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眼中的日出:马凡综合征的双侧超鼻上晶状体半脱位。

Sunrise in the eye: Bilateral superonasal lens subluxation in Marfan syndrome.

机构信息

Department of Ophthalmology, Moti Lal Nehru Medical College, Prayagraj, India.

出版信息

Rom J Ophthalmol. 2024 Apr-Jun;68(2):170-172. doi: 10.22336/rjo.2024.31.

Abstract

To describe the case of a patient with Marfan syndrome who had bilateral superonasal lens subluxation. The case of a male patient, aged 18, who complained of having impaired vision in both eyes (BE) since he was a toddler, was presented. On examination of the patient, features suggestive of Marfan syndrome were revealed, as well as bilateral intraocular lens subluxation. The patient was refracted and glasses were recommended, which improved his vision. The patient was referred to the cardiology, orthopedic, and dental departments for a multidisciplinary approach to prevent complications and further management. Lens subluxation is frequently presented as a primary clinical manifestation of Marfan syndrome. It can vary from asymptomatic, which is seen only after pupillary dilation, to significant subluxation, in which the equator of the lens in the pupillary axis causes diplopia or decreased vision. This case underscored the importance of considering the rare feature of Marfan syndrome.

摘要

描述一位患有马凡综合征的患者,其患有双侧超鼻上晶状体半脱位。介绍了一位 18 岁男性患者的病例,他从幼儿时期就主诉双眼视力下降。对患者进行检查时,发现有马凡综合征的特征,以及双侧晶状体半脱位。对患者进行了屈光检查并建议佩戴眼镜,这提高了他的视力。患者被转介到心脏科、骨科和牙科部门进行多学科治疗,以预防并发症和进一步管理。晶状体半脱位常作为马凡综合征的主要临床表现出现。它可以从无症状(仅在瞳孔扩张后才能看到)到明显的半脱位,其中晶状体的赤道在瞳孔轴上引起复视或视力下降。这个病例强调了考虑马凡综合征罕见特征的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0fce/11238859/6eb6d3192e5e/RomJOphthalmol-68-170-g001.jpg

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