Freedman H L, Kushner B J
University of Washington School of Medicine, Redmond, USA.
J Pediatr Ophthalmol Strabismus. 1997 Jan-Feb;34(1):10-6. doi: 10.3928/0191-3913-19970101-04.
We have the impression that congenital aberrant innervations are more common than previously reported. Many varieties exist and typically involve the sixth nerve. The most common ocular miswirings are Duane's syndrome and Marcus Gunn jaw-winking ptosis. The second most common miswiring involves lateral rectus activation in upgaze causing a "Y" pattern exotropia (pseudo inferior oblique overaction). This commonly is confused with inferior oblique overaction but surgery on the obliques does not cure the condition. Lateral rectus recession and elevation are required.
We selected demonstrative cases from our practices to illustrate a variety of congenital aberrant innervations. A literature search for previous reported cases of aberrant innervations was performed. This report is an eclectic collection of observations of individual selected cases.
We found aberrant innervations of unusual varieties. These miswirings involve simultaneous firing of the lateral rectus with other muscles, including: the ipsilateral superior rectus, causing "pseudo inferior oblique overaction"; the contralateral lateral rectus, causing synergistic divergence; the ipsilateral superior rectus, in upward saccades only; and the masticatory muscles, causing exotropia with sucking. The third nerve by itself rarely is involved in congenital miswirings, but commonly shows aberrant regeneration after traumatic injuries. We know of no cases of aberrant innervation involving the fourth nerve.
We present cases describing these congenital aberrant innervations and discuss a unifying hypothesis as to their typical involvement of the sixth nerve.
我们感觉先天性异常神经支配比之前报道的更为常见。存在多种类型，通常累及第六神经。最常见的眼部神经错连是杜安综合征和 Marcus Gunn 下颌瞬目性上睑下垂。第二常见的神经错连是上视时外直肌激活导致“Y”型外斜视（假性下斜肌亢进）。这常被误诊为下斜肌亢进，但对斜肌进行手术并不能治愈该病症。需要进行外直肌后徙和上徙术。
我们从临床实践中挑选了具有代表性的病例以说明各种先天性异常神经支配情况。对先前报道的异常神经支配病例进行了文献检索。本报告是对个别精选病例观察结果的综合汇集。
我们发现了不同寻常类型的异常神经支配。这些神经错连包括外直肌与其他肌肉同时放电，其中有：同侧上直肌，导致“假性下斜肌亢进”；对侧外直肌，导致协同性散开；仅在向上扫视时的同侧上直肌；以及咀嚼肌，导致吸吮时出现外斜视。动眼神经本身很少参与先天性神经错连，但在创伤后常出现异常再生。我们尚未知晓涉及滑车神经的异常神经支配病例。
我们展示了描述这些先天性异常神经支配的病例，并讨论了一个关于它们通常累及第六神经的统一假说。
