不典型Budd-Chiari综合征一例

病例资料 患者,男,48岁.皮肤黄染1个月人院,伴乏力,食欲不振,无肝炎,酗酒史,无长期服药史.查体:神清,巩膜黄染,胸腹壁、腰背部及下肢未见静脉曲张,肝大(肋下3 cm),质地韧,脾肋下可及,移动性浊音阴性,双下肢无水肿.CT检查:平扫示肝脏体积增大,以尾状叶增大明显,脾大,腹膜后奇静脉、半奇静脉明显扩张增粗;增强示肝脏淤血肿大,表面呈网格状改变,下腔静脉、肝右静脉明显扩张,奇静脉、半奇静脉增粗、纡曲,下腔静脉肝段未见明显狭窄及缺如(图1).CI诊断:考虑布-加综合征可能,建议下腔静脉DSA.     

血液透析患者中心静脉狭窄的介入治疗

目的探讨血液透析患者中心静脉狭窄介入治疗的方法及疗效。方法 2010年5月至2011年11月共收治20例发生中心静脉狭窄的血液透析患者,所有患者均具有中心静脉狭窄的临床症状、体征,并行MRA或CTA明确诊断,经静脉造影明确病变长度、范围和程度,行血管球囊扩张成形术(PTA)对病变部位进行治疗,成形术失败时,行血管支架植入术。术后随访患者的临床症状、MRA、超声等影像学检查。结果所有患者均成功行静脉造影,成功处理了20例患者共17条狭窄静脉,其中成功进行PTA 15例,支架植入成功2例,术后狭窄静脉血管通畅,侧支循环消失。首次PTA后再狭窄发生率为11.8%,再次行P1A。所有手术成功病例随访至今未出现再狭窄。结论血液透析患者中心静脉狭窄的介入治疗安全、有效,静脉通畅率良好。     

肝移植术后肝静脉和下腔静脉梗阻的诊断及介入治疗

目的 探讨肝移植术后肝静脉、下腔静脉梗阻的诊断及介入治疗技术.方法 在831例原位肝移植(OLT)、26例活体肝移植(LDLT)患者中,共有11例在移植术后2～111 d经血管造影证实为肝静脉、下腔静脉梗阻并进行了介入治疗.其中肝静脉吻合口狭窄或闭塞5例、下腔静脉吻合口狭窄5例、肝静脉狭窄伴下腔静脉吻合口狭窄1例.11例中,5例为成人OLT、4例为LDLT、2例为儿童减体积OLT,介入治疗前9例接受了肝脏CT、2例接受了MR增强扫描.术后随访患者肝肾功能指标、临床症状及肝静脉、下腔静脉血流状况.对11例患者的影像资料、介入治疗技术要点和治疗效果进行回顾性分析.介入治疗前后梗阻两端静脉压力差比较,采用配对t检验.结果 11例患者,CT或MR检查均可明确显示肝脏淤血范围、肝静脉或下腔静脉梗阻部位及程度;其中4例肝静脉梗阻和5例下腔静脉梗阻者行支架植入治疗,1例肝静脉梗阻者行经皮腔内血管球囊扩张术(PTA),1例肝静脉伴下腔静脉梗阻者,行肝静脉PTA和下腔静脉支架植入,介入治疗手术均成功.术后检测梗阻两端静脉压力差为(2.9±1.7)mm Hg(1 mm Hg=0.133 kPa),较术前(16.5±4.1)mm Hg明显下降(t=11.5,P＜0.01).术后10例患者临床症状改善,肝肾功能恢复;1例肝功能恶化,于术后第9天死于多器官功能衰竭.患者术后随访9～672 d,2例肝静脉PTA治疗者术后1个月内发生血管再狭窄,支架植入治疗者未发生再狭窄,无严重并发症发生.结论 支架植入是治疗肝移植术后肝静脉和下腔静脉梗阻安全、有效的方法;术前CT或MR对明确肝淤血范围及静脉梗阻具有重要价值.     

土三七致肝窦阻塞综合征的临床及CT特征研究

目的 探讨土三七致肝窦阻塞综合征(PA-HSOS)患者的临床、肝损伤及CT特征,为PA-HSOS的早期诊断和治疗奠定基础。方法 选取31例PA-HSOS患者的临床资料和CT图像、与同期收治的46例肝静脉型布加综合征(HVBCS)患者的资料行对比分析,观察PA-HSOS患者具有特征性的临床表现及CT征象。结果 PA-HSOS与HV-BCS患者相比,超急性/急性期多见,年龄较大,肝损伤程度更高。第二肝门处“爪形”强化、肝静脉和下腔静脉肝段变细但通畅、肝尾状叶体积不大、肝内侧枝循环少见、门静脉周围晕征为PA-HSOS患者的CT特征表现;而HV-BCS则呈第一肝门“扇形”强化,肝静脉可见狭窄、闭塞及充盈缺损,肝尾状叶增大多见,多伴肝内侧支循环开放,常见脾脏增大。结论 有服用土三七史、病程短、病情急、肝功能损伤重为PA-HSOS临床特征表现;第二肝门处“爪形”强化、肝静脉变细但通畅、肝尾状叶体积不大、肝内侧枝循环少见、门静脉周围晕征是其特征CT表现,易于鉴别HV-BCS。     

自展式血管内支架治疗Budd—Chiari综合征

笔者报告9例在经皮腔内血管成形术(PTA)基础上置入自展式血管内支架(stent)治疗 Budd-Chiari syndrome(BCS)的研究结果。下腔静脉支架7例,副肝右静脉支架1例,下腔静脉及肝左静脉双支架1例。下腔静脉平均压力术前为3.54±0.91kPa(1mmH_2o=0.098kPa),术后即刻降为2.025±0.98kPa,术后随访1～8个月(平均2.6个月),下腔静脉保持通畅,支架扩张良好,无移位。主要临床症状和体征消失者7例,明显改善者2例,无严重并发症发生。笔者还讨论了 BCS 内支架置入的适应症和副肝右静脉肝左静脉置入支架的临床意义。     

支架置入术治疗原发性肝癌并下腔静脉阻塞的近期疗效观察

目的:探讨支架置入术治疗原发性肝癌并下腔静脉阻塞的近期疗效。方法:回顾性分析对30例因原发性肝癌并下腔静脉阻塞行介入治疗的患者资料,其中24例仅行下腔静脉支架置入术,4例行下腔静脉及右心房联合支架置入术,2例行下腔静脉及肝静脉联合支架置入术。观察其近期疗效,并对其进行评价分析。结果:30例患者支架置入术均成功,未发现有任何严重手术并发症,术后患者Child-Push分级及临床症状较术前明显改善(P<0.05)。结论:支架置入术治疗肝癌并发下腔静脉阻塞的短期疗效显著,可使患者获得进一步积极治疗的机会。     

单纯球囊扩张成形术治疗肝静脉型Budd-Chiari综合征(附10例报告)

目的 评价经皮肝穿刺单纯球囊扩张成形术(PTA)在肝静脉型布-加综合征介入治疗中的应用价值和疗效.方法 10例肝静脉狭窄或闭塞的布-加综合征患者,采用B超引导下经皮肝静脉穿刺的方法,实施单纯肝静脉球囊扩张血管成形术.结果 10例患者在实施肝静脉球囊扩张血管成形术后效果显著,肝静脉狭窄闭塞处内径较前增宽,肝静脉压力下降,由PTA前平均32.6 cmH2O降到PTA后平均16.9 cmH2O,血流通畅.手术成功率100%.无严重并发症,术后腹水、肝功能损害、下肢水肿等症状均有明显改善.结论 经皮肝穿刺肝静脉单纯球囊扩张成形术对肝静脉狭窄或梗阻型布-加综合征患者症状的缓解有显著疗效.     

肝静脉型Budd-Chiari综合征的介入治疗现状

Budd-Chiarj综合征(Budd-Chiari syndrome,BCS)是由于肝静脉和(或)下腔静脉阻塞导致肝静脉和(或)下腔静脉回流障碍而产生的门脉高压和(或)下腔静脉高压的一系列临床症状和体征.文献报道肝静脉型布加综合征占该病总数的5.0%～32.5%[1-2].由于各肝静脉之间广泛存在潜在的侧支循环,在肝静脉阻塞或狭窄后,肝内血管的解剖变得更为复杂,是BCS介入治疗中的难题之一.本文综述肝静脉型BCS的介入治疗.     

The Interventional Treatment on Budd Chiari Syndrome with Long Segmental Inferior Vena Cava Thrombosis and Upper Gastrointestinal Haemorrhage

目的 探讨Budd-Chiari综合征(BCS)合并下腔静脉长节段血栓及上消化道出血的介入治疗方法,评价下腔静脉多支架顺序置入固定血栓+下腔静脉成形术+肝静脉成形术的治疗价值.资料与方法 搜集2009年1月至2011年3月6例BCS合并下腔静脉长节段血栓及上消化道出血患者.上消化道出血控制稳定1周后,均以如下介入方法治疗:下腔静脉多支架顺序置入固定血栓+下腔静脉成形术+必要时肝静脉成形术.术后予以抗凝并随访.结果 6例患者以下腔静脉多支架顺序置入压迫血栓+下腔静脉成形术+必要时肝静脉成形术治疗,均获成功.近中期临床疗效明显.主要并发症为无症状性肺栓塞,共2例.无手术致死病例.结论 下腔静脉多支架顺序置入固定血栓+下腔静脉成形术+肝静脉成形术,对治疗BCS合并下腔静脉长节段血栓及上消化道出血是安全、有效的,可以作为BCS伴下腔静脉长节段血栓及上消化道出血的治疗选择.     

肝脏恶性肿瘤所致下腔静脉狭窄及阻塞的内支架治疗

目的：探讨肝脏恶性肿瘤所致下腔静脉狭窄或阻塞内支架置入术的疗效。材料和方法：108例肝脏恶性肿瘤所致下腔静脉狭窄或阻塞患者先作下腔静脉造影，用导丝通过狭窄段，置放Z型国产金属内支架，观察侧支循环、造影剂通过状况，测量狭窄段宽度及静脉压差。结果：108例均成功置入血管内支架，之后行经肝动脉化疗栓塞术。100例获良好血流动力改善，静脉压差明显降低，狭窄段宽度显著提高，侧支关闭；临床症状与体征在2－7天内减轻或消失；本组未出现明显并发症；80例随访6个月，支架保持通畅。结论：内支架置入术是治疗肝脏恶性肿瘤所致肝段下腔静脉狭窄的有效方法。     

Ultrasonically guided percutaneous transhepatic hepatic vein stent placement for Budd-Chiari syndrome.

PURPOSE: To evaluate the utility of ultrasonically guided hepatic vein stent placement in the treatment of Budd-Chiari syndrome (BCS) in patients with short hepatic vein obstruction. MATERIALS AND METHODS: Twenty-five patients with BCS, each with three obstructed hepatic veins diagnosed with ultrasound (US), color Doppler, probing with guide wire, and echo contrast, underwent hepatic vein stent placement under US guidance. Nine patients had hepatic vein obstruction alone, and 16 had hepatic vein obstruction along with primary inferior vena cava (IVC) obstruction. In each patient, only one of the hepatic veins was selected for recanalization and stent placement. In patients with primary IVC lesions, a stent was placed in the IVC first. Clinical and US examinations were performed at 3-6-month intervals on every patient during follow-up. RESULTS: Hepatic vein stents were successfully placed in 23 of the 25 patients, a success rate of 92%. The mean +/- SD hepatic vein pressure decreased from 25.57 mm Hg +/- 9.46 to 9.67 mm Hg +/- 2.31 (P < .01), and the flow direction in the hepatic vein became centripetal and its spectral analysis showed a normal phasic flow. Twenty-two patients experienced a significant improvement in hepatic outflow, as evidenced by disappearance of ascites, remission of hepatosplenomegaly, improvement in liver function, and alleviation of esophageal varices. Severe intraperitoneal hemorrhage occurred in one patient. No other serious procedure-related complications were observed. During follow-up of 1-43 months (mean, 23 months), stent reocclusion occurred in one patient. The other stents remained patent, and clinical features of BCS did not recur. CONCLUSION: Percutaneous transhepatic hepatic vein stent placement is a reasonable treatment for BCS in patients with hepatic vein obstruction, and the procedures can be performed safely and accurately with US.     

肝静脉阻塞型Budd－Chiari综合征的介入治疗（附10例报告）

肝静脉阻塞型Ｂｕｄｄ－Ｃｈｉａｒｉ综合征（ＢＣＳ）既是内外科临床治疗的“难治之症”，也是介入放射学领域尚未完全解决的课题。作者采用肝静脉开通术（ＰＴＡ及ＥＭＳ置入术等）和经颈静脉肝内门腔静脉内支架分流术（ＴＩＰＳＳ）对１０例肝静脉阻塞型ＢＣＳ进行了治疗，取得了满意的临床效果。作者认为：肝静脉开通术是治疗肝静脉口部狭窄或闭塞型ＢＣＳ的较为合理而且安全有效的非手术方法。尽管它较下腔静脉开通术的难度和风险更大些，但临床效果显著持久，因而，应将其作为此类ＢＣＳ的首选治疗方法。但对不适于此项治疗者，即肝静脉广泛阻塞型ＢＣＳ则可将ＴＩＰＳＳ作为主要的治疗手段。     

肝静脉阻塞的血管造影表现

目的 评价肝静脉阻塞的血管造影表现。方法 肝静脉阻塞患者45例，男23例，女22例；年龄9～54岁。全部病例均行下腔静脉造影和肝静脉造影。结果 肝静脉阻塞而下腔静脉通畅37例，肝静脉和下腔静脉同时阻塞8例。肝静脉阻塞而下腔静脉造影表现为肝内段下腔静脉局限性或普遍性狭窄31例，肝内段下腔静脉管径正常者12例，肝静脉开口处出现隔膜膨出征5例，副肝静脉开口处出现隔膜膨出征4例，肝静脉之间交通支形成45例。结论 下腔静脉造影能对肝静脉有无阻塞做出初步判断，隔膜膨出征是肝静脉和副肝静脉开口处膜性阻塞的直接征象，选择性肝静脉造影是诊断肝静脉阻塞的可靠依据。     

3D DCE MRA在诊断Budd-Chiari综合征中的应用价值

目的评价三维对比动态增强磁共振血管成像(3D DCE MRA)在诊断Budd-Chiari综合征(BCS)中的价值。方法8例BCS术前接受了常规MRI和3D DCE MRA检查,着重于回顾性分析其3D DCE MRA表现。结果8例3D DCE MRA检查显示下腔静脉阻塞3例,下腔静脉和肝静脉阻塞5例。下腔静脉阻塞部位在肝段5例、膈段1例及膈上段2例,阻塞表现形式包括膜性1例及节段性7例。伴有肝静脉阻塞的5例中肝静脉开口处阻塞3例,肝静脉分支阻塞2例。8例中有5例显示肝内侧枝循环,包括肝包膜下静脉侧枝循环、叶间静脉侧枝循环及肝内未定型侧枝循环。8例均显示肝外侧枝循环,包括深层静脉侧枝循环、中层静脉侧枝循环、浅层静脉侧枝循环及门静脉侧枝循环。8例BCS的其他征象包括肝实质信号异常、肝脏形态改变、下腔静脉内血栓形成、脾大、腹水及胸水。结论3D DCE MRA在诊断BCS中具有很高的价值,是诊断和治疗前后全面评价BCS的无创性血管成像技术。     

Application of expandable metallic stents to the venous system.

Expandable metallic stents were successfully introduced in 12 patients; 6 with superior vena cava (SVC) obstruction due to tumor invasion or lymph node metastases, 3 with inferior vena cava (IVC) obstruction or stenosis due to lymph node metastases or hepatic tumor, one with common iliac vein (CIV) obstruction due to lymph node metastases, one with idiopathic obstruction of the hepatic IVC and Budd-Chiari syndrome, and one with CIV obstruction following a dialysis shunt. The length of the lesions was between 2 and 15 cm. Multiple (2-7) stents in tandem were inserted percutaneously from a femoral venous approach through a 12 to 16 F (4.0-5.3 mm) Teflon sheath. Postoperatively, all 12 patients became free from symptoms such as SVC syndrome or IVC syndrome. In 11 patients, the symptoms did not recur during the follow-up periods of 1 to 21 months.     

3.0T磁共振对肝静脉型布-加氏综合征的诊断价值

目的:探讨3.0T磁共振对肝静脉型布-加氏综合征(HVBCS)的诊断价值。方法:57例HVBCS患者均行3.0T磁共振平扫及三维对比增强MR血管成像(3D CE MRA)检查,观察肝静脉(HV)改变、侧枝循环的建立及肝实质改变情况。全部患者均行HV造影证实,分析磁共振结果。结果:HV:共显示126支,其中HV广泛阻塞21支;开口阻塞93支;以上HV阻塞病例中有血栓形成6支;正常HV12支。以血管造影为金标准,磁共振显示HV灵敏度为91.3%,特异度为100%。副肝静脉(AHV):显示38支,其中17支开口狭窄。侧枝循环:肝内侧枝循环形成53例,4例无肝内侧枝循环;全部病例均有肝实质改变。结论:3.0T磁共振能准确显示HV病变,肝内侧枝循环,肝实质改变,在诊断HVBCS中有重要价值。     

Posttransplant Complex Inferior Venacava Balloon Dilatation After Hepatic Vein Stenting

Orthotopic and living related liver transplantation is an established mode of treatment of end-stage liver disease. One of the major causes of postoperative complications is vascular anastomotic stenosis. One such set of such complications relates to hepatic vein, inferior vena cava (IVC), or portal vein stenosis, with a reported incidence of 1–3%. The incidence of vascular complications is reported to be higher in living donor versus cadaveric liver transplants. We encountered a patient with hepatic venous outflow tract obstruction, where the hepatic vein had been previously stented, but the patient continued to have symptoms due to additional IVC obstruction. The patient required double-balloon dilatation of the IVC simultaneously from the internal jugular vein and IVC.     

Membranous obstruction of the inferior vena cava with Budd-Chiari syndrome: MR imaging findings.

Membranous obstruction of the inferior vena cava (IVC) is a curable cause of a primary type of Budd-Chiari syndrome. Magnetic resonance (MR) imaging and vena cavography were performed on nine patients with membranous obstruction of the IVC. The MR findings were retrospectively analyzed and compared with computed tomographic findings in seven patients. The morphologic features of membranous obstruction of the IVC on spin-echo MR images were a curvilinear soft-tissue membrane (five cases) or an obliterated lumen of a hepatic segment of the IVC (four cases) in transverse or sagittal views. The lumen below the obstruction revealed flow-related signal (seven cases), intraluminal thrombus (one case), and thrombotic occlusion (one case). The hepatic veins were narrow and disoriented without connection to the hepatic segment of the IVC just below the diaphragm. On T2-weighted images, inhomogeneity with high signal intensity was shown more prominently in the hepatic parenchyma in Simson type II or III membranous obstruction. Other findings were hepatosplenomegaly, enlarged caudate lobe, cirrhotic liver, associated hepatoma, and presence of various collaterals.     

肝静脉阻塞之下腔静脉造影表现及临床意义

目的：探讨肝静脉阻塞之下腔静脉造影征象及临床意义。方法：２３例肝静脉阻塞患者，采用股静脉穿刺插管行下腔静脉造影，分析肝静脉开口征象并指导临床治疗（ＰＴＡ和ＥＭＳ）。结果：２３例患者中，杯口征１０例，指压迹征３例，盲袋征４例，未显示６例。采用腔静脉途径开通肝静脉１４例。结论：识别阻塞肝静脉造影征象是介入治疗的关键。杯口征和指压迹征皆易开通，盲袋征和未显示者开通则较难     

Radionuclide venography as a clue to the diagnosis of Budd-Chiari syndrome

A patient was examined with radionuclide venography (RVG) to investigate unilateral leg oedema which might be due to deep vein thrombosis. RVG with Tc-99m MAA demonstrated no findings to suggest deep vein thrombosis of the right leg. However, collateral flow derived from the left common iliac vein and truncated inferior vena cava (IVC) were revealed. Contrast venography confirmed the obstruction of IVC and collateral flow from the left common iliac vein to the left ascending lumbar vein. It also showed the obstruction of hepatic veins and the patient was finally diagnosed as Budd-Chiari syndrome. Although unilateral leg oedema is an atypical symptom in Budd-Chiari syndrome, the findings on RVG led us to conduct further imaging studies to reach the diagnosis.