[Management of blepharophimosis, ptosis, epicanthus inversus syndrome at a referral center in Tunisia]. / Prise en charge du syndrome de blépharophimosis ptôsis épicanthus inversus (SBPE) dans un centre de référence en Tunisie.

ABSTRACT

INTRODUCTION: Blepharophimosis ptosis epicanthus inversus syndrome (BPES) is a rare congenital hereditary abnormality. It includes complex orbital-palpebral malformations, causing aesthetic and functional ramifications. Management of BPES requires two steps  diagnosis and treatment. PATIENTS AND METHODS: We performed a retrospective descriptive study of 44 patients (88 eyelids) with blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). In our series, we opted for two-stage surgery in 28 cases  epicanthus-telecanthus surgery followed by ptosis surgery. Simultaneous surgery was performed in 5 cases. RESULTS: The mean age at the first visit was 6 years (6.1±6.4). The mean age of our patients at the time of the first surgery was 6.6 years. Epicanthus surgery was performed in 35 cases. The two techniques used to correct epicanthus were Y-V plasty in 30 cases (85.7%, n=35) and Y-V+double Z plasty in 5 cases (14.3%, n=35). Correction of the telecanthus was performed at the same time by a medial canthal tendon plication in 31 cases (88.6%, n=35) or transnasal canthopexy in 4 cases (11.4%, n=35). The mean age at the time of ptosis surgery was 7.23 years (±6.25), ranging from 8 months to 27 years. Ptosis surgery was performed in 41 cases (79 eyelids), of which 3 patients underwent unilateral ptosis surgery due to asymmetrical ptosis. The techniques used were levator resection in 64 eyelids and frontal suspension in 15 eyelids. CONCLUSION: BPES is often clinically diagnosed. The difficulty in management lies in the complex surgery required. There is no established consensus regarding surgical techniques or the timing of the surgeries.