Kowal Lionel, Yahalom Claudia, Shuey Neil H
Royal Victorian Eye & Ear Hospital and Centre for Eye Research Australia, 19 Simpson Street, East Melbourne, Victoria 3002, Australia.
Binocul Vis Strabismus Q. 2006;21(1):18-26.
Strabismus has been previously reported as a rare presenting feature of the Type 1 Chiari malformation.
We report a case series of twelve patients with Chiari 1 malformations with either strabismus or diplopia as part of their initial presentation. Ten patients had diplopia at the time of presentation, while 2 young children (ages 2 and 6) presented with esotropia without complaints of diplopia. Of the 10 patients with diplopia, 7 were constantly or frequently tropic while 3 had symptomatic phorias. One or more unusual features of the strabismus led to further investigations and the diagnosis of Chiari in these patients. The most common oculomotor disturbance was a comitant esotropia. Most patients were managed with prism glasses. One patient had strabismus surgery as primary treatment with early orthotropia. Three patients underwent neurosurgical decompression, with minimal improvement of their strabismus; one of these underwent subsequent successful strabismus surgery.
Chiari 1 malformation may present with strabismus or diplopia as the major finding. Associated neurological features may be nonspecific (e.g., headache), subtle (e.g., gaze-evoked nystagmus), or delayed. Although neurosurgery may be required in some cases, primary strabismus management (surgical or prismatic correction) can be successful, particularly when strabismus is the lone (or sole specific) finding.
斜视先前已被报道为1型Chiari畸形的一种罕见表现特征。
我们报告了一组12例Chiari 1畸形患者的病例系列，其初始表现包括斜视或复视。10例患者在就诊时有复视，而2名幼儿（2岁和6岁）表现为内斜视，无复视主诉。在10例有复视的患者中，7例持续或频繁斜视，3例有症状性隐斜视。斜视的一个或多个不寻常特征导致对这些患者进行进一步检查并诊断为Chiari畸形。最常见的眼球运动障碍是共同性内斜视。大多数患者采用棱镜眼镜治疗。1例患者以斜视手术作为主要治疗方法，早期获得正位。3例患者接受了神经外科减压手术，斜视改善甚微；其中1例随后成功接受了斜视手术。
Chiari 1畸形可能以斜视或复视为主要表现。相关的神经学特征可能不具有特异性（如头痛）、不明显（如凝视诱发的眼球震颤）或出现较晚。虽然在某些情况下可能需要神经外科手术，但原发性斜视治疗（手术或棱镜矫正）可能会成功，尤其是当斜视是唯一（或唯一的特定）表现时。
