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眼部神经纤维瘤病

Ocular Neurofibromatosis.

作者信息

Alkhairy Saba, Baig Mahad M

机构信息

Ophthalmology, Dow International Medical College, Dow University of Health Sciences, Karachi, PAK.

出版信息

Cureus. 2021 Sep 6;13(9):e17765. doi: 10.7759/cureus.17765. eCollection 2021 Sep.

Abstract

A 14-year-old boy presented with a right orbital lid mass, which had slowly grown over the last 4.5 years, as well as some impaired visual acuity in the affected (right) eye. We assessed the patient by taking a detailed history and physical examination. A Snellen chart was used to assess visual acuity, which revealed decreased acuity in the right eye as compared to the left eye. Pupillary reactions, including relative afferent pupillary reflexes, were unremarkable; anterior and posterior chamber assessment was normal including that of the optic disc and macula. Additionally, the intraocular pressure was within acceptable limits. The mass was excised surgically as it had caused significant disfigurement and posed risk to the patient in terms of the possibility for the lesion to increase in severity. It was an approach utilizing a blepharoplasty incision, horizontal wedge resection, and a frontalis sling done under general anesthesia. A biopsy of the mass identified it as a plexiform lesion of the orbit such as that attributed to neurofibromatosis type 1.

摘要

一名14岁男孩出现右侧眶睑肿物,该肿物在过去4.5年中缓慢生长,同时患眼(右眼)视力也有所下降。我们通过详细询问病史和体格检查对患者进行了评估。使用斯内伦视力表评估视力,结果显示右眼视力低于左眼。瞳孔反应,包括相对传入瞳孔反射,均无异常;前房和后房评估正常,包括视盘和黄斑。此外,眼压在可接受范围内。由于该肿物已导致明显毁容,且病变有加重的可能性,对患者构成风险,因此通过手术切除。手术采用睑成形术切口、水平楔形切除术,并在全身麻醉下进行额肌悬吊术。对肿物进行活检后确定其为眼眶的丛状病变,如1型神经纤维瘤病所致。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/564c/8494155/de8aa54ab9c1/cureus-0013-00000017765-i01.jpg

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