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[马库斯·冈恩综合征。3例罕见病例的研究]

[Marcus Gunn Syndrome. Study of 3 rare cases].

作者信息

Benlahbib M, Bencherifa F, Imdary I, El Berdaoui N, Ali H, Bernoussi A, Chefchaouni M C, Berraho A

出版信息

Bull Soc Belge Ophtalmol. 2013(322):125-32.

Abstract

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.

摘要

目的

报告三例罕见的马库斯·冈恩综合征病例,并分析该病症的临床特征。

方法

摩洛哥拉巴特CHU眼科B科室治疗的三例马库斯·冈恩综合征患者的病例报告。

结果

我们的患者为两名1岁和2岁的男孩以及一名31岁患有马库斯·冈恩综合征的女性。特殊之处在于,男孩的病例中该综合征为双侧性,而另一例在成人中与同侧下直肌先天性纤维化临床占主导有关。由于年龄小,未对儿童进行手术治疗。成年患者接受了下直肌后徙和上直肌折叠手术。被动牵拉试验证实下直肌存在纤维化。鉴于残留的眼球运动障碍和暴露性角膜炎风险,该患者未进行上睑下垂和联带运动的手术矫正。

结论

马库斯·冈恩综合征在成人中较为罕见。这可以通过睑退缩随年龄增长而减轻的趋势来解释。马库斯·冈恩综合征双侧受累罕见。其与眼外肌先天性纤维化的关联有助于更好地从病因学角度理解该综合征,突出其核上起源。

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