Choi Ki-Hwan, Kyung Sungeun, Oh Sei Yeul
Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
J Pediatr Ophthalmol Strabismus. 2006 Sep-Oct;43(5):285-8. doi: 10.3928/01913913-20060901-03.
To investigate the factors influencing visual development in blepharophimosis-ptosis-epicanthus inversus syndrome.
The study population comprised 20 patients with blepharophimosis-ptosis-epicanthus inversus syndrome who were referred for an oculoplastic opinion. The ocular examination included measurement of Snellen visual acuity, cycloplegic refraction, ocular movements and alignment, and the presence of amblyopia. Patients were treated with spectacle correction, occlusion therapy, strabismus surgery, and oculoplastic surgery if necessary. Minimum follow-up was 2 years.
Nine (45%) patients had amblyopia; 5 (25%) of these patients had unilateral amblyopia and 4 (20%) had bilateral amblyopia. Six (67%) patients with amblyopia had a significant coexisting strabismus. A significant strabismus was present in 11 (55%) patients; 6 (55%) of these patients had unilateral or bilateral amblyopia. Patients with strabismus were more likely to have amblyopia than those without. A total of 14 (70%) patients had refractive error; 6 (43%) of these patients had amblyopia.
Patients with blepharophimosis-ptosis-epicanthus inversus syndrome had a high rate of amblyopia, especially bilateral amblyopia. Patients with strabismus were more likely to have amblyopia than those without. Patients also had a high incidence of refractive errors.
探讨睑裂狭小-上睑下垂-内眦赘皮综合征影响视觉发育的因素。
研究对象为20例因眼部整形问题前来就诊的睑裂狭小-上睑下垂-内眦赘皮综合征患者。眼部检查包括测量斯内伦视力、睫状肌麻痹验光、眼球运动与眼位以及有无弱视。必要时对患者进行眼镜矫正、遮盖疗法、斜视手术和眼部整形手术。最短随访时间为2年。
9例（45%）患者有弱视；其中5例（25%）为单眼弱视，4例（20%）为双眼弱视。6例（67%）弱视患者同时存在明显的斜视。11例（55%）患者有明显斜视；其中6例（55%）有单眼或双眼弱视。有斜视的患者比没有斜视的患者更易患弱视。共有14例（70%）患者有屈光不正；其中6例（43%）有弱视。
睑裂狭小-上睑下垂-内眦赘皮综合征患者弱视发生率高，尤其是双眼弱视。有斜视的患者比没有斜视的患者更易患弱视。患者屈光不正的发生率也很高。
