Congenital Fibrosis of the Extraocular Muscles: a Rare Entity
JJK Ip, MM Marcet, KW Yan, WWM Lam, MT Chau
Hong Kong J Radiol 2013;16:236-40
DOI: 10.12809/hkjr1311071
Congenital restriction of ocular movements could be due to various abnormalities. One of the rarest causes is fibrosis of the extraocular muscles. Congenital fibrosis of the extraocular muscles is a congenital non-progressive strabismus syndrome, characterised by diffusely infiltrative orbital lesions with secondary involvement of extraocular muscles, resulting in variable restrictive external ophthalmoplegia due to the cicatricial process. Congenital fibrosis of the extraocular muscles can be unilateral or bilateral and affect any or all of the muscles, and the degree of fibrosis of a muscle can be total or partial. We report on a patient with congenital fibrosis of the extraocular muscles, and review the clinical presentation, magnetic resonance imaging features, and correlation with intraoperative and histopathological findings. A brief literature review is also provided.
中文摘要
先天性眼外肌纖維化：一種罕見病症
葉精勤、MM Marcet、殷堅榮、林慧文、周明德
先天性眼球運動受限的病因多樣化，最罕見的原因之一是眼外肌纖維化。眼外肌纖維化是一種先天、非進行性斜視症，特徵為眶內瀰漫性浸潤性病變並累及眼外肌，因瘢痕修復而導致不同程度的限制性眼外肌麻痺。先天性眼外肌纖維化可以是單側或雙側，亦可影響任何一條或所有肌肉，而單條肌肉纖維化的程度可以是全部或部份。本文報告一個先天性眼外肌纖維化的病例，並回顧患者的臨床症狀、磁共振成像特徵，以及與術中發現及病理結果的相關性。此外，亦作一簡短的文獻回顧。