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迟发性脊椎骨骺发育不良的角膜改变

Corneal changes in spondyloepiphyseal dysplasia tarda.

作者信息

Hirata Y, Watanabe H, Maeda N, Inoue Y, Shimomura Y, Tano Y

机构信息

Department of Ophthalmology, Osaka University Medical School, Suita, Japan.

出版信息

Jpn J Ophthalmol. 2000 Jan-Feb;44(1):29-32. doi: 10.1016/s0021-5155(99)00146-x.

Abstract

BACKGROUND

A new type of corneal opacity with prominent corneal nerve fibers as an ocular complication of spondyloepiphyseal dysplasia tarda (SEDT).

CASE

A 58-year-old woman, diagnosed with SEDT at 5 years of age, underwent a complete ophthalmological examination.

OBSERVATIONS

The patient had no complaints and no history of eye disease. No relatives were reported to have suffered from SEDT. Slit-lamp examination disclosed a diffuse opacity in the central cornea in both eyes, which was localized in the middle to deep stroma. Dot opacities in the central and paracentral cornea were located in the middle of the stroma in both eyes. Optically clear regions were observed in the peripheral cornea of both eyes. More interestingly, corneal nerve fibers were visible passing from the limbus to the central cornea in both eyes.

RESULTS

The etiology of the corneal opacities of this patient with SEDT is unknown. However, collagen and proteoglycan abnormalities in the skin of patients with SEDT have been reported. Therefore, such abnormalities may also be present in the cornea and these alterations may lead to corneal complications.

摘要

背景

迟发性脊椎骨骺发育不良(SEDT)的一种眼部并发症是出现一种新型角膜混浊且伴有明显的角膜神经纤维。

病例

一名58岁女性,5岁时被诊断为SEDT,接受了全面的眼科检查。

观察结果

患者无眼部不适且无眼部疾病史。未报告有亲属患SEDT。裂隙灯检查发现双眼中央角膜有弥漫性混浊,位于基质中深层。双眼中央和旁中央角膜的点状混浊位于基质中部。双眼周边角膜可见光学透明区。更有趣的是,双眼均可见角膜神经纤维从角膜缘向中央角膜走行。

结果

该SEDT患者角膜混浊的病因不明。然而,已有报道称SEDT患者皮肤中的胶原蛋白和蛋白聚糖存在异常。因此,角膜中可能也存在此类异常,且这些改变可能导致角膜并发症。

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