【双语病例】角膜葡萄肿一例CT影像表现

Description

A 5-year-old girl with blackish mass protruding from the left eye since birth

5岁女孩，出生时即发现左眼突出性黑色肿块。

LegendsAxial plain CT images show an out-pouching on the anterior aspect of the left globe, with enlargement of the anterior chamber. Lens and posterior chamber appear normal. No evidence of intraocular calcifications.

CT平扫示左眼球前房增大，并可见囊袋样向前突出。晶状体及后房未见明显以。眼球内无明显钙化。

(Corneal) Staphyloma

角膜葡萄肿

Background

Staphyloma refers to thinning (ectasia) of the uvea and scleral layers of the globe. It results in enlargement of the globe with a focal bulge, usually in the posterior wall.
葡萄肿指眼球的葡萄膜和巩膜变薄，可引起眼球的局限性增大、凸起。多发生于眼球后壁。
Anterior staphyloma can be the result of an inflammatory/infectious process or secondary to developmental defect, due to faulty differentiation of the anterior mesoderm. The congenital type is rare.发生于眼球前部的葡萄肿一般是感染性病变所致，或由于中胚层分化异常所致的发育不良。先天性葡萄肿临床罕见。

Clinical PresentationPatients usually present with enlarged, protruding, and opacified eye with loss of vision.

患者常表现为眼球增大、突出、呈乳白色，伴有视觉丧失。

Key Diagnostic Feature Imaging is needed to establish the presence or absence of a mass within the enlarged eye. In staphyloma, there is ectatic protruding cornea with focal out-pouching and enlargement of the anterior segment and without an intraocular mass.

影像学检查需明确增大的眼球内是否存在肿块。

葡萄肿一般是角膜膨隆、局限性囊袋样凸出、前房增大，而不伴有眼球内肿块。

DDx

Bupothalmus (secondary to congenital glaucoma)继发于先天性青光眼的Bupothalmus
Axial myopia轴性近视
Coloboma角膜缺损

注：小编未查到Bupothalmus一词的释义，但根据文献，先天性青光眼可有眼压增高所致前房增大，可与本病类似。

Penetrating keratoplasty穿透性角膜成形术
Enucleation (in servere cases)重症患者需摘除眼球

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