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首页> 外文期刊>Journal of AAPOS: The official publication of the American Association for Pediatric Ophthalmology and Strabismus >Possible association of congenital Brown syndrome with congenital cranial dysinnervation disorders
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Possible association of congenital Brown syndrome with congenital cranial dysinnervation disorders

机译:先天性布朗综合征与先天性颅神经失调症的可能联系

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Background: Congenital cranial dysinnervation disorders (CCDDs) are known to arise from abnormal development of individual and multiple cranial nerve nuclei or abnormalities in cranial nerve axonal transport. We report our findings for several patients with Brown syndrome in association with other known abnormalities characteristic of CCDDs. Methods: The medical records of patients presenting during a 4-year period with congenital Brown syndrome were retrospectively reviewed. Patients with Brown syndrome confirmed by forced ductions were included in the study if the Brown syndrome was associated with either an abnormal development of the superior oblique muscle or superior oblique paresis, ptosis, Duane syndrome, or other known CCDDs. Results: A total of 9 patients with Brown syndrome were identified. Of these, 3 also demonstrated a contralateral superior oblique palsy; 2, a contralateral Duane syndrome; 1, an ipsilateral congenital ptosis; and 3, a moderate to severely hypoplastic ipsilateral superior oblique muscle. Conclusions: Some patients with congenital Brown syndrome are associated with and possibly in the spectrum of CCDDs. We propose that Brown syndrome may be due to abnormal development of the trochlear nerve, which results in physical changes in the superior oblique muscle-tendon-trochlea complex. This results in a tendon that is either long and lax, absent, or abnormally inserted (ie, superior oblique paresis) or a tendon that is restricted in its movements through the trochlea (Brown syndrome).
机译:背景:先天性颅神经失调症(CCDD)已知是由于单个和多个颅神经核的异常发育或颅神经轴突运输异常引起的。我们报告了几例患有布朗综合征的患者与CCDDs的其他已知异常特征相关的发现。方法:回顾性分析4年来先天性布朗综合征患者的病历。如果布朗综合征与上斜肌或上斜肌异常发育,上睑下垂,杜安综合征或其他已知的CCDD有关,则通过强迫引诱证实的布朗综合征患者被纳入研究。结果:共鉴定出9例布朗综合征患者。其中3例还显示出对侧上斜性麻痹。 2,对侧杜安综合征; 1,同侧先天性上睑下垂; 3,中度至重度发育不良的同侧上斜肌。结论:一些先天性布朗综合征患者与CCDD有关,可能与CCDD有关。我们提出布朗综合征可能是由于滑车神经发育异常,导致上斜肌-肌腱-滑车复合体的物理改变。这会导致肌腱长而松弛,缺失或不正常插入(即上斜肌麻痹)或肌腱通过滑车运动受限(布朗综合征)。

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