静脉成形术和经颈静脉肝内门体静脉分流术治疗肝静脉型巴德-吉亚利综合征回顾性分析

Hepatic venousaplasty and transjugular intrahepatic portosystemic shunt in the treatment of BuddChiari syndrome with occlusion of the hepatic veins

【目的】分析肝静脉闭塞型巴德-吉亚利综合征（BCS）的治疗方法。【方法】回顾性分析1995年5月至2012年2月收治的50例肝静脉型BCS患者的临床资料。男性23例，女性27例｜平均年龄（39±11）岁，肝功能Child-Pugh评分（9.6±2.6）分。肝静脉近段闭塞12例，肝静脉闭塞合并下腔静脉狭窄6例，全肝静脉闭塞19例，肝小静脉闭塞13例。急性3例，亚急性或慢性47例。患者主要表现为食管静脉曲张破裂出血和顽固性腹水，采用多普勒超声、CT血管造影或磁共振血管造影、上消化道钡餐、血管造影明确诊断。治疗方法包括：肝静脉成形术12例，肝静脉成形加下腔静脉成形术6例，经颈静脉肝内门体静脉分流术（TIPS）13例，改良TIPS 19例。【结果】所有患者均成功完成手术。TIPS或改良TIPS术后，门静脉压力由（41±10）cmH2O（1 cmH2O=0.098 kPa）下降至（27±6）cmH2O（t=20.20，P=0.001），门静脉血流速度由（14±10）cm/s增至（52±14）cm/s（t=15.02，P=0.001）。患者出血控制，腹水渐消退，肝功能明显好转。住院期间因肝功能不全死亡1例，肝门分流道急性阻塞1例。术后随访12～204个月，平均随访时间（82±46）个月，分流道狭窄行扩张内支撑2例，肝静脉狭窄再扩张2例。【结论】肝静脉成形术和TIPS是治疗肝静脉型BGS的有效方法。

Objective To determine the outcome of hepatic venousaplasty and transjugular intrahepatic portosystemie shunt （TIPS） in the treatment of the Budd-Chiari syndrome with occlusion of the hepatic veins. Methods Fifty patients of the Budd-Chiari syndrome with occlusion of the hepatic veins （23 males and 27 females, with a mean age of （ 39 ± 11 ） years） were elected for venousaplasty or TIPS. The average of Child-Pugh scores was 9.6 ± 2. 6. Three patients had a acute course of the disease, while 47 patients had a subacute or a chronic course of the disease. The clinical presentation was ascites in all 50 cases, with concomitant upper gastrointestinal bleeding in 10 patients, hepatorenal syndrome in 4 patients and impaired liver function in all patients. Hepatic venousplasty was performed for 12 patients with occlusion of hepatic venous. Hepatic and inferior eaval venousplasty were performed for 6 patients with occlusion of hepatic and inferior eaval vein. TIPS was performed for 13 patients with occlusion of small hepatic vein. Modified TIPS was performed for 19 patients with extensive occlusion of hepatic vein. Results The procedure of treatment was successfully performed in all patients. The shunt reduced the portosystemic pressure gradient from （41 ± 10） to （27 ± 6） cmH20 （ 1 cmH2 0 = 0. 098 kPa, t = 20. 20, P = 0. 001 ） and improved the portal flow velocity from （ 14 ± 10） to （52 ± 14） cm/s （t = 15.02, P =0. 001 ） after TIPS or modified TIPS. Clinical symptoms and the biochemical test results improved significantly during 3 weeks after hepatic venousplasty and shunt treatment. During the hospitalization, the death occurred in 1 case due to hepatic failure and the acute occlusion of shunt was treated with secondary intervention in another case. The mean follow-up was （82 ± 46） months. The revisions of shunt with TIPS were needed in 2 patients and the inflation of stenosised hepatic vein in another 2 patients during the follow-up. All patients were still observed. Conclusion Hepatic venousaplasty and TIPS provide an excellent outcome in patients of Budd-Chiari syndrome with occlusion of the hepatic veins.