One Patient with Klippel-Trenaunay Syndrome: Treatment with Charles' Procedure,-One Case Report and Literature Review

以Charles' procedure治療Klippel-Trenaunay症候羣病人－病歷報告及文獻回顧

施秉庚(Pin-Keng Shih) ； 陳信翰(Hsin-Han Chen) ； 許永昌(Yung-Chang Hsu) ； 吳肇毅(Chao-I Wu) ； 張家寧(Sophia Chia-Ning Chang) ； 陳宏基(Hung-Chi Chen)

《臺灣整形外科醫學會雜誌》 21卷2期 (2012/06) Pp. 164-168

https://doi.org/10.7096/TJTSPS.201206.0164

摘要

背景：Klippel-Trenaunay症候羣是一種罕見病因不明的先天疾病，主要以靜脈畸形或靜脈曲張，皮膚毛細血管畸形，肢體軟組織肥大為特徵。Charles' procedure已被廣泛的使用於特定下肢嚴重水腫的病患。把Charles' procedure應用在治療Klippel-Trenaunay症候羣病人肥大的軟組織上文獻上從未被提及。目的和目標：在這份報告中，我們提出一個Klippel-Trenaunay症候羣的病人接受Charles' procedure的過程。臨床病史，手術方法和術後追蹤都在本文中敘述。材料和方法：一位30歲Klippel-Trenaunay症候羣的男性因為右腳趾及趾缝慢性感染、下肢腫脹和疼痛、皮膚萎縮併反覆流血來院求診。由於慢性感染，病人接受Charles' procedure併腳趾截肢，術後病人追蹤長達3年。結果：功能性結果在3年的追蹤期間均令人滿意，儘管有兩次皮膚擦傷引發的皮膚潰瘍但是右下肢的感染沒有再發生過。結論：我們的結果建議Charles' procedure對於Klippel-Trenaunay症候羣的病人可以立即減緩下肢腫大及不對稱且避免反覆的感染，如果後續趾頭反覆的發炎感染則趾頭的截肢有時候還是需要的，病患行走的功能在三年的追蹤是可以接受的。

並列摘要

Background: Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder of unknown etiology characterized by venous malformations or varicose veins, cutaneous capillary malformation and hypertrophy of soft tissues of the limb. Charles' procedure has been used for patients with lower limb advanced lymphoedema in selected cases. There are no literatures talking about Charles' procedure in KTS patients. Aim and objectives: In this report, we present a case of KTS undergoing Charles' procedure. The clinical history, surgical procedure and post-operative follow-up are illustrated in this article.Materials and Methods:A 30-year-old male presented to our hospital with chronic infection of right web and toes, lower limb swelling and pain, and skin atrophy with impending bleeding. The patient underwent Charles' procedure with toes amputation due to chronic infection. Since then, the patient was followed up for 3 years.Results:Functional outcomes in the 3-year follow-up period were satisfactory in spite of two episodes of skin ulceration induced by skin abrasion without any right lower limb cellulitis.Conclusion:This case suggested the Charles' procedure provided KTS patients with immediate volume and circumference reduction and reduced opportunity of skin infection. Toes amputation was necessary if chronic infection over toes were noted. Ambulatory function was acceptable in 3-year follow-up.

並列關鍵字

Charles' procedure ； Klippel-Trenaunay syndrome