CONCLUSION:The two stages management using simple procedures as Y to V medial canthoplasty with MCT plication followed by frontalis suspension or maximum levator resection yielded satisfactory results in the correction of complicated deformity as BPES.
INTRODUCTIONBlepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is an uncommon congenital disorder characterized by a narrowed horizontal palpebral aperture, ptosis, epicanthus inversus, and telecanthus. It typically has bilateral features, although not always symmetrical [1,2] . This syndrome is inherited as an autosomal disease, but also can be manifested as a result of new genetic mutations and sporadic cases without family history of the disease can occur. Several studies confirmed the presence of mutations such as deletion or translocation of the FOXL2 gene, which maps to chromosome 3q21-24 [3,4] . Surgical treatment for BPES is one of the most complex in the field of eyelid surgery due to the presence of multiple deformities. Many surgical techniques have been described to address the epicanthus inversus and accompanying telecanthus including Y to V flaps, the Mustarde technique, the 5-flap technique, medial canthal tendon shortening or plication, and transnasal wiring, and none of them is free from criticism [5][6][7][8][9] . Some authors recommended that medial canthoplasty should be performed first followed by ptosis correction, while others recommended one stage. Therefore, there is no unanimous consensus on BPES correction with respect to either
MATERIALS AND METHODS:Prospective interventional case series which included 15 cases of BPES. Two stages management was used in all cases. The first stage was Y to V medial canthoplasty with MCT plication. 3 months later, ptosis was corrected by bilateral frontalis suspension or maximum levator resection in the more ptotic eyelid in patients with asymmetric ptosis. Data regarding margin reflex distance 1 (MRD1), horizontal palpebral fissure length (HPFL), inner intercanthal distance (IICD), and the ratio of IICD to HPFL were recorded before and after surgery and were statistically analyzed. Post operative IICD/HPFL ratio of 1.5 or less was considered successful outcome. RESULTS: The mean age of patients was 3.7±0.8 years. According to the postoperative IICD/HPFL, 12 patients (80%) had successful outcome. The preoperative IICD had a mean of 37.6±1.5 mm, which was reduced post operative to a mean of 33.0±2.3 mm. The HPFL improved from preoperative mean 21±1.2 mm to postoperative mean 24.2±1.4 mm. The mean preoperative IICD/HPFL ratio was 1.8±0.2 which was reduced post operative to a mean of 1.37±0.2. The MRD1 improved from preoperative mean of 1±0.4 mm to 3.6±0.3 mm post operative. The main complication recorded was undercorrection which was observed in 3 patients (20%).