平阳霉素碘油乳剂血管内介入治疗Klippel-Trenaunay综合征7例临床分析

Intravascular administration of pingyangmycin lipiodol emulsion for Klippel-Trenaunay syndrome:clinical analysis of 7 cases

目的探讨Klippel-Trenaunay综合征(KTS)的动脉造影表现及以平阳霉素碘油乳剂进行血管内介入治疗的可行性.方法对7例KTS患者行患肢动脉造影,经患肢动脉主干灌注平阳霉素碘油乳剂(平阳霉素8～16 mg,碘油4～6m1)行血管内硬化治疗,观察疗效和术后并发症.结果术前动脉造影显示5例患肢动脉二、三级分支增多,软组织内广泛毛细血管样染色伴混杂血窦显影.术后随访3～30月,5例肢体肿胀明显好转、1例无明显变化、1例复发.并发症主要为血清转氨酶异常、感觉异常和皮肤坏死.结论动脉造影可明确KTS患肢血管和软组织病变,动脉内灌注平阳霉素碘油乳剂硬化治疗对改善患肢肥大具有较好疗效.

Objective To define the arteriographic features of Klippel-Trenaunay sydrome (KTS) and explore the feasibility of the treatment with intravascular administration of pingyangmycin lipiodol emulsion (PLE). Methods Seven patients with KTS underwent arteriography followed by therapy with PLE administration (containing pingyangmycin 8-16 mg and lipiodol 4-6 ml) into the trunk of the arteries of all impaired extremities. The therapeutic effect and postoperative complications were observed. Results Arteriography revealed abundant small branches of the runoff arteries, showing sinusoid changes of varied sizes in 5 cases by staining of the soft tissue. During the 3 to 30-month follow-up, swelling of the extremities was obviously improved in 5 cases after PLE therapy, which failed to produce obvious effect in 1 case and recurrence was detected in another. The major complications included paresthesia of the limbs, serum transaminase disorder and local skin necrosis. Conclusion Arteriography can detect the lesions of the arteries and soft tissues in the affected extremity of patients with KTS, intravascular administration of PLE was effective to alleviate the hypertrophy of the limbs.