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

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.