CMV neuroretinitis in a post-transplant patient.

This report presents an atypical case of neuroretinitis in a post-transplant patient. A man in his 40s presented to a provincial ophthalmology service with unilateral left visual loss. He was immunosuppressed following orthotopic liver transplantation for end-stage liver cirrhosis secondary to primary sclerosing cholangitis. He had received his third Pfizer-BioNTech COVID-19 booster vaccine 34 days prior to symptom onset. His presenting left visual acuity was 6/36. His left optic nerve head was grossly swollen with peripapillary haemorrhage, intraretinal and subretinal fluid extending to involve the fovea. His serological and radiological investigations were all negative except for serum IgG and IgM positivity to cytomegalovirus (CMV). Following the commencement of antiviral treatment and systemic steroids, his neuroretinitis improved with visual recovery to 6/4.5. This report describes an atypical presentation of neuroretinitis in an immunocompromised patient without AIDS but with evidence of CMV exposure.

Palmerston North Interventional Rapid Avastin Treat and Extend (PIRATE) study protocol; meeting the medical retina service needs of our ageing population.

INTRODUCTION: As the rates of age-related macular denegation exponentially increase, new innovation is required to address the challenges faced by our ageing population. The aim of the Palmerston North Interventional Rapid Avastin Treat and Extend (PIRATE) study is to establish the safety and efficacy of rapid treatment extension of bevacizumab (Avastin) in patients with low-risk neovascular age-related macular degeneration (nAMD). METHODS AND ANALYSIS: The PIRATE study is a monocentric, non-blinded, open-label randomised control trial. Participants over the age of 50 years with low-risk nAMD characteristics will be recruited in a prospective manner and randomised into treatment and control groups. Rapid treatment extension by 4 weeks will be applied in the treatment group, with the standard 2-week treatment extension occurring among controls. Participants will enter the trial after initial treatment induction consisting of three bevacizumab injections, 1 month apart. The primary outcome of best-corrected visual acuity will be assessed along with predetermined secondary outcomes at a study duration of 12 months (initial) and 24 months (total). TRIAL REGISTRATION NUMBER: ACTRN12622001246774p.

The Atypical Red Eye: A Case of Scleritis with Peripheral Ulcerative Keratitis Secondary to Granulomatosis with Polyangiitis.

This report presents a rare case of scleritis with peripheral ulcerative keratitis secondary to granulomatosis with polyangiitis (GPA). A 65-year-old Caucasian male presented to a regional ophthalmology service with an atypical red eye. His immune work-up demonstrated positive anti-neutrophil cytoplasmic IgG autoantibodies (ANCA) with anti-proteinase 3 antibody (PR3) elevation. Multi-system vasculitis was discovered including lung, liver, bladder, prostate, nasal and paranasal sinuses involvement. His ocular sequelae included significant peripheral corneal thinning requiring cyanoacrylate gluing, juxtalimbal conjunctival resection, and bandage lens placement. He was treated with systemic methylprednisolone and rituximab achieving remission with ongoing prednisone and methotrexate maintenance therapy. This case demonstrates the importance of recognizing ocular manifestations of GPA as a first presentation of systemic vasculitis.

Congenital Hypertrophy of the Retinal Pigment Epithelium (CHRPE) as a Screening Marker for Familial Adenomatous Polyposis (FAP): Systematic Literature Review and Screening Recommendations.

Purpose: Familial adenomatous polyposis (FAP) has an almost 100% colorectal cancer risk warranting early detection in gene carriers. This study presents congenital hypertrophy of the retinal pigment epithelium (CHRPE) as a highly specific phenotypical marker for FAP that can be used in screening at-risk individuals. Screening recommendations including morphological subclassification were formulated with supporting literature. Methods: A systematic literature review with a comprehensive search strategy was conducted using online databases. Manual searches of bibliographies and reference lists were also performed. Studies meeting inclusion criteria were graded with respect to their hierarchy of evidence and strength of recommendations according to the National Health and Medical Research Council (NHMRC) guidelines of Australia. Results: Almost 4500 participants were analysed across 28 included studies. The mean specificity of CHRPE as a phenotypical screening marker of FAP was 89% (standard deviation (SD); 14) with a mean sensitivity of 79% (SD; 8). The mean prevalence of CHRPE amongst FAP participants; at-risk participants were found to be 76% (SD; 24) and 37% (SD; 21) respectively. Bilateralism and multiple lesion number ≥3 are features highly specific for FAP. Conclusion: CHRPE was found to be a non-invasive, rapid, early phenotypical screening marker of FAP. Clinical recognition further allows increased gene analysis efficiency. The absence of CHRPE alone cannot exclude FAP. Our screening recommendations provide guidance to clinicians on evidence based CHRPE assessment. We would advocate inclusion of ocular examinations as part of a three-pronged approach, along with endoscopy and genetic testing, for efficient, timely FAP assessment in at-risk individuals.