双眼Duane眼球后退综合征31例的临床表现

Clinical features of 31 cases with bilateral Duane′s retraction syndrome

目的观察３１例双眼Ｄｕａｎｅ眼球后退综合征（Ｄｕａｎｅ′ｓｒｅｔｒａｃｔｉｏｎｓｙｎｄｒｏｍｅ，ＤＲＳ）的临床表现，并对其鉴别诊断进行讨论。方法回顾性总结３１例双眼ＤＲＳ，并对其临床表现包括主诉、年龄、性别分布、疾病类型、第一眼位的眼位偏斜、外转及内转受限、内转时眼球后退、眼球急速上转及下转等进行了分析。结果３１例中，男性１４例，女性１７例；男女之比为０．８１。主诉包括眼球运动异常者１４例（４５％），眼位偏斜者１０例（３２％）。本征的最常见类型为Ⅰ型２９例（９４％），余２例为Ⅲ型（６％），其临床表现包括企图内转时眼球后退伴同侧睑裂缩小，常有外转受限伴不同程度的内转受限及患眼内转时出现急速上转和／或下转。结论非典型患者内转时，眼球后退不明显，诊断ＤＲＳ时应与眼球运动异常疾病即外展神经麻痹、Ｍｏｅｂｉｕｓ综合征、先天性眼球运动不能和先天性或婴儿型内斜视相鉴别。

Objectives To summarize the clinical features of 31 cases with bilateral Duane＇s retraction syndrome （DRS） and discuss its differential diagnosis. Methods We retrospectively summ arized 31 cases with bilateral DRS from 1979 to 1996. Its clinical features including chief eomplaints, age and sex distribution, types of presentation, defects in abduction and adduction, retraction of the globe, upshots and downshots in abduction, etc. were analyzed. Results There were 14 males and 17 females with a female-to-male ration 1：0.8. The chief complaints comprised 14 casos （ 45% ） with ocular mottiity disorders and 10 cases （ 32% ） with ocular deviations. The most common form of the syndrome was type Ⅰ（ 29 cases. 94% ）. the rein ain ing 2 cases （ 6% ） with type Ⅲ.Its clinical features consisted of retraction of the globe With narrowing of the palpebral fissure in attern pted adduction, limitation in abduction with variable extent also in adduction, and upshot and/or down shot of the affected eye du ring adduction. Conclusions In atypical cases, the rctraction of th eglobe in adduction was not obvious and the diagnosis of DRS must be differentiatcd from the following ocular motility disorders, namely, abducens nerve palsy, Moebius syndrome, congenital oculomotor aprax ia and congenital or infantile esotropia.