青少年布加综合征临床特点及介入治疗

Budd-Chiari syndrome in youth： clinical features and interventional therapy

目的 探讨青少年布加综合征（Budd-Chiari syndrome,BCS）临床特点并评估介入治疗的疗效.方法 徐州医学院附属医院自1990年1月至2012年4月收治227例年龄在29岁以下的BCS患者,均经彩超及血管造影证实,其中下腔静脉型87例、肝静脉型105例、混合型35例.通过经皮血管腔内血管成形术（percutaneous transluminal angioplasty,PTA）、血管内支架置入术及置管溶栓术开通闭塞血管.术后给予抗凝治疗、定期随访.结果 227例患者均以门脉高压的症状和体征为最初临床表现.210例患者初次介入手术取得成功,其中下腔静脉阻塞型成功率100％,肝静脉阻塞型85.7％,混合型94.3％.介入治疗成功后的下腔静脉平均压力由术前的（26.52±8.16） cm H2O下降至术后（14.28 ±4.08） cm H2O（P＜0.05）.肝静脉平均压力由术前（35.70±13.26） cm H2O下降至术后（18.36±8.16） cm H2O（P＜0.05）.术后随访1个月至15年,平均（46±37）个月.再狭窄发生率为21.4％（45/210）,其中下腔静脉型狭窄率为13.8％（12/87）,肝静脉阻塞型31.1％ （28/90）,混合型15.2％ （5/33）,肝静脉型患者再狭窄发生率明显高于其他两型.再狭窄患者介入治疗方法同初次治疗,44例再狭窄患者再次介入治疗取得成功.结论 青少年布加综合征患者以肝静脉阻塞型最多见,门脉高压症状和体征为主要临床表现特点,肝静脉型介入治疗后复发率高于其他两型.

Objective To present the clinical features and to evaluate interventional therapy for Budd-Chiari syndrome in Chinese youth. Methods From January 1990 to April 2012, 227 cases who hospitalized at the age 〈 29 underwent color Doppler ultrasound scan and digital subtraction angiography （DSA）. 87 cases were with occlusive inferior vena eava （ IVC type） , 105 cases with occlusive hepatic veins （HV type） and 35 cases with occlusive inferior vena cava and hepatic veins （MIX type）. The occlusive veins were opened by pereutaneous transluminal angioplasty （PTA）, endovascular stent placement, intravenous catheter thrombolysis or combination. Postoperative antieoagulation was given to all patients. Results The symptoms and signs of portal hypertension disappeared or were alleviated in successful cases. Technical success was achieved in 210 patients. The success rate was 100% in IVC type, 85.7% in HV type and 94. 3% in MIX type. IVC pressure decreased from （26. 52 ＋_ 8. 16） cm H20 to （ 14. 28 _＋ 4. 08） cmH20（P 〈 0.05） and HV pressure dropped from（35.70 -＋ 13.26） cm H20 to（18.36 -＋ 8.16） cm H20 （P 〈0. 05）. Restenosis or occlusion was found in 21.4% （45/210） patients after a follow- up of 1 month to 15 years. The rate was 13.8% （12/87） in IVC type, 31.1% （28/90） in HV type and 15.2% （5/33） in MIX type. These patients were managed by iuterventional procedures. Technical success was achieved in 44 cases with restenosis. Conclusions Hepatic vein occlusion was the most common type of BCS in Chinese youth. The symptoms and signs of portal hypertension were the initial clinical manifestations. Postoperative recurrence rate in HV type was higher than that in the other two types.