布-加综合征下腔静脉病变隔膜形态学特征研究

目的 观察分析布-加综合征下腔静脉(IVC)病变隔膜的形态学特征,探讨病变隔膜的形成机制.方法 大体观察2007年10 月至2010年l2月根治性切除手术切除的10例布-加综合征下控静脉病变隔膜形态特征,并利用光镜、电镜对比观察病变隔膜与 7例正常肝后段下腔静脉壁的组织结构差异.结果 术中见病变隔膜位于下腔静脉入右心房2.0 cm范围内.隔膜边缘较厚,中央偏薄,表面与IVC内膜相连.9例隔膜下方IVC有附壁血栓,5例病变隔膜局部有钙化.光镜下病变隔膜组织由粗大的均质胶原纤维及成纤维细胞构成,胶原排列紊乱,部分胶原纤维呈透明变性.隔膜边缘为不等程度的纤维结缔组织增生,少量肉芽组织和新生血管等病理改变;正常下腔静脉壁内膜、中膜和外膜3层结构完整.电镜下病变隔膜组织中可见较多的纤维细胞,表面有少量内皮细胞.内皮细胞内线粒体大部分嵴和膜脱落融合、模糊不清或缺失,粗面内质网轻度扩张.偶见凋亡细胞,但体积缩小;正常下腔静脉壁大内皮细胞排列规则,呈条带状分布,内皮细胞内可见线粒体、粗面内质网及吞饮小泡,内皮细胞下为少量排列较稀疏的胶原纤维及弹性纤维.结论 布-加综合征下腔静脉病变隔膜形成以纤维细胞增生为主,肝后段下腔静脉的损伤和炎症反应可能参与了隔膜的形成过程.     

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.     

直视下病变隔膜切除治疗布-加综合征(附172例报告)

１９９０年５月至１９９５年１０月应用病变隔膜切除术治疗膜型布－加综合征１７２例。术中以球囊控制肝、腔静脉血流。在常温、直视下切除病变隔膜。手术死亡率２．９％（５／１７２），有效率８６．６％（１４９／１７２），复发率１０．５％（１８／１７２）。认为直视下病变隔膜切除创伤较小，不用体外循环即能完成，下腔静脉内不留病变隔膜碎片，是治疗膜型布－加综合征的有效方法。     

经右心房下腔静脉扩张并放置支架治疗布-加综合征1例

病人　男 ,32岁。腹胀、纳差 4个月并下肢水肿。查体 :腹部膨隆 ,胸腹壁浅静脉怒张 ,肝肋缘下 2 0ｃｍ ,腹水 ,双下肢浅静脉曲张并水肿。下腔静脉测压 2 4ｃｍＨ2 Ｏ(1ｃｍＨ2 Ｏ =0 0 98ｋＰａ)。下腔静脉造影显示下腔静脉肝段完全闭塞 ,上腔静脉同时双重造影 ,狭窄段长度约 2 0ｃｍ ,肝静脉不显影。彩色超声检查示肝大 ,肝静脉狭窄 ,下腔静脉肝段完全闭塞 ,长约 2 1ｃｍ ,无血流通过。诊断 :布—加综合征。于当地医院放置下腔静脉支架失败后转入我院。2 0 0 1年 6月手术。经右心房探查 ,下腔静脉无血流冲击感 ,完全闭塞。沿左肝静脉开…     

血管支架在布-加综合征治疗中的应用

布-加综合征(Budd-Chiari syndrome,BCS)是由于肝段下腔静脉和/或肝静脉阻塞,或狭窄引起的下腔静脉和/或肝静脉血流受阻导致的一组临床症候群。以往治疗BCS主要依靠外科手术,随着介入放射学技术的广泛开展,尤其是经皮血管腔内成形术(PTA)和血管内支架的应用,对该病的治疗取得了更理想的效果。介入治疗已经成为BCS的一种安全有效的非外科手术治疗方法[1]。据文献报道及我们的经验,单纯球囊血管成形术,膜性病变的良好开通率约为50%,节段性病变几乎无效,而血管内支架置入术是在PTA显示不足或失败后发展起来的,对球囊扩张不佳的无论是膜…     

布-加综合征治疗进展

布-加综合征(Budd-Chiari syndrome,B-CS)在西方国家多指Chiari病,即主肝静脉(main hepatic vein,MHV)阻塞,以血栓形成多见.在我围等亚洲国家出现的下腔静脉膜性梗阻(membranous obstruction 0f inferior vena cava, MOVC),其表现类似Chari病.     

布—加综合征的介入或半介入治疗

目的　探讨布 加综合征的介入或半介入治疗方法。方法　自 1986年起 ,我们采用多种介入或半介入方法治疗该病 173例 :①下腔静脉PTA76例 ;②下腔静脉PTA加支架置放术 5 9例 ;③经皮经肝静脉再通术 3例 ;④下腔静脉置管溶栓 4例 ;⑤经右心房及股静脉联合破膜、扩张2 2例 ;⑥联合破膜、扩张加支架置入术 17例 ;⑦根治术加支架置放 3例 ;⑧下腔静脉单纯介入治疗后附加其它手术 2 3例。结果　介入治疗即时技术成功率 90 .1% ,半介入治疗技术成功率 10 0 %。治疗前后下腔静脉压力下降范围为 3～ 2 9cmH2 O。发生并发症者 8例。死亡 5例。随访结果 ,下腔静脉单纯PTA后复发率 14.5 % ,下腔静脉PTA加支架置放组复发率仅 1.7% ,联合破膜组复发率18.2 % ,其余各组尚未发现复发。结论　①下腔静脉或肝静脉膜性阻塞或狭窄且无继发新鲜血栓者 ,PTA应为首选疗法。②下腔静脉破膜、扩张后出现弹性回缩者 ,应放置支架。③对于破膜困难者 ,应改行经右心房和股静脉联合破膜术。④下腔静脉病变合并肝静脉闭塞者 ,行下腔静脉介入治疗后可附加降低门脉高压的手术。     

布-加综合征下腔静脉阻塞合并血栓形成的治疗

目的 探讨下腔静脉阻塞并血栓形成的布-加综合征的治疗方法.方法 回顾分析近6年间收治的75例下腔静脉阻塞并血栓形成的布一加综合征患者的临床资料.其中行根治性隔膜切除、血栓取出22例,腔-腔(房)人工血管架桥41例,放射介入+内支架放置12例.结果 73例手术成功,手术死亡2例,手术后下腔静脉压力明显下降.临床症状及体征消失或缓解.随访6个月至6年,复发4例;其中人造血管堵塞2例,再发下腔静脉狭窄2例.结论 对下腔静脉阻塞合并血栓形成的布一加综合征患者,根据阻塞节段及血栓长度选择相应手术方式,大多可以取得满意效果.     

布-加综合征诊治现状与展望

布-加综合征(BCS)在西方国家多指Chiari病,即主肝静脉(MHVs)阻塞,以血栓形成多见。在我国等亚洲国家出现的下腔静脉膜性梗阻(MOVC),其表现类似Chiari病。因此,我们目前认为广义的BCS是指MHVs出口部和/或肝后段下腔静脉血流受阻所引起的肝后性门静脉高压和/或下腔静脉高压综合征[1-2]。自首例报道BCS至今已经160余年,随着科学技术的发展和认识的不断提高,大量病例逐渐被发现。中国、日本、印度和南非等为高发国家,国内河南、山东、安徽和江苏等省为高发区。BCS发病多为青壮年,综合国内近年来发表的文献BCS病例已经超过6000例。研…     

经皮球囊导管成形支架植入术治疗布-加综合征的临床观察

目的 探讨经皮下腔静脉球囊成形加支架植入术治疗布加综合征(BCS)的临床疗效。方法 120例患者均经彩色多普勒超声、下腔静脉及肝静脉造影等检查明确诊断，并进行经皮下腔静脉球囊成形(PTA)加支架植入术(EMS)治疗。所有病例随访6-108个月。结果 扩张前静脉狭窄段内径0-6mm(平均3mm)，肝静脉内径2mm。扩张并置放血管内支架后，腔静脉、肝静脉内径分别为18—20mm和8mm。扩张前下腔静脉至右房压力差为18-35cmH20(1．76-3．43Da)，平均20．3cmH20(1．989kPa)，扩张后压差消失，119例手术顺利，1例左肝静脉轻度损伤。术后96例主要症状及体征消失或基本消失，19例明显改善。120例(100％)获1-108(平均67．35)个月随访，其中门5例在随访期间血管内支架形态良好，支架内血流通畅；3例在术后8，11，16个月发生腔静脉再阻塞，经二次PTA EMS治愈；2例于术后2—3个月发生3支肝静脉再阻塞，转为外科手术。结论 经皮下腔静脉球囊成形、支架植入术是非长段阻塞型布—加综合征良好的治疗方法。     

Extended indication for living donor liver transplantation in patients with hepatocellular carcinoma

BACKGROUND: Liver transplantation is an accepted treatment option for patients with otherwise untreatable hepatocellular carcinoma (HCC). The present study assessed the outcome of living donor liver transplantation (LDLT) under extended selection criteria based on a single-center experience. METHODS: A total of 60 patients who underwent LDLT for HCC were included. Our indication for LDLT included HCC without extrahepatic spread or macroscopic vascular invasion. The size and number of HCC nodules were not limited. Recurrence-free survival rates according to various factors were compared to identify risk factors for recurrence. RESULTS: Forty patients (67%) preoperatively exceeded the Milan criteria. The median follow-up was 437 days (range: 23-1,385 days). The overall 1- and 3-year actuarial survival rates were 88.4 and 68.6%, respectively. HCC recurred in eight patients (14.3%) within a mean follow-up of 288 days; all were patients who exceeded the Milan criteria. The 1-, 2- and 3-year recurrence-free survival rates of patients who fulfilled the Milan criteria were 100%, 100%, and 100%, respectively, whereas those of patients who exceeded the criteria were 83.0%, 74.0%, and 74.0%, respectively. Tumor diameter >5 cm was significantly associated with worse prognosis, but the number of tumors was not. A preoperative des-gamma-carboxy prothrombin value >300 mAU/ml was strongly associated with the high recurrence rate. These two variables were significant in multivariate analysis. CONCLUSIONS: LDLT was shown to offer acceptable results in patients who exceeded the Milan criteria. The indication for LDLT can therefore be expanded beyond the Milan criteria, especially for patients with small multiple tumors <5 cm.     

ABO-incompatible liver transplantation and patients with hepatopulmonary syndrome

From 1991 to 2000, more than 100 ABO-incompatible liver transplantations were performed in 12 institution in Japan. The overall survival rate is 60%. Survival data appear to have improved in these years even in adult cases, although acute vascular rejection causing hepatic necrosis, infection, and intrahepatic bile duct injury are major complications to be resolved. Hepatopulmonary syndrome (HPS) was considered to be a contraindication to transplantation in the 1980s. However, even severe cases can recover from hypoxia after liver transplantation, and improved survival data were reported in the 1990s. Intensive respiratory therapy after transplantation with NO inhalation allows expansion of the indications for transplantation in HPS.     

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.     

Outcomes of liver transplantation for patients with Alagille syndrome: the studies of pediatric liver transplantation experience

Alagille syndrome (ALGS) is a multisystem disorder that manifests as childhood cholestasis. Reports of liver transplantation (LT) for patients with ALGS have come largely from single centers, which have reported survival rates of 57% to 79%. The aim of this study was to determine LT outcomes for patients with ALGS. We performed a retrospective analysis of the Studies of Pediatric Liver Transplantation database, which contains information about 3153 pediatric LT recipients. Data were available for 91 patients with ALGS and for 236 age-matched patients with biliary atresia (BA). The frequency of complex cardiac anomalies was lower in the LT group with ALGS versus published ALGS series (5% versus 13%). The pretransplant glomerular filtration rate (GFR) was <90 mL/minute/1.73 m(2) in 18% of the LT patients with ALGS and in 5% of the LT patients with BA (P < 0.001). The height deficit at listing was worse for the ALGS patients (66%) versus the BA patients (22%). The 1-year patient survival rates were 87% for the ALGS patients and 96% for the BA patients (P = 0.002). The deaths in the ALGS group mostly occurred within the first 30 days. No pretransplant factors associated with death were identified in the ALGS group. A survival analysis revealed that biliary (P = 0.02), vascular (P < 0.001), central nervous system (CNS; P < 0.001), and renal complications (P < 0.001) after LT were associated with death in the ALGS group. Renal insufficiency in the ALGS patients worsened after LT, and at 1 year, GFR was <90 mL/minute/1.73 m(2) in 22% of the LT patients with ALGS but in only 8% of the patients with BA (P = 0.0014). More LT pediatric patients with ALGS either were currently receiving special education (50% versus 30% for BA patients, P = 0.02) or had received special education in the past (60% versus 36%, P = 0.01). Vascular, CNS, and renal complications were increased in the ALGS patients after LT, and this reflected multisystem involvement. Although the 1-year survival rate was modestly lower for the ALGS patients versus the BA patients, the clustering of deaths within the first 30 days is notable and warrants increased vigilance and further investigation.     

Outcomes in patients with hepatopulmonary syndrome undergoing liver transplantation

Hepatopulmonary syndrome (HPS) is a condition of significant hypoxia due to intrapulmonary shunting (IPS) in patients with advanced liver disease. Reversibility of HPS after liver transplantation (LT) has been suggested, but the results of LT for HPS remain poorly defined. We studied 78 patients with decompensated liver disease who underwent LT after a preoperative evaluation including contrast echocardiography. We compared the baseline characteristics and outcomes after LT in patients with HPS (n = 13) with those of patients without HPS (n = 65, controls). Before LT, prolongation of prothrombin time was more severe and an advanced Child-Pugh class were more frequent among HPS, patients compared with controls (INR 2.5 +/- 0.8 vs 1.9 +/- 0.7, P = .01; Child-Pugh class A:B:C = 0%:31%:69% vs 14%:65%:21%, P < .01). After LT, no significant differences were observed between the two groups in: clinical outcomes, duration of endotracheal intubation (4.5 +/- 7.7 vs 4.4 +/- 15.0 days), duration of intensive care unit stay (12.0 +/- 8.7 vs 14.4 +/- 19.4 days), duration of total hospital stay (40.0 +/- 33.5 vs 39.8 +/- 23.0), rate of pulmonary complications (7.7% vs 9.2%), or 3-month survival rates (92.3% vs 86.1%). These findings suggest that the presence of HPS does not significantly affect LT outcomes in patients with decompensated liver disease.     

Multifocal biliary papillomatosis: an indication for liver transplantation

Biliary papillomatosis is a uncommon disease. Because of the high rate of recurrence and the possibility of malignant transformation, liver resection or transplantation was recommended. A case of diffuse bilobar biliary papillomatosis, in a 60 years old patient, responsible for cholangitis, cholestasis and for high portal pressure (esophageal varices grade I and II and hypersplenisme) is reported. The patient had had an external biliary drainage leading to an great loss of hydroelectrolytic component important. Opacification and biopsies under endoscopic control assert the right diagnosis. He was treated by a orthotopic liver transplantation. Post operative course was simple. In the 9th month, it was asymptomatic with a completely satisfactory evolution. The other therapeutic modalities was discussed, as well as the review of the literature.     

布—加综合征的围手术期处理体会

手术治疗布加综合征５０２例。围手术期处理措施包括：①术前进行下腔静脉与肝静脉造影，以选择适当术式；纠正营养不良、贫血及肝功能异常。②术中经上腔静脉属支建立输液通路；自体血回输；解除下腔静脉阻塞后，应逐渐增大回心血量，以防心衰；病变远心侧血栓应彻底取出，以防肺动脉栓塞。③术后应用抗凝祛聚药物。结果显示，手术治疗的有效率８５７％，死于围手术期并发症１４例（２８％）。提示加强围手术期处理对减少并发症、提高疗效，具有重要意义。     

Surgical limitation for biliary atresia: indication for liver transplantation

Of 245 patients undergoing corrective operations for biliary atresia, jaundice was cleared in 113. In January 1988, 84 of them were living and free of jaundice and the other 19 were alive with jaundice. A vast majority of long-term survivors showed normal growth and development, and were leading normal lives for their respective ages. Portal hypertension, a common late complication, improved spontaneously or after sclerotherapy in jaundice-free patients. Therefore, liver transplantation is not recommended for jaundice-free patients even with esophageal varices. Patients with persistent severe jaundice (serum bilirubin over 10 mg/dL) and those with moderate jaundice (serum bilirubin 5 to 10 mg/dL) and severe esophageal varices require liver transplantation. Patients with moderate jaundice having no or slight varices should be carefully followed. When varices become worse or serum bilirubin rises, liver transplantation is indicated. Patients with mild jaundice (serum bilirubin lower than 5 mg/dL) have a possibility of improvement in their condition before the age of 15 years, and are not recommended for liver transplantation. The high value of the lowest postoperative bilirubin level suggests the necessity of liver transplantation in early childhood. Liver transplantation as the primary treatment for biliary atresia may be indicated only for patients over 120 days of age with an enlarged and hard liver.