Duane眼球后退综合征201例的临床特征

Clinical features of 201 cases with Duanes retraction syndrome

目的总结２０１例Ｄｕａｎｅ眼球后退综合征（Ｄｕａｎｅ′ｓｒｅｔｒａｃｔｉｏｎｓｙｎｄｒｏｍｅ，ＤＲＳ）的临床特征，并讨论其鉴别诊断。方法自１９７９～１９９６年回顾性地总结了２０１例ＤＲＳ，对它的临床特征，包括主诉、性别分布、初诊年龄、侧向状态、表现类型、原在位的眼位偏斜、屈光不正、弱视、眼球后退、睑裂改变、内转时眼球急速上转和下转、双眼单视及其伴随的眼部和非眼部异常等予以分析。结果２０１例患者中，男９９例，女１０２例，男女之比为１∶１。６５８８％ＤＲＳ左眼受累，以２∶１之比，左眼易患病。本综合征最常见类型为Ⅰ型（１８４例，占９１５４％）。原在位最常见的眼位偏斜为外斜（７２例，占３５８２％）。在１１８例中，多数表现外转受限、内转时眼球后退和面转以保持双眼单视。最常见到的眼部异常为鳄鱼泪（２６例，占１２９３％）。结论典型的ＤＲＳ诊断并不困难，但是儿童患有双眼外转受限类似ＤＲＳ者，应与下列４种眼球运动异常鉴别，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。

Objective To summarize the clinical features of 201 cases with Duanes retraction syndrome (DRS) and discuss its differential diagnosis Methods The 201 cases in 19791996 were retrospectively summarized The clinical features including chief complaints, sex distribution, age at first visit, laterality, type of presentation, ocular deviation in the primary position, refractive errors, amblyopia, globe retraction, change of the palpebral fissure, upshoot and downshoot in adduction, binocular single vision, and its associated ocular and nonocular anomalies were analyzed Results There were 99 males and 102 females with a femaletomale ratio of 116588% of DRS cases had left eye involvement with twotoone predilection for the left eye The most common form of the syndrome was type 1 (184 patients, 9154%) Exotropia was the most common deviation in the primary gaze (72 patients, 3582%) Among 118 patients, most had abduction deficits, globe retraction in adduction, and faceturn for maintaining single binocular vision Crocodile tears (26 patients, 1293%) were the most frequently encountered ocular abnormality Conclusions Diagnosis of DRS in a typical case is not difficult, however, children with bilateral abduction deficits which may mimic DRS must be differentiated from the following four motility disorders, namely, abducens nerve palsy, Moebius syndrome, congenital oculomotor apraxia, and congenital or infantile esotropia