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光性折射角膜切削术联合丝裂霉素 C 治疗神经纤维瘤病性角膜营养不良

Treatment of lisch corneal dystrophy with photorefractive keratectomy and mitomycin C.

机构信息

Department of Ophthalmology, New York-Presbyterian Hospital, Weill Cornell Medical College of Cornell University, New York, NY, USA.

出版信息

Cornea. 2011 Apr;30(4):481-5. doi: 10.1097/ICO.0b013e3181ec8e26.

Abstract

PURPOSE

To describe a case of Lisch dystrophy; review the clinical, histopathologic, and electron microscopic features of this entity; and discuss a novel treatment approach using photorefractive keratectomy (PRK) and mitomycin C (MMC).

METHODS

A 45-year-old man with a feathery, comet-shaped, right-sided, corneal lesion was treated with excimer laser PRK and 20 seconds of MMC. The uninvolved fellow eye underwent traditional PRK without the use of MMC. Epithelial scrapings were sent for histopathologic analysis.

RESULTS

Histopathologic analysis showed vacuolated cells in the epithelial layer. Electron microscopy revealed empty intracytoplasmic vacuoles, electron-dense whorled inclusions, and reduced tonofilaments. Surface ablation and MMC was successful in treating the initial lesion, with only minimal recurrence noted in the affected eye. Surprisingly, a new asymptomatic lesion was noted in the unaffected eye but dissipated over time.

CONCLUSIONS

Although the whorled inclusions represent a novel finding, the overall clinical and microscopic analysis was consistent with Lisch dystrophy. Surface ablation with MMC should be considered as a treatment option for this disease.

摘要

目的

描述一例神经纤维瘤病性错构瘤;回顾该实体的临床、组织病理学和电子显微镜特征;并讨论使用光折射性角膜切削术(PRK)和丝裂霉素 C(MMC)的新治疗方法。

方法

一名 45 岁男性,右侧角膜出现羽毛状、彗星形、单侧性病变,接受准分子激光 PRK 和 20 秒 MMC 治疗。未受累的对侧眼接受传统的 PRK 治疗,未使用 MMC。刮取上皮细胞进行组织病理学分析。

结果

组织病理学分析显示上皮层有空泡化细胞。电子显微镜显示空的细胞内空泡、电子致密的漩涡状包涵体和减少的张力丝。表面消融和 MMC 成功治疗了初始病变,受累眼仅观察到轻微复发。令人惊讶的是,在未受影响的眼睛中发现了一个新的无症状病变,但随着时间的推移而消散。

结论

尽管漩涡状包涵体是一个新的发现,但总体的临床和显微镜分析与神经纤维瘤病性错构瘤一致。表面消融联合 MMC 应被视为该疾病的一种治疗选择。

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