经颈内静脉肝内门体分流术治疗肝静脉广泛闭塞型布加综合征

目的探讨经颈内静脉肝内门体分流术(TIPS)治疗肝静脉广泛闭塞型布加综合征的临床疗效。方法采用TIPS治疗11例广泛肝静脉闭塞型布加综合征患者,其中3例为急性,8例为亚急性或慢性。患者表现为食管静脉曲张破裂出血和顽固性腹水,采用超声多普勒、CT或MRI、上消化道钡餐、血管造影和肝活检明确诊断。TIPS将肝内分流道建于肝后下腔静脉与门静脉分支,支架直径为10 mm,随访时间(63±43)个月。结果所有患者均成功完成TIPS,肝门部门静脉分叉处出血1例,1周后出血控制再植内支撑;肝内分流道建立后门体压力梯度由(41．2±10．5)cm H2O(1 cm H2O=0．098 kPa)下降至(12．4±4．7)cm H2O,门静脉血流速度由(11．2±2．8)cm／s增加至(52．2±13．7)cm／s。患者出血控制,腹水渐消退,肝功能指标明显好转。住院期间因肝功能衰竭死亡1例。术后随访,2例分流道狭窄分别行分流道再扩张或再植内支撑,其余8例无相关并发症。结论TIPS是治疗肝静脉广泛闭塞型布加综合征的重要方法,具有良好的远期疗效。

Transjugular intrahepatic portosystemic shunt in the treatment of Budd-Chiari syndrome with extensive occlusion of the hepatic veins

OBJECTIVE: To explore the outcome of a transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of 11 patients with Budd-Chiari syndrome with extensive occlusion of the hepatic veins. METHODS: Eleven patients with Budd-Chiari syndrome with extensive occlusion of the hepatic veins were elected for TIPS. Three patients had a acute; eight, a subacute or a chronic course of the disease. All patients were associated with variceal bleeding and massive ascites. The diagnosis of Budd-Chiari syndrome was established by duplex-sonography, CT, MRI, upper digestive barium meal, angiography of hepatic veins and IVC, and liver biopsy. The shunt with diameter of 10 cm was established between the inferior caval vein and the intrahepatic portal vein with self-expandable stents in all patients. The mean follow-up was 63 +/- 43 months. RESULTS: The shunt reduced the portasystemic pressure gradient from 41.2 +/- 10.5 to 12.4 +/- 4.7 cm H2O and improved the portal flow velocity from 11.2 +/- 2.8 to 52.2 +/- 13.7 cm/s. Clinical symptoms and the biochemical test results improved significantly during 3 weeks after shunt treatment. Ten patients are alive without clinical symptoms except one death due to hepatic failure. Revision in 2 patients was needed during the follow-up. The inflation of stenosing shunt was performed in 1 patient, and the reimplantation of stent in another patient. Eight patients had no revisions. CONCLUSIONS: TIPS provided an excellent outcome in patients with Budd-Chiari syndrome with extensive occlusion of the hepatic veins. It might be regarded as a treatment for the acute and long-term management of these patients.