Klippel-Trenaunay综合征的动脉造影表现和介入治疗

Klippel-Trenaunay syndrome: the angiographic manifestations and endovascular treatment with pingyangmycin-lipiodol emulsion

目的 观察K1ippel-TrenaunTdy综合征(KTS)的动脉造影表现并采用新的介入方法对其进行治疗。方法 观察7例KTS患肢动脉DSA的影像表现。采用经导管患肢动脉内注射平阳霉素碘油乳剂(PLE,平阳霉素用量6-12 mg,超乳化碘油4-8 m1)治疗。观察术后疗效、副反应和并发症。结果 动脉造影显示患肢少数动脉2、3级分支增粗,软组织内可见部分斑片状染色伴大小不等血窦显影(5例),引流静脉增粗(4例)和浅静脉迂曲扩张(5例)。病变区碘油沉积良好者6例,1例碘油沉积欠佳。术后随访13-30个月,肢体肥大明显回缩者6例,大腿的周长接近正常肢体,1例病情无明显变化。1例于1年后局部轻度复发。主要术后反应:均有肢体中至重度肿胀和轻度发热,另外有一过性血清转氨酶升高2例和肢端麻木1例。并发症为局限性皮肤坏死1例,经治疗痊愈;另1例出现患侧足拇指背屈无力,治疗无效。结论 患肢动脉造影可以显示KTS部分畸形血管。PLE动脉内灌注治疗KTS引起的患肢肥大具有较好疗效。由于本疗法可能有较严重的副反应和并发症,应慎用。

Objective To observe the angiographic manifestations of Klippel-Trenaunay syndrome (KTS) and to treat it by intra-arterial injection of pingyangmycin-lipiodol emulsion (PLE) . Methods Seven youngpatients (age range 12-19 years, mean 15.2 years) with KTS in the single low limb were examined by arteriography. Then, PLE (mixed with pingyangmycin 6-12 mg, lipiodol 4-8 ml) was injected by transcatheter into the femoral artery. The effects, side-effects, and complications of the therapy were observed. Results The arteriography revealed a few distended small arteries with staining of venous sinus of different size in the soft tissue (5/7 ) , as well as drainage vein enlargement (4/7 ) and superficial varicose vein (5/7). PLE deposited visibly in the abnormal sinus except one case. During 13-30 months' follow-up,6 cases had good effects on limb hypertrophy after the treatment,and the limbs with lesions were obviously shrank and the thigh circumference became near to the normal limb. Another case had no obvious change. One had mild recurring around the knee one year later. The major side-effects included medium to extreme swelling of the limbs (7/7 ) , serum transaminase elevation (2/7 ) , and numbness of the distal end of the limb ( 1/7). The complications included a small piece of skin necrosis (1/7 ) and the first toe-drop (1/ 7). Conclusion The arteriography in KTS can demonstrate a part of vascular malformations. Transcatheter intra-arterial PLE injection was effective in treating the hypertrophy of the limb caused by KTS. Because the therapy could result in some serious side-effects and complications, it should be used carefully.