Occlusive retinal vasculitis in an immunocompetent woman: rare presentation of ocular melioidosis

Burkholderia pseudomallei is a known great mimicker responsible for melioidosis. Ocular involvement in melioidosis is unusual, with eyelid and orbital infection as the commonest presentation. We describe a 41-year-old, healthy woman who complained of reduced vision in her left eye. On examination, vision in the left eye was 6/9. There was evidence of occlusive retinal vasculitis on fundoscopy examination. Fundus fluorescein angiogram showed extensive capillary fallout. Diagnosis was established by a rise in the serum antibody titre for the bacterium and further supported by clinical improvement of vision after completion of treatment antibiotics. Sectoral panretinal photocoagulation at the capillary fallout area successfully arrested the sequelae of retinal ischaemia. Occlusive retinal vasculitis is a rare presentation of melioidosis. Early prompt diagnosis in an immunocompetent individual helps prevent visual-related morbidity. The ability of this bacteria to cause recurrent infection in an endemic area should not be underestimated.

P048 Cytomegalovirus retinitis in a patient with rheumatoid arthritis on tofacitinib

Background/Aims Cytomegalovirus (CMV) retinitis is one of the most common opportunistic ocular infections and has traditionally been associated with immunocompromised individuals. We present a case of CMV retinitis following 3 months of treatment with tofacitinib in conjunction with methotrexate (MTX) for rheumatoid arthritis (RA) in an elderly lady with no other co-morbidities. Methods The case is presented below. Results 69-year-old lady with long standing seronegative, anti-cyclic citrullinated peptide negative, erosive RA since 2016. She was intolerant of sulphasalazine, leflunamide, methotrexate and etanercept. Hydroxychloroquine was avoided due to idiopathic cystoid macular oedema in the right eye with a baseline visual acuity of 6/18. Baseline fundus fluorescein angiogram (FFA) was negative for any vascular, inflammatory or occlusive disease. She was escalated to tofacitinib in August 2019. Approximately 3 months later, she noticed significantly reduced vision in her right eye from 6/18 down to 6/60. She had an FFA and Indocyanine Green Angiography, which showed quite extensive retinal ischaemia and vasculitis. Her bloods revealed a raised serum ACE and a T-spot test from 2018 was reactive followed by a repeat in 2019 which was equivocal. Tofacitinib was discontinued. She was commenced on rifampicin and isoniazid as 3 months prophylaxis for latent tuberculosis (LTBI). Additionally, she was started on oral prednisolone 60mg daily for presumed sarcoid related retinal vasculitis. She failed to improve despite a week’s treatment and subsequently had a vitreous aspirate which was strongly positive for CMV PCR. Her CMV viral load was 267copies/ml. The aspirate was negative for other infectious agents. She was commenced on valganciclovir for CMV retinitis. Unfortunately, her vision remained unchanged despite the viral load becoming undetectable a few weeks later. Despite having LTBI, there was no reactivation of this with her immunosuppressive therapy. Conclusion CMV retinitis is usually associated with immunocompromised individuals and is rarely encountered related to tofacitinib therapy for rheumatoid arthritis. Tofacitinib suppresses T cell function by inhibiting STAT signalling pathways, modulating T cell activation and cytokine production. Our case highlights the preferential development of CMV retinitis over active TB in the presence of LTBI, which is a recognised risk with the use of JAK kinase inhibitors. TB risk has been associated with genetic mutations in IL-12, IFN-γ and STAT1 pathways while CMV activation may be influenced by mutations in HLA ,Toll-like receptors, immunoglobulin light chain antibodies, IFN lambda region as well as alternative transcripts of the CMV viral major immediate early genes. Disclosure K. Beharry: None. S. Kaur: None. E. Papagiannuli: None. S. Moses: None. D. De Lord: None.

Rubeosis iridis and macular hemorrhages from multiple Corkscrew retinal vessels in a patient with NF1

Purpose: To report a case of a patient with NF1 presenting with ocular findings of AV malformation, multiple retinal hemorrhages, and neovascular glaucoma in the absence of retinal ischemia. Methods: Review of the medical record was conducted in accordance with the local IRBt. Results: A 60-year-old female patient with diagnosis of Neurofibromatosis type1 (NF1) and sudden decrease of vision in her left eye was found to have rubeosis iridis and high intraocular pressure (IOP). On fundus exam multiple corkscrew retinal vessels and retinal hemorrhages were present in her left eye. On Optical Coherence Tomography (OCT) the foveal hemorrhages appeared as outer layer hyperreflective retinal infiltrates whereas in the parafoveal area the hyperreflectivity was present between the RPE and neurosensory retina. Fluorescein Angiogram (FA) showed normal perfusion and no areas of leakage or ischemia. Treatment with anti-angiogenics in a timely manner correlated with a good visual outcome. Conclusions: We present a unique patient with NF1, rubeosis iridis, high IOP, and macular hemorrhages from multiple corkscrew retinal vessels in a well perfused retina, who underwent treatment with a single dose of intravitreal Bevacizumab and had an excellent response

Delayed-Onset Propionibacterium acnes Endophthalmitis in the Absence of Capsular Complex Following Scleral-Fixated Intraocular Lens: A Case Report

Purpose: This work reports a case of delayed-onset Propionibacterium acnes endophthalmitis in a patient with scleral-fixated intraocular lens (IOL), successfully treated with intravitreal antibiotics and steroids. Methods: Patient underwent complete ophthalmic examinations over 2 years. Vitreous cultures, optical coherence tomography, anterior segment and fundus photographs, and fluorescein angiogram were performed. Results: A 78-year-old man with dislocated IOL underwent IOL removal and scleral-fixated AO60. He did well for 2 months but returned 9 months later with vision loss. Examination revealed low-grade inflammation and multiple IOL opacities. Vitreous culture grew P acnes. He was treated with intravitreal clindamycin followed by dexamethasone for macular edema without IOL explantation. A year after treatment no inflammation or macular edema was noted. Conclusion: To our knowledge, this is the first case of P acnes endophthalmitis following scleral sutured IOL. Treatment with intravitreal injections alone, without IOL explantation, was effective, possibly because of the absence of capsular complex.

Twelve-Year-Old Girl with Acute Macular Neuroretinopathy: A Multimodal Retinal Evaluation

We report a 12-year-old female patient with acute macular neuroretinopathy assessed with multimodal retinal exams. Initial fluorescein angiogram and optical coherence tomography angiography (OCT-A) were both normal, and after 4 months OCT-A showed abnormal vascular flow with normal flow in superficial retina layers, choroid, and choriocapillaris.

Evaluation of the clinical utility of optical coherence tomography angiography in age-related macular degeneration

Background/AimsTo evaluate the ability of optical coherence tomography angiography (OCTA) to identify the presence or absence of choroidal neovascularisation (CNV) and CNV activity in age-related macular degeneration (AMD).MethodsClinical parameters, fundus fluorescein angiogram and spectral-domain optical coherence tomography (SD-OCT) were used as the gold standard to determine disease activity. OCTA imaging was performed on the same day and was graded by two masked retina specialists for the presence or absence of CNV. Traditional multimodal imaging and OCTA findings were compared.ResultsOne hundred and fifty-two eyes of 106 patients with AMD were retrospectively reviewed. Of these, 59 eyes had wet AMD and 93 had dry AMD with high-risk drusen. OCTA had 85.4% and 79.3% specificity and sensitivity, respectively, in determining the presence or absence of CNV. OCTA was 69.5% accurate in determining active CNV. False positives and negatives were 21.6% and 8.0%, respectively.ConclusionsThis study suggests that en-face OCTA images allow a moderate ability to identify CNV and that OCTA alone is weak at recognising active CNV requiring treatment in AMD.

An Atypical Presentation of Giant Cell Arteritis with Bilateral Choroidal Ischaemia

Background: Giant cell arteritis is an immune-mediated, medium to large vessel vasculopathy affecting individuals over 50 years of age. It can cause sudden, severe and irreversible loss of vision, most commonly from an arteritic posterior ciliary artery occlusion causing anterior ischaemic optic neuropathy. The optic nerve appearance would typically be swollen and chalky white. Visual reduction secondary to choroidal ischaemia is a much less frequent presentation, the signs of which can be more subtle in appearance, making its early recognition potentially more challenging. Case Presentation: A 51-year-old male presented to eye casualty complaining of a one-week history of neck pain, intermittent headaches and jaw claudication, associated with reduced vision in his right eye. Presenting visual acuity was hand movements and 6/5 in the right and left eyes respectively. On examination, he was noted to have a right relative afferent pupillary defect, a pale macular area in the right eye with a possible cherry red spot. There was no optic disc swelling. ESR was 34 and CRP was 46 and he was wrongly diagnosed with a non-arteritic central retinal artery occlusion. He subsequently re-presented 5 weeks later with vision loss in his left eye. Best corrected visual acuity was now 6/60 and 6/12 in the right and left eyes respectively. Dilated fundoscopy showed multiple yellow-white lesions in the posterior pole of the left eye and a retinal cotton wool spot. The right optic nerve was pale, and left was normal. A fundus fluorescein angiogram showed delayed choroidal filling and the temporal artery biopsy was suggestive of GCA. He was started on 110mgs of oral prednisolone. After 4 weeks of steroids his BCVA was 6/36 and 6/6 in the right and left eyes respectively. His neck pain, headaches and jaw claudication had all resolved and his ESR and CRP had returned to normal levels. Conclusion: Our case highlights the need for increased awareness of this uncommon presentation of this potentially blinding disease, to allow prompt and appropriate treatment. Our case is unusual in that despite a delayed diagnosis of 5 weeks, visual acuity initially improved with treatment.

Diagnosis of keratoconus in a young male after the hint of electrophysiological test: A Case Report and Review of the Literature

Background: Keratoconus (KCN) is a bilateral and usually asymmetrical disease in which the ectatic cornea becomes conical. Diagnosis of unimproved visual acuity (VA) as KCN might sometimes be missed out due to lack of consideration. However, combination of the electrophysiology test and other common ophthalmological examinations could help to locate the lesion for the unimproved VA and realize the possibility of the existence of KCN, which could be diagnosed via corneal topography. The purpose of this report is to describe the diagnosis process of a case of KCN after the hint of lesion location by electrophysiological tests. Case presentation: A 23-year-old young male presented to our ophthalmology clinic complaining of decreased visual acuity in the left eye for 5 months. Clinical evaluation showed best corrected visual acuity of 1.0 OD, and 0.06 OS. The dilated fundus examination revealed no specific abnormality. Spectral-domain optical coherence tomography (SD-OCT) of macular revealed no obvious change on macular and the thickness of peripapillary nerve fiber layer on both eyes. No leakage of fluorecein was found under Fluorescein angiogram (FFA) of both eyes. Indocyanine green angiography (ICGA) did not showed abnormality. The full-field electroretinogram (ffERG) revealed no obvious changes of amplitudes in all responses. Pattern visual evoked potentials (PVEP) detected a reduced amplitude and delayed phase in P100-wave in both eyes. The amplitude and latency of P2-wave in Flash VEP (FVEP) were comparable in both eyes and were within normative ranges. Corneal topography was finally performed and KCN was diagnosed with the presence of asymmetrical bowtie pattern in both eyes, which was worse in the OS. Conclusions: The hint of lesion location by electrophysiological studies (ffERG, PVEP and FVEP) could be of favor to diagnose the conditions with unimproved VA, such as KCN. Consideration of KCN should be suspected in patients with unimproved VA and significant irregular stigmatism, while no obvious lesion exists in other parts of the eye.

Recurrent retinal and choroidal ischemia in a case of ocular ischemic syndrome

A 62 year-old male patient presented with fluctuating vision in both eyes. On fundus fluorescein angiogram, there was an area of choroidal and retinal ischemia in the left eye. The carotid evaluation revealed complete stenosis of the right internal carotid artery and 90% stenosis of the left internal carotid artery for which he underwent left modified endarterectomy. The vision and angiographic features improved after the procedure. Eleven months after the procedure, he again presented with choroidal ischemia due to recurrent left internal carotid artery blockage which improved gradually after carotid stenting.