Kobus Kazimierz, Wójcicki Piotr, Wysocki Mariusz
Z Kliniki Chirurgii Plastycznej Akademii Medycznej we Wrocławiu.
Klin Oczna. 2008;110(4-6):166-71.
The orbitoblepharophimosis syndrome is an autosomal-dominant congenital malformation involving the orbitopalpebral region. The condition is associated with orbital phimosis, upper eyelid ptosis, epicanthus and telecanthus.
We retrospectively reviewed the records of 60 patients with blepharophimosis who underwent surgery in the Hospital of Plastic Surgery in Polanica Zdrój from January 1975 to January 2006. The study population consisted of 23 (38.3%) females and 37 (61.7%) males patients whose age at the time of first surgical stage ranged from 1 to 48 years (mean 8 years). Surgical management consisted of epicanthus correction using modified technique of Mustardé followed by eyelid suspension to the frontalis muscle with using temporal fascia. In patients with orbitoblepharophimosis type II, correction of the lower lid ectropion and antimongoid slanting palpebral fissures, were performed before or after Mustardé procedure.
In our series of 60 patients, good result of treatment was obtained in 80.1% and satisfactory in 18.3% of patients. Poor outcome was obtained only in one patient with orbitoblepharophimosis type II.
Orbitoblepharophimosis management should consists of gradual and complex surgical treatment. During the first stage epicanthus correction is performed. As a second stage, blepharoptosis is treated by frontalis suspension technique with using temporal fascia.
眼眶睑裂狭小综合征是一种常染色体显性遗传性先天性畸形，累及眼眶睑裂区域。该病症与眼眶狭窄、上睑下垂、内眦赘皮和睑裂增宽有关。
我们回顾性分析了1975年1月至2006年1月在波兰尼察兹德鲁伊整形外科医院接受手术的60例睑裂狭小患者的病历。研究对象包括23例（38.3%）女性和37例（61.7%）男性患者，首次手术时年龄在1至48岁之间（平均8岁）。手术治疗包括采用改良的Mustardé技术矫正内眦赘皮，然后使用颞筋膜将眼睑悬吊于额肌。对于II型眼眶睑裂狭小患者，在Mustardé手术之前或之后进行下睑外翻和睑裂反蒙古样倾斜的矫正。
在我们的60例患者系列中，80.1%的患者治疗效果良好，18.3%的患者效果满意。仅1例II型眼眶睑裂狭小患者治疗效果不佳。
眼眶睑裂狭小的治疗应包括逐步的综合手术治疗。第一阶段进行内眦赘皮矫正。第二阶段，采用颞筋膜额肌悬吊技术治疗上睑下垂。
