The activity and damage of blepharokeratoconjunctivitis in children

Abstract

The syndrome of childhood blepharokeratoconjunctivits (BKC) is frequently underestimated. Total control of the disease is not possible in many cases. Measuring success has been based on clinical experience only. We are implementing a new classification of BKC to define the degree of activity and damage at any stage of the disease and hence measuring success of disease management. It will objectively measure success of our BKC management protocol. A retrospective review of all children with BKC over the last 10 years at tertiary referral centre. Children with significant systemic disorders, atopy, vernal keratoconjunctivitis, or perennial allergic conjunctivitis were excluded. Activity/damage score of the worst affected eye at initial presentation and at last follow-up were included in the statistical analysis. Clinical features were graded, before and after instituting a hierarchical therapeutic protocol comprising lid hygiene, topical/systemic antibiotics, intensive topical glucocorticoids, topical lubricants, and nutritional supplement. Activity (A0-A3) and damage (D0-D3) scoring was applied to grade BKC. Complete success was defined as ability to fully control disease activity i.e. e. from any grade to A0. Partial success meant reduction in activity scoring but not reaching A0 grade. Failure if there was no change or worsening of disease activity. We identified a cohort of 42 patients (84 eyes) with BKC; mean age at onset of symptoms was 4.6 years (0.4-14 years) and at presentation to our center 7 (1-15 years). The median duration of symptoms prior to presentation was 2.22 years (range, 0.01-10 years). 17 Asian and 25 Caucasian patients were followed up for 3.2 years (3 months-10 years). The protocol was followed in 90% of children. All cases where scored as per activity and damage scoring system. All risk factors were analysed. Complete success was achieved in 50%, partial success in 38%, and failure in 12% of children). Recurrences ranged from 0 to 7 times (mean, 2.1). BKC is underestimated in white children. Total control of the disease is not always possible. A protocol based on topical steroids and following a strict regime of treatment is vital for disease control. A classification of BKC based on activity and damage score is an appropriate method to describe the disease and classify it. It allows measuring success of treatment and to compare various modalities of treatments. |Younger age and longer duration of symptoms were common in the failure group. Those with acne rosacea had the worst prognosis and this was statistically significant. Our protocol resulted in full control of the disease in 50% of patients but failed in 12% of cases. Whites with BKC were at risk of severe phenotype that is resistant to treatment. The management of BKC in children is very challenging. Suboptimal treatment of BKC in children may permit a progressively destructive sight-threatening phenotype, which may last into adulthood and cause visual disability. Appropriate aggressive topical steroids and following a strict regime of treatment are vital for disease remission. A novel scoring system, which takes into consideration both activity and damage of BKC, will allow appropriate disease description and classification, measuring success of treatment, and comparing various treatment protocols.