Klippel-Trenaunay综合征DSA表现及平阳霉素碘油乳剂介入治疗探讨

The DSA appearance and discusssion on interventional therapy with PLE of Klippel-Trenaunay syndrome

目的观察及分析Klippel-Trenaunay综合征(KTS)的DSA表现,以及探索介入治疗的方法及疗效。方法观察5例Klippel-Trenaunay综合征DSA表现,采用导管患肢动脉内平阳霉素碘油乳剂(PLE)灌注的介入治疗,评价疗效。结果DSA5例显示患肢动脉分支增多、增粗,尤其以2、3级动脉分支明显,其中4例可见小或微小动静脉畸形,可见供血分支动脉增粗、纡曲,引流静脉增粗且早期显影,浅静脉增多、增粗和(或)纡曲。4例行PLE动脉内灌注治疗,术后再次造影显示异常血管网部分消失或明显减少,局部碘油沉积。结论患肢DSA可显示KTS部分畸形血管,PLE动脉内灌注治疗KTS有一定的疗效,较全面的治疗及远期疗效有待进一步探讨。

Objective To observe and analyse the DSA appearance of Klippel-Trenaunay syndrome （KTS）, and to search for the method and curative effect of interventional therapy. Method By observing the DSA appearance of 5 KTS cases, applying therapy of infusion of Pingyangmycin-lipiodol emulsion （PLE） by catheter in affected limb artery. Evaluation KTS shows artery branch＇s multiplication and thickening on affected limb, obviously in 2-3 level sub-branch artery （5 cases）, some micro-AVM also can be seen（4 cases）, the blood-supply artery branch becomes large and winding, draining vein becomes large also and visualization in earlier period of contrast agent injection, superficial veins multiple, thicken and wind. Four cases underwent therapy of infusion of PLE by catheter, angiograph shows absent or decrease of abnormal vas- cular net after therapy, lipiodol＇s deposition can be seen locally. Conclusion DSA can desplay some abnormal vessel of KTS, therapy of infusion of PLE by catheter has some curative effect, but full-scale and long-term efficacy need more search.