Surgical strategy for congenital blepharophimosis syndrome

Background So far, most of the surgical techniques for congenital blepharophimosis syndrome are two-stage procedures. In this study, we investigated a modified one-stage procedure to reduce the suffering of patients. Methods From 2003 to 2005, we adopted an one-stage technique combining blepharoptosis correction with medial canthoplasty in 16 patients with congenital blepharophimosis syndrome （10 male, 6 female; aged from 6 to 21）. All the patients had bilateral severe blepharoptosis, epicanthus inversus, and flat dorsum nasi. The movement of the upper lid was 0 to 3 mm, vertical length of the eye fissure 2 to 4 mm, horizontal length 13 to 22 mm, and the distance between the eyes was 35 to 39 mm. The patients were followed up for one half to 2 years after the operation. Results In all the patients, after the operation, the horizontal length of the eyelid 〉25 mm, the vertical length 〉 6 mm. and the distance between the eyes 〈 35mm. The appearance of their double eyelids was satisfying. Conclusion The modified one-stage technique combining blepharoptosis correction with medial canthoplasty can achieve favorable outcomes for patients with congenital blepharophimosis syndrome.

Prevalence of amblyopia in congenital blepharoptosis: a systematic review and Meta-analysis

AIM: To conduct a systematic review and Meta-analysis of the published literature to evaluate the pooled prevalence rate of amblyopia in patients with congenital ptosis.METHODS: We searched the PubMed, Embase, the Cochrane Central Register of Controlled Trials, China National Knowledge Infrastructure, Wanfang Data, and Chongqing VIP databases for studies reporting the prevalence of amblyopia in patients with congenital ptosis. The reference lists of relevant studies were scanned. Heterogeneity of effect sizes across studies was tested. We calculated prevalence ratios to compare prevalence estimates for different causes of amblyopia in patients with congenital ptosis, as well as for different geographical regions, year of publication and sample size in subgroup analyses. A systematic review and Meta-analysis were performed.RESULTS: We identified 29 eligible surveys with a total population of 2436. Prevalence rates of amblyopia ranged from 13.8% to 69%. We noted substantial heterogeneity in prevalence estimates for amblyopia in congenital ptosis(Cochran’s χ2 significant at P<0.0001; I2=90%). The pooled prevalence using random-effects models of 29 studies was 32.8%(95%CI: 27.3%-38.4%) in the overall population. Compared to the overall pooled prevalence, amblyopia prevalence was higher in studies in which only subjects with blepharophimosis syndrome were included.CONCLUSION: We confirm that nearly one-third of congenital ptosis patients are suffering from or at risk for amblyopia. Patients with blepharophimosis syndrome are more likely to develop amblyopia. The identificationand management of amblyopia should be integral to the treatment of congenital ptosis.