Combination of Frontalis Sling,Y-V Plasty, and Medial Canthopexy in Blepharophimosis Syndrome

1. Wulan Aprianti
2. Ira Sudarmadji
3. Hernawita Suharko

University of Indonesia, Jakarta, Indonesia

Purpose
To report correction of Blepharophimosis Ptosis Epicanthus inversus and telecanthus Syndrome (BPES) in a 6 -year old Indonesian boy with combination of Y-V plasty, medial canthopexy and frontalis sling.

Methods
A 6 year-old boy with epicanthus inversus, ptosis,and telecantus of both eye since he was a baby. Visual acuity of both eye was 6/7.5. The horizontal palpebral fissure 17 mm of both eyes, vertical palpebral fissure height 3 mm of the right eye, 4 mm of the left eye. The margin reflex distance was -3 mm of the right eye, and -2 of the left eye. The intercanthal distance was 37 mm. Levator action of both eye was 2mm. The reconstruction procedure was frontalis sling procedure followed by medial canthopexy and y-v plasty.

Results
Two weeks after surgery, horizontal palpebral fissure 27 mm of right eye and 29 mm of left eye , vertical palpebral fissure height was 6 mm of both eyes. Margin reflex distance was 2 mm of the right eye, and 1 mm of the left eye. Intercanthal distance was 24 mm. There were lagophthalmus 5 mm of right eye, and 4 mm of left eye with no corneal expose. Suture removal was done.

Conclusions
BPES requires complex surgery correction with variable techniques and indications. One stage surgery with Y-V plasty, medial canthopexy and frontalis sling give good result and hopefully will bring better visual function and cosmesis.