婴幼儿牵牛花综合征的临床特征分析

Analysis of clinical features in pediatric morning glory syndrome

目的分析总结婴幼儿牵牛花综合征患者的一般临床特征和眼底表现。方法回顾性分析2006年6月至2010年6月于上海交通大学医学院附属新华医院眼科就诊的牵牛花综合征患儿的临床资料，统计分析患者性别、年龄、眼别及视力等情况。观察指标包括视力检查、眼内压检查、眼前节检查、眼底检查以及屈光度检查等。对病例进行计数并求百分比。结果共有13例（14眼）被诊断为牵牛花综合征。患儿就诊时的年龄为2个月-6岁，平均（30．6＋24．7）个月。其中男性7例（54％），女性6例（46％）；6只（43％）为左眼，8只（57％）为右眼；除1例患者（8％）为双眼发病外，其余12例患者均为单眼发病。能够进行视力检查的8例患者的最佳矫正视力均低于0．1。所有患者眼底表现均非常典型，似盛开的牵牛花。其中有4例（4眼，28％）合并有永存原始玻璃体增生症，9例（9眼，64％）合并有黄斑异位。1例（2眼，14％）合并有视网膜脱离。结论牵牛花综合征是一种严重影响患者视觉功能的先天缺陷综合征。准确及时的诊断和定期随访有利于保存这类患儿的视功能。

Objective To analyze and summarize the clinical features and ocular fundus of pediatric patients with morning glory syndrome. Methods In this retrospective study, the clinical data of pediatric patients with morning glory syndrome, who were treated from June 2006 to June 2010 at the Department of Ophthalmology, Xinhua Hospital, including sex, age, right or left eye and visual acuity, were analyzed. Main outcome measures were best corrected visual acuity （BCVA）, intraocular pressure, ocular fundus and dioptroscopy. Results Thirteen patients （14 eyes）, 7 males （54%） and 6 females （46%）, were diagnosed with morning glory syndrome. They were diagnosed at an average age of （30.6±24.7）months （range from 2 months to 6 years of age）. The syndrome presented in 6 left eyes （43%） and 8 right eyes （57%）. Only one patient presented bilaterally. Visual acuity measurement was possible in 8 patients and their BCVA were all below 0.1. The ocular fundus of all patients＇ was exactly like a blooming morning glory. Four patients （4 eyes, 28%） had persistent hyperplastic primary vitreous, 9 patients （9 eyes, 64%） had heterotopia of the macula, while one patient （2 eyes, 14%） had retinal detachment. Conclusion Morning glory syndrome is a type of congenital deficiency syndrome that leads to severe visual impairment. Accurate and prompt diagnosis and regular follow-up is important for maintaining the visual acuity of these patients.