One-stage correction of blepharophimosis-ptosis-epicanthus inversus syndrome using a frontalis muscle transfer technique

Abstract

Blepharophimosis-ptosis-epicanthus inversus (BPES) is a rare genetic disease involving a complex eyelid malformation. The surgical treatment approach for BPES is highly complex and a subject of controversy. This study reports the results of a one-stage frontalis muscle transfer technique to correct BPES. This retrospective, interventional study included 21 patients with BPES who had been followed-up for a minimum of 1 year. The one-stage intervention was a combination of three surgical techniques: Mustardé medial canthoplasty, Fox lateral canthoplasty, and the frontalis muscle transfer technique. Preoperative and postoperative measurements of the horizontal lid fissure length (HLFL), vertical lid fissure width (VLFW), inner intercanthal distance (IICD), and the IICD/HLFL ratio were analyzed by Wilcoxon's signed rank test. The mean preoperative measurements were 4.73 ± 0.32 mm for VLFW, 19.98 ± 3.74 mm for HLFL, 40.85 ± 4.46 mm for IICD, and 2.11 ± 0.45 mm for the IICD/HLFL ratio. The mean postoperative measurements were 7.86 ± 0.41 mm for VLFW, 24.47 ± 3.35 mm for HLFL, 32.52 ± 4.16 mm for IICD, and 1.35 ± 0.22 mm for the IICD/HLFL ratio (p < 0.0001 for all preoperative vs postoperative values). Postoperative complications included eyelid fold deformities, lagophthalmos, and conspicuous scars. Most of these complications gradually resolved. One-stage correction of BPES is safe and efficient with the surgical techniques described.