Risk Factors for the Development of Cataract in Children with Uveitis

PURPOSE: To determine the risk factors for the development of cataract in children with uveitis of any etiology. DESIGN: Cohort study. METHODS: Two hundred forty-seven eyes of 140 children with uveitis were evaluated for the development of vision-affecting cataract. Demographic, clinical, and treatment data were collected between the time of presentation and the first instance cataract was recorded or findings at final follow-up. Main outcome measures included the prevalence of cataract and distribution by type of uveitis, incidence of new onset cataract time to cataract development, and risk factors for the development of cataract. RESULTS: The prevalence of cataract in our cohort was 44.2% and was highest among eyes with panuveitis (77.1%), chronic anterior uveitis (48.3%), and intermediate uveitis (48.0%). The overall incidence of newly diagnosed cataract was 0.09 per eye-year, with an estimated 69% to develop uveitis-related cataract with time. The main factors related with cataract development were the number of uveitis flares per year (hazard ratio [HR] = 3.06 [95% confidence interval {CI}, 2.15–4.35], P < .001), cystoid macular edema (HR = 2.87 [95% CI, 1.41–5.82], P = .004), posterior synechia at presentation (HR = 2.85 [95% CI, 1.53–5.30], P = .001), and use of local injections of corticosteroids (HR = 2.37 [95% CI, 1.18–4.75], P = .02). Treatments with systemic and topical corticosteroids were not significant risk factors. CONCLUSIONS: In this study, we found that development of cataract is common among pediatric eyes with uveitis and is most strongly related to the extent of inflammation recurrences and ocular complications. We suggest that controlling the inflammation, even using higher doses of systemic and topical corticosteroids, is of importance in preventing ocular complications, such as cataract. Uveitis accounts for 10–15% of blindness in the developed world.1 Although pediatric uveitis is relatively uncommon, accounting for only 5–10% of all uveitis cases,2 it affects young patients, who in most cases are otherwise healthy. Vision loss results from ongoing inflammation that leads to ocular structural changes, such as cataract, corneal opacities, optic neuropathy, and retinal lesions. The most common causes of vision loss in children with uveitis are cataract, glaucoma, and chronic cystoid macular edema (CME).2, 3 In addition, any chronic visual obstruction can result in the development of amblyopia in younger children, with vision loss persisting after the inciting cause is treated.4 Such changes, together with the need for long-term treatment and continuous monitoring, can have a profound impact on their development, independence, and education. The prevalence of cataract in eyes with uveitis ranges from 20–64%,4, 5, 6, 7 and it is the most common complication of uveitis in children,8 occurring in approximately 35% of children with juvenile idiopathic arthritis (JIA)-associated uveitis9 and increasing ≤80% in adults.10, 11 Cataract progression can be the result of persistent intraocular inflammation,12, 13 can be caused by surgery for uveitis complications (eg, trabeculectomies and repair of retinal detachments), or can be a consequence of uveitis treatment, particularly the use of local or systemic corticosteroids.14, 15, 16, 17 It results in reduced visual acuity and can have a detrimental effect on the development and academic achievements of these children.18 Studies have examined risk factors for the development of cataract among children with JIA-associated uveitis, identifying risk factors such as the presence of posterior synechiae (PS) at presentation,12, 19 the use of systemic corticosteroids,13 topical corticosteroid therapy exceeding 3 drops a day,12 or persistent, uncontrolled active inflammation,3 while early treatment with methotrexate delayed cataract progression.19 However, JIA is a unique cause of uveitis, often localized to the anterior chamber, with frequent intraocular structural changes and the early use of systemic immunosuppressive agents. It may not represent the same risks as other causes of pediatric uveitis. We examined disease- and treatment-related risk factors for cataract development in children with uveitis of any etiology. We investigated clinical and ophthalmologic characteristics, as well as treatment strategies in relation to the time interval between the first presentation with uveitis and cataract development

Clinical Outcome of Retinal Vasculitis and Predictors for Prognosis of Ischemic Retinal Vasculitis

PURPOSE: To determine factors affecting the visual outcome in eyes with retinal vasculitis and the rate of neovascularization relapse in ischemic vasculitis. DESIGN: Retrospective cohort study. METHODS: We reviewed 1169 uveitis patients from Moorfields Eye Hospital, London, UK. Retinal vasculitis was observed in 236 eyes (121 ischemic, 115 nonischemic) that were compared with a control group (1022 eyes) with no retinal vasculitis. Ultra-widefield fluorescein angiography images were obtained in 63 eyes with ischemic vasculitis to quantify area of nonperfusion measured as ischemic index. RESULTS: The risk of vision loss was significantly more in the retinal vasculitis compared with the non-vasculitis group (hazard ratio [HR] 1.67, 95% confidence interval [CI] 1.24–2.25, P = .001). Retinal vasculitis had twice the risk of macular edema compared to the non-vasculitis group. Macular ischemia increased the risk of vision loss in vasculitis eyes by 4.4 times. The use of systemic prednisolone in eyes with vasculitis was associated with a reduced risk of vision loss (HR 0.36, 95% CI 0.15–0.82, P = .01). Laser photocoagulation was administered in 75 eyes (62.0%), out of which 29 (38.1%) had new vessel relapse and required additional laser treatment. The median ischemic index was 25.8% (interquartile range 10.2%–46%). Ischemia involving ≥2 quadrants was associated with increased risk of new vessel formation (HR 2.7, 95% CI 1.3–5.5, P = .003). CONCLUSION: Retinal vasculitis is associated with an increased risk of vision loss, mainly secondary to macular ischemia, and has a higher risk of macular edema compared to eyes with no vasculitis. Ischemia involving ≥2 quadrants is a risk factor for new vessel formation

UNICORNS: Uveitis in childhood prospective national cohort study protocol [version 1; peer review: 1 approved, 1 approved with reservations]

Background: Childhood uveitis is a rare inflammatory eye disease which is typically chronic, relapsing-remitting in nature, with an uncertain aetiology (idiopathic). Visual loss occurs due to structural damage caused by uncontrolled inflammation. Understanding of the determinants of long term outcome is lacking, including the predictors of therapeutic response or how to define disease control. Aims: To describe disease natural history and outcomes amongst a nationally representative group of children with non-infectious uveitis, describe the impact of disease course on quality of life for both child and family, and identify determinants of adverse visual, structural and developmental outcomes. Methods: UNICORNS is a prospective longitudinal multicentre cohort study of children newly diagnosed with uveitis about whom a core minimum clinical dataset will be collected systematically. Participants and their families will also complete patient-reported outcome measures annually from recruitment. The association of patient (child- and treatment- dependent) characteristics with outcome will be investigated using logistic and ordinal regression models which incorporate adjustment for within-child correspondence between eyes for those with bilateral disease and repeated outcomes measurement. Discussion: Through this population based, prospective longitudinal study of childhood uveitis, we will describe the characteristics of childhood onset disease. Early (1-2 years following diagnosis) outcomes will be described in the first instance, and through the creation of a national inception cohort, longer term studies will be enabled of outcome for affected children and families