Severe Blepharokeratoconjunctivitis in Children-The Toronto Experience

Abstract

Purpose: To report on the clinical characteristics and treatment outcomes of a large cohort of pediatric patients who presented with severe blepharokeratoconjunctivitis (BKC).

Design: Retrospective consecutive cohort study.

Subjects: Patients aged under 18 years with BKC presented to the Cornea Service at the Hospital for Sick Children, Toronto, Canada, between 2006 and 2021.

Methods: Children with severe BKC, defined as BKC with inflammation, new vessel formation, scarring, thinning, and lipid deposits affecting the peripheral and/or central cornea, and a follow-up of at least 6 months were included. Basic demographics, best corrected visual acuity (BCVA), refractive error, disease laterality, type and duration of antibiotic therapy, flare-up rate, and extent of corneal involvement were analyzed.

Main outcome measures: BCVA, refraction, extent of corneal involvement, procedures, flare-up of BKC, and time to flare-up rate after cessation of systemic antibiotics treatment.

Results: Out of 2542 patients with blepharitis, 197 patients (315 eyes) were identified with severe BKC. The mean age at the first presentation was 7.6 years, with a 2:1 female predominance. At the presentation, 65% had a history of chalazion, and 28% had BCVA worse than 0.3 logMAR, which improved to 17% at final follow-up (P = .007). The most striking corneal pathologies affecting the peripheral and central cornea were scarring (83%) and new vessel formation (49%). One hundred forty-two patients (72%) required systemic antibiotics (azithromycin, clarithromycin, erythromycin, or doxycycline), of whom 23% experienced flare-ups requiring an escalation in treatment with topical steroids and/or systemic antibiotics, and 75% of these occurred within the first year of treatment cessation. Ninety-one percent of patients achieved complete response, defined as no disease activity and no use of topical steroids or systemic antibiotic treatment. Fifteen patients required surgical intervention.

Conclusions: BKC is a serious disease in children, potentially causing irreversible ocular morbidity and vision loss. Central scarring and astigmatism can affect vision in a significant proportion of children. Therefore, early identification and treatment are necessary to reduce disease morbidity. Close follow-up is crucial as one in four patients may experience a flare-up, especially within the first year of treatment cessation. Most patients with severe BKC require both topical steroids and systemic antibiotics to control disease activity.