肝移植治疗布加综合征九例

目的 总结原位肝移植术治疗布加综合征的经验和体会.方法 回顾性分析2003年12月至2010年4月9例布加综合征患者接受肝移植治疗的临床资料,总结术前影像学评估和手术经验,并观察术后并发症的发生情况及受者存活情况.结果 术前9例患者均经腹部CT强化扫描及血管重建等检查明确诊断,并行腔静脉造影明确梗阻情况及分型.9例患者中,接受尸体供肝移植者8例,均采用经典非转流术式;接受亲属活体右半供肝移植1例.术后免疫抑制方案为他克莫司+吗替麦考酚酯+皮质激素.9例受者术后平均随访时间为32.8个月(13～61个月),期间出现癫痫发作1例,十二指肠乳头及功能不良1例,小肝综合征1例,以及肺部感染4例;1例接受了中度脂肪肝供肝的受者于术后12d因原发性移植肝无功能行再次肝移植;1例受者于术后6个月因发生胆道并发症导致移植肝功能丧失而行再次肝移植,该例患者术后35个月死于肝脏肿瘤复发.所有患者未发生流出道梗阻及布加综合征复发.9例受者术后1和2年存活率为100％(9/9),术后3年存活率为88.9％(8/9).结论 通过准确的影像学评估和选择适宜的手术方式,肝移植已成为治疗布加综合征的重要手段,受者预后良好.     

活体肝移植治疗布加综合征并下腔静脉狭窄

目的 报道笔者利用尸体下腔静脉（IVC）替代受体肝后IVC施行成人间活体肝移植（LDLT）治疗布加综合征（BCS）并IVC狭窄的经验。方法 1例35岁男性BCS并肝后IVC狭窄的患者，曾接受内科治疗，并于9个月前经放射介入置入金属扩张器，但症状无缓解。最终患者被施行了成人间LDLT，术中采用了尸体IVC替代受体肝后IVC进行重建。结果 患者术后过程平稳，效果满意。结论 笔者认为采用LDLT及利用尸肝IVC重建受者肝后IVC治疗BCS并肝后IVC狭窄的术式可推荐作为一种新的术式。     

肝移植治疗布加综合征

肝移植治疗布加综合征杨松山１严以群２杨甲梅２布加综合征（Ｂｕｄｄ－ＣｈｉａｒｉＳｙｎｄｒｏｍｅ，ＢＣＳ）是由于肝静脉和／或肝后下腔静脉梗阻所致的肝后性门脉高压症。其病因复杂，内科疗效不佳，临床常用各种分流减压术和根治性手术进行治疗，尚存在着术后并发症...     

再次肝移植手术临床病例分析:附六例

目的 探讨影响再次肝移植预后的因素及再次移植手术问题.方法 回顾性总结2000年7月至2006年4月北京大学第三医院完成的6例再次肝移植病例临床资料,分析再次肝移植的原因、手术方法及病人转归.结果 6例再次肝移植的原因中:移植肝肝癌复发1例,乙型肝炎复发1例,慢性排斥反应2例,肝动脉血栓形成1例,药物性肝损害1例;再次肝移植时间距第一次肝移植平均(12.9±10.0)个月;术中出血平均(14 050±8 215)m1;平均手术时间(12.7±2.0)h.围手术期死亡3例.结论 再次肝移植病人术前一般情况差,手术风险大,正确把握手术时机及手术适应证,术中精细操作,减少出血,手术后采取个体化免疫抑制剂治疗方案等是提高再次肝移植病人存活率的关键因素.     

背驮式肝移植技术改良（附41例次临床病例分析）

目的：探讨背驮式肝移植在终末期肝病中应用的利弊。方法：自1995年9月至2000年7月,对39例患施行了41次背驮式肝移植。对供肝静脉回流通道重建技术进行了逐步改变,由供肝下腔静与受体成型的肝静脉重建,到供、受下腔静脉的端－侧吻合及侧－侧吻合,并进行了供体下腔静脉与受体下腔静脉－右心房的桥式吻合和原旁位供肝肝上下腔静脉－右心房重建悬吊。结果：手术技术的改良既避免了肝静脉回流不同程度受阻的弊端,同时缩短了无肝期和下半躯体淤血时间。结论：背驮式肝移植技术改良减少了术后并发症,缩短了手术时间,并拓宽了手术适应证。     

布加综合征的肝移植

报告一例曾接受过脾肺固定术的布加综合征病例，因终末期肝硬化，下腔静脉阻塞而行原位肝移植术。术野局部粘连严重，侧支丰富，特别是第二肝门解剖困难，采用右房肝上下腔静脉吻合术，重建下腔静脉通道。行髂总静脉、门静脉至右房插管转流，使用ＷＭＯ液保存供肝５小时４５分。完成手术肝血液循环良好，当天即有胆汁排出。术后第１３天死于肝排斥反应脏肝动脉栓塞，肝、肾功能衰竭 。     

布加综合征治疗的研究进展

通过综合分析布加综合征的内科非手术治疗、外科治疗及介入治疗方法及其研究进展,在研究如何有效利用布加综合征的各种治疗方法,并进一步探讨更为有效的治疗措施。结果显示：1）抗凝、溶栓等内科非手术治疗疗效欠佳;2）根据病变部位采用不同的手术方法可获得较好的疗效,但手术创伤大,术后并发症多;3）严格掌握适应证的介入治疗可获得满意的治疗效果且明显降低了复发率。对各种类型的布加综合征,多能进行有效的介入和（或）手术治疗。介入疗法简便易行、创伤小、疗效确切,值得临床推广。     

布加综合征疑难病例与介入治疗相关并发症的处理（附1859例报道）

目的总结布加综合征行介入治疗过程中的疑难病例与介入治疗相关并发症的类型,提出相应的解决对策。方法回顾性分析1990年1月至2014年9月期间于笔者所在医院行介入诊断和治疗的1859例（2214例次）布加综合征患者的临床资料。结果本组患者在介入治疗过程中,发生与手术操作有关的并发症31例次,发生率为1.40%（31/2214）,其中成功处理并发症25例次,成功率为80.65%（25/31）。途中放弃治疗7例,术中和术后死亡6例,死亡率为0.32%（6/1852）。396例次疑难病例中,9例次未予处理、随访观察,余387例次中成功施行介入手术372例次,成功率为96.12%（372/387）。术后获访1553例,随访时间为10-284个月,平均100.9个月。获访病例中发生再狭窄209例,再狭窄发生率为13.46%（209/1553）。结论布加综合征的介入治疗已经进入成熟阶段,及时发现和正确处理术中并发症是提高介入治疗疗效和成功率的重要环节。     

布加综合征治疗方式的选择

自20世纪80年代以来,人们对布加综合征的认识日益深入,一些陈旧的治疗手段被摒弃,而一些新的治疗方法不断涌现。目前,对布加综合征的治疗方法已经有很大的改变,微创介入治疗和根治性病变切除已经成为治疗布加综合征的主流方法。现将布加综合征的分型及其常见的治疗方法介绍如下。     

深低温停循环布加综合征手术治疗(附2例报告)

为探讨布加综合征的病理解剖特点及手术技术。1998年2～6月为2例布加综合征病人采用深低温停循环法直视下矫治下腔静脉机化性血栓梗阻及肝静脉阻塞性病变,以恢复下腔静脉及肝静脉的通畅血流,取得了十分满意的疗效。采用此法优点是视野清楚,直视下更能精确、比较彻底根除下腔静脉系各种梗阻性病变,疗效更好。     

Liver transplantation in patients with Budd-Chiari syndrome

Patients with Budd-Chiari syndrome (obstruction of the hepatic veins) and associated hepatic insufficiency may be candidates for orthotopic liver transplantation (OLT). In our series of 405 OLT patients, 3 were transplanted due to Budd-Chiari syndrome (0.7%). The indication for liver transplantation in these patients was severe hepatic insufficiency (chronic in two and acute in the third one). Morphologic study of the obstructions revealed apparently different causes, including thrombi, membranous webs in hepatic veins, and hydatid cyst compression. The surgical technique employed in these transplantations was similar to that for other etiologies. Due to its implications for the future course of OLT, it is important to determine the exact etiology of Budd-Chiari syndrome in the pretransplant period and to treat the patients with early and long-term anticoagulant therapy to avoid syndrome recurrence.     

Liver transplantation for PNH with Budd-Chiari syndrome

A 54-year-old male patient with end-stage liver failure from Budd-Chiari syndrome due to paroxysmal nocturnal hemoglobinuria (PNH) underwent liver transplantation (OLT) in 1989. Retransplantation became necessary 1 year later when thrombotic occlusion of the portal vein and common hepatic artery led to graft loss after withdrawal of anticoagulation therapy because of several gastrointestinal bleeding episodes. The patient is now alive 3 years after the first OLT. To the best of our knowledge and according to the literature, this is, to date, the longest that any PNH patient has survived after liver transplantation. Although the course of this patient was complicated in a way similar to that reported for other cases in the literature, patients with PNH should not, in principle, be excluded from liver transplantation. Lifelong anticoagulation with coumarin and the use of steroids together with cyclosporin reduce the risk of recurrent thrombosis and PNH crises.     

Budd-Chiari 综合征根治术后复发的介入治疗：附15例报告

目的：探讨布加综合征（BCS）根治术后复发患者介入治疗的可行性及疗效。 方法：回顾性分析2007年3月—2013年9月15例BCS根治术后复发行介入治疗患者的临床资料。 结果：15例患者,年龄30~68岁,平均46.4岁;共行18例次介入治疗（1例3次,1例2次）,其中下腔静脉球囊扩张成形术10例次,置管溶栓后下腔静脉球囊扩张成形术5例次,下腔静脉、肝静脉及副肝静脉球囊扩张成形术2例次,下腔静脉球囊扩张成形术并下腔静脉支架植入1例次。围手术期未出现肺动脉栓塞、心包填塞等并发症 。15例患者随访12~106个月,平均41.9个月,13例未出现复发症状及体征,1例肝静脉闭塞在外院行球囊扩张支架成形术,1例下腔静脉再次闭塞保守治疗。 结论：对于BCS根治术后复发患者,选择及时介入治疗安全有效,可以改善累积通畅率及预后。     

Liver transplantation for Budd-Chiari syndrome-palliation or cure?

This report documents two cases of Budd-Chiari syndrome (BCS) with essential thrombocytosis and antithrombin (AT) III deficiency as underlying etiological factors. Orthotopic liver transplantation was successfully performed in both patients but with different therapeutic intention. In the patient with essential thrombocytosis, hepatic transplantation only relieved the symptoms of the predisposing thrombogenic condition; it dìd not cure the underlying disorder. Prophylactic long-term anticoagulation, as well as adjuvant therapy for the causative disease, remained necessary. On the other hand, in the patient with AT III deficiency, liver transplantation was curative, resulting in complete reconstitution of serum AT III activity with resolution of the hypercoagulable state postoperatively. Thus, depending on the underlying etiology, liver transplantation for BCS can be considered as palliative, necessitating long-term adjuvant therapy, or as curative, with correction of a metabolic defect.     

Recurrence of Budd-Chiari syndrome after liver transplantation in paroxysmal nocturnal hemoglobinuria

Venous thrombembolism is a major complication of paroxysmal nocturnal hemoglobinuria (PNH). Often, veins of atypical localization are afflicted, resulting in cerebral, mesenteric, or hepatic venous thrombosis. We present a patient who received an orthotopic liver graft for chronic Budd-Chiari syndrome in 1988. PNH was the only thrombophilic predisposition identified in this patient. After transplantation, he repeatedly suffered from hemorrhage. Subsequently, the patient discontinued prophylactic anticoagulation nearly 10 years after transplantation. Within 6 months Budd-Chiari syndrome recurred, but stabilized after anticoagulation therapy with low-molecular-weight heparin was reinstituted. The patient is clinically stable 14 years after receiving the liver graft. Eleven cases of relapsing Budd-Chiari syndrome have been reported in the literature. Of these, four patients suffered from PNH. All patients transplanted for PNH-associated Budd-Chiari syndrome in these reports suffered from either major bleeding or thrombosis. In conclusion, patients afflicted with PNH appear to be at high risk of incurring complications after liver transplantation.     

原位肝移植术后痫性发作六例病因分析

目的探讨原位肝移植术后痫性发作的病因和防治措施。方法回顾性分析 83例原位肝移植术的临床资料并结合文献进行讨论。结果术后发生痫性发作共有 6例 ,发生率为 7% ,发生于移植术后 4～ 15d ,平均 (9 3± 1 2 )d。均表现为强直 阵挛性发作。其中 3例有癫痫病史 ,2例合并脑血管意外。 3例死亡 ,与肝移植术后痫性发作相关的病死率为 4 %。结论肝移植术后痫性发作可能提示患者中枢神经系统存在着导致痫性发作的病灶 ,免疫抑制药物毒性、代谢紊乱和肺部感染等具有促发作用。及时地控制症状和针对导致痫性发作病因的综合治疗有利于改善预后。     

Liver transplantation for Budd-Chiari syndrome: A challenging but handable procedure

BackroundEstablishing venous outflow in liver transplantation for patients with Budd-Chiari syndrome is crucial and requires various surgical techniques. The outcomes of these patients exibits distinct problems including vasculary thrombosis and biliary complications.MethodsIn this single center study, the outcomes and surgical features of 33 patients with Budd-Chiari syndrome who were carried out liver translantation (27 patients from living donor). Another group was formed among patients underwent liver transplantation due to other etiologies and the outcomes were compared.ResultsThe most-seen type was the classical type of Budd-Chiari syndrome (25, 75.8%). For twenty-six patients inherited or acquired prothrombotic disorders were identified (78.2%) in Budd-Chiari group. Average follow-up was 29.7 ± 15.5 months. We have observed no recurrence of disease in our BCS patients. When the two groups was compared in terms of thrombotic complications, there was a significantly increased risk in BCS group (p = 0.014). Our 1 and 3-year survival rates for the BCS group were 81.8% and 78.8%, respectively. In the control group, 1 and 3-year survival rates were 93.3% and 88.9%, respectively. Log-rank test analyses showed no statistically significant results.ConclusionLiver transplantation with individual surgical and postoperative treatment strategy for patients with Budd-Chiari syndrome provides comparable outcomes.     

Liver transplantation for Budd-Chiari syndrome: A retrospective study

A retrospective study was performed on 11 patients who underwent orthotopic liver transplantation for Budd-Chiari syndrome (BCS), 3 of whom had fulminant type BCS and 8, chronic type BCS. Both the 3- and 5-year actuarial survival rates were 64%, after one patient with fulminant, and three with chronic disease died of sepsis or multiple organ failure following transplantation. Anticoagulation therapy in the early postoperative period was tailored to each individual patient. Most of the patients received heparin for several days and were then converted to Coumadin therapy, although some were not given heparin in the immediate postoperative period but were instead commenced on oral Coumadin after the prothrombin time had recovered to wihtin the normal range. All the long-term survivors had received Coumadin therapy and there was no recurrence of BCS and no early thrombotic or hemorrhagic event. One patient developed late thrombosis of the portal vein despite having received apparently adequate Coumadin therapy. It was thus concluded that liver transplantation is an effective therapy for both fulminant and chronic BCS, and that immediate postoperative heparinization is not mandatory for all patients.     

亲属活体供肝移植治疗儿童终末期肝病六例

目的 探讨亲属活体供肝移植(LDLT)治疗儿童终末期肝病的效果,并总结治疗经验.方法 2005年9月至2007年1月对6例终末期肝病患儿进行了LDLT.6例患者中,原发病为肝内外胆管弥漫性囊性扩张症Ⅳ型伴肝硬化1例、肝豆状核变性2例、门静脉海绵样变性3例;供者为患儿的母亲3例、父亲2例及舅父1例,分别切取供者的右半肝1例和左半肝5例作为供肝;在切除受者全部病肝和保留下腔静脉后,对受者施行部分供肝的原位肝移植.术后依据供、受者的临床表现、血液学指标和影像学检查,对活体供肝移植的治疗效果进行评价.结果 术后对供、受者随访了6～21个月.6例供者均健康存活,未发生并发症.6例受者中,1例于术后第4天死于门静脉血栓形成,1例于术后5个月时死于肝内静脉血栓形成,其余4例均长期健康存活.结论 亲属活体供肝移植是治疗儿童终末期肝病的有效方法.术前进行仔细的供、受者选择和完善的影像学检查,术中应用精确的手术技术,术后给予严格的管理是儿童LDLT成功的关键.