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S. Hamada, A. K. O. Denniston, S. Rauz; Severe Blepharokeratoconjunctivitis in Caucasian Children and Adolescents: Identifying a Severely Destructive Phenotype. Invest. Ophthalmol. Vis. Sci. 2010;51(13):2382.
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The syndrome of childhood blepharokeratoconjunctivits (BKC) has recently gained recognition as a disease entity that is frequently undertreated. The condition is particularly prevalent and aggressive amongst Asian (Indo-Pakistani) or Middle Eastern populations, but effective, early treatment can prevent permanent sight loss. In this study we characterize a rare recalcitrant and destructive phenotype in Caucasian children and adolescents that persists into early adulthood necessitating systemic immunosuppression to control disease.
A cohort of 10 Caucasian patients (20 eyes), median age 15.8[range 6-27] years) with a diagnosis of BKC, was referred to a tertiary regional centre in the United Kingdom. In all cases, the reason for referral was ‘failure to control disease’. Clinical features were graded and all patients underwent cultures of the lids/conjunctiva to exclude hypercolonization by microbes. Therapeutic strategies included a range of interventions: lid hygiene, topical/systemic antibiotics, intensive topical glucocorticoids, cyanoacrylate gluing of the cornea, and systemic immunosuppression.
The median duration of clinical history prior to presentation was 4.3[range 0.5-10.5] years when patients were either treated in secondary care and/or non-specialized general ophthalmology clinics, or by the general (family) practitioner prior to referral to tertiary care. All eyes at presentation had 360o of peripheral corneal vascularization and limbitis of which a further 10 eyes (6 patients) had corneal vascularization encroaching or involving the visual axis. In 3 eyes (2 patients) severe disease led to one or more corneal perforations. None of the eyes were culture positive. Implementing aggressive steroid-based therapeutic strategies enabled disease control in 11 eyes (7 patients). In the remaining 6 eyes (3 patients), persistent active disease with progressive corneal damage (including perforation and corneal glue (3 eyes)) necessitated systemic immunosuppression (Azathioprine (2), Mycophenolate (1)) with 1 patient requiring a short induction course of oral prednisolone. In all 3 cases, disease control was achieved. No side effects were experienced during the follow up period (9, 10 & 72 months).
Delayed referral or suboptimal treatment of BKC in a Caucasian subgroup of children may trigger a progressively destructive sight-threatening phenotype (requiring systemic immunosuppression), which may persist beyond puberty and into adulthood. Early referral to specialists’ centers is vital.
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