Portal biliopathy: a multitechnique imaging approach

Portal biliopathy (PB) is a disorder characterized by biliary ductal and gallbladder wall abnormalities seen in patients with extrahepatic portal vein obstruction. These abnormalities consist mainly of bile duct compression and tethering, stenoses, fibrotic strictures and dilatation of both extrahepatic and intrahepatic bile ducts, as well as gallbladder varices. In this pictorial essay, we describe the imaging findings of PB, which allow differentiation of this entity from other diseases that may have similar imaging findings including cholangiocarcinoma, extrinsic compression of the bile duct caused by metastatic adenopathy or sclerosing cholangitis.     

Biliary parasitic diseases including clonorchiasis,opisthorchiasis and fascioliasis

Parasitic infection of the biliary tree is caused by liver flukes, namely Clonorchis sinensis and Opisthorchis viverrini. These flukes reside in the peripheral small bile ducts of the liver and produce chronic inflammation of the bile duct, bile duct dilatation, mechanical obstruction, and bile duct wall thickening. On imaging, peripheral small intrahepatic bile ducts are dilated, but the large bile ducts and extrahepatic bile ducts are not dilated or slightly dilated. There is no visible caused of obstruction. Sometimes, in heavy infection, adult flukes are demonstrated on sonography, CT or MR cholangiography as small intraluminal lesions. The flukes in the gallbladder may appear as floating, small objects on sonography. Chronic infection may result in cholangiocarcinoma of the liver parenchyma or along the bile ducts. Human infection of Fasciola hepatica, a cattle flukes, may occur inadvertently, and the flukes migrate in the liver (hepatic phase) and reside the bile ducts (biliary phase). Image findings in the hepatic phase present with multiple, small, clustered, necrotic cavities or abscesses in the peripheral parts of the liver, showing “tunnels and caves” sign, reflecting parasite migration in the liver parenchyma. In the biliary phase, the flukes are demonstrated in the intra- and extrahepatic bile ducts and the gallbladder as small intraluminal flat objects, sometimes moving spontaneously. Bile ducts are dilated.     

Vascular and biliary abnormalities mimicking cholangiocarcinoma in patients with cavernous transformation of the portal vein: role of color Doppler sonography.

OBJECTIVE: The purpose of this study was to describe and evaluate the sonographic and color Doppler features of tumorlike biliary and venous changes in patients with cavernous transformation of the portal vein (CTPV). METHODS: The sonographic studies of 24 patients with CTPV were reviewed. Sonographic evaluation of the biliary system included measurement of intrahepatic and extrahepatic biliary duct caliber changes and common bile duct (CBD) wall thickening and character. Color Doppler features of the portoportal collateral circulation at various locations (intrahepatic, periportal, gallbladder, pancreatic, and gastric regions) were carefully evaluated. RESULTS: Biliary abnormalities were detected in 13 (54%) of 24 patients with CTPV. All 13 patients (100%) had intrahepatic biliary dilatation; 11 patients (85%) had CBD abnormalities: diffuse CBD wall thickening causing diffuse narrowing of the true lumen in 7 (54%) and CBD dilatation proximal to the focal area of narrowing due to pericholedochal compressing venous collaterals in 4 (30%). A tumorlike solid mass appeared on the gray scale images of 2 patients (8%): 1 at the porta hepatis and the other at the pancreatic head level. Color Doppler imaging evaluation showed venous-type flow, suggesting a bulk of varicosities. CONCLUSIONS: Portoportal collaterals in patients with CTPV may alter the biliary and venous systems, causing biliary wall thickening, stenosis, intrahepatic and extrahepatic dilatation, and pseudotumors. Detailed sonographic and color Doppler imaging assessment can show and facilitate the correct diagnosis of those changes, thus avoiding the need for a more invasive modality such as endoscopic retrograde cholangiopancreatography or a more expensive investigation such as magnetic resonance cholangiopancreatography.     

Caroli disease: Computed tomographic diagnosis

Computed tomographic (CT) scan findings in patients with cavernous ectasia of the biliary tract (Caroli disease) have been described. Our patient had the isolated form of Caroli disease characterized by saccular dilatation of intrahepatic bile ducts and no evidence of portal hypertension. CT scan of the liver showed tiny dots with strong contrast enhancement within dilated intraheptic bile ducts (the central dot sign). This finding when correlated with sonogram indicated portal radicles surrounded by dilated intrahepatic bile ducts.     

磁共振胰胆管成像的探讨(附52例报告)

目的：用ＭＲ显示胰、胆管扩张情况，并提出评价。材料与方法：检查５２例病人，其中胆道梗阻３５例，其它１７例。应用呼吸门控快速自旋回波，重Ｔ２加权成像技术及图像叠加后处理。其中２３例进行了手术治疗。结果：胆道梗阻病人均能显示梗阻部位及扩张的胆管，非梗阻病人可显示胆总管及部分左右肝管。结论：ＭＲＣＰ能显示胆管的狭窄部位、程度及扩张的胆管、胰管。与ＭＲＩ结合，能更准确地诊断梗阻部位及原因。与Ｂ超、ＣＴ及ＥＲＣＰ相比较，ＭＲＣＰ在诊断胆道梗阻的病因方面，具有独特的优越性。     

Ultrasound appearances of intra- and extrahepatic biliary ascariasis

Background: To determine the range of ultrasonographic (US) appearances of Ascaris lumbricoides roundworms in the biliary system, their distribution in the intra- and extrahepatic parts of the system, and associated features. Methods: All cases of biliary ascariasis during a 10-year period were reviewed. There were 42 cases in which diagnosis was based on established US criteria, and 2 cases diagnosed surgically in which US had been negative. These 44 cases occurred in 36 patients. Results: In the US-diagnosed cases, worms were present in intrahepatic ducts in 32 cases, in the main duct in 37, and in the gallbladder in eight. In six cases, the liver was lifted off the main portal vein by a bundle of worms obliterating the lumen of the main bile duct. Worms packing and dilating intrahepatic ducts produced pseudotumorous appearances in the liver in four cases and bundlelike appearances in three. Worms were also seen in the pancreatic duct in two cases. Stones in intrahepatic ducts were present in 12 patients. Conclusion: Biliary ascariasis should be searched for inside and outside the liver. The appearances of bundles and boluses are different from those of single worms. Intra- and extrahepatic biliary stones may be present. Received: 7/30/96/Accepted: 9/4/96     

肝门部冷冻对肝脏血流动力学影响的实验研究

目的：探讨第一肝门深低温冷冻对肝脏血流动力学的影响。方法：选健康小猪１５只,实验组８只,阻断肝门,用冷冻头深低温冷冻第一肝门。对照组７只,仅阻断肝门。以彩色多普勒观察肝动脉及门静脉血流动力学变化,同时做肝功能及肝脏病理检查。结果：实验组动物术后肝动脉阻力指数、搏动指数增加,肝动脉、门静脉血流减慢、血流量减少,肝动脉、门静脉结构无明显改变;胆管系统不可逆损伤;肝功能进行性损害。结论：深低温冷冻第一肝门会严重影响肝动脉、门静脉血流动力学     

Reconstruction of intrahepatic bile ducts in congenital biliary atresia.

Macroserial reconstruction of the main intralobular bile ducts was made in 7 cases of biliary atresia; 2 cases of type I, 1 case of type II and 4 cases of type III according to Kasai's classification. From the results of these reconstruction studies, it was confirmed that the main interlobular bile ducts are usually patent through the liver regardless of the type of atresia of the extrahepatic bile ducts. A microserial reconstruction of bile ducts and ductules of a small portal tract performed in one case disclosed that a number of ductules make a network around the main duct and have some communications with the main duct. These results were compatible with the fact that active excretion of bile was obtained in many postoperative patients with biliary atresia. As observed in one case of the present series, postoperative complication of severe ascending cholangitis seemed to be an important cause of destruction or disappearance of intrahepatic bile ducts, which has also seen in older infants with this disease without complicaion of cholangitis. In view of these facts the operation of an early stage of life is recommended in biliary atresia.     

Radiographic demonstration of common bile duct varices

Varicose veins may occur along the course of the common bile duct in patients with extrahepatic obstruction of the portal vein. These may cause partial biliary obstruction or excessive bleeding during biliary surgery. The cholangiographic appearance of choledochal varices is described.     

Anatomic variation of portal venous anatomy in the porta hepatis: ultrasonographic evaluation.

The appearance of the portal vein as it crosses anterior to the inferior vena cava and enters the liver at the porta hepatis was evaluated on 100 longitudinal and 100 transverse ultrasonograms. Three major variations were noted on the parasagittal scans, while four major variations were evident on the transverse images. These variations were primarily related to the size of the left portal vein and the angle at which the left portal vein coursed away from the main portal vein. The appearance and course of the proximal right portal vein was extremely constant and may therefore be used as a landmark to detect pathologic processes in the porta hepatis. Detection of altered anatomy in this region may be especially helpful in correctly differentiating dilated intrahepatic bile ducts from normal portal veins.     

PTBD治疗肝内胆管微扩张型梗阻性黄疸

目的探讨超声实时引导结合X线透视下,经皮肝胆管穿刺置管引流术（PTBD）治疗肝内胆管微扩张型梗阻性黄疸的操作技术及其临床应用价值。方法回顾性分析9例肝内胆管微扩张型梗阻性黄疸患者的临床资料,6例行右肝胆管PTBD,3例行左肝胆管PTBD。结果胆管穿刺置管成功率100％,术后短暂性发热1例、一过性血性胆汁1例,未出现腹腔出血及胆汁性腹膜炎等严重并发症,术后一周胆红素平均下降（75．4±29．6）μmol／L。引流时间10d～5个月。结论超声引导结合X线透视下PTBD治疗肝内胆管微扩张型梗阻性黄疸是安全可行的。     

Anatomic relationship of intrahepatic bile ducts to portal veins

To assess the anatomic relationship between the intrahepatic bile ducts and corresponding portal veins, we studied anterior-posterior relationship of the segmental bile ducts to the accompanying portal veins using sonograms and cadavers. On sonograms of the 64 segments in 16 patients with biliary dilation, the segmental bile ducts lay anterior to the corresponding portal veins in 34%, posterior in 39%, superior in 2%, tortuous in 13%, and undetermined in 13%. The relationship was inconsistent in terms of hepatic segments and thus bile ducts lay anterior to the portal vein in one segment and posterior in the other segment within the same liver. Sagittal sections in 11 segments of the 3 cadaveric livers revealed that the bile ducts were anterior to the corresponding portal veins in 1 segment, posterior in 2 segments, superior in 5 segments, anterosuperior in 1 segment, and posterosuperior in 2 segments. It is concluded that, contrary to the common belief, there is no constant anterior-posterior relationship between the intrahepatic bile ducts and the corresponding portal veins.     

Doppler ultrasound evaluation of advanced portal vein pulsatility in patients with normal echocardiograms

The hypothesis tested that mechanisms other than retrograde transsinusoidal fluid wave transfer reported in patients with right heart failure are responsible for the ultrasonographic sign of advanced portal vein pulsatility (APP). Within a time-period of 3 years we have seen 13 patients with APP, defined as temporary portal flow reversal in the face of a normal echocardiogram. Nine of these patients had biopsy-proven liver cirrhosis and four with liver disease were without cirrhosis or cardiac pathology. A randomly selected control group of 18 healthy subjects was studied. Doppler ultrasound evaluation of the hepatic veins as well as the intra and extrahepatic portal vein territories was performed in both groups. Hepatopetal portal flow with APP reversed to hepatofugal flow in follow up studies in two patients. In another two hepatopetal flow with APP in the main portal vein and hepatofugal flow in the intrahepatic portal radicles was recorded during the same examination. The remaining group displayed APP in the intra and extrahepatic portal vein territories. None of the normal subjects presented with APP. Hepatic venous outflow obstruction associated with excessive arterioportal shunting is likely to account for APP of all of our patients. Based on a causal link between angiographic ‘to-and-fro’ flow pattern and the sonographic APP sign in patients with sinusoidal outflow obstruction we suggest, that APP expresses a short, transitional period of portal hypertension just before the occurence of flow reversal.     

Hereditary hemorrhagic telangiectasia: demonstration of portosystemic venous shunts using power Doppler sonography and sonographically guided percutaneous transhepatic portovenography.

Hereditary hemorrhagic telangiectasia is a rare autosomal dominant inherited disease associated with vascular abnormalities, which may occur in any organ. Cases of hereditary hemorrhagic telangiectasia accompanied by intrahepatic portosystemic venous shunts, however, have rarely been described. We report a case of hereditary hemorrhagic telangiectasia in which intrahepatic portosystemic shunts were detected using power Doppler sonography and portovenography with percutaneous transhepatic contrast agent injection. On gray-scale sonography, the common hepatic artery was dilated, and dilated tubular structures mimicking dilated biliary tracts were found. Power Doppler sonography demonstrated the continuity of tortuous vascular channels connecting a branch of the right portal vein to a branch of the right hepatic vein. The dilated vascular channels and tributaries of the right hepatic vein showed a monophasic waveform pattern on spectral analysis. Portovenography showed a tangle of vascular structures connecting with a branch of the right hepatic vein.     

CT of agenesis and atrophy of the right hepatic lobe

Background: To identify and differentiate agenesis and severe atrophy of the right hepatic lobe on computed tomography (CT). Methods: The CT examinations of three cases of agenesis and 11 cases of severe atrophy of the right hepatic lobe were reviewed. We evaluated visibility of the three hepatic veins, the two main portal veins (including their branches if necessary), the dilated intrahepatic ducts, enlargement of the medial and lateral segments of the left lobe and caudate lobe of the liver, presence of a retrohepatic gallbladder, hyperattenuation of the atrophic liver parenchyma, posterolateral interposition of the hepatic flexure of the colon, and upward migration of the right kidney. Results: In the three cases of agenesis, no structure can be recognized as the right hepatic vein, right portal vein, or dilated right intrahepatic ducts. In the 11 cases of severe lobar atrophy, the right portal vein (or its branches) was recognized in eight cases, the right hepatic vein in four cases, and the dilated right intrahepatic ducts in 11 cases. The degree of enlargement of the lateral segment does not necessarily change inversely with the size of the medial segment and the caudate lobe. The retrohepatic gallbladder is present in eight cases (two in agenesis and six in atrophy). The phenomenon of hyperattenuation of the atrophic liver parenchyma was noted in six cases. Conclusion: Even though a retrohepatic gallbladder and a severely distorted hepatic morphology due to compensatory hypertrophy of the left and caudate lobes may raise a suspicion of agenesis of the right lobe of the liver, absence of visualization of all of the right hepatic vein, right portal vein and its branches, and dilated right intrahepatic ducts is a prerequisite of the diagnosis of agenesis of the right hepatic lobe on CT. In severe lobar atrophy, at least one of these structures is recognizable. Received: 1 March 1997/Accepted after revision: 25 June 1997     

The anatomy and pathology of the porta hepatis demonstrated by gray scale ultrasonography

Gray scale ultrasound has proved to be a simple, rapid, and reliable means for the investigation of cholestatic jaundice. The distended biliary tree has a characteristic but sometimes confusing appearance on ultrasonic tomography. Gray scale ultrasound can diagnose portal hypertension by visualizing an enlarged, tortuous portal vein. This can be differentiated from the typical shape of the dilated common bile duct by the condition of the intrahepatic biliary canaliculi and by examination of the spleen.     

梗阻性黄疸的CT和ERCP诊断

目的 通过分析梗阻性黄疸的CT和ERCP表现,总结出有意义的征象,以提高本病的诊断水平。方法 回顾性分析经病理证实的梗阻性黄疸病例的ERCP(68例)及CT(45例)表现。结果 良性梗阻性黄疸一般表现为胆总管扩张较轻,肝内胆管呈枯枝状,胆总管由上至下逐渐变细;恶性者则一般表现为胆管中-重度扩张,肝内胆管如软藤状,扩张的胆管由上至下突然中断。结论 CT和ERCP均能对梗阻性黄疸作出较准确的定位及定性诊断,但互有长短,疑胆总管下端梗阻时联合运用两种检查方法,可提高诊断准确率。     

经T管超声胆管造影评估肝移植术后早期肝内外胆管

目的 探讨以经T管超声胆管造影(CEUSC)评估肝移植术后早期肝内外胆管的可行性。方法 纳入17例接受肝移植患者,均于术后早期(中位时间28天)因血清学检查异常接受经T管二维、三维CEUSC及X线胆管造影检查;对比3种方法显示肝外胆管(尤其是吻合口区域),左、右叶肝内胆管及其不同级别分支的能力。结果 17例中,3例发生胆道并发症,包括T管脱出2例、胆管结石1例。二维、三维CEUSC均可清晰显示并明确诊断;X线胆管造影仅显示2例T管脱出,未显示胆管结石。除2例因T管脱出未能显示肝内外胆管外,3种检查对其余15例均能完整显示肝外胆管及1、2级肝内胆管,符合诊断要求。3种检查方法所示肝右叶最远端胆管级别均高于肝左叶(P均＜0.05),其间差异无统计学意义(P＞0.05)。3种方法对1~4级肝内胆管评分差异均无统计学意义(P均＞0.05),对5级肝内胆管评分差异有统计学意义(P＜0.01),其中二维CEUSC评分高于X线胆管造影和三维CEUSC (P均＜0.05)、X线胆管造影与三维CEUSC差异无统计学意义(P＞0.05)。结论 经T管CEUSC可于肝移植术后早期显示肝内外胆管,辅助诊断胆道并发症。     

Diagnosis of intramural bile duct varicose veins ("pseudo-cholangiocarcinoma sign"

We report the case of a patient with portal hypertension and portal vein thrombosis who was admitted to hospital for evaluation prior to a scheduled portosystemic shunt operation. Ultrasound examination revealed dilatation of intrahepatic bile ducts and echogenic thickening of the walls of both right and left main bile ducts as well as the common bile duct, highly suspicious of a carcinoma. Further evaluation, however, showed a varicosis of the bile duct walls to be the cause of the thickening of the walls, the varicosis resulting from the long-standing cavernous transformation of the portal vein and the portal hypertension. This tumour-like thickening of the walls, caused by numerous tiny varicose veins, is also known as "Pseudo-cholangiocarcinoma sign" in the Anglo-American world.     

Sonographic differentiation between the umbilical portion of the left portal vein and intrahepatic bile ducts

The method of differentiating between the portal vein and the bile ducts by means of their intrahepatic portions was studied. The left branch of the portal vein courses horizontally as the transverse portion, then veers anteriorly at an acute angle to make the umbilical portion. This characteristic form was demonstrated in all normal controls and 95% of the jaundiced patients studied. The left hepatic duct does not curve anteriorly at an acute angle, but branches off to the lateral segment running superior to the umbilical portion. This anatomic relationship and characteristic form of the umbilical portion were useful in differentiating the portal vein and the bile ducts.