节段性狭窄闭塞布-加综合征的介入治疗

目的　探讨布 加综合征 (BCS)下腔静脉节段性狭窄、闭塞合并肝静脉阻塞的介入治疗。方法　 2 8例节段性狭窄、节段闭塞患者 ,多数合并 1～ 3支肝静脉闭塞 ,无合并下腔静脉血栓形成。应用球囊导管或房间隔穿刺针行下腔静脉开通术 ,下腔静脉开通后用 2 .0～ 2 .3cm球囊扩张后放入金属内支架。术后抗凝治疗 2年。结果　 2 7例患者成功进行了经皮穿刺球囊破膜扩张 ,即经皮腔内血管成形术 (PTA)和血管内支架植入治疗。闭塞肝静脉多数扩张下腔静脉后同时开通。患者肝、脾缩小 ,腹水吸收。 1例穿破下腔静脉急诊手术止血。随访 2～ 36个月未见复发及消化道出血。结论　下腔静脉狭窄者注意有肝静脉闭塞 ;下腔静脉闭塞破膜后 ,2 0例肝静脉闭塞者 19例随之开放 ,无需特殊处理 ;安放支架时注意保护肝静脉入口。     

Diagnosis of Budd-Chiari syndrome: three-dimensional dynamic contrast enhanced magnetic resonance angiography

Objective

To investigate the characteristics and diagnostic value of three-dimensional contrast-enhanced magnetic resonance angiography (3D CE-MRA) in the diagnosis of Budd?CChiari syndrome (BCS).

Methods

One hundred thirty-three BCS patients underwent 3D CE-MRA, 64 patients had primary BCS, and 69 had secondary BCS.

Results

Fifty five cases (41.4%) showed a segmental stenosis of the inferior vena cava, 9 cases (6.8%) a membranous obstruction of the inferior vena cava, 5 cases (3.8%) an unobstructed inferior vena cava and hepatic veno-occlusive condition, 16 cases (12.0%) an inferior vena cava stenosis and hepatic veno-occlusive disease, and 48 cases (36.1%) an intraluminal filling defect in the inferior vena cava. In 52 cases (39.1%), collateral blood vessels were formed, with deep, medium, and shallow portal veins and intrahepatic collateral veins in 88 groups. Among these, 41 (46.6%) had deep venous collateral channels, 24 (27.3%) had medium venous collateral channels, 9 (10.2%) had superficial venous collateral channels, 5 (5.7%) had portal vein collateral channels, and 9 (10.2%) had intrahepatic venous collateral channels.

Conclusion

3D CE-MRA is important in the clinical diagnosis and treatment planning of BCS and displays hepatic veins, the inferior vena cava system, and collateral vessels.     

微创时代下腔静脉开放手术的治疗机遇

因下腔静脉在解剖学及组织学方面的特性,治疗上具有特殊性。本文通过回顾既往文献报道,对下腔静脉综合征、平滑肌肉瘤及布加综合征等下腔静脉疾病外科治疗方式的进展进行论述。下腔静脉综合征为下腔静脉部分或完全阻塞所致的临床症候群,较常见且病因多样,占位性病变中下腔静脉平滑肌肉瘤发病罕见,根治性切除是目前唯一有效的治疗手段,其中下腔静脉及肾静脉的处理为手术难点。布加综合征表现为肝后性门脉高压,外科治疗包括手术治疗及介入治疗。     

Recurrent acute Budd–Chiari syndrome after right hepatectomy: US color-Doppler vascular pattern and left hepatic vein stenting for treatment

After extended right hepatectomy remnant liver can be affected by outflow obstruction due to torsion of the inferior vena cava or kinking of the left hepatic vein. Remnant liver fixation is therefore suggested to avoid postoperative acute Budd–Chiari syndrome. Despite remnant liver reposition during surgery, a 76-years-old woman developed complete outflow obstruction. This clinical situation, due to left hepatic vein kinking, was suspected by US examination and confirmed by CT scan that showed a pathological intrahepatic vascular pattern. Patient required urgent relaparotomy and the liver was replaced in normal position. However, recurrence of outflow obstruction occurred and it was ultimately treated by inferior vena cava angiogram with left hepatic vein stenting.     

Budd-Chiari syndrome: hepatic venous obstruction by an elevated diaphragm

We describe two cases of Budd-Chiari syndrome detected by magnetic resonance imaging that resulted from compression of the inferior vena cava by an elevated right hemidiaphragm. Magnetic resonance images demonstrated elevation of the right hemidiaphragm and medial deviation of the inferior vena cava with short segmental narrowing. The hepatic veins and inferior vena cava were patent but discontinuous. Hepatic venous drainage was assisted by multiple large intrahepatic collaterals. Received: 13 April 1998/Accepted: 3 June 1998     

布-加综合征肝脏病理学与肝静脉和下腔静脉阻塞类型的相关性研究

目的:探讨布-加综合征肝脏病理学改变与肝静脉、下腔静脉病变的关系。方法:27例布-加综合征患者术前行彩超检查,术中常规行下腔静脉造影和选择性肝静脉造影或经皮肝穿刺肝静脉造影了解肝静脉、下腔静脉阻塞情况;影像引导下经皮肝穿刺组织活检了解肝脏病理学变化。根据病变累及血管分为肝静脉型、下腔静脉型、肝静脉和下腔静脉混合型;根据血管阻塞程度将其分为狭窄型和完全闭塞型。将肝脏病理学改变与肝静脉、下腔静脉阻塞情况进行相关性分析。结果:25例穿刺组织达满意组织学诊断要求,其中肝静脉阻塞型11例,下腔静脉阻塞型4例,肝静脉和下腔静脉混合阻塞型10例;狭窄型7例,完全闭塞型18例。肝纤维化程度与血管阻塞程度密切相关;肝血窦扩张和肝细胞变性与血管阻塞程度无关,肝脏病变与阻塞部位无相关性。结论:肝静脉和下腔静脉阻塞对肝脏的损害程度是一致的,血管阻塞程度较阻塞部位对肝脏损害的影响更大。     

Budd-Chiari syndrome associated with liver cirrhosis: A case report

BACKGROUNDBudd-Chiari syndrome (BCS) is a rare heterogeneous liver disease characterized by obstruction of the hepatic venous outflow tract. The incidence of BCS is so low that it is difficult to detect in general practice and difficult to include within the scope of routine diagnosis. The clinical manifestations of BCS are not specific; hence, BCS tends to be misdiagnosed.CASE SUMMARYWe report the case of a 33-year-old Chinese woman who presented with progressive distension in the upper abdomen. She was initially misdiagnosed with liver cirrhosis (LC) due to abnormalities on an upper abdominal computed tomography scan. Although she was taking standard anti-cirrhosis therapy, her symptoms did not improve. Magnetic resonance imaging showed caudate lobe hypertrophy; and dilated lumbar and hemiazygos veins. Venography revealed membranous obstruction of the inferior vena cava owing to congenital vascular malformation. A definitive diagnosis of BCS was made. Balloon angioplasty was performed to recanalize the obstructed inferior vena cava and the patient’s symptoms were completely resolved.CONCLUSIONBCS lacks specific clinical features and can eventually lead to LC. Clinicians and radiologists must carefully differentiate BCS from LC. Correct diagnosis and timely treatment are vital to the patient''s health.     

Tunnelled central venous catheter-induced thrombosis: a rare case of superior vena cava syndrome

Thrombotic venous obstruction in patients with a tunnelled central venous catheter is a cause of superior vena cava syndrome that is not routinely encountered by emergency physicians. Diagnosis requires identifying patients at risk (e.g., those under treatment for cancer and those who have a tunnelled central venous catheter), recognizing the signs and symptoms of superior vena cava syndrome, usually dyspnea and dilated neck or thoracic veins, and imaging the venous obstruction using computer tomography or sonography. Management involves anticoagulation and local thrombolytic administration. We report the case of a 28-year-old woman who presented with a 2-day history of face, chest and bilateral arm swelling who had been receiving maintenance chemotherapy for acute lymphoblastic leukemia through a Hickman catheter. This case demonstrates the need to be vigilant for thrombus formation in patients with long-term, indwelling central venous catheters.     

Embryogenetic malformations of the inferior vena cava, and their manifestations in adults

Five male patients with congenital anomalies of the inferior vena cava aged 20 to 43 were examined. The diagnosis and the level of aplasia were established on the basis of complex instrumental examination (duplex scanning of inferior extremity veins, pelvic veins, and retroperitoneal veins; computed and magnetic resonance tomography of the abdominal cavity; pelvic phlebography; retrograde cavagraphy). Together with inferior vena cava anomalies, other malformations such as pulmonary arterial stenosis or duplication of renal collector system were diagnosed in two patients. In three or 60% of the patients the disease had first manifested by the clinical picture of peripheral thrombosis (shin and femoral edema); fever, chill and subsequent edema of both legs had been first manifestations in two patients. Aplasia of the infrarenal segment of the inferior vena cava was revealed in two patients; in other two patients aplasia of the infrarenal, renal, and partly suprarenal segments of this magistral vessel was found; in one patient the whole vein was aplastic except a small part of the suprarenal segment, where the hepatic veins and the right suprapolar renal vein flew into. To establish an early diagnosis of a congenital inferior vena cava anomaly, the protocol of examination of patients with venous diseases should include ultrasound mapping of the suprarenal and infrarenal segments of the vena cava; in cases of agenesia it should include computed and magnetic resonance tomography and retrograde cavagraphy.     

Computed tomography of hepatic venous hypertension: The reticulated-mosaic pattern

A reticulated-mosaic pattern of the liver was identified on contrast-enhanced computed tomography in 4 of 20 patients with constrictive pericarditis or congestive heart failure. Reflux of contrast into a distended inferior vena cava and the hepatic veins was identified in 3 of the 4 patients. This abnormal enhancement pattern combined with hepatic venous or caval reflux of contrast indicates the presence of hepatic venous hypertension, and should not be mistaken for other abnormalities that may result in inhomogeneous hepatic enhancement.     

多层螺旋CT血管成像评价肝移植受者移植前后血管结构

背景:多层螺旋CT具有先进的扫描技术和强大的图像后处理功能,其亚秒级的扫描速度及各向同性的成像功能,在血管成像上有很多优势.目的:探讨多层螺旋CT血管成像技术对肝移植受者移植前后血管结构评价的应用价值.设计、时间及地点:回顾性分析,以肝移植受者肝脏血管为观察对象,自身对照观察,于2003-10/2006-12在中山大学附属第五医院放射科完成.对象:选择拟行原位肝移植患者27例,年龄31～67岁,男17例,女10例.方法:轴位螺旋扫描范围为膈顶上方2 cm至双肾下极以下.电压120 kV,动脉期准直0.75 mm,平扫、门脉期、肝静脉期准直1.5 cm;使用非离子型对比剂优维显,注射速度为3 mL/s,三期增强扫描延迟时间分别为20～25 s,50～55 s,75～80 s.重建增强的薄层图像序列,动脉期层厚1 mm,间隔0.7 mm;门脉期、肝静脉期层厚2 mm,间隔1 mm.将图像序列传入后处理工作站,以MIP、VR等方式显示腹腔干系、门脉系、肝静脉及下腔静脉血管的三维结构.主要观察指标:肝移植前后患者血管三维结构.结果:移植前所有患者腹腔干系均得到良好显示,21例肝动脉走形正常,6例患者肝动脉变异,其中门静脉海绵样变2例,门脉多发血栓1例,因不适宜手术此3例未进行肝移植.24例患者第二肝门及下腔静脉肝内段血管结构显示清楚,肝右静脉单独汇入下腔静脉、肝左静脉和肝中静脉先汇合后再注入下腔静脉18例,肝有静脉、肝中静脉及肝左静脉分别单独汇入下腔静脉6例,下腔静脉肝后段狭窄2例.17例肝移植后CT血管成像发现肝动脉吻合口部狭窄2例,门脉高压侧支迂曲缓解10例,另7例患者因检查费用问题选择B超检查.结论:多层螺旋CT血管成像能够清晰显示肝移植患者移植前后血管结构,对适宜手术的病例筛选、指导手术方案及移植后血管结构的评价具有重要意义.     

Cement-associated thrombotic embolism in the inferior vena cava and bilateral iliac veins after percutaneous vertebroplasty: a case report

Percutaneous vertebroplasty (PVP) was first reported in 1987 for treating vertebral hemangiomas. PVP is also an effective treatment for osteoporotic vertebral compression fracture that mainly involves a percutaneous injection of polymethylmethacrylate. Severe complications of PVP have been reported in recent years. However, to the best of our knowledge, cement-associated thrombotic embolism in the inferior vena cava and bilateral iliac veins after PVP has rarely been reported. We experienced a patient with cement-associated thrombotic embolism in the inferior vena cava and bilateral iliac veins after PVP. Fortunately, after conservative therapy, we achieved a satisfactory result. Unfortunately, we could not explain the cause of the cement leakage and formation of the thrombotic embolism. We believe that surgeons should have a better understanding of the fracture pattern and anatomy of the vertebral venous net system. They should also perform meticulous imaging monitoring with slower pushing of the cement to minimize the risks during the PVP. This case report highlights a rare, but potentially life-threatening, complication of PVP. Surgeons need to be aware of the possibility of cement leakage and the formation of cement-associated thrombotic embolism so that they are much more vigilant when performing PVP.     

自然组织谐波显像技术评价左室收缩功能

目的：评价自然组织谐波显像技术（ＮＴＨＩ）测量左室收缩功能的准确性。方法：利用测定２０例患者的左室容量和射血分数,并与左室造影的测值进行比较。结果：ＮＴＨＩ所测左室容量和射血分数与左室造影相应测值高度相关,左室舒张末期容量和收缩末期容量及射血分数的相关系数分别为０．８７,０．９６,０．９２。ＮＴＨＩ左室容量测值低估约３０％。结论：超声ＮＴＨＩ技术能准确可靠测定左室容量和射血分数,为评价左室收缩功能提供简便易行的方法。     

Foramen ovale: an ultrasonographic study of its relation to the inferior vena cava, ductus venosus and hepatic veins.

According to the literature, oxygenated blood from the ductus venosus and hepatic veins may either enter the right atrium before flowing through the foramen ovale to the left atrium, or flow directly from the ductus venosus and the hepatic veins to the foramen ovale, bypassing the right atrium. To address this problem, 103 normal fetuses were examined by two-dimensional imaging, M-mode and color Doppler at an average gestational age of 27 weeks (range, 15-40 weeks). The position of the ventricular septum and foramen ovale, and the angle and flow direction of the inferior vena cava, ductus venosus and hepatic veins were recorded. Two pathways for blood were described: a left ductus venosus-foramen ovale pathway that delivers blood directly to the foramen ovale circumventing the right atrium, and a right inferior vena cava-right atrium pathway that delivers blood into the right atrium through the right portion of the proximal inferior vena cava at an angle of 13 degrees to the long axis of the spine. The left and medial hepatic veins enter the left ductus venosus-foramen ovale pathway, and the right hepatic vein enters the right inferior vena cava-right atrium pathway. This supports the hypothesis that oxygenated blood from the ductus venosus and left hepatic veins flows directly through the foramen ovale to the left atrium avoiding extensive mixture in the inferior vena cava and an intermediate entrance to the right atrium.     

Obstruction of the inferior vena cava in the hepatic portion and hepatocellular carcinoma

High incidence of hepatocellular carcinoma in patients with obstruction of the inferior vena cava in the hepatic portion (Budd-Chiari syndrome) was previously pointed out by us from the review of Japanese literature of autopsied cases. This was confirmed by the follow-up study of 16 patients with obstruction of the inferior vena cava in the hepatic portion hospitalized in 1958 to 1974. Follow-up information was available on 13 of the 16 patients. Deaths due to hepatocellular carcinoma occurred in 6 patients (46%), and those due to other causes in 4 patients (31%). Three patients were alive. Hepatocellular carcinoma occurred most frequently in the patients who were found to have obstruction of the inferior vena cava at less than 44 years of age and were followed up for more than 10 years.     

Ultrasound examination of the liver: Normal vascular anatomy

Various treatments for liver diseases, including liver transplant (particularly partial liver resection from a living donor), treatment of liver tumors, and TIPS, require detailed knowledge of the complex vascular anatomy of the liver. The hepatic artery and portal vein provide the organ with a double blood supply whereas venous drainage is furnished by the hepatic veins.Multislice computed tomography and magnetic resonance imaging provide undeniably excellent information on these structures. On ultrasound, the inferior vena cava, the openings of the hepatic veins, and the main branch of the portal vein can always be visualized, but intrasegmental vessels (portal, arterial, accessory hepatic venous branches) can be only partially depicted and in some cases not at all.In spite of its difficulty and limitations, hepatic sonography is frequently unavoidable, particularly in critically ill patients, and the results are essential for defining diagnostic and therapeutic strategies. For this reason, a thorough knowledge of the sonographic features of hepatic vascular anatomy is indispensable.     

Experimental evaluation of portal venous pulsatile flow synchronized with heartbeat intervals: effects of vascular clamping on portal hemodynamics

Purpose

We previously showed that blood flow in the portal vein was pulsatile and influenced by both the inferior vena cava and the arterial system in a complex manner (Nihei et al., 38:141–149, 2011). The objective of the present study is to identify determinants of blood flow and to clarify the source of pulsatile flow in the portal vein.

Methods

Three-breed terminal crossbreed mini-pigs underwent general anesthesia. Pressure and flow in the portal vein, inferior vena cava, hepatic artery, and mesenteric artery were measured simultaneously. Vascular occluders were placed in the inferior vena cava, hepatic artery, and mesenteric artery to examine the effects of clamping on portal venous flow.

Results

Clamping of the mesenteric artery altered pressure and flow waves in the portal vein to waveforms similar to those in the inferior vena cava. Waves resembling those of the inferior vena cava superimposed on portal venous flow appeared later than waves of the inferior vena cava. Clamping of the inferior vena cava promptly altered portal venous pressure and flow. Because clamping of the inferior vena cava led to a sharp rise in portal venous pressure, detailed evaluations were not feasible. Clamping of the hepatic artery had no effect on flow-wave pulsation in the portal vein.

Conclusions

In the hepatic circulation, flow-wave pulsation in the portal vein is influenced by flow in the inferior vena cava via the sinusoids and by flow in the mesenteric artery via the capillary vessels of the intestine.     

Sonography in Budd-Chiari syndrome.

OBJECTIVE: The objective of this presentation is to provide an overview of sonographic manifestations of Budd-Chiari syndrome (BCS). METHODS: Patients were scanned with ultrasound systems using mainly a 2- to 5-MHz curvilinear transducer and in some patients a 5- to 12-MHz linear transducer. The patients were asked to fast from the previous night or for at least 6 hours. Color and spectral Doppler sonography was performed in all patients. RESULTS: Commonly seen findings in BCS include inferior vena cava (IVC) webs and thrombi, IVC narrowing, hepatic venous thrombosis, enlarged caudate lobes, ascites, intrahepatic or extrahepatic collaterals, monophasic to absent flow in the hepatic veins, and high flow velocities in areas of stenosis in the IVC or hepatic veins. Inferior vena cava stents used in the treatment of BCS could also be seen. CONCLUSIONS: Budd-Chiari syndrome is an uncommon disorder; outcome is poor in many cases; and the condition is often misdiagnosed or underdiagnosed. Sonography is a noninvasive and effective modality for diagnosis of BCS.     

Visualization of calf veins by color flow imaging

Both indirect and direct noninvasive tests have been shown to be useful for the detection of deep venous thrombi involving the proximal veins (popliteal to inferior vena cava). However, thrombi that are confined to the calf veins will not affect venous outflow and cannot be detected by the plethysmographic methods. B-mode imaging, while excellent for the major deep veins, is also limited in the calf region because the veins are small, and imaging resolution is not always adequate to permit visualization of these smaller veins. With the availability of duplex scanning with "slow flow" color capability, these veins lend themselves to study. In 30 normal subjects, we were able to visualize all paired veins from the level of the ankle to the popliteal fossa. The clinical implications of this approach are discussed.     

预先腹内控制下腔静脉防治肝切除术中肝静脉及下腔静脉损伤大出血

作者对7例半肝以上肝切除术及2例ⅦⅧ肝段切除应用止血带在腹腔内围绕肝下肾上及膈下肝上之下腔静脉,同时用橡皮管控制肝门血管,而暂不阻断,以预防术中可能发生的肝静脉或/和下腔静脉损伤大出血,其中2例还同时控制膈下腹腔动脉以上的腹主动脉。9例中7例无意外,2例术中发生肝中静脉损伤大出血,经止血带迅速控制,修补裂口,抢救成功。本文对肝叶切除术中误伤肝静脉或/和下腔静脉的原因和预防处理方法进行了讨论,认为在病变接近或累及第二肝门或下腔静脉的肝叶肝段切除,以及在半肝或三叶切除术中常规控制下腔静脉和肝门是一个简而有效的防治肝静脉或/和下腔静脉损伤大出血的好方法。