两阶段斜视手术治疗罕见的单侧Möbius综合征的成功结果:1例报告。

Beyoglu Eye Journal Pub Date : 2025-03-25 eCollection Date: 2025-01-01 DOI:10.14744/bej.2024.71601
Anupam Singh, Srishti Sharma, Umesh Yadav, Pramod Kumar Pandey, Barun Kumar
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引用次数: 0

摘要

Möbius综合征(MBS)是一种罕见的先天性疾病,以面部和外展神经麻痹为特征,通常是双侧的,可能涉及多个颅神经。其他特征,如颅面异常,舌畸形和肢体缺陷,也可能存在。这种综合征的病因尚不清楚,但脑干缺血性坏死导致脑神经核发育不全被认为是最可能的原因。MBS导致大角度内斜视,伴双侧外展受限,诊断延迟,单次手术矫正非常具有挑战性。关于MBS中斜视的手术结果只有少数报道。在这个病例报告中,我们描述了一个不寻常的单侧MBS病例,他从小就表现为大内斜视,并通过两阶段斜视手术进行治疗,包括单侧内侧直肌(MR)肌肉萎缩和垂直直肌转位。最后的主要位置对准是令人满意的。因此,MR肌肉收缩是MBS患者轻度至中度内斜视的有效首选手术,而严重的病例可能进一步需要垂直直肌移位。由于弱视是一种先天性疾病,早期手术干预可以预防弱视,并为双眼单眼视力的发展提供机会。因此,在这些病例中,及时诊断和早期手术干预是至关重要的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Successful Outcome of Two-Stage Strabismus Surgery in a Rare Case of Unilateral Möbius Syndrome: A Case Report.

Möbius syndrome (MBS) is a rare congenital disorder characterized by facial and abducens palsy which is usually bilateral and may involve multiple cranial nerves. Additional features, such as craniofacial anomalies, tongue malformations, and limb defects, may also be present. The etiology of this syndrome is not well established but the ischemic necrosis of the brainstem leading to hypoplasia of the cranial nerve nuclei is the supposed to be most likely one. MBS leads to a large angle esotropia along with bilateral limitation of abduction with delayed diagnosis, which is very challenging to correct with a single procedure. There are only a few reports illustrating the surgical outcome of strabismus in MBS. In this case report, we describe an unusual case of unilateral MBS who presented with large esotropia since childhood and was managed by two-stage strabismus surgery, including unilateral medial rectus (MR) muscle recession followed by vertical recti transposition. The final primary position alignment was satisfactory. Therefore, MR muscle recession alone is an effective first procedure of choice for mild-to-moderate esotropia in patients with MBS, whereas severe cases may further require the transposition of vertical recti. As it is a congenital condition, early surgical intervention may prevent amblyopia and provide an opportunity for the development of binocular single vision. Hence, prompt diagnosis and early surgical intervention are of utmost importance in these cases.

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