马库斯·冈恩综合症。[3例罕见病例的研究]。

M Benlahbib, F Bencherifa, I Imdary, N El Berdaoui, H Ali, A Bernoussi, M C Chefchaouni, A Berraho
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引用次数: 0

摘要

目的:报告3例罕见的Marcus Gunn综合征,分析其临床特点。方法:对3例在摩洛哥拉巴特市眼科治疗的Marcus Gunn综合征患者进行病例报告。结果:我们的患者是两名1岁和2岁的男孩和一名31岁的马库斯·冈恩综合征女性。作为特殊的特征,在男孩病例中,这种综合征是双侧的,而在另一个病例中,它与先天性纤维化有关,临床上以成人同侧下直肌为主。由于儿童年龄小,没有提供手术治疗。成人患者受益于下直肌的衰退和上直肌的褶皱。强迫诱导试验显示下直肌纤维化。考虑到残留的眼肌运动障碍和暴露性角膜炎的风险,该患者尚未指出手术矫正上睑下垂和联动性。结论:Marcus Gunn综合征在成人中较为少见。这可以用眼睑收缩随年龄增长而减弱的趋势来解释。Marcus Gunn综合征的双侧病变很少见。它与先天性眼外肌纤维化的关联,使得更好的病因途径强调了该综合征的核上起源。
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[Marcus Gunn Syndrome. Study of 3 rare cases].

Purpose: reporting three rare cases of Marcus Gunn Syndrome and analyzing the clinical features of this entity.

Methods: Case reports of three patients with a Marcus Gunn Syndrome, treated at the Ophthalmology B CHU service Rabat - Morocco.

Results: Our patients are two boys aged 1 and 2 years old, and a 31 year old woman with a Marcus Gunn Syndrome. As special features, in the boys' cases this syndrome is bilateral, as for the other case it is associated with congenital fibrosis clinically predominant of the Ipsilateral inferior rectus muscle in an adult. Surgical treatment was not offered for children because of their young age. The adult patient has benefited from a recession of the inferior rectus muscle and a pleating in the superior rectus muscle. The forced duction test objectified a fibrosis of the inferior rectus muscle. Surgical correction of Ptosis and Synkinesis has not been indicated in this patient, given the residual oculomotor disorder and the risk of exposure keratitis.

Conclusion: Marcus Gunn Syndrome is exceptional in adults. This can be explained by the tendency of the lid retraction to fade with age. Bilateral involvement in Marcus Gunn Syndrome is rare. Its association with congenital fibrosis of extraocular muscles has allowed a better etiopathogenic approach highlighting a supra nuclear origin of this Syndrome.

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