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Clinical and Radiological Features of Intramuscular Orbital Amyloidosis: A Case Series and Literature Review
Ophthalmic Plastic and Reconstructive Surgery ( IF 2 ) Pub Date : 2022-05-01 , DOI: 10.1097/iop.0000000000002061
Jessica Y Tong 1 , Valerie Juniat 2 , Penelope A McKelvie 3 , Brett A O'Donnell 4 , Thomas G Hardy 5 , Alan A McNab 5 , Dinesh Selva 2
Affiliation  

Purpose: 

Orbital amyloidosis of the extraocular muscles (EOMs) is a rare condition, and its clinicoradiological features are not well elucidated. This study describes the characteristic clinical signs, MRI features, and potential treatment options.

Methods: 

Retrospective multicenter case series and literature review of EOM amyloidosis.

Results: 

Five cases were identified for inclusion. Common clinical findings were diplopia, ophthalmoplegia, and proptosis. Systemic amyloidosis was more likely to present with multiple muscle involvement, but no particular pattern was observed with localized disease. On MRI, amyloid deposition was characterized as a heterogeneous intramuscular mass with T2 hypointensity and post contrast enhancement. Management is dependent on the extent of disease and functional impairment; options include surgical debulking and radiation therapy.

Conclusion: 

EOM amyloidosis is uncommon. The combination of clinical and radiologic findings described in this study should lead to its clinical suspicion.



中文翻译:

肌内眶淀粉样变性的临床和放射学特征:病例系列和文献回顾

目的: 

眼外肌(EOMs)的眼眶淀粉样变性是一种罕见的疾病,其临床放射学特征尚不清楚。本研究描述了典型的临床体征、MRI 特征和潜在的治疗方案。

方法: 

EOM 淀粉样变性的回顾性多中心病例系列和文献回顾。

结果: 

五个案例被确定纳入。常见的临床表现是复视、眼肌麻痹和眼球突出。系统性淀粉样变性更可能表现为多处肌肉受累,但未观察到局部疾病的特殊模式。在 MRI 上,淀粉样蛋白沉积被表征为具有 T2 低信号和对比增强后的不均匀肌内肿块。管理取决于疾病和功能障碍的程度;选项包括手术减瘤和放射治疗。

结论: 

EOM 淀粉样变性并不常见。本研究中描述的临床和放射学发现的组合应导致其临床怀疑。

更新日期:2022-05-01
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