Cystinosis is a rare autosomal recessive disorder characterized by the accumulation of cystine within the cells of different organs. Infantile nephropathic cystinosis is the most common and severe phenotype. With the success of renal transplantation, these patients are now living longer and thus more long-term complications within different organs are becoming apparent. Ophthalmic manifestations range from corneal deposits of cystine crystals to pigmentary retinopathy. With increasing age, more severe ocular complications have been reported. Photophobia is a prominent symptom for patients. With prolonged survival and increasing age, this symptom, along with corneal erosions and blepharospasm, can become debilitating. This review revisits the basic pathogenesis of cystinosis, the ocular manifestations of the disease, and the treatment of corneal crystals.

Wickham et al. [1] have highlighted the impact of COVID policies on ophthalmology services. Their findings are supported by our experience in Scotland. Rhegmatogenous retinal detachments (RRDs) are acute conditions that require prompt surgery [2]. The annual incidence of RRDs in Scotland was reported as 12.05/100,000 in 2010 [3]. The SARS-CoV-19 outbreak has been unprecedented. Healthcare services have worked hard to stay afloat. Subspecialty care has taken a back seat to intensive care and acute COVID wards. With UK eye departments providing only emergency care, outpatient clinics have been cancelled and workloads reduced. Anecdotal evidence from units all over the country suggests that RRD presentations have declined. Fortuitously, since August 2019 a study has been running across Scotland, prospectively collating all new RRDs, aiming to establish the incidence 10 years after the Scottish Retinal Detachment Study [3]. This has allowed us to assess the impact of the COVID-19 lockdown on RRD presentations. Fifteen surgeons in all six Vitreoretinal units across Scotland prospectively recorded all RRD presentations since August 2019. These records were collated centrally. Details such as age, sex, laterality and macular status were documented. We examined the data prior to and 5 weeks after the UK lockdown on the 23 rd March 2020. As the announcement was late in the afternoon, most of the RRDs operated on the 24 th would have presented prior to this. We therefore started counting the post lockdown numbers from 25 th March.

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Cystinosis is a rare, autosomal recessive disorder leading to defective transport of cystine out of lysosomes. Subsequent cystine crystal accumulation can occur in various tissues, including the ocular surface. This review explores the efficacy of cysteamine hydrochloride eye drops in the treatment of corneal cystine crystal accumulation and its safety profile.

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