SPECT imaging in the diagnosis of Budd-Chiari syndrome

We recently performed planar and single photon emission computed tomographic (SPECT) liver/spleen scintigraphy in a patient with Budd-Chiari syndrome. While planar imaging revealed only nonspecific hepatocellular dysfunction, tomographic images demonstrated increased radiotracer accumulation in the caudate lobe of the liver, the classical scintigraphic finding in this entity. This case suggests that SPECT may enhance the sensitivity of the scintigraphic diagnosis of this uncommon condition, especially when the occlusion of the hepatic veins is incomplete.     

CT diagnosis of primary Budd-Chiari syndrome--membranous obstruction of the inferior vena cava

Five cases with primary Budd-Chiari syndrome due to membranous obstruction of the hepatic segment of the inferior vena cava were examined by CT. In all cases, CT demonstrated caudate lobe enlargement, reticular low density within the liver parenchyma, splenomegaly, and collaterals via the ascending lumbar veins and azygous system. Pathological study revealed liver cirrhosis or fibrosis in all cases. In two cases, calcification was shown in the region of the hepatic segment of the inferior vena cava. Our results suggested that the CT appearance of primary Budd-Chiari syndrome was rather characteristic and useful in diagnosis, although membranous obliteration could not be shown directly on CT.     

Hepatic outflow obstruction: enhancement patterns of the liver on MR angiography

Our purpose was to present the enhancement patterns of the liver on MR angiography in patients with hepatic outflow obstruction. Twenty-three patients with Budd-Chiari syndrome (4 in acute stage and 19 in chronic stage of the disease) were examined with 3D contrast-enhanced MR angiography. During early and late portal venous phase of MR angiography the pattern of parenchymal enhancement was assessed on source images. The enhancement patterns were evaluated under 4 groups as following: (a) central (b) peripheral (c) patchy and (d) homogeneous enhancement. The morphologic changes in the liver (lobar hypertrophy or atrophy, hepatic surface irregularities) were also recorded. In the acute stage global liver enlargement (75%) with caudate hypertrophy (100%) and central enhancement of the liver (75%) were suggestive findings of the hepatic outflow obstruction. The left lobe hypertrophy (53%) associated with the caudate lobe hypertrophy (72%) and irregular surface (26%) were predominant in the chronic stage of the disease. The enhancement patterns seen in chronic disease were variable and reflected the persistent stasis of the portal blood flow (patchy enhancement in 32% of the patients) or the altered hemodynamics of the liver due to the development of subcapsular collaterals (peripheral enhancement in 21% of the patients). Homogeneous enhancement of the liver in Budd-Chiari syndrome may indicate the chronicity of the outflow obstruction (37%) and shows a more stable hepatic perfusion that occurs after the formation of intra and extrahepatic collateral veins. The morphological and perfusional features on multiphase contrast-enhanced MR angiography are valuable in understanding the effects of the hepatic outflow obstruction on the liver parenchyma.     

Liver scan in Budd-Chiari syndrome.

Seventeen liver scans were performed in seven patients with occulsion of hepatic veins: the Budd-Chiari syndrome. When some, but not all liver veins were occluded, markedly diminished uptake over the affected segments was usually seen (absence of uptake may occur in acute infarction and in chronic cases). When all major liver veins are occluded, markedly diminished uptake is seen over the peripheral parts of the right and left lobes with a triangular midline area of normal or excessive activity. The latter effect is probably caused by uptake in segments surrounding the inferior vena cava (frequently the caudate lobe) that have direct venous drainage. This liver-scan appearance is characteristic enough to warrant consideration of the Budd-Chiari syndrome as the first diagnosis. Good correlation existed between selective venography and liver-scan findings. The usefulness of liver scans in the followup after portocaval shunting is illustrated in one patient.     

Pseudo-Budd-Chiari syndrome

A patient with clinical, laboratory, radiographic and scintigraphic findings resembling the Budd-Chiari syndrome is described. However, at autopsy there was no thrombotic occlusion of any of the intrahepatic veins. The right hepatic vein was completely constricted by the hypertrophied left lobe of the liver, and the left hepatic vein was narrowed. We have referred to this clinical entity as the pseudo-Budd-Chiari syndrome. It represents a potentially treatable form of the Budd-Chiari syndrome.     

布加综合征的CT和MRI诊断

布加综合征(BCS)是一种因肝静脉流出道阻塞而导致的少见病变,本文对BCSCT和MRI表现进行了综述,CT和MRI检查可以显示直接征象即静脉阻塞的部位和范围,对于肝内外的侧支血管、肝尾叶增大、肝脏不均匀强化、再生结节等静脉阻塞间接表现也能较好显示,认识这些表现有助于病变的早期诊断和选择合适的治疗方法。     

布-加综合征的CT诊断

目的探讨布-加综合征的CT影像特征及CT诊断价值。方法对11例经DSA证实的布-加综合征及2例误诊为布-加综合征的病例的CT图像进行回顾性分析。结果肝脏体积增大伴尾叶增大9例,下腔静脉病变11例,肝静脉病变3例。结论CT对下腔静脉病变导致的布-加综合征有较高的诊断价值,但对肝静脉病变的诊断价值有限。     

Budd-Chiari syndrome: spectrum of imaging findings

OBJECTIVE: The objective of our study was to illustrate the imaging findings of Budd-Chiari syndrome, including CT, MRI, sonographic, and angiographic findings. CONCLUSION: The key imaging findings in Budd-Chiari syndrome are occlusion of the hepatic veins, inferior vena cava, or both; caudate lobe enlargement; inhomogeneous liver enhancement; and the presence of intrahepatic collateral vessels and hypervascular nodules. Awareness of these findings is important for early diagnosis and appropriate treatment.     

Technetium-99m-medronate uptake in hepatic necrosis associated with Budd-Chiari syndrome.

A 99mTc-MDP bone scan performed on a 52-yr-old female for possible bone metastasis revealed prominent hepatic uptake. Subsequently, a 99mTc-SC scan revealed tracer uptake in the caudate lobe with diminished uptake in the remainder of the liver. Further imaging with Doppler ultrasound and hepatic venography confirmed a diagnosis of Budd-Chiari syndrome. Hepatic necrosis, demonstrated on CT imaging, was secondary to Budd-Chiari syndrome and was felt to be the cause of 99mTc-MDP hepatic uptake in this patient.     

Budd-Chiari syndrome: US evaluation

Twelve patients with proved Budd-Chiari syndrome (eight acute and four chronic cases) were examined, using real-time ultrasonography (US). In all acute cases, US study showed at least one hepatic vein with findings suggestive of the syndrome, such as stenosis, dilatation, thick wall echoes, thrombosis, abnormal course, or extrahepatic anastomosis. In chronic cases, hepatic veins were usually not visible. Modifications of liver morphology were present in all patients except those with recent onset of the disease. Caudate lobe hypertrophy was present in only six cases. US study is therefore the procedure of choice for initial diagnosis of acute Budd-Chiari syndrome. Pitfalls were the failure to detect two caval thromboses and one hepatic vein web. Cavography should still be performed systematically, but hepatic phlebography is useful in selected cases only.     

Budd-Chiari综合征的MRI诊断

目的 探讨ＭＲＩ对Ｂｕｄｄ－Ｃｈｉａｒｉ综合征的诊断价值。材料与方法 分析１９例Ｂｕｄｄ－Ｃｈｉａｒｉ综合征的ＭＲＩ表现,并与超声和静脉造影进行对比。结果 肝脏增大１８例（９５％）,肝尾叶增大１４例（７４％）,肝脏信号不均匀１４例（７４％）,７例（３７％）显示下腔静脉阻塞,４例（２１％）下腔静脉内见有血栓,肝静脉狭窄或阻塞１９例（１００％）,１７例（８９％）显示有肝内侧支血管,１７例（８９％）显示     

Budd-Chiari综合征的磁共振影像分析

目的：探讨MRI对Budd-Chiari综合征的诊断价值.方法：回顾性分析24例经DSA证实的Budd-Chiari综合征MRI表现.所有病例均采用FSPGR T1和FRFSE T2序列扫描.其中12例行FSPGR T1动态增强扫描.结果：肝静脉型6例,下腔静脉型8例,混合型10例,其中合并肝静脉或下腔静脉血栓6例.急性6例,亚急性6例,慢性12例.所有病例均有不同程度的肝肿大.肝脏尾叶增大20例,肝实质信号不均匀12例,肝内侧支血管17例,副肝静脉2例,肝外侧枝血管16例,脾大腹水16例,合并肝癌和脾梗死各1例.结论：MR能直接显示下腔静脉和肝静脉的狭窄,明确诊断并分型.并能根据不同时期信号特点和侧支血管分布的影像特征对其进行分期,判断疾病的病程和预后.MR是一个独立的非侵袭性的多方面评价Budd-Chiari综合征的方法.     

Hepatic caudate vein in Budd-Chiari syndrome: Depiction by using magnetic resonance imaging

Objective

To study the visibility of the caudate vein and its diameter on MR imaging in healthy people and in patients with Budd-Chiari syndrome.

Materials and methods

In this study there were 14 patients with Budd-Chiari syndrome and 54 healthy subjects without hepatic lesion or liver disease, all of whom had upper abdominal enhanced MRI. The visibility of the caudate vein and its diameter on MR images was compared between Budd-Chiari patients and healthy subjects, and among Budd-Chiari patients, the correlation between the visibility of caudate vein and extrahepatic collaterals were compared.

Results

Caudate vein was noted in 64% of patients with Budd-Chiari syndrome and in 7% of healthy subjects (P = 0.000). The diameter of the caudate vein visualized on MR imaging in Budd-Chiari syndrome was significantly larger than that in healthy group (7.3 ± 3.9 mm vs 2.6 ± 0.6 mm, P = 0.037). Among Budd-Chiari patients, both caudate vein and extrahepatic collateral veins were noted in 9 patients, only extrahepatic collateral veins were noted in 4 patients and neither caudate vein nor extrahepatic collateral veins were noted in 1 patient. No correlation was found between the visibility of caudate vein and that of extrahepatic collateral vein in patients with Budd-Chiari (P = 0.375).

Conclusion

Gadolinium enhanced dynamic MR imaging can visualize hepatic caudate vein frequently. The visibility and dilation of hepatic caudate veins on MR imaging in Budd-Chiari syndrome were more frequent than in control subjects. MR depiction of a caudate vein may help differentiate Budd-Chiari from cirrhosis.     

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.     

Budd-Chiari综合征的磁共振诊断

目的：探讨柏一查氏综合征（Ｂｕｄｄ－Ｃｈｉａｒｉｓｙｎｄｒｏｍｅ）的磁共振（ＭＲ）表现,分析比较各种检查方法。方法：８例本病患者的磁共振成像检查,采用ＳＥ、ＦＳＥ和ＧＲ序列,轴位,冠状位和矢状位扫描,其中２例做了磁共振血管造影（ＭＲＡ）。结果：ＭＲ表现为肝静脉和／或下腔静脉（ＩＶＣ）狭窄、阻塞和栓塞及“逗点样”肝内侧支循环;肝脏充血肿大或尾叶代偿性增大;脾大;门脉增粗;腹水和肝外侧支循环。结论：Ｂｕｄｄ－Ｃｈｉａｒｉ综合征在ＭＲ上有较为典型的表现,ＭＲ是目前诊断该病的最佳选择     

Angiographic abnormalities in partial Budd-Chiari syndrome.

Partial Budd-Chiari syndrome was produced in 8 monkeys by obstructing 2 or 3 lobar hepatic veins with a rapidly polymerizing plastic. Hepatic angiography done 1-3 months later revealed crowded tortuous arteries and a dense hepatogram in the lobe with venous occlusion. Unobstructed lobes were hypertrophied. Retrograde injection of persistently patent hepatic veins demonstrated a "spiderweb" collateral system. Retrograde venography to demonstrate this pathognomonic plexus is the procedure of choice when partial Budd-Chiari syndrome is suspected.     

Budd-Chiari syndrome: dynamic CT

A retrospective multi-institutional study was carried out on a series of 38 patients with histologically proved Budd-Chiari syndrome: Five patients had acute disease, and 33 had subacute or chronic disease. All patients underwent dynamic CT scanning. Angiography was performed in 20 cases, inferior cavography in 22, and wedge-hepatic venography in 16. In all acute cases, CT showed global liver enlargement with diffuse hypodensity on plain scans and patchy enhancement after contrast material injection. Thrombosis of the three main hepatic veins was always demonstrated. In subacute or chronic disease, plain CT scans showed abnormalities of liver morphology and hypodensity either in atrophic areas (19 cases) or in the periphery of the liver (eight cases). With dynamic CT, patchy enhancement was present in 28 cases. Correlation with angiography in 15 cases revealed a normal portal blood flow in enhanced areas and an inversed portal blood flow in atrophic areas. Different morphologic and enhancement patterns on CT scans could be related to the direction of portal blood flow, which changes with different stages of Budd-Chiari syndrome.     

MRI of the Budd-Chiari syndrome

Five of six patients with angiographically proved Budd-Chiari syndrome (hepatic venous outflow obstruction) showed multiple specific MRI abnormalities: striking reduction in caliber or complete absence of the hepatic veins, "comma-shaped" intrahepatic collateral vessels, and/or marked constriction of the intrahepatic inferior vena cava. The sixth patient had angiographically proven sinusoidal hepatic venous obstruction and patent central hepatic veins; MRI showed ascites but revealed no specific features of the Budd-Chiari syndrome. Patients with end-stage cirrhosis also showed compressed, distorted hepatic veins; however, these cirrhotic livers were distinguished by their small size, nodular surface, and extrahepatic collateral varices. In patients without cirrhosis or the Budd-Chiari syndrome, normal hepatic, portal, and inferior caval veins were routinely seen when technically adequate MRI examinations were obtained (94 of 100 cases). Four of the six patients with Budd-Chiari syndrome had been treated surgically. In three, MRI identified patent portocaval shunts. In the fourth, angiographically confirmed shunt stenosis was demonstrated by MRI.     

三维对比剂增强MR血管成像诊断布加综合征的价值

目的观察三维对比剂增强MR血管成像（3DCEMRA）上布加综合征（BCS）的各种表现,并初步评价该项新技术的价值。方法33例BCS患者行3DCEMRA检查。23例为继发性BCS,分别继发于肝细胞癌（21例）、右肾上腺癌（1例）或血栓性静脉炎（1例）。10例为原发性BCS。观察肝静脉、下腔静脉（IVC）和门静脉的开放性,观察有无肝内外侧支、肝实质病变和门静脉一体静脉间曲张静脉。10例患者行下腔静脉造影术,2例行肝右静脉穿刺造影术,把3DCEMRA所获的诊断结果与造影相对照。结果3DCEMRA可显示BCS的各种表现。肝静脉表现包括：癌栓形成（19例）、肿瘤压迫（2例）、肝静脉未显示（4例）和局限性狭窄（4例）。IVC表现为严重狭窄或闭塞（10例）、肿瘤直接侵犯（2例）、癌栓形成（3例）、血栓性静脉炎（1例）和隔膜形成（3例）。9例显示肝内侧支形成,其中2例显示“蜘蛛网”征象。所见的肝外侧支包括扩张的奇静脉和半奇静脉（13例）,以及左肾一膈下一心包膈静脉侧支（2例）形成。2例患者发现门静脉左支闭塞,10例患者发生门静脉．体静脉间静脉曲张。3DCEMRA发现的肝实质病变有：尾叶增大（7例）、不均匀强化（18例）和并发肿瘤（18例）。12例3DCEMRA诊断结果均与造影结果一致。结论3DCEMRA能显示BCS的各种征象,并能帮助提供正确诊断。     

Budd-Chiari综合征的CT与超声、静脉造影对比研究

目的：评价ＣＴ、超声、静脉造影对Ｂｕｄｄ－Ｃｈｉａｒｉ综合征的诊断价值。方法：回顾性分析４４例Ｂｕｄｄ－Ｃｈｉａｒｉ综合征的ＣＴ、超声、静脉造影表现,比较三种检查方法显示肝脏的形态、肝静脉、下腔静脉、肝内外的侧枝血管情况。结果：ＣＴ显示肝尾叶增大４２例（９５％）,肝脏密度不均或呈低密度表现,１９例（４３％）显示有肝内侧枝血管,４２例（９５％）见有肝外侧枝血管,其中奇静脉扩张３５例（８０％）,半奇静脉扩张３９例（８９％）,下腔静脉钙化７例（１６％）。超声显示３８例（８６％）有下腔静脉狭窄或阻塞,１６例（３６％）有肝静脉狭窄或阻塞,３７例（８４％）显示有肝内侧枝血管,１６例（３６％）显示有肝外侧枝血管。静脉造影显示下腔静脉狭窄或阻塞２３例（５３％）,肝静脉狭窄或阻塞５例（１１％）,其余１６例（３６％）同时累及下腔静脉和肝静脉,３８例（８４％）显示有肝内侧枝血管,４４例（１００％）见有肝外侧枝血管。结论：ＣＴ对显示肝脏形态、下腔静脉钙化、肝外侧枝血管尤其是奇静脉和半奇静脉扩张有优势,超声则对显示下腔静脉和肝静脉狭窄或阻塞、肝内侧枝血管有优势,ＣＴ、超声和静脉造影相互补充有助于本病的正确诊断