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原发性眼眶嗜酸性血管中心性纤维化为鼻腔内延伸型。

Primary orbital eosinophilic angiocentric fibrosis with intranasal extension.

机构信息

Department of Otorhinolaryngology, University of Sassari, Sassari, Italy.

出版信息

Head Neck. 2014 Jan;36(1):E8-E11. doi: 10.1002/hed.23396. Epub 2013 Sep 11.

DOI:10.1002/hed.23396
PMID:23733241
Abstract

BACKGROUND

Eosinophilic angiocentric fibrosis is a chronic, idiopathic disorder that usually involves the upper respiratory tract and features progressive submucosal perivascular fibrosis of unknown etiology. To our knowledge, only 5 cases of eosinophilic angiocentric fibrosis with primary orbital involvement have been reported.

METHODS AND RESULTS

We report the case of a 46-year-old man with right proptosis and lateral globe displacement caused by a primary eosinophilic angiocentric fibrosis extending from the orbit into the anterior ethmoid. The nasal extension of the lesion helped in establishing the correct diagnosis.

CONCLUSION

Physicians involved in the treatment of orbital pathologies should be familiar with this entity, because it may manifest as an intraorbital mass growing primarily or secondly into the orbit. The clinical manifestations of eosinophilic angiocentric fibrosis with orbital involvement often mimic other more common ophthalmological diseases. Biopsies are necessary for diagnosis and treatment planning, although cures are usually of palliative effect.

摘要

背景

嗜酸性血管中心性纤维化为一种慢性、特发性疾病,通常累及上呼吸道,其特征为病因不明的进行性黏膜下血管周围纤维化。据我们所知,仅有 5 例原发性眼眶受累的嗜酸性血管中心性纤维化病例报告。

方法和结果

我们报告了 1 例 46 岁男性病例,其右侧眼球突出伴外侧眼球移位,由原发于眼眶并延伸至前筛窦的嗜酸性血管中心性纤维化引起。病变的鼻腔延伸有助于确立正确的诊断。

结论

参与治疗眼眶病变的医生应熟悉该疾病,因为它可能表现为主要或次要生长至眼眶的眶内肿块。伴有眼眶受累的嗜酸性血管中心性纤维化的临床表现常类似于其他更为常见的眼科疾病。活检对于诊断和治疗计划是必要的,尽管通常疗效为姑息性。

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