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1型神经纤维瘤病中以搏动性眼球突出为表现的蝶骨翼发育异常的多学科治疗:1例罕见病例报告

A multidisciplinary approach to sphenoid wing dysplasia presenting with pulsatile proptosis in neurofibromatosis Type 1: A rare case report.

作者信息

Prathibha S, Parasar Vandana, Yasmin S, Seetha Pramila V V

机构信息

Department of Ophthalmology, Rajarajeswari Medical College and Hospital, Bengaluru, Karnataka, India.

Department of Radiology, Rajarajeswari Medical College and Hospital, Bengaluru, Karnataka, India.

出版信息

Indian J Ophthalmol. 2018 Jan;66(1):157-160. doi: 10.4103/ijo.IJO_429_17.

Abstract

Neurofibromatosis (NF) with sphenoid wing dysplasia is a rare clinical entity. Herewith, we present a case of NF with sphenoid wing dysplasia which presented with pulsatile progressive proptosis. Other ocular symptoms or visual disturbances were absent. Diagnosis of the condition was not easy and the management was a challenging task which needed multidisciplinary approach as there were ocular, neurological, orthopedic, and dermatological manifestations. With neurosurgical intervention, reconstruction of the sphenoid wing was possible. Proptosis was corrected without any disturbance of vision.

摘要

伴有蝶骨翼发育异常的神经纤维瘤病(NF)是一种罕见的临床病症。在此,我们报告一例伴有蝶骨翼发育异常的NF病例,该病例表现为搏动性进行性眼球突出。无其他眼部症状或视觉障碍。该病症的诊断并不容易,且治疗是一项具有挑战性的任务,由于存在眼部、神经、骨科和皮肤科表现,需要多学科方法。通过神经外科干预,蝶骨翼的重建成为可能。眼球突出得以矫正,且视力未受任何影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/39eb/5778558/3f783eea9560/IJO-66-157-g001.jpg

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