Childhood blepharokeratoconjunctivitis: characterising a severe phenotype in white adolescents.

BACKGROUND: The syndrome of childhood blepharokeratoconjunctivitis (BKC) is frequently underestimated. While prevalent and aggressive among Indo-Pakistani/Middle-Eastern populations, we observe a recalcitrant destructive phenotype in white children/adolescents that persists into early adulthood and may require systemic immunosuppression. METHODS: A cohort of 10 white patients (20 eyes), median age 15.2 (range 6-27) years were identified among 62 patients with BKC attending a tertiary referral centre. Clinical features were graded and lid/conjunctiva swabs were performed, before instituting a hierarchical therapeutic protocol comprising lid hygiene, topical/systemic antibiotics, intensive topical glucocorticoids and systemic immunosuppression. RESULTS: The median duration of symptoms prior to presentation was 4.3 (range 1.2-16.3) years, with 14 eyes (nine patients) demonstrating 360° peripheral corneal vascularisation associated with encroachment/involvement of the visual axis in 10 eyes (six patients). Corneal perforation(s) occurred in three eyes (two patients). Intensive topical glucocorticoids enabled disease control in 10 eyes (seven patients). In six eyes (three patients), persistent active disease necessitated systemic immunosuppression (azathioprine (2), mycophenolate mofetil (1), prednisolone (1)) achieving disease remission within three months with no adverse events reported. CONCLUSIONS: Suboptimal treatment of BKC in white children may permit a progressively destructive sight-threatening phenotype, which may last into adulthood and require immunosuppression. Appropriate aggressive steroid-based and steroid-sparing strategies are vital for disease remission.

Severity of pediatric blepharokeratoconjunctivitis in Asian eyes.

PURPOSE: To describe and evaluate the severity of pediatric blepharokeratoconjunctivitis in Asia. DESIGN: Retrospective case series. METHODS: Clinical records of patients diagnosed with pediatric blepharokeratoconjunctivitis at a tertiary referral center in Singapore from 1991 through 2010 were reviewed. Patients were graded as having mild (corneal involvement without scarring), moderate (corneal scarring), or severe (corneal scarring with thinning or perforation) disease based on recorded clinical findings. RESULTS: Fifty-one patients were diagnosed with pediatric blepharokeratoconjunctivitis. The mean age at presentation was 10.2 ± 3.6 years, most patients were female (80.4%), and the mean duration of follow-up was 58.9 ± 44.0 months. Chinese (56.9%) subjects made up most of the cases. Most subjects had moderate (56.9%), followed by severe (37.4%) and mild (5.9%), disease. Four patients (7.9%) had an associated dermatologic disease. All patients were treated with topical antibiotics, and 98% were treated with topical steroids. Nineteen (37.3%) patients received systemic antibiotic therapy, and 1 received systemic steroid therapy. Three patients required deep lamellar keratoplasty (2 tectonic and 1 optical), and 2 underwent cornea gluing alone; all 5 of them were Chinese. Patients graded as having severe disease were more likely to undergo surgical intervention (26.3%) than patients who were graded as having moderate (0%) and mild (0%) disease (P < .05). The main complication of treatment was raised intraocular pressure in 7 (13.7%) patients requiring medical therapy. Overall, best-corrected visual acuity improved by 0.10 logarithm of the minimal angle of resolution units (P < .001) after appropriate medical and surgical intervention. CONCLUSIONS: Pediatric blepharokeratoconjunctivitis patients in Asia seem to have a more severe clinical presentation and course. Early and adequate management can arrest the disease process and can minimize visual morbidity.

Blepharokeratoconjunctivitis in children: diagnosis and treatment.

AIM: Blepharokeratoconjunctivitis (BKC) is a poorly described entity in children. This study characterises this syndrome in childhood and evaluates epidemiology, clinical grading, and treatment strategies. METHODS: 44 children (20 white, 22 Asian, 2 Middle Eastern, median age 5.4 (range 1-14) years) with a diagnosis of BKC were followed for a median of 7 years. Diagnostic criteria included recurrent episodes of chronic red eye, watering, photophobia, blepharitis including recurrent styes or meibomian cysts, and a keratitis. Clinical features were graded as mild, moderate, or severe. The lids and conjunctiva were cultured. The treatment regimen incorporated lid hygiene, topical and/or systemic antibiotics, and topical corticosteroids. RESULTS: The disease was most severe in the Asian and Middle Eastern children (p <0.001), who had a statistically higher risk of subepithelial punctate keratitis (p = 0.008), corneal vascularisation (p <0.001), and marginal corneal ulcerations (p = 0.003), than the white group. 15 children had culture positive lid swabs. Most children had a reduction in symptoms and signs with treatment, and progression of disease after the age of 8 was rare. CONCLUSIONS: BKC in children can be defined as "a syndrome usually associated with anterior or posterior lid margin blepharitis, accompanied by episodes of conjunctivitis, and a keratopathy including punctate erosions, punctate keratitis, phlyctenules, marginal keratitis, and ulceration." BKC is common in children in a tertiary referral corneal and external diseases clinic, with the more severe manifestations in the Asian and Middle Eastern populations. Therapy is effective and loss of sight can be prevented in most cases.