Department of Radiology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea.
Ophthalmology. 2010 Nov;117(11):2208-13.e1-2. doi: 10.1016/j.ophtha.2010.02.017. Epub 2010 Jun 8.
Congenital cranial dysinnervation disorders (CCDDs) are developmental abnormalities involving the hypoplasia or aplasia of the cranial nerves with muscle dysinnervation. This study aimed to determine if congenital superior oblique palsy with superior oblique hypoplasia also can be classified as a CCDD.
Observational case series.
Ten patients with superior oblique hypoplasia.
Ophthalmologic examination and thin-section magnetic resonance imaging (MRI) at the brainstem level as well as across the orbit were performed in 10 patients with superior oblique hypoplasia. To confirm the accuracy of the procedure, the results obtained were compared with those of a control group comprising 12 children by using the same technique.
Examination of ductions and versions, 3-step test, angle of deviation, and presence of the trochlear nerve and superior oblique hypoplasia on MRI.
Trochlear nerve was absent on the affected side in 10 patients with confirmed hypoplastic superior oblique muscles. The trochlear nerves as well as normal-sized superior oblique muscles were observed in 24 (100%) of 24 eyes screened as controls.
Congenital superior oblique palsy with superior oblique hypoplasia also can be classified as a CCDD by the MRI documentation of congenital aplasia of the trochlear nerve.
FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
先天性颅神经发育不良（CCDD）是一种发育异常，涉及颅神经的发育不良或发育不全，伴有肌肉神经支配异常。本研究旨在确定先天性上斜肌麻痹伴上斜肌发育不良是否也可归类为 CCDD。
观察性病例系列研究。
10 例上斜肌发育不良患者。
对 10 例上斜肌发育不良患者进行眼科检查和脑桥水平及眼眶内薄层磁共振成像（MRI）检查。为了确认该程序的准确性，使用相同的技术将获得的结果与包括 12 名儿童在内的对照组的结果进行比较。
扫视和上下转运动检查、3 步试验、偏斜角以及 MRI 上滑车神经和上斜肌发育不良的存在。
10 例确诊的上斜肌发育不良患者中，患侧滑车神经缺失。24 例（100%）作为对照组筛查的眼均观察到滑车神经和正常大小的上斜肌。
通过 MRI 记录先天性滑车神经发育不全，可以将先天性上斜肌麻痹伴上斜肌发育不良归类为 CCDD。
作者没有与本文讨论的任何材料有关的专有或商业利益。
