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先天性遗传性内皮层营养不良的撕囊内皮角膜移植术成功。

Successful Descemet stripping endothelial keratoplasty in congenital hereditary endothelial dystrophy.

机构信息

Cornea and Anterior Segment Services, Sanjivni Eye Care, Model Town, Ambala City, Haryana, India.

出版信息

Cornea. 2011 Mar;30(3):354-6. doi: 10.1097/ICO.0b013e3181e8441a.

DOI:10.1097/ICO.0b013e3181e8441a
PMID:21045677
Abstract

PURPOSE

To report successful Descemet stripping endothelial keratoplasty (DSEK) in a patient with congenital hereditary endothelial dystrophy (CHED).

METHODS

A 19-year-old boy presented with complaints of decreased vision, hazy corneas, and nystagmus since birth. Six months prior, the right eye had undergone penetrating keratoplasty. In the left eye, he was planned for DSEK.

RESULTS

Successful Descemet membrane (DM) scoring and stripping was achieved with a reverse Sinskey hook after trypan blue staining. Manually dissected posterior stromal donor tissue was transplanted using Busin glide. Corneal edema resolved completely with a final best-corrected visual acuity of 20/100 at 6 months. Serial anterior segment optical coherence tomography scans showed maximum decrease in stromal thickness in the first month. Despite resolution of corneal edema, mild stromal haze persisted, which decreased with time. Histopathology of removed DM showed thickened DM and absence of endothelial cells which was suggestive of CHED.

CONCLUSIONS

To the best of our knowledge, this is the first reported case of successful DSEK in a patient with CHED.

摘要

目的

报告 1 例先天性遗传性角膜内皮营养不良(CHED)患者行去表皮角膜内皮移植术(DSEK)成功的病例。

方法

一名 19 岁男性,自出生以来即有视力下降、角膜混浊和眼球震颤的主诉。6 个月前右眼行穿透性角膜移植术。左眼计划行 DSEK。

结果

在眼表面麻醉下行孟加拉玫瑰红染色后,使用反向 Sinskey 钩成功进行了后弹力层(Descemet 膜,DM)划开和撕除。利用 Busin 滑行技术将手动分离的后板层供体组织移植。角膜水肿在 6 个月时完全消退,最终最佳矫正视力为 20/100。连续的眼前节光学相干断层扫描显示,第 1 个月时基质厚度的最大下降。尽管角膜水肿消退,但仍存在轻度的基质混浊,随着时间的推移逐渐减轻。切除的 DM 的组织病理学检查显示 DM 增厚,且无内皮细胞,提示 CHED。

结论

据我们所知,这是首例 CHED 患者行 DSEK 成功的报告。

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