Sequential correction of blepharophimosis syndrome: comparison of anatomical outcomes for surgery before and after 24 months of age

Abstract

Purpose: To evaluate the safety and morphometric outcomes of early sequential surgical correction in pediatric patients with blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) with significant functional impairment, comparing patients under and over 24 months of age. Methods: The medical records of consecutive BPES patients undergoing two-stage treatment of Y-V epicanthoplasty followed by silicone frontalis suspension from 2019 to 2021 were retrospectively analyzed. Primary outcomes included morphometric parameters: ratio of inner intercanthal distance (IICD) to horizontal palpebral fissure length (HPFL), margin reflex distance-1 (MRD1), and vertical palpebral fissure length (VPFL). Secondary outcomes were visual axis clearance, compensatory posture resolution, and complications. Statistical analysis employed repeated measures ANOVA with Bonferroni correction. Results: A total of 20 patients were included. Mean age at surgery was 43.4 ± 32.4 months (range, 6-120), with 11 patients (55%) under 24 months. At 6 months' follow-up, all morphometric parameters demonstrated significant improvement (P < 0.001): IICD/HPFL ratio decreased from 1.82 ± 0.23 to 1.13 ± 0.08; VPFL increased from 3.92 ± 0.71 mm to 8.22 ± 0.44 mm; and MRD1 improved from 0.45 ± 0.91 mm to 4.17 ± 0.40 mm. Visual axis clearance and resolution of compensatory chin-up posture were achieved in 100% of cases; surgical success (IICD/HPFL <1.3), in 95% of patients (19/20). Age-stratified analysis revealed no significant differences in outcomes between cohorts (P > 0.05). Grade I complications occurred in 5 patients (25%) and resolved conservatively. Conclusions: In select infants with severe BPES, early two-stage sequential correction can achieve visual axis clearance and anatomical outcomes comparable to those achieved in older children.