Corneal perforation in Paediatric Blepharokeratoconjunctivitis

1. Livia Teo^1,2
2. Donald T Tan^1,2
3. Jodhbir S Mehta^1,2

¹Singapore National Eye Centre
²Singapore Eye Research Institute

Purpose:
To describe a series of patients with paediatric blepharokeratoconjunctivitis (PBKC) who developed corneal perforations and their subsequent surgical management.

Method:
This is a retrospective case series. Clinical records of patients diagnosed with PBKC at a tertiary referral centre in Singapore from 1991 to 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 at any point during the follow up period. Those with corneal perforations were singled out and data evaluated.

Results:
51 patients were diagnosed with PBKC. The mean age of presentation was 10.2 years ± 3.6 years, the majority were female (41 subjects (80.4%), and the mean duration of follow up was 58.9 ± 44.0 months (range 3 - 227 months). Chinese (29 subjects, (56.9%)) subjects made up the majority of the cases. Most subjects had moderate disease, (56.9%) followed by severe (37.4%) and mild (5.9%) disease .4 patients (7.9%) had an underlying associated systemic disease (3 with acne rosacea, 1 with acne vulgaris). All patients were treated with topical antibiotics and 98% of them were treated with topical steroids. 19 patients received systemic antibiotic therapy and one received systemic steroid therapy. 4 patients in our series developed corneal perforations as a direct result of the disease. 3 patients required deep lamellar keratoplasty (2 of them tectonic and 1 optical) and 2 underwent cornea glueing alone, without graft surgery. Patients who were graded as severe were more likely to undergo surgical intervention (26.3%) than patients who were graded moderate (0%) and mild (0%) (p< 0.05). The main complication of treatment was raised intraocular pressure in 7 (13.7%) patients requiring medical therapy. Overall, best corrected visual acuity improved by LogMAR 0.10 (p<0.001) after appropriate medical and surgical intervention.

Conclusion:
PBKC and the complications associated with its treatment can be sight threatening but early and adequate management can arrest the disease process and minimize visual morbidity.