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眼眶及眶周淀粉样变性:4例报告

Orbital and peri-orbital amyloidosis: a report of four cases.

作者信息

Medel Jiménez Ramón, Sánchez España Juan C, Vasquez Luz M, Tapia Bahamondes Alejandra, Rondón Max, Francesc Treserra, Barroso Eva Ayala

机构信息

a Oculoplastic , Instituto de Microcirugía Ocular (IMO) , Barcelona , Spain.

b Histopathology , Quiron-Deixeus , Barcelona , Spain.

出版信息

Orbit. 2019 Apr;38(2):148-153. doi: 10.1080/01676830.2018.1449868. Epub 2018 Mar 22.

Abstract

Our objective was to describe our experience with orbital amyloidosis, and illustrate the different forms of presentation. This was a retrospective case series of four patients with biopsy-proven orbital amyloi- dosis, over the period from 2014 to 2016. We describe its diagnostic and clinical characteristics, management and systemic study. The series comprised three women and one man of mean age 52 ± 9.4 years. Affected sites were the lacrimal gland, tarsal conjunctiva, lacrimal sac and orbit. In three of the four patients, calcifications were observed. Three patients had associated ptosis. The patient with orbital involvement suffered an unusual vascular complication during surgery and systemic disease was detected. Management included debulking and complete resection of the lesion. In conclusion, orbital amyloidosis presents as a wide variety of forms. Its diagnosis is biopsy-based. Calcifications in biopsy specimens or images should raise suspicion of amyloidosis. It is important to always check for systemic amyloidosis.

摘要

我们的目的是描述我们在眼眶淀粉样变性方面的经验,并阐述其不同的表现形式。这是一项回顾性病例系列研究,涵盖了2014年至2016年期间4例经活检证实为眼眶淀粉样变性的患者。我们描述了其诊断和临床特征、治疗方法及全身检查情况。该系列包括3名女性和1名男性,平均年龄为52±9.4岁。受累部位为泪腺、睑结膜、泪囊和眼眶。4例患者中有3例观察到钙化。3例患者伴有上睑下垂。眼眶受累的患者在手术期间出现了罕见的血管并发症,并检测出全身性疾病。治疗方法包括病变减容和完全切除。总之,眼眶淀粉样变性表现形式多样。其诊断基于活检。活检标本或影像中的钙化应引起对淀粉样变性的怀疑。始终检查是否存在全身性淀粉样变性很重要。

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