Factors Affecting Visual Outcome in Patients with BPES (Blepharophimosis, Ptosis and Epicanthus Inversus syndrome).

Abstract

Abstract: : Purpose: To determine the risk factors for amblyopia in BPES Methods: A retrospective review of all patients with BPES presenting to Moorfields Eye Hospital, a tertiary referral centre in the UK over a 23–year period. Putative risk factors for amblyopia included refractive error, strabismus, degree of ptosis leading to stimulus deprivation amblyopia and timing of ptosis surgery. Amblyopia was defined as a visual acuity <= 20/40 at the final follow–up visit. Patients who had undergone ptosis surgery elsewhere were specifically excluded. Logistic regression was used for data analysis. Results: Our final cohort consisted of 100 patients. 42 % developed amblyopia. The odds of amblyopia were much higher in patients with strabismus than without (OR 4.2 { 1.33, 12.95}) P = 0.01 ) and in patients with stimulus deprivation due to ptosis (OR 23.85 {2.78, 180.7} P = 0.004). Patients with a moderate degree of ptosis (palpebral aperture 4–8mm) were more likely to develop stimulus deprivation amblyopia than those with a severe ptosis (palpebral aperture <4mm). Odds ratio (1.4 {0.49, 4.01} P = 0.5 ) . The median age of surgery in the moderate ptosis group was 5.7 years compared with 3.25 years in the severe ptosis group Anisometropia was not found to be a significant risk factor for amblyopia in this study . Conclusions: There is a high incidence of amblyopia in BPES . Strabismus and stimulus deprivation due to ptosis are significant risk factors. Surprisingly, the risk of stimulus deprivation amblyopia was found to be higher in the moderate ptosis group compared to the severe ptosis group and this may be related to the relative delay in surgery in this group. This group should therefore be carefully monitored and may benefit from earlier surgery.

Keywords: eyelid • strabismus: treatment • visual development