Introduction To measure the prevalence of hydroxychloroquine retinopathy in patients attending a hydroxychloroquine monitoring service using 2018 Royal College of Ophthalmologists diagnostic criteria. Methods A service evaluation audit of a hydroxychloroquine retinopathy monitoring service was undertaken. Results of Humphrey 10-2 field tests, spectral-domain optical coherence tomography and fundus autofluorescence were collected with data on dose, weight, duration of treatment, estimated glomerular filtration rate, and concurrent tamoxifen therapy. Visual field tests were assessed as reliable or unreliable, and classified as normal, hydroxychloroquine-like, poor test or related to other pathology. Cases of definite and possible retinopathy were identified using the 2018 RCOphth criteria. Results There were 1976 attendances over two years of 1597 patients. Seven hundred and twenty-eight patients had taken hydroxychloroquine for less than 5 years and 869 had taken hydroxychloroquine for 5 years or more. Fourteen patients were identified with definite hydroxychloroquine retinopathy (1.6%), and 41 patients with possible retinopathy (4.7%). Sixtyseven per cent of 861 visual fields were performed reliably, with 66.9% classified as normal, 24.9% as poor test, 5.2% hydroxychloroquine-like and 3.0% abnormal due to other pathology. Conclusions The 1.6% prevalence of hydroxychloroquine retinopathy is lower than the previously reported prevalence of 7.5% as reported by Melles and Marmor JAMA Ophthalmol 132: 1453-60 (2014). This is because of a difference in the diagnostic criteria. Both definite and possible retinopathy would meet the diagnostic criteria of the Melles and Marmor study; 6.3% in our data, compared with 7.5%, a much smaller difference and likely to be explained by differences in the risk characteristics of the two groups.

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Despite the paucity of high quality evidence, the existing literature suggests that the use of CH for procedural sedation in children appears to be an effective alternative to general anaesthesia, and it can be safe when administered in the hospital setting with appropriate monitoring and vigilance for intervention.

Purpose: To present the natural course and describe the characteristic findings of a case of unilateral retinal pigment epithelium dysgenesis (URPED) and highlight the optical coherence tomography angiography characteristics of this rare fundus pattern. Methods: Case report. Results: A 52-year-old male was referred 8 years ago to our clinic due to a distinctive unilateral lesion in his left fundus, of which he was aware from early adulthood. Clinical evaluation revealed an irregularly shaped patch of retinal pigment epithelium atrophy surrounding his left optic nerve, with a characteristic scalloped border and with severe distortion of the overlying retinal and vascular tissue. Retinal pigment epithelial hyperplasia was noted in the margin as well as in distinct lacunae clustered around the periphery of the lesion. Right fundus examination was normal. The patient was diagnosed with URPED and was followed annually with multimodal imaging ever since. No choroidal neovascularization or other complication was noted. However, the lesion appeared to slowly but steadily grow to eventually affect the foveal region causing severe visual loss. Best corrected visual acuity was 20/40 at baseline and 20/200 at last follow-up 8 years after the original diagnosis. Conclusion: URPED is a rare retinal disease with unique clinical characteristics that can progress relentlessly even in the absence of secondary complications.

Purpose: To present a rare case report of Onodi cell-associated optic neuropathy, conducting a review of the literature. Methods: A 36-year-old male presented with an 18-h history of acute deterioration of vision in his left eye (LE). Ophthalmic examination and Magnetic Resonance Imaging (MRI) were consistent with an Onodi cell-associated compressive optic neuropathy. Results: Despite immediate, successful surgical decompression, severe optic nerve atrophy and permanent visual loss occurred during early postoperative period. The reported case gives rise to different hypotheses regarding pathophysiology that may lead to irreversible blindness. A systematic review of the respective literature is provided attempting to compare different approaches in the management of Onodi cell-associated compressive optic neuropathy and assess their efficacy in the final visual outcome. Poor initial visual acuity (VA) may represent a bad prognostic factor. Moreover, age and gender do not seem to significantly influence the outcome. Conclusion: This report and associated literature review highlight the importance of the radiologic characteristics and early diagnosis in the final visual outcome of the Onodi cell-associated optic neuropathy. High level of suspicion is crucial for early diagnosis of mucoceles, which must be treated promptly by surgical and medical means to enhance visual recovery.