIVE in the left eye. He was diagnosed with ocular toxoplasmosis and treated accordingly based on positive serologic testing and negative work-up for other entities, including BD. The disease course was favorable, but 1 year later a similar neuroretinitis developed in the right eye. Extraocular features of BD became evident only at the time of the second eye involvement, and the patient received corticosteroid and immunosuppressive therapy. Swept source (SS) OCT showed at the acute phase in both eyes a typical "mushroom-shaped" prepapillary hyperreflectivity of the PIVE. SS OCT angiography (OCTA) demonstrated a corresponding prepapillary hypointense area due to shadowing effect, decreasing in size while scanning deeper layers. It also detected peripapillary retinal hypervascularity in both eyes and a sectoral area of flow signal loss in the first involved left eye. Visual acuity improved following the resolution of the PIVE and associated acute inflammatory changes in both eyes. The left eye showed residual optic disc pallor and retinal nerve fiber layer defects. Conclusion: Sequential bilateral neuroretinitis associated with PIVE may occur before other clinical features of BD become evident. SS OCT and OCTA can provide useful information for the diagnosis and management of this rare, but typical, ocular manifestation of BD uveitis. [ABSTRACT FROM AUTHOR]

Background: The purpose of this single case report was to report the use of anterior segment optical coherence tomography for the diagnosis and management of a retained vegetal intraocular foreign body. Results: A 23-year-old otherwise healthy male presented with a progressive vision loss in the right eye (RE). He reported a mild ocular trauma with a tree leaf 1 year ago followed by recurrent episodes of redness and pain in the RE that partially resolved after a self-medication with topical steroids. Visual acuity of the RE was limited to light perception. Slit-lamp examination of the RE showed an iris granuloma with overlying exudate and associated anterior chamber inflammatory reaction. Film X-rays, contact B-scan ultrasonography, and CT scan showed no abnormalities. Anterior segment optical coherence tomography revealed an enclaved iris foreign body. The foreign body was removed after a short course of local antibio-corticosteroid therapy. This was followed 2 months later by cataract surgery with intraocular lens implantation, with subsequent improvement of visual acuity to 20/40. Conclusions: A missed intraocular foreign body can lead to sight-threatening complications. Anterior segment optical coherence tomography may be useful for detecting non-clinically evident intraocular foreign body involving the anterior segment masquerading as chronic anterior uveitis. [ABSTRACT FROM AUTHOR]

Background: Varicella is a common infectious disease primarily of childhood that is usually benign and self-limited. It is, however, increasingly seen in adults who are at a higher risk of severe infection. Ocular complications of varicella are relatively uncommon and have been rarely described in adults. We describe herein five adults who developed ocular involvement in association with primary varicella-zoster virus infection. Findings: Ocular manifestations included acute anterior uveitis in four eyes, with associated stromal keratitis in one of them, epithelial ulcerative keratitis in the two eyes of one patient, and acute retinal necrosis in one eye. One patient with acute anterior uveitis was treated with topical steroids and cycloplegic agents. The four other patients received topical or systemic antiviral drugs, with subsequent resolution of acute ocular inflammatory disease. Conclusions: The spectrum of chickenpox-associated ocular complications in adults is wide. Early diagnosis and appropriate management are mandatory to improve visual outcome. [ABSTRACT FROM AUTHOR]

Posterior microphthalmia (PM) is a relatively rare autosomal recessive condition with normal anterior segment and small posterior segment resulting in high hyperopia and retinal folding. It is an uncommon subtype of microphthalmia that has been mostly reported to coexist with several other ophthalmic conditions and to occur in sporadic cases. The membrane-type frizzled-related protein ( MFRP) is the only gene so far reported implicated in autosomal recessive, non-syndromic and syndromic forms of PM. Here, we performed a clinical and genetic analysis using six consanguineous families ascertained from different regions of Tunisia and affected with non-syndromic PM that segregates as an autosomal recessive trait. To identify the disease-causing defect in these families, we first analysed MFRP gene, then some candidate genes ( CHX10, OPA1, MITF, SOX2, CRYBB1-3 and CRYBA4) and loci (MCOP1, NNO1 and NNO2) previously implicated in different forms of microphthalmia. After exclusion of these genes and loci, we performed a genome-wide scan using a high density single nucleotide polymorphism (SNP) array 50 K in a large consanguineous pedigree. SNP genotyping revealed eight homozygous candidate regions on chromosomes 1, 2, 3, 6, 15, 17 and 21. Linkage analysis with additional microsatellite markers only retained the 2q37.1 region with a maximum LOD score of 8.85 obtained for D2S2344 at θ = 0.00. Further investigations are compatible for linkage of four more families to this region with a refined critical interval of 2.35 Mb. The screening of five candidate genes SAG, PDE6D, CHRND, CHRNG and IRK13 did not reveal any disease-causing mutation. [ABSTRACT FROM AUTHOR]

Microspherophakia seems to be the most specific feature of the Weill–Marchesani Syndrome, which could be due to mutations within the ADAMTS10 gene. As the locus responsible for isolated microspherophakia is still unknown, because the reported cases are rare, we checked whether the ADAMTS10 gene is involved in isolated microspherophakia in a Tunisian family. A consanguineous family (MSP-M), including six family members and two patients, presented with decreased vision secondary to bilateral isolated microspherophakia. A linkage analysis was carried out using microsatellite markers flanking the ADAMTS10 candidate gene. In the MSP-M family, isolated microspherophakia is likely inherited as an autosomal-recessive disease. Using a homozygosity-mapping strategy, haplotypic analysis using four STRs showed an exclusion of linkage between the ADAMTS10 gene and the disease locus in this family. Our study suggests that isolated microspherophakia and the Weill–Marchesani Syndrome are not allelic to the ADAMTS10 gene. [ABSTRACT FROM AUTHOR]

Background: White dot syndromes (WDS) are a group of idiopathic multifocal inflammatory conditions that can be recognized and distinguished by lesion morphology, other specific clinical features, imaging findings, and disease course. Our purpose is to describe an atypical case of WDS with choriocapillaris ischemia shown by multimodal imaging including swept-source OCT angiography (OCTA) in a 30-year-old woman with latent tuberculosis.Findings: At presentation, visual acuity in the left eye was 20/500. Clinical findings included macular granularity, diffuse outer retinal discoloration with satellite yellow-white dots, and disc margin blurring. Fluorescein angiography showed early confluent areas of choroidal hypofluorescence and late perifoveal punctate hyperfluorescence. There was choroidal hypofluorescence in a geographic configuration throughout the indocyanine green angiography sequence. OCTA showed confluent geographic areas of loss of signal in the choriocapillaris. Work-up revealed latent tuberculosis. The patient received corticosteroids and prophylactic anti-tubercular treatment. Nine months later, visual acuity had improved to 20/20, and there were some residual retinal pigment epithelium changes.Conclusion: Atypical WDS associated with choriocapillaris hypoperfusion may show features of multiple evanescent white dot syndrome and acute posterior multifocal placoid pigment epitheliopathy melting together. [ABSTRACT FROM AUTHOR]

Background: Acute multifocal retinitis is a rare condition that has been considered to be often idiopathic. The purpose of this study was to analyze clinical features and causes of acute multifocal retinitis.Results: This study is a retrospective review of the charts of 35 patients with acute multifocal retinitis. Patients with three or more retinal lesions in at least one eye, with at least one lesion of less than 500 μm in size were included. All patients had complete ophthalmological examination, fundus photography, and fluorescein angiography. Twelve patients (34.3%) had optical coherence tomography. An extensive work-up was performed including a detailed comprehensive medical history, examination by an internist and an infectious disease specialist, a chest X-ray, Mantoux test, and laboratory tests for syphilis, human immunodeficiency virus, Bartonella, and Rickettsia. Of the 35 patients, 25 (71.4%) had bilateral involvement and 10 (28.6%) had unilateral involvement (total number of eyes: 60). Mean best-corrected visual acuity (BCVA) was 20/25 (range, 20/1000-20/20). Retinal lesions ranged from 3 to more than 20 in number in at least 1 eye, and from 150 to 1500 μm in size. Associated findings included mild anterior chamber inflammation in 5 eyes (8.3%), mild vitritis in 46 eyes (76.7%), optic disc swelling in 9 eyes (15%), macular star in 4 eyes (6.7%), exudative retinal detachment in 6 eyes (10%), and branch retinal artery occlusion in 6 eyes (10%). Acute multifocal retinitis was found to be caused by Rickettsia conorii infection in 20 patients (57.1%), Rickettsia typhi infection in 4 patients (11.4%), cat-scratch disease in 8 patients (22.9%), and syphilis in 1 patient (2.9%). It was idiopathic in two patients (5.7%). Retinal lesions resolved without scarring in 3 to 12 weeks in all but three eyes (5%), in which residual retinal pigment epithelial changes were noted.Conclusion: Rickettsial disease was the most common cause of acute multifocal retinitis. Other identified causes included cat-scratch disease and syphilis, and a very small subset of patients was diagnosed with idiopathic multifocal retinitis. [ABSTRACT FROM AUTHOR]