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Background The risk of developing hydroxychloroquine retinopathy is considered sufficient to justify national monitoring programmes. There are an estimated 71,144–77,170 long-term hydroxychloroquine users in the UK. However, the number of patients diagnosed with retinopathy is unknown. This study aimed to identify the number of cases and clinical characteristics of hydroxychloroquine retinopathy diagnosed annually in hospital eye services across the UK. Methods A nationwide, prospective case ascertainment study was undertaken using the British Ophthalmological Surveillance Unit, which sends approximately 1420 reporting cards to UK Ophthalmologists monthly. The case definition was two abnormal tests suggestive of hydroxychloroquine retinopathy. Demographic and clinical data relating to hydroxychloroquine use and retinopathy were collected from identified cases using a standardised questionnaire over a 1-year period (2018–2019). Results Sixty-six cases of hydroxychloroquine retinopathy were reported, and 46 questionnaires were received (73% response rate). Twenty-four incident cases of hydroxychloroquine retinopathy were identified (24-43 cases following adjustment). The median duration of drug therapy was 19 years (range: 4–50 years, IQR: 14.5–23 years). Fourteen patients were asymptomatic, and 9 symptomatic at diagnosis. A trend towards a lower mean deviation on visual field testing was observed in the symptomatic group (−11.55 dB versus −6.9 dB; P = 0.15). Conclusion Between 1 in 1655 and 3215 (0.03–0.06%) long-term hydroxychloroquine users were diagnosed with retinopathy over the study period. We estimate that monitoring was available for 1.9–3.8% of long-term users, accounting for a lower than expected incidence. The high proportion of symptomatic retinopathy at diagnosis underlines the importance of monitoring to detect pre-symptomatic disease.

Although adeno-associated viral (AAV) vector-mediated retinal gene therapies have demonstrated efficacy, the mechanisms underlying dose-dependent retinal inflammation remain poorly understood. Here, we present a quantitative analysis of cellular immune response to subretinal AAV gene therapy in mice using multicolor flow cytometry with a panel of key immune cell markers. A significant increase in CD45+ retinal leukocytes was detected from day 14 post-subretinal injection of an AAV8 vector (1 × 109 genome copies) encoding green fluorescent protein (GFP) driven by a ubiquitous promoter. These predominantly consisted of infiltrating peripheral leukocytes including macrophages, natural killer cells, CD4 and CD8 T cells, and natural killer T cells; no significant change in resident microglia population was detected. This cellular response was persistent at 28 days and suggestive of type 1 cell-mediated effector immunity. High levels (80%) of GFP fluorescence were found in the microglia, implicating their role in viral antigen presentation and peripheral leukocyte recruitment. When compared against AAV.GFP in paired eyes, an equivalent dose of an otherwise identical vector encoding the human therapeutic transgene Rab-escort protein 1 (REP1) elicited a significantly diminished cellular immune response (4.2-fold; p = 0.0221). However, the distribution of immune cell populations remained similar, indicating a common mechanism of AAV-induced immune activation., Graphical abstract, The mechanism of gene therapy-associated uveitis (GTAU) following AAV-mediated retinal gene therapy remains poorly understood. Chandler, Xue, and colleagues utilize multicolor flow cytometry to characterize the cellular immune response to subretinal AAV injections in mice, demonstrating substantial populations of myeloid and T cells from 14 days post-injection.

Inherited retinal diseases are a frequent cause of severe visual impairment or blindness in children and adults of working age. Across this group of diseases, there is great variability in the degree of visual impairment, the impact on everyday life, disease progression, and the suitability to therapeutic intervention. Therefore, an early and precise diagnosis is crucial for patients and their families. Characterizing inherited retinal diseases involves a detailed medical history, clinical examination with testing of visual function, multimodal retinal imaging as well as molecular genetic testing. This may facilitate a distinction between different inherited retinal diseases, as well as a differentiation from monogenic systemic diseases with retinal involvement, and from mimicking diseases.Erbliche Netzhauterkrankungen sind eine häufige Ursache für eine schwere Sehbehinderung oder Erblindung bei Kindern und Erwachsenen im erwerbsfähigen Alter. Aufgrund einer großen Heterogenität besteht eine hohe Variabilität hinsichtlich Einschränkungen der Sehfunktion, Auswirkungen auf das alltägliche Leben, auf die Lebensplanung sowie hinsichtlich neuer Therapieverfahren. Insofern ist eine frühzeitige und präzise Diagnose für Patienten und ihre Familien von Bedeutung. Die Charakterisierung einer erblichen Netzhauterkrankung umfasst eine detaillierte Anamnese, eine umfassende klinische Untersuchung mit Testung der Sehfunktion, eine multimodale retinale Bildgebung als auch eine molekulargenetische Diagnostik. Neben der Unterscheidung verschiedener erblicher Netzhauterkrankungen ist eine Abgrenzung zu monogenen Systemerkrankungen mit einer Netzhautbeteiligung, sowie eine Abgrenzung zu Erkrankungen, die eine Netzhautdystrophie imitieren, wichtig.

Retinopathy in Malignant Hypertension A 55-year-old man presented with a 1-week history of headache and blurred vision. His blood pressure was 250/170 mm Hg. Funduscopic examination revealed disk e...

MERTK (MER proto-oncogene, tyrosine kinase) is a transmembrane protein essential in regulating photoreceptor outer segment phagocytosis. Biallelic mutations in MERTK cause retinal degeneration. Here we present the retinal phenotype of three patients with missense variants in MERTK. All patients underwent a full clinical examination, fundus photography, short-wavelength fundus autofluorescence and optical coherence tomography imaging. Two patients also underwent Goldmann visual field testing and electroretinography was undertaken for the third patient. Molecular genetic testing was undertaken using next generation or whole-exome sequencing with all variants confirmed by Sanger sequencing. The first patient was a 29-year-old female heterozygous for a missense variant (c.1133C>T, p.Thr378 Met) and a nonsense variant (c.1744_1751delinsT, p.Ile582Ter) in MERTK. The second patient was a 26-year-old male homozygous for a c.2163T>A, p.His721Gln variant in MERTK. The third patient was an 11-year-old female heterozygous for a deletion of exons 5–19 and a missense variant (c.1866 G>C, p.Lys622Asn) in MERTK. Reduced night vision was the initial symptom in all patients. Fundoscopy revealed typical signs of retinitis pigmentosa (RP) with early-onset macular atrophy. All three MERTK missense variants affect highly conserved residues within functional domains, have low population frequencies and are predicted to be pathogenic in silico. We report three missense variants in MERTK and present the associated phenotypic data, which are supportive of non-syndromic RP. MERTK is a promising candidate for viral-mediated gene replacement therapy. Moreover, one variant represents a single nucleotide transition, which is theoretically targetable with CRISPR-Cas9 base-editing.

Cadherins are a superfamily of calcium-dependent intercellular adhesion molecules that are widely expressed in living tissues. Within the retina and retinal pigment epithelium (RPE), cadherins contribute to tissue morphogenesis, neural circuit formation, adherens junctions of the outer blood-retinal barrier, photoreceptor disc morphogenesis, maintenance and survival. Four monogenic disorders involving genes which encode cadherins have been identified as causes of inherited retinal degeneration: the retinal cadherinopathies (CDHR1, CDH23, PCDH15, CDH3). Biallelic variants in CDHR1 result in cone-rod dystrophy, rod-cone dystrophy or late-onset macular dystrophy which may be misclassified as dry age-related macular degeneration. Biallelic variants in CDH23 and PCDH15 underlie Usher Syndrome type 1D and 1F. Hypotrichosis with juvenile macular dystrophy results from biallelic variants in CDH3, which contributes to adherens tight junctions between RPE cells. In this review, we summarise the classification of cadherins, and the role of cadherins in the physiology and morphogenesis of the inner and outer retina. Cadherins expressed in primate photoreceptors (CDHR1, CDH23 and PCDH15) have evolved complex roles in outer segment disc morphogenesis and maintenance involving intracellular heterophilic interactions which are as yet incompletely characterised. We highlight what is currently unknown about the molecular function of these cadherins, and review the pathogenesis, clinical phenotype and molecular genetics of each monogenic retinal cadherinopathy. Genes regulating the expression and post-translational modification of retinal cadherins, or those coding for as yet unidentified interacting partners, are candidates for unsolved cases of retinal degeneration. This group of disorders is potentially treatable; we summarise the likely molecular therapeutic approaches and future directions for each retinal cadherinopathy.

The Royal College of Ophthalmologists (RCOphth) has published new recommendations for retinal monitoring for hydroxychloroquine and chloroquine users in the United Kingdom (2020).1 The recommendations aim to protect both patients and prescribers by reducing the risk of irreversible visual loss from hydroxychloroquine retinopathy. Importantly, as a consequence of new evidence, baseline retinal testing is no longer required and an updated monitoring timetable is recommended.

To validate the Cdhr1Deep phenotyping of Cdhr1Cdhr1The Cdhr1

Purpose To describe the clinical and electrophysiological features of adult-onset macular dystrophy, due to novel combinations of CDHR1 alleles, and compare the associated phenotypes with previous reports. Methods The clinical records of patients with macular dystrophy and biallelic variants in CDHR1 were reviewed. Data analysed included best corrected visual acuity (BCVA), fundus images: autofluorescence (AF) and optical coherence tomography (OCT); full field electroretinography (ERG) and pattern ERG (PERG). Results Seven patients from six pedigrees were ascertained. One patient was homozygous for a known synonymous variant p.(Pro261=), four were compound heterozygous for the p.(Pro261=) variant and a novel allele of CDHR1: p.(Gly188Ser), p.(Met1?), or p.(Val458Asp); one patient was compound heterozygous for two previously unreported variants: c.297+1G>T in trans with p.(Pro735Thr). The range of BCVA at the last clinic review was (6/5–6/60). Autofluorescence showed macular flecks of increased AF in mild cases and patches of reduced AF in severe cases. The OCT showed attenuation of the ellipsoid zone (EZ) in mild cases and loss of the EZ and the outer nuclear layer in severe cases; one patient had subfoveal hyporeflective region between the EZ and the retinal pigment epithelium. The full field ERG was normal or borderline subnormal in all cases, and the PERG was subnormal in mild cases or undetectable in severe cases. Conclusions This report corroborates previous observations that genotypes distinct from those causing pan-retinal dystrophy can cause a milder phenotype, predominantly affecting the macula, and expands the spectrum of these genotypes. The findings in this cohort suggest a potential macular susceptibility to mild perturbations of the photoreceptor cadherin.

Effective treatment of retinal diseases with adeno-associated virus (AAV)-mediated gene therapy is highly dependent on the proportion of successfully transduced cells. However, due to inflammatory reactions at high vector doses, adjunctive treatment may be necessary to enhance the therapeutic outcome. Hydroxychloroquine and chloroquine are anti-malarial drugs that have been successfully used in the treatment of autoimmune diseases. Evidence suggests that at high concentrations, hydroxychloroquine and chloroquine can impact viral infection and replication by increasing endosomal and lysosomal pH. This effect has led to investigations into the potential benefits of these drugs in the treatment of viral infections, including human immunodeficiency virus and severe acute respiratory syndrome coronavirus-2. However, at lower concentrations, hydroxychloroquine and chloroquine appear to exert immunomodulatory effects by inhibiting nucleic acid sensors, including toll-like receptor 9 and cyclic GMP-AMP synthase. This dose-dependent effect on their mechanism of action supports observations of increased viral infections associated with lower drug doses. In this review, we explore the immunomodulatory activity of hydroxychloroquine and chloroquine, their impact on viral infections, and their potential to improve the efficacy and safety of retinal gene therapy by reducing AAV-induced immune responses. The safety and practicalities of delivering hydroxychloroquine into the retina will also be discussed.

Background: Gene editing has shown huge potential in correcting aberrant splicing and Cas13 has been identified as being particularly suitable for targeting RNA. It has therefore become increasingly important to highlight new splice site mutations that may be correctable, particularly in genes that are too large to be encoded by AAV vectors. About 20% of Usher Type 1 cases are caused by mutations in CDH23. Purpose: To report a novel splice site mutation of CDH23 associated with Usher Type 1D. Materials and Methods: Case report. Results: A 35-year-old Caucasian female who is congenitally deaf with vestibular dysfunction presented with visual acuity of 6/12 in both eyes. Fundus examination revealed findings typical of retinitis pigmentosa with foveal preservation of photoreceptor layer. Next generation sequencing analysis revealed a novel homozygous variant, c.9319 + 1G>T in CDH23 consistent with the diagnosis of Usher Syndrome Type 1D. The c.9319 + 1G>T variant is predicted to affect splicing at the exon 65/intron 65 boundary, which highly likely leads to complete skipping of exon 65. Conclusions: We describe a case of a typical Usher Syndrome Type 1D caused by a novel splice site variant in CDH23. Currently there are no treatments for CDH23 related retinal degeneration, partly because the cDNA size of 10kb is too large for AAV vector gene augmentation therapy. Alternative strategies include CRISPR-Cas9 adenine base editors and RNA editing with CRISPR-Cas13. Single-nucleotide editing represents a promising approach for targeting this variant in CDH23 to restore the wildtype splice donor site at this position.

Purpose To validate the Cdhr1−/− mouse as a model for human CDHR1-associated retinal degeneration, which may present as cone-rod dystrophy or geographic atrophy. Methods Deep phenotyping of Cdhr1−/− (n = 56) and C57BL6J wildtype control mice (n = 45) was undertaken using in vivo multimodal retinal imaging and dark- and light-adapted electroretinography (ERG) over 15 months to evaluate rod- and cone-photoreceptor responses and retinal morphology. Results Cdhr1−/− retinas exhibited outer retinal thinning on optical coherence tomography (OCT) at 1-month versus C57BL6J (mean 14.6% reduction; P < 0.0001), with progressive degeneration to 15 months. The OCT layer representing photoreceptor outer segments was more significantly shortened in Cdhr1−/− eyes at 1 month (mean 33.7% reduction; P < 0.0001), remained stable to 3 months and was not identifiable at later timepoints. Outer retinal thinning was more pronounced at inferior versus superior retinal locations in Cdhr1−/− eyes (P < 0.002 at 3–9 months). Dark-adapted ERG identified severe functional deficits in Cdhr1−/− mice at 1 month of age versus C57BL6J (mean 62% reduction) that continued to decline to 15 months (P < 0.0001). Light-adapted flicker identified severe deficits in cone function at 1 month (mean 70% reduction), with improved function to 3 months followed by progressive decline (P < 0.0001). Conclusions The Cdhr1−/− mouse exhibits structural and functional evidence of progressive outer retinal degeneration at a slow rate. Early functional deficits affecting both rod and cone photoreceptors in the context of relatively mild structural changes reflect the human phenotype. This study validates the use of the Cdhr1−/− mouse for the pre-clinical evaluation of therapeutics for human CDHR1-associated retinal degeneration.

Objective To describe the conception and evaluation of a novel educational intervention to teach direct ophthalmoscopy with retinal simulators using a peer-assessed group objective structured clinical examination (OSCE) format. Design Prospective, single-centre educational trial at Oxford University Medical School, Oxford, U.K. Participants A total of 160 consecutive undergraduate fifth-year medical students participated in the study. Methods Students identified prior experience, teaching, examination, and feedback relevant to direct ophthalmoscopy. Students self-evaluated their perceived confidence across 6 domains of direct ophthalmoscopy examination before and after the educational intervention using a Likert-type psychometric scale. Wilcoxon matched pair testing was used to determine statistical significance for each domain. Results The group OSCE intervention increased confidence in direct ophthalmoscopy overall from 2.5% to 63.8% (p < 0.001). Confidence improved in all 6 domains, most significantly in controls of ophthalmoscope (p < 0.001) and sequence of examination (p < 0.001) but also in the identification and interpretation of retinal signs (p < 0.001). Students rated the tutorial as very effective or effective across all 6 domains, and 96.29% rated the tutorial as effective overall. Conclusions Retinal simulation, integrated with a peer-assessed group OSCE format, is effective in increasing confidence in all aspects of direct ophthalmoscopy. It may be insufficient alone for training in the identification and interpretation of posterior segment clinical signs. Diminishing ophthalmology clerkships worldwide require ophthalmologists to identify innovative teaching methods, using modern technology and pedagogy to deliver high-quality, yet high-throughput, training in direct ophthalmoscopy. This novel teaching strategy may be considered by ophthalmologists responsible for direct ophthalmoscopy training in a teaching hospital context.

Purpose To determine the functional and structural effects of trabeculectomy surgery on patients with advanced glaucoma and central visual field defects in the early post-operative period. Methods Thirty consecutive adult subjects with advanced glaucoma requiring trabeculectomy surgery and an established visual field defect within 10° of fixation underwent microperimetry (MAIA MP-1, CenterVue, Padova, Italy) and optic disc optical coherence tomography (OCT) imaging (Spectralis, Heidelberg Engineering, Germany) pre-operatively, and 1 month and 3 months following trabeculectomy surgery. Main outcome measures were post-trabeculectomy change in mean threshold on microperimetry and nerve fibre layer thickness on OCT. Fellow eyes were used as controls. Results The mean change in MP average threshold values from pre-operative to post-operative was 0.6 ± 1.9 dB for treated eyes and 0.1 ± 1.3 dB for control eyes (p = 0.14) at 1 month and 0.2 ± 2.3 and −0.3 ± 1.6 dB at 3 months (p = 0.22). Mean change in global nerve fibre layer thickness was −0.6 and −0.5 µm for operated and control eyes, respectively (p = 0.83), at 1 month and 0.8 and −0.4 µm at 3 months (p = 0.88). The kappa agreement for structure–function correlation between OCT and MP was 0.735 (confidence interval 0.59–0.88) (p Conclusions Central visual function and retinal nerve fibre layer thickness appear to be preserved in glaucoma patients with central visual field defects undergoing trabeculectomy surgery in the early post-operative period. These data may inform glaucoma surgeons considering trabeculectomy surgery in this patient group.

Purpose To determine whether microperimetry or optical coherence tomography (OCT) imaging can detect early disease in the fellow eye of patients with unilateral focal ischaemic glaucoma. Methods Thirty-seven consecutive adult patients with unilateral focal ischaemic glaucoma with a unilateral split-fixation visual field defect on standard automated perimetry (SAP) with normal SAP in the fellow eye were selected. All patients underwent microperimetry (MAIA, CenterVue, Italy) of the central 10 degrees and OCT imaging (Spectralis, Heidelberg Engineering, Germany) of the retinal nerve fibre layer of both eyes. The main outcome measures were reduced retinal sensitivity on microperimetry and/or retinal nerve fibre layer thinning on OCT imaging of the fellow eye. Results Thirty fellow eyes had abnormal global thresholds on microperimetry, and 20 had abnormal OCT imaging studies. Kappa agreement between tests in fellow eyes was poor (p = 0.2546). Fixation was significantly poorer in fellow eyes on microperimetry when compared to eyes with glaucoma (p Conclusion Microperimetry detects reduced retinal sensitivity close to fixation and OCT detects focal thinning of the retinal nerve fibre layer in the fellow eye of most patients with presumed unilateral focal ischaemic glaucoma. Further studies are required to correlate specific optic disc features on OCT imaging with microperimetry in the fellow eye of this patient group.

We read with interest the article by McGill and Ambrose on lupus in young people.1 They reiterate that patients should receive retinal screening after 5 years of exposure to hydroxychloroquine.1 The Royal College of Ophthalmologists has made a collaborative recommendation for systematic retinal screening in users of hydroxychloroquine and chloroquine in the United Kingdom.

FLVCR1 (feline leukemia virus subgroup c receptor 1) is a transmembrane protein involved in the trafficking of intracellular heme. Homozygous variants in FLVCR1 have been described in association with a clinical syndrome of posterior column ataxia with retinitis pigmentosa (PCARP). Here, we describe a patient with non-syndromic retinitis pigmentosa homozygous for a splice-site variant in FLVCR1 (c.1092 + 5G>A) without evidence of posterior column ataxia or cerebellar degeneration. We suggest an association between intronic splice-site variants in FLVCR1 and the absence of posterior column degeneration and suggest a hypothesis to explain this observation. Should this association be proven, it would provide valuable prognostic information for patients. Retinal degeneration appears to be the sole clinical manifestation of this FLVCR1 variant; gene therapy approaches using an adeno-associated viral vector with sub-retinal delivery may therefore represent a therapeutic approach to halting retinal degeneration in this patient group.

A 42-year-old male refugee of North African descent presented to ophthalmology department with a 2-year history of reduced vision in his right eye, noted by the optometrist. There was no redness, photophobia, ocular pain, floaters, or photopsia at the time of presentation. There was no significant medical history. He did not take any regular medications and denied use of any intravenous drug. There was no family history of eye disease. The patient had been living in the United Kingdom for the past 16 years. He had presented to ophthalmology 13 years before, complaining of chronic reduced vision in his left eye over the preceding years associated with occasional periocular aching. Visual acuity was 20/15 in the right eye and 20/20 in the left eye with no evidence of anterior chamber inflammation or vitritis at that time. Fundus examination revealed an occluded peripheral retinal vessel with an associated pigmented scar in the left eye only. His macular examination showed clinically normal results. No treatment or follow-up was recommended.

Purpose: The purpose of the study was to audit the use of non-contact ultra-widefield retinal imaging in infants with suspected abusive head trauma (AHT) using the Optos P200MA Scanning Laser Ophthalmoscope. Patients and methods: A retrospective, observational case series. Ten eyes of five consecutive infants (aged 1–15 months) with suspected (or in 1 case, known) AHT referred for an ophthalmological opinion were included. Each infant underwent non-contact ultra-widefield retinal imaging using the Optos P200MA scanning laser ophthalmoscope. Optos fundus fluorescein angiography (FFA) was performed in one infant with oral sedation. The other four infants did not require sedation. The main outcome measure was the acquisition of a single, definitive ultra-widefield retinal image in each eye. Safety was audited by determining adverse changes in heart rate and oxygen saturations that required cessation of imaging. Results: The Optos P200MA ultra-widefield scanning laser ophthalmoscope acquired good quality retinal images in all infants. Documentation of acute, widespread retinal haemorrhages contributed to a diagnosis of AHT in three infants. Chronic pre-macular haemorrhage and macular schisis were documented by FFA in a fourth infant. The absence of retinal haemorrhages was documented in a fifth infant contributing to the exclusion of a diagnosis of AHT. There were no adverse safety signals in any infant in this series. Conclusion: The Optos P200MA ultra-widefield scanning laser ophthalmoscope appears safe to use in infants with suspected AHT, providing high-quality retinal images in a single frame without ocular contact. Optos P200MA may be used as alternative to RetCam to document retinal haemorrhages in stable infants with suspected AHT.

Purpose The aim of this case series is to raise awareness of the emerging issue of serious retinal damage caused by the prolonged use of hydroxychloroquine (HCQ) and the importance of adequate and appropriate monitoring of visual function during treatment. Patients and methods This is a small retrospective case series of 3 patients on long-term HCQ who developed serious symptomatic retinal toxicity confirmed on imaging and functional testing. Results All 3 patients were treated with HCQ for over 15 years; two for rheumatoid arthritis (RA), and the third for systemic lupus erythematosus (SLE). All 3 patients had macular involvement varying in severity confirmed with characteristic features on imaging and functional testing (Optical Coherence Tomography (OCT), Autofluorescence (AF) and Humphrey 10-2 visual fields). Conclusion HCQ is widely used to treat autoimmune conditions with a proven survival benefit in patients with SLE. However, longterm use can be associated with irreversible retinal toxicity. These cases highlight that HCQ, like chloroquine, can also cause visual loss in susceptible individuals. Early detection of presymptomatic retinal changes by the introduction of appropriate screening and monitoring is mandatory to limit the extent of irreversible visual loss due to HCQ retinal toxicity.

We thank Gallina and coauthors for their comments relating to our report of normal-tension glaucoma occurring prematurely in a 27-year-old male with chronic, recurrent intracranial hypotension. We can report that since the implantation of the ProGAV cerebrospinal fluid (CSF) shunt, his recurrent left optic disc hemorrhages have not recurred. His intraocular pressures at last review were 15mm Hg in the right eye and 14mm Hg in the left using Latanoprost 0.005% once at night to each eye. His split-fixation visual field defect in the left eye, reported in our description,1 has remained unchanged. His symptoms of headache have improved postoperatively, and therefore, intracranial pressure monitoring has not been required, nor has external adjustment of the magnetic valve that forms part of ProGAV shunt.

Introduction: Toxoplasma chorioretinitis is a leading cause of infectious posterior uveitis worldwide.Methods: We report an atypical presentation of Toxoplasma chorioretinitis presenting after uneventful cataract surgery in an 81-year-old male, with known hypernephroma and rheumatoid arthritis, treated with prednisolone and methotrexate.Results: He was treated for acute retinal necrosis and cytomegalovirus retinitis before Toxoplasma chorioretinitis was confirmed by vitreous biopsy 11 months after presentation. He developed a secondary rhegmatogenous retinal detachment, treated successfully with pars plana vitrectomy, silicone oil and endolaser. Visual acuity at discharge was 6/12 following silicone oil removal.Discussion: Necrotising chorioretinitis in immunosuppressed or elderly patients may present with an atypical phenotype. Clinical diagnosis in this context remains challenging. We discuss the clinical reasoning behind investigation and management of this patient group in whom viral and Toxop...