Levator muscle changes in Marcus Gunn jaw-winking syndrome and other forms of ocular congenital cranial dysinnervation disorder.

IF 1.1 4区 医学 Q3 OPHTHALMOLOGY
Arquivos brasileiros de oftalmologia Pub Date : 2025-04-28 eCollection Date: 2025-01-01 DOI:10.5935/0004-2749.2024-0236
Hind Manaa Alkatan, Nawaf Alkuhaimi, Adel H Alsuhaibani, Azza M Y Maktabi
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引用次数: 0

Abstract

Purpose: This study was conducted to report the histopathological and clinical features of the Marcus Gunn phenomenon and other similar conditions of upper eyelid misfiring.

Methods: This was a retrospective study of patients with congenital ptosis with Marcus Gunn phenomenon who have undergone surgical repair over a period of 12 years and another two patients with upper eyelid misfiring in association with extraocular movements to identify their histopathological findings as subtypes representing ocular congenital cranial dysinnervation disorder.

Results: Among 136 patients with congenital ptosis, 11 (8%) patients with Marcus Gunn phenomenon or misfiring were identified, of whom 9 (6.6%) had typical known Marcus Gunn phenomenon and 2 (1.4%) had eyelid misfiring similar to Marcus Gunn phenomenon. In all patients, the histopathological changes of the excised levator muscle included overall loss and/or atrophy of muscle fibers and irregular-modified Gomori trichrome staining.

Conclusion: The Marcus Gunn phenomenon and similar misfiring conditions with synkinetic extraocular muscle movements share findings that are consistent with the neurogenic type of muscle atrophy. This result suggests a common underlying etiology with variable clinical findings, representing the ocular counterpart of congenital cranial dysinnervation disorder, which has been reported as ocular congenital cranial dysinnervation disorder.

Marcus Gunn眨眼综合征和其他形式的眼部先天性颅神经支配障碍的提上提肌改变。
目的:报道Marcus Gunn现象及其他类似上睑失火的组织病理学和临床特征。方法:回顾性研究先天性上睑下垂伴Marcus Gunn现象的患者,并对另外两例伴有眼外运动的上睑下垂患者进行手术修复,以确定其组织病理学表现为代表眼部先天性颅神经支配障碍的亚型。结果:136例先天性上睑下垂患者中,鉴定出Marcus Gunn现象或不亮11例(8%),其中已知典型Marcus Gunn现象9例(6.6%),与Marcus Gunn现象相似的眼睑不亮2例(1.4%)。在所有患者中,切除提肌的组织病理学改变包括肌纤维的整体丢失和/或萎缩,不规则修改的Gomori三色染色。结论:Marcus Gunn现象和类似的眼外肌运动失燃情况与神经源性肌萎缩类型一致。这一结果提示了一个共同的潜在病因,具有不同的临床表现,代表先天性颅神经失调的眼部对应,这已被报道为眼部先天性颅神经失调。
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来源期刊
CiteScore
1.60
自引率
0.00%
发文量
200
审稿时长
6-12 weeks
期刊介绍: The ABO-ARQUIVOS BRASILEIROS DE OFTALMOLOGIA (ABO, ISSN 0004-2749 - print and ISSN 1678-2925 - (ABO, ISSN 0004-2749 - print and ISSN 1678-2925 - electronic version), the official bimonthly publication of the Brazilian Council of Ophthalmology (CBO), aims to disseminate scientific studies in Ophthalmology, Visual Science and Health public, by promoting research, improvement and updating of professionals related to the field.
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