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一名3岁儿童的克-特综合征:病例报告

Klippel Trenaunay syndrome in a 3-year-old: A case report.

作者信息

Karmacharya Robin Man, Vaidya Satish, Yadav Binay, Joshi Sahasra, Karkee Diwas, Marasini Sujita, Khadka Sadiksha, Bhatt Swechha

机构信息

Cardiothoracic and Vascular Surgery Unit, Department of Surgery, Kathmandu University School of Medical Sciences, Dhulikhel, Kavre, Nepal.

Cardiothoracic and Vascular Surgery Unit, Department of Surgery, Kathmandu University School of Medical Sciences, Dhulikhel, Kavre, Nepal.

出版信息

Int J Surg Case Rep. 2022 Oct;99:107673. doi: 10.1016/j.ijscr.2022.107673. Epub 2022 Sep 17.

Abstract

INTRODUCTION AND IMPORTANCE

Klippel-Trenaunay syndrome (KTS) is characterized by a triad of port-wine stain, varicose veins and soft tissue or bony hypertrophy of lower limb. Varicose veins in Klippel Trenaunay syndrome are mostly distributed in the lateral aspect of the lower limb. The exact etiology of KTS is not known, and the treatment usually starts with conservative management- limb elevation, compression stockings and physiotherapy. However, some cases are severe enough to warrant surgical management.

CASE PRESENTATION

Here we present a case of a 3-year-old male child with clinical features suggestive of Klippel Trenaunay Syndrome managed successfully with sclerotherapy of persistent lateral marginal vein of servelle. At one month follow-up the vein was sclerosed and there was a significant reduction in varicosities of leg.

CLINICAL DISCUSSION

Starting treatment of varicose veins in Klippel Trenaunay Syndrome in children is effective in preventing long-term complications in adults. Thus, treating venous malformation with sclerotherapy is warranted in early childhood to prevent venous hypertension and chronic venous insufficiency.

CONCLUSION

Varicose veins in KT syndrome can be managed successfully by sclerotherapy of lateral marginal vein of servelle resulting in significant reduction in varicosities of leg.

摘要

引言与重要性

克-特综合征(KTS)的特征为葡萄酒色斑、静脉曲张以及下肢软组织或骨肥大三联征。克-特综合征中的静脉曲张大多分布于下肢外侧。KTS的确切病因尚不清楚,治疗通常始于保守治疗——抬高患肢、穿弹力袜和物理治疗。然而,有些病例严重到需要手术治疗。

病例介绍

在此,我们报告一例3岁男童,其临床特征提示克-特综合征,通过对塞尔维永存外侧缘静脉进行硬化治疗成功治愈。在1个月的随访中,静脉已硬化,腿部静脉曲张明显减轻。

临床讨论

儿童期开始治疗克-特综合征中的静脉曲张,对于预防成人期的长期并发症有效。因此,在儿童早期采用硬化疗法治疗静脉畸形,以预防静脉高压和慢性静脉功能不全是有必要的。

结论

通过对塞尔维外侧缘静脉进行硬化治疗,可成功治疗KT综合征中的静脉曲张,使腿部静脉曲张明显减轻。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8cf3/9568750/3ef75835cbbb/gr1.jpg

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