Improvement of 1-Stage Comprehensive Operation Technique for Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome.

BACKGROUND

This retrospective case series presents the clinical results of a modified 1-stage comprehensive surgical method for blepharophimosis-ptosis-epicanthus inversus syndrome (BPES).

METHODS

A total of 25 patients with BPES underwent a modified reverse Z-plastic surgery method to bisect the epicanthus into 2 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 composite flap technique. We studied the inner intercanthal distance (IICD), interpupillary distance (IPD), horizontal palpebral fissure length (HPFL), marginal reflex distance 1 (MRD-1), IICD/IPD ratio, IICD/HPFL ratio, Vancouver Scar Scale, and overall postoperative satisfaction.

RESULTS

The 25 patients included 12 girls and 13 boys (mean age, 8.3 ± 6.8 years), 19 of whom had a family history of BPES. The mean IPD value 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 0.8 ± 0.1 preoperatively to 0.6 ± 0.1 postoperatively ( P < 0.05), and IICD/HPFL from 1.98 ± 0.33 preoperatively to 1.22 ± 0.10 postoperatively ( P < 0.01). HPFL 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 Vancouver Scar Scale score was 3.68 ± 1.07. Patient satisfaction was rated as 8.8 ± 1.08, with a relatively high overall satisfaction rate.

CONCLUSIONS

The corrective effect on BPES was noticeable, improving the IICD/HPFL and IICD/IPD ratios. Postoperative scars were not evident, and patient satisfaction was high.

CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.