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.

IntroductionAs 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 analysisThe 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 numberACTRN12622001246774p.

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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.

this article is available in open access under Creative Common attribution-Non-Commercial-No derivatives 4.0 International (CC BY-NC-Nd 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially IntroductIonSuccessive vaccination against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was a primary public health strategy during the COV-ID-19 pandemic. Pfizer-BioNTech COVID-19 (Pfizer), is the nationally approved and disseminated vaccine in New Zealand (NZ) [1]. This mRNA vaccine is provided as an intramuscular injection with two sequential doses required at a 3-4 week interval to achieve satisfactory immunogenicity [2]. Due to the relative infancy of Pfizer's commercial use, its ocular side effect profile is continuing to emerge, particularly from anecdotal accounts. This case re-port describes a temporal relationship of unilateral neuroretinitis following the second Pfizer vaccine, providing evidence for a potentially rare vaccine-related side effect. case presentatIonA 16-year-old male of Maori, Pacific Islander, and NZ European background presented to a regional ophthalmology service with a 12-day history of gradual vision loss in his left eye and pain on eye movement. A review of the systems was negative, with no constitutional symptoms. He had