Improvement of one-stage comprehensive operation technique for blepharophimosis-ptosis-epicanthus inversus syndrome

Abstract

Background: This retrospective case series aimed to evaluate the clinical effect of a modified one-stage comprehensive surgical method for blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). Methods: Twenty-five patients with BPES underwent a modified reverse Z-plastic surgery method to change the Epicanthus into two unequal V-shaped flaps and suture them alternately. The medial canthus ligament was partially incised, shortened, and sutured; the outer canthus was opened with a "V-Y" flap. The gray line of the outer canthus was cut to extend the eyelid margin length. Ptosis was corrected using the orbicularis oculi-frontalis cross-flap technique. We studied the internal canthal distance (IICD), interpupillary distance (IPD), horizontal palpebral fissure length (HPFL), marginal reflex distance-1 (MRD-1), IICD/IPD ratio, IICD/HPLF ratio, Vancouver Scar Scale (VSS), and overall postoperative satisfaction. Results: The 25 patients included 12 females and 13 males (8.3±6.8 years), 19 of whom had a family history of BPES. The mean value of IPD was 50.15±7.43 mm. IICD decreased from 39.38±2.39 mm preoperatively to 31.64±2.37 mm postoperatively, IICD/IPD from 0.80±0.12 preoperatively to 0.64±0.07 postoperatively (p<0.05), and IICD/HPLF from 1.98±0.33 preoperatively to 1.22±0.10 postoperatively (p<0.01). HPLF increased from 20.08±3.53 mm preoperatively to 26.04±1.36 mm postoperatively (p<0.01) and MRD-1 from -1.12±0.71 mm preoperatively to 3.24±0.47 mm postoperatively (p<0.01). The postoperative VSS score was 3.68 ±1.07. The patients’ satisfaction was 8.8±1.08, with a relatively high overall satisfaction. Conclusions: The corrective effect on patients with BPES was noticeable, improving the IICD/HLFL and IICD/IPD ratios. Postoperative scars were not evident, and patient satisfaction was high.