球囊扩张血管成形治疗布—加综合征

采用球囊扩张下腔静脉成形术治疗布－加综合征４０例，４０例中男１８例，女２２例，病程１－２４年。病理类型为膜性下腔静脉阻塞和短节段性下腔静脉阻塞。本组病例总扩张成功率为９２．５％。１例因肺梗塞而死亡；５例下腔静脉侧壁被穿刺导丝刺穿，后者均经非手术治疗痊愈。     

膜性布-加综合征的治疗--介入或手术?

目的　探讨膜性BuddChiari综合症(BCS)的治疗方法,评价放射介入和根治性病变隔膜切除术对膜性BCS的治疗效果。方法　对1990年5月～1997年12月我院收治的342例膜性BCS病人的临床资料进行回顾性分析。结果　手术治疗182例(其中18例为介入治疗失败者),采用常温直视下病变隔膜切除;介入178例,采用经皮腔下腔静脉球囊扩张成形术(PTA)156例,经皮腔下腔静脉球囊扩张成形术及经皮肝肝静脉扩张成形术22例,其中10例加用血管内支架(Stent)放置。手术组随访177例中总有效率898%,复发率102%;介入组总有效率810%,死亡率17%(3例),复发率190%,介入失败112%。结论　大部分膜性BCS病人经PTA或PTA加血管内支架治疗可取得良好效果,对介入治疗失败者、下腔静脉内有血栓者、介入治疗后复发者或下腔静脉内为斜或厚膜的病人应选择根治性膜切除术。     

下腔静脉及肝静脉短段病变型布-加氏综合征的外科治疗

目的 探讨布-加氏综合征的下腔静脉短段闭塞及肝静脉主干病变根治性切除的优缺点、术中的注意事项。方法 下腔静脉闭塞或狭窄段切除、人工血管原位移植术42例,肝静脉主干闭塞段切除、肝静脉流出道成形、人工血管原位移植术10例,肝静脉主干闭塞段切除、肝静脉-右心房吻合3例,经皮肝穿肝或副肝静脉破膜、扩张10例。结果 1例死于并发症,随访58例,平均30个月,介入复发3例,手术复发1例。结论 下腔静脉或肝静脉病变段切除、人工血管原位移植治疗布-加氏综合征,能根治病变、符合正常解剖生理、远期疗效好。     

布-加综合征的外科治疗

目的　分析多种手术及介入方法治疗布加综合征的疗效。方法　113例中行经皮腔内血管成形术(PTA)22例,PTA加支架置术术16例,脾肺固定加大网膜包肺术27例,经右房及股静脉联合破膜扩张并置支架术33例,下腔静脉右房人工血管转流术10例,直视根治加支架置入术5例。结果　术后病情缓解,下腔静脉压力由术前26～40cmH2O下降至术后15～25cmH2O,门静脉压力由术前37～51cmH2O下降至术后22～33cmH2O。结论　对下腔静脉或肝静脉膜性阻塞或狭窄且无新鲜血栓者,PTA加支架置入为首选措施,不宜用此法的患者以选择适当的手术加介入治疗为佳。     

单球囊导管扩张及内支架置放治疗布-加综合征

目的评价单球囊导管扩张及内支架置放治疗膜性及段性布-加综合征.方法 3 4例布-加综合征接受了上、下腔静脉同时插管造影及下腔静脉单球囊导管扩张和内支架置放治疗.结果 33例穿刺、扩张成功.下腔静脉阻塞段分别被扩张至10～20 mm,9例置入金属内支架,阻塞下方下腔静脉压力从平均(2.71±0.78) kPa下降至(1.98±0.85) kPa.结论上下腔同时插管造影,阻塞段部位及长度显示清楚,单个大球囊置入简便,扩张确切,段性者配合内支架置入可在一定程度上预防再狭窄.     

布-加综合征的外科治疗进展

本文就近年来发展的外科治疗布一加综合征的国外相关文献作一综述。对分流术的主要典型术式在治疗布一加综合征的适用条件、手术操作要点、血管移植的选用、随访疗效方面进行了比较，对肝移植治疗布一加综合征的适应证进行了概述。     

布-加综合征的治疗

目的：探讨布－加综合征（BCS）各种治疗方法的疗效。方法：回顾性分析260例BCS病人采用不同治疗方法的临床资料。结果：260例中采用介入、手术或介入联合手术治疗273例次，其中下腔静脉成形术或／和支架置入术149例160例次，肝静脉或副肝静脉成形术或／和支架置入术15例17例次，下腔静脉加肝静脉或融肝静脉成形术或／和支架置入术10例；常温下直视根治术8例，肠腔转流术16例，肠颈转流术5例，肠房转流术8例，腔房转流术6例，脾肺固定术18例，肠腔转流联合下腔静脉成形术和支架置入术17例，经右房手指破膜术联合下腔静脉成形术和支架置入术8例。手术死亡2例，1例介入病人心包填塞死亡，1例手术病人大出血死亡。全组病人经随访3月－6年，20例复发，其中7例死于肝功能衰竭，其余病人恢复满意。结论：布－加综合征首选介入治疗，不宜介入或介入失败和复发者应选用手术或介入联合手术治疗，治疗效果良好。     

合并上腔静脉阻塞的布-加综合征的处理

198 8年 9月至 2 0 0 1年 5月我们共收治 4 83例布 加综合征 (Budd Chiarisyndrome ,BCS)病人 ,其中合并上腔静脉阻塞者 3例 ,现报告如下。1.资料与方法 :(1)对象 :3例病人均为男性 ,分别为 38、4 4、4 8岁 ,均有头颈部浮肿及颈静脉怒张 ,劳累后心慌、腹胀 ,胸腹壁静脉曲张 ,双下肢静脉曲张及色素沉着。其中 2例合并上消化道出血。全组病人均经彩色Doppler和腔静脉造影确诊为下腔静脉肝后段阻塞或狭窄合并上腔静脉阻塞或狭窄 ,其中 1例上腔静脉血栓延续至两侧颈内静脉。 (2 )造影方法 :以Seldinger法分别经颈内静脉和股静脉行上、下腔静…     

介入治疗布加综合征312例经验

目的　总结球囊扩张及支架治疗布加综合征 (Budd Chiarisyndrome ,BCS)的经验。方法　对 312例BCS患者行下腔静脉 (inferiorvenacava ,IVC)造影 ,确定病变部位、类型 ,再用导丝硬头或穿刺针穿通阻塞部位、球囊扩张、内置支架。结果　本组 312例 ,扩张成功 2 71例 ,其中IVC 2 6 0例 ,肝静脉 (hepaticvein ,HV) 11例 ,放置IVC支架 195例 ,HV支架 1例 ,无一例发生肺栓塞。术后发生急性肾衰 6例 ,肝昏迷 1例 ,急性心衰 2 1例 ,死亡 1例。并发IVC急性血栓形成 2例 ,死亡 1例。 2 0 3例获随访 6～ 10 4个月 ,复发 2 1例。结论　介入治疗BCS适应于多种病理类型 ,安全、有效。     

膜性布加综合征480例的治疗分析

目的：探讨膜性布－加综合征的最佳治疗方法，方法：对480例膜性布－加综合征患者手术的长期疗效和复发情况进行回顾性分析。结果：破膜组52例，随访率84．62％（44／52），随访时间6个月至10年，有效率61．4％，复发率38．6％，介入组238例，随访率86．55％（206／238），随访时间6个月至8年，有效率91．7％，复发17例，复发率8．3％，根治组190例，随访率87．37％（166／190），随访时间9个月至8年，有效率90．4％，复发率9．6％，介入组，根治组的长期效果明显优于破膜组（P＜0．05），复发率低于破膜组（P＜0．05），结论：膜性布－加综合征患者治疗应首选介入球囊扩张术，对于病变范围大，膜厚和介入治疗后复发的病例，则应用直视下病变隔膜切除术。     

Transatrial membranotomy for Budd-Chiari syndrome

In a 7-year period, transatrial membranotomy was performed in 11 patients with membranous obstruction of the inferior vena cava. There were 5 men and 6 women, ranging in age from 23 to 53 years. Clinical symptoms included jaundice in 4 patients, hepatomegaly in 4, leg edema or varicose veins in 10, and venous collaterals over the abdominal and chest wall in all 11 patients. Transatrial membranotomy was performed through a median sternotomy in all patients. When inferior vena cava venography revealed that the obstruction was accompanied by long segmental thrombosis, additional dilation was performed with a Hegar dilator. There was no surgical mortality. Early operative complications included pulmonary embolism in 2 patients and bleeding requiring reoperation in 1. In a mean follow-up period of 30.6 months (range, 2 to 88 months), 9 patients had no symptoms, transient pericardial constriction developed in 1 patient and resolved 1 month later, and restenosis of the inferior vena cava developed in another patient 1 year after the first operation. This latter patient received a second transatrial membranotomy followed by percutaneous balloon angioplasty of the inferior vena cava, with a satisfactory result at 8 months follow-up. We conclude that transatrial membranotomy is an effective and safe procedure for patients with membranous obstruction of the inferior vena cava.     

Liver transplantation for Budd-Chiari syndrome

BACKGROUND: Budd-Chiari syndrome (BCS) is a clinical condition characterized by hepatic venous outflow obstruction secondary to an underlying systemic predisposition to thrombosis. METHODS: We reviewed our experience of 19 adult patients who underwent orthotopic liver transplantation for BCS from April 1988 to May 1999 to assess their long-term outcome and specific complications related to this procedure. RESULTS: Of these patients, 13 presented with chronic and 6 with acute liver failure. At presentation predisposing factors included polycythemia rubra vera in five, an undefined myeloproliferative disorder in four, essential thrombocythemia in two, presence of lupus anticoagulant in one, antiphospholipid antibody positivity in one, post-gestational in one, oral contraceptive pill in one, and idiopathic in four. Five patients had undergone previous porto-systemic shunt. Of the 19 patients, 16 are alive at a median follow-up of 89 months (range 1-119) with 2 patients developing disease recurrence at 4 months and 7 years posttransplant, respectively. Four patients have been retransplanted: one for progressive graft dysfunction due to nodular regenerative hyperplasia secondary to azathioprine toxicity, two for hepatic artery thrombosis (one soon after and the other 47 months posttransplant), and one for recurrent BCS. Three patients have died: one from an intra-abdominal bleed secondary to acute hemorrhagic pancreatitis 8 years posttransplant, another from acute myeloid leukemia at 6 years posttransplant, and the third patient from graft failure secondary to severe rejection 1 month posttransplant. CONCLUSION: Liver transplantation for BCS provides good long-term survival with acceptable morbidity. Long-term survival may be prejudiced by progression of the underlying hematological disorders.