Keratoconus, Dry Eye Syndrome, Overnight Orthokeratology, Achromatopsia and Uveitis in Children

Keratoconus (KC) is a progressive corneal ectasia, leading to high astigmatism and myopia, associated with corneal scarring and visual impairment that may necessitate transplantation in severe cases. Despite an estimated incidence of between 50 – 230/100,000 in those younger than 18 years, KC remains underdiagnosed in children and the correct diagnosis is often not made for years. At the same time, the clinical course is thought to be more aggressive in children with faster progression and a greater risk of corneal opacification [1]. In 2003, the pioneering technique of corneal crosslinking (CXL) was introduced into clinical practice. This low-cost and minimally invasive technology has since been shown to halt progression in many cases and thereby reduce the subsequent requirement for corneal transplantation. The original Dresden protocol, involving 30 min of riboflavin instillation followed by 30 min of 3 mW/cm2 of UVA light with a total fluence of 5.4 J/cm2, has been shown in studies to stabilize the corneal topo- and tomographic findings and achieve better visual acuity outcomes in the long term [2]. However, the relatively long duration and less cooperation in young patients have led to attempts to reduce the treatment time in children while preserving the clinical efficacy of the procedure. Xanthopoulou et al. [3] report in this issue their longterm results in 41 eyes (25 male and 5 female patients, mean age 15.3 ± 1.2 years) with an accelerated epithelium-off CXL technique, involving accelerated riboflavin instillation for 20 min and UVA light irradiation 9 mW/cm2 for only 10 min (Epi-off A-CXL). They report successful stabilization of KC progression in patients younger than 18 years even more than 2 years after CXL. Interestingly, they observed evidence of “pseudoprogression” up to the 6-week follow-up, but they report this did not affect the longterm outcome. They conclude that Epi-off A-CXL is “a safe and effective protocol for the successful stabilization of KC and achieves a tomographic improvement 2 years postoperatively, which can be maintained even after > 2 years post-CXL”.