EARLY SINGLE STAGE CORRECTION OF BLEPHAROPHIMOSIS SYNDROME
Author: Zoran Zikic
Base Hospital / Institution: Milos Eye Clinic, Belgrade, Serbia and Eye Clinic, University Hospital Zvezdara, Belgrade
Rapid fire oral presentation
Abstract ID: 24-464
Purpose
Traditionally, the treatment of blepharophimosis epicanthus inversus syndrome (BPES), has been performed in 2 stages, between the ages of 3 and 5 years of age. Usually the epicanthus inversus and telecanthus are corrected first, followed by ptosis correction about 12 months later. There have been proposals to correct the ptosis first. In recent years, a single stage approach has been advocated, but the minimum age of patients treated was 6-7 years. We present a single stage technique, that can be applied to patients as early as one year of age.
Methods
Four children with BPES, 1 female, and 3 male, all with severe ptosis, frontalis compensation, head tilt and impending amlyopia, were indicated for early ptosis correction. The mean age was 14 months. The parents were properly informed and given the option of ptosis correction only, or simultaneosly with epicanthus and telecanthus reduction. All opted for the latter. The surgical technique incorporated a Y-V plasty with medial canthal tendon resection, followed by a direct frontalis palpebral suspension, using 3 polypropilene 4-0 sutures between the frontalis muscle and tarsal plate.
Results
In 3 of 4 patients, a good clearance of the visual axis was achieved, whereas in 1 patient an initial undercorrection was present on one eye, which had to be revised. Another early complication was chemosis of the bulbar conjunctiva in one child, which resolved with lubricants. With regard to late complications, there was a medial canthus granuloma in one child, which also required surgical reintervention. Overall parent satisfaction with the procedure was 4.75, on a scale of 1-5.
Conclusion
The concept of a single stage correction of BPES is based on the intention of reducing the number of general anesthesias in affected children. The technical difficulty of a single stage approach is due to the different force vectors, applied simultaneously to the upper eyelids. Severe ptosis requires early surgical treatment and direct frontalis suspension obviates the need to do a silicone sling as a first line approach, since fascia lata frontalis suspension cannot be performed before 4 years of age.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Isidora | Janicijevic | Milos Eye Clinic, Belgrade, Serbia and Eye Clinic, University Hospital Zvezdara, Belgrade |
| Andjela | Pusonja | Milos Eye Clinic, Belgrade, Serbia and Eye Clinic, University Hospital Zvezdara, Belgrade |
