Abstract Listings 2024

Purpose

This study aimed to assess the long-term functional, cosmetic and visual rehabilitation outcomes of patients treated for Blepharophimosis Syndrome (BPES).

Methods

Records of BPES patients who underwent two-stage surgical correction for blepharophimosis syndrome treated between 1996 and 2022 were retrospectively assessed. Parameters including best corrected visual acuity, refractive errors, presence of amblyopia, vertical interpalpebral fissure height (IPFH), horizontal palpebral fissure length (HPFL) and inner intercanthal distance (IICD) were assessed during preoperative and postoperative periods.

Results

A total of 42 patients (84 eyes) were included. The mean age at initial presentation was 68 ± 126.4 months. Firstly Mustarde double Z-plasty (61.9%, n=26) or Y-V plasty (38.1%, n=16) was performed for epicanthus inversus repair, afterwards frontal suspension surgery with silicone rod (71.4%, n=30) or autogenous fascia lata (28.6%, n=12) was performed for ptosis repair. The mean follow-up period after ptosis surgery was 98.4 ± 61.3 months. Postoperatively, astigmatism values decreased from a mean of -2.50 ± 0.98 D to a mean of -1.66 ± 1.0 D (p<0.001). Amblyopia was detected in 52.2% of patients (n=22) at the final visit (mean age 17.6 years ± 12.8 years), positive correlations observed with increasing age at initial examination (p=0.017), age at ptosis surgery (p=0.042) and postoperative astigmatism values (p=0.001). The IICD/HPFL ratio decreased from 2.25 ± 0.19 to 1.27 ± 0.33 postoperatively (p<0.001) and IPFH measurements increased from 3.04 ± 0.7 mm to 7.23 ± 0.79 mm (p<0.001).

Conclusion

BPES is a syndrome with functional, cosmetic and psychological implications, necessitating early surgery due to the risk of developing amblyopia. A stepwise surgical approach in the management of these patients followed by regular follow-up is crucial.

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