Surgical Treatment Of Congenital Blepharophimosis-ptosis-epicanthus Inversus Syndrome

ObjectiveTo evaluate the effect of Congenital Blepharophimosis syndrome (BPES) patients with surgical treatment.MethodsThis is a retrospective interventional case series study of8BPES patients (4male patients,4females) who were treated by the Eye Center, Second Affiliated Hospital of Zhejiang University from February2010to February2013. The one-stage intervention combined two different surgical procedures in the following order:YV-epicanthoplasty for the epicanthus, and a bilateral fascia lata sling for ptosis correction.ResultsThe patients were followed up for2months to3years, with an average follow-up of16.4months, the observation criteria include:horizontal lid fissure length (HFL), vertical lid fissure width (VFW), nasal intercanthal distance (ICD), lid margin arc, corneal exposure and symmetry of both eyes. Mean preoperative HFL of the affected eyes was15.6mm(13.0mm-19.0mm). Mean preoperative VFW of the affected eyes was2.6mm(1.0mm-4.0mm).Mean preoperative ICD of the affected eyes was36mm (32.0mm-40.0mm). At the end of the follow-up period, the appearances of those who underwent the surgery obviously improved compared with the preoperative faces. Mean postoperative HFL of the affected eyes was17.6mm (15.0mm-20.0mm). Mean preoperative VFW of the affected eyes was6.9mm (4.0mm-8.0mm).Mean preoperative ICD of the affected eyes was33.1mm (28.0mm-35.0mm). Bilateral palpebral fissure height of all the patients were almost symmetrical and looked natural, function and form of the double eyelid were normal.Conclusionfrontalis suspension combined with the epicanthoplasty is a safe and effective surgical method for the treatment of congenital blepharophimosis syndrome.