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基因型确诊的von Hippel-Lindau病合并视网膜毛细血管瘤的牵引性视网膜脱离的松解性视网膜切除术

Relaxing retinectomy for tractional retinal detachment with retinal capillary hemangiomas in genotypically confirmed von hippel-lindau disease.

作者信息

Peng Chi-Hsien, Cheng Cheng-Kuo, Li Sing-Chung, Mu Shu-Chi, Lee Chin-Cheng

机构信息

From the *Department of Ophthalmology, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan; † School of Medicine, Fu Jen Catholic University, Taipei, Taiwan; ‡Institute of Clinical Medicine and School of Medicine, National Yang-Ming University, Taipei, Taiwan; §Department of Ophthalmology, College of Medicine, National Taiwan University, Taipei, Taiwan; ¶School of Nutrition and Health Sciences, Taipei Medical University, Taipei, Taiwan; and Departments of **Pediatrics ††Pathology, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.

出版信息

Retin Cases Brief Rep. 2010 Fall;4(4):309-11. doi: 10.1097/ICB.0b013e3181af7b0c.

DOI:10.1097/ICB.0b013e3181af7b0c
PMID:25390905
Abstract

PURPOSE

To report the outcome of tractional retinal detachment with retinal capillary hemangiomas after pars plana vitrectomy (PPV) and relaxing retinectomy in genotypically confirmed von Hippel-Lindau disease (VHL).

METHODS

Interventional case report of a 28-year-old patient with multiple and large retinal capillary hemangiomas underwent PPV combined with relaxing retinectomy.

RESULTS

Over the next several weeks, the retina recovered to reattachment and remained this way for 2 years. In excised angiomas, the finding of a missense Arg167Trp mutation and immunohistochemistry staining confirmed the diagnosis of VHL.

CONCLUSION

Patients with tractional retinal detachment with hemangiomas secondary to VHL may benefit from PPV with relaxing retinectomy.

摘要

目的

报告在基因确诊的von Hippel-Lindau病(VHL)患者中,经扁平部玻璃体切除术(PPV)和松解性视网膜切除术治疗牵引性视网膜脱离合并视网膜毛细血管瘤的结果。

方法

对一名28岁患有多个大型视网膜毛细血管瘤的患者进行介入性病例报告,该患者接受了PPV联合松解性视网膜切除术。

结果

在接下来的几周内,视网膜恢复到附着状态,并保持这种状态达2年。在切除的血管瘤中,错义Arg167Trp突变的发现及免疫组织化学染色确诊为VHL。

结论

继发于VHL的牵引性视网膜脱离合并血管瘤患者可能从PPV联合松解性视网膜切除术中获益。

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