A one-stage correction of the blepharophimosis syndrome using a standard combination of surgical techniques

Abstract

Background: The aim of this study was to evaluate the efficacy of a one-stage treatment for the blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) using a combination of standard surgical techniques.

Methods: This is a retrospective interventional case series study of 21 BPES patients with a 1-year minimum follow-up period. The one-stage intervention combined three different surgical procedures in the following order: Z-epicanthoplasty for the epicanthus, transnasal wiring of the medial canthal ligaments for the telecanthus, and a bilateral fascia lata sling for ptosis correction. Preoperative and postoperative measurements of the horizontal lid fissure length (HFL), vertical lid fissure width (VFW), nasal intercanthal distance (ICD), and the ratio between the intercanthal distance and the horizontal fissure length (ICD/HFL) were analyzed using Student's t test for paired variables.

Results: The mean preoperative measurements were 4.95 ± 1.13 mm for the VFW, 20.90 ± 2.14 mm for the HFL, 42.45 ± 2.19 mm for the ICD, and 2.04 ± 0.14 mm for the ICD/HFL ratio. The mean postoperative measurements were 7.93 ± 1.02 mm for the VFW, 26.36 ± 1.40 mm for the HFL, 32.07 ± 1.96 mm for the ICD, and 1.23 ± 0.09 mm for the ICD/HFL ratio. All these values and their differences were statistically significant (P < 0.0001). All of the patients developed symmetric postoperative inferior version lagophthalmus, a complication that tended to decrease over time.

Conclusion: One-stage correction of BPES is safe and efficient with the surgical techniques described.