Caroli disease: high-frequency US and pathologic findings

Three infants with nonobstructive bile duct dilatation (Caroli disease) are described. The bile duct pathology was associated with autosomal recessive polycystic kidney disease in two patients and with severe destruction of the renal parenchyma of unknown origin in the other. Sonograms of the liver showed, besides bile duct dilatations, intraluminal bulbar protrusions, bridge formation across dilated lumina, and portal radicles partially or completely surrounded by dilated bile ducts. Liver biopsy was performed in two patients, and in one patient, the biopsy sample confirmed the findings noted on sonograms. These findings support the hypothesis that the normal embryogenesis of intrahepatic bile ducts is arrested in the pathogenesis of this disease.     

The portal venous tree simulating dilated biliary ducts on computed tomography of the liver

Intrahepatic portal veins may be identified on routine computed tomography (CT) of the liver. These venous structures appear as linear and branching, low-density regions in the liver which appear to converge at the porta hepatis. Intrahepatic portal veins may simulate the appearance of dilated intrahepatic bile ducts. Venous structures may be distinguished from dilated bile ducts by their obliteration following intravenous infusion of urographic contrast agents. CT numbers of portal veins tend to be somewhat higher than those of dilated bile ducts, but enough overlap of values exists to make reliance upon these numbers in an individual case suspect.     

Computed tomographic diagnosis of obstructive jaundice in the absence of intrahepatic ductal dilatation.

Six patients with obstructive jaundice had computed tomography (CT) scans showing a dilated extrahepatic biliary tree in the absence of intrahepatic ductal dilatation. Dilated extrahepatic bile ducts were most easily identified by means of intravenous injection of urographic contrast material, which permitted them to be seen as low density structures. Findings in these patients emphasize that (1) demonstration of dilated intrahepatic bile ducts is not a prerequisite for the CT diagnosis of obstructive jaundice and (2) careful CT evaluation of the extrahepatic biliary tree is necessary to identify patients with isolated dilatation of the common hepatic or common bile duct.     

16层螺旋CT曲面重建在诊断胆总管扩张疾病中的应用

目的评价16层螺旋CT曲面重建对诊断胆总管扩张病变的应用价值。方法 51例经手术及其中43例又经病理证实的胆总管扩张患者,包括34例恶性病变（14例胆总管癌,11例胰腺癌,9例肝癌或肝转移瘤）和17例良性病变（8例胆总管结石,4例胆总管炎性狭窄,3例胆囊切除术后,2例胰腺炎）均经16层螺旋CT腹部平扫,随后将源像传送至工作站进行后处理曲面重建（CPR）。结果 CPR能直接全程显示扩张的胆总管,准确判断其梗阻部位、范围、形态以及与邻近解剖结构的关系,并能鉴别良恶性病变的CT征象,例如,胆总管癌表现为胆总管中段管腔内结节影和肝内、外胆管重度扩张,而胰腺炎合并胆总管结石表现为胆总管与胆囊扩张,横轴位像显示胆总管胰头处结节状结石影。结论 CPR在诊断胆总管扩张及鉴别其良恶性病变中起重要作用,它应作为诊断本病的首选方案。     

肝细胞癌合并胆管癌栓的影像表现

目的 探讨胆管癌栓的影像表现,以提高影像诊断水平.方法 回顾性分析经手术病理证实的肝细胞癌(HCC)胆管癌栓13例患者资料,其中3例进行了CT和MR检查,2例仅行CT检查,8例仅行MR检查,7例进行了MR胰胆管成像检查,13例均进行了超声检查.采用四格表Fisher 确切概率检验方法比较超声与CT、MR诊断HCC胆管癌栓的准确性.结果 13例HCC肿瘤及胆管癌栓均在CT或MRI上显示.4例胆管癌栓在CT上表现为胆管内软组织块影,动脉期可见癌栓轻度增强,癌栓远端胆管扩张.11例胆管癌栓在T1 WI上均呈稍低信号,T2 WI为稍高信号,增强后可见轻、中度强化.MR胰胆管成像上胆管癌栓表现为:胆管阻塞中断、狭窄或不规则充盈缺损伴有梗阻上方胆管扩张,胆管突然截断或呈"鼠尾"状(5例);肝内胆管扩张,癌栓充满整个胆总管.胆总管不显示(2例).超声检查准确诊断胆管癌栓7例,误、漏诊6例.CT、MRI准确诊断12例,误诊4例,超声与CT、MRI诊断胆管癌栓差异无统计学意义(P=0.270).结论 CT或MRI对诊断HCC合并胆管癌栓及明确癌栓范围有价值.     

华支睾吸虫肝病的CT诊断：附139例报告

作者分析了１３９例华支睾吸虫肝病患者的ＣＴ表现。发现其肝内胆管扩张可分为四型、细长枝型：肝内胆管呈管状扩张为主且长度大于６ｃｍ，３４例（２４．４６％）；小囊型：胆管以囊状扩张为主，２０例（１４．３９％）；细短枝型：胆管以管状扩张为主，但长度小于６ｃｍ，１２例（８．６３％）；混合型：兼具上述２种或３种改变，７３例（５２．５２％）。     

CT evaluation of the bile ducts in patients with fatty liver

Computed tomographic (CT) evaluation of the bile ducts in the fatty liver can be difficult, since hepatic attenuation decreases with increased triglyceride content, and liver parenchyma may become isodense with bile. Forty-seven patients with fatty infiltration of the liver were retrospectively identified. In 7 of these patients, attenuation of liver and bile differed by less than 10 HU. In 2 patients, dilated intrahepatic ducts were invisible using CT, because bile was isodense with fatty liver parenchyma. Thus, the fatty liver presents a potential pitfall in CT evaluation of the bile ducts. Obstructive jaundice can be diagnosed in patients with fatty liver, if the extrahepatic ducts and peribiliary vascular plexus region are carefully scrutinized. For maximal accuracy, scans should be obtained both before and after administration of intravenous urographic contrast material.     

Mucin-hypersecreting intrahepatic biliary neoplasms

The radiologic findings are described in six patients with "mucin-hypersecreting" intrahepatic biliary neoplasms (biliary cystadenocarcinoma or papillary cholangiocarcinoma). Massive amounts of mucin were confirmed in the dilated bile ducts at surgery or autopsy. Computed tomography (CT) and ultrasound (US) demonstrated severe dilatation of the intrahepatic bile ducts distal to the tumor in all patients and of extrahepatic ducts in five. On cholangiograms, large, amorphous filling defects caused by retention of mucin were found in the dilated ducts in three patients. The tumor was multilocular and cystic in five patients; in the other patient it was too small to be detected at CT or US. The presence of liver tumor (mostly multilocular and cystic), marked biliary dilatation distal to the tumor, and filling defects in the dilated bile ducts are important findings that indicate a mucin-hypersecreting intrahepatic biliary neoplasm.     

Hepatic arterial color doppler signals in Caroli's disease

Three siblings with congenital dilatation of the intrahepatic bile ducts (Caroli's disease) are presented. Bile duct pathology was associated with congenital hepatic fibrosis and polycystic renal disease in all three patients. On color Doppler imaging (CD imaging), multiple small color Doppler signals were observed in the vascular radicles within the dilated bile ducts or in the center of the lumen apart from the vascular radicles, as well as in other well-known sonographic findings such as bile duct dilatations and bilary calculi. Doppler frequency spectral analysis confirmed all these color signals as arterial in origin in all patients, revealing pulsatile wave patterns. In spite of the fact that portal venous radicles have been well described on conventional sonograms or computed tomography (CT), continuous wave patterns of venous flow on spectral analysis were not detected in all patients. Identification of such less emphasized arterial flow may add another clue in the diagnosis and pathogenesis of this rare disease entity. In conclusion, color Doppler signals of arterial wave pattern within the dilated bile ducts are another helpful diagnostic criteria in previously reported sonographic findings, and these color signals are easily depicted on sonograms with color mapping.     

肝内胆管脓肿的CT特征探讨

目的 肝内胆管脓肿（intrahepatic bile duct abscess,IBDA）的CT表现特点及病理基础。方法 回顾性研究1989年10月至1999年2月经手术及临床抗炎治疗后复查证实的IBDA连续性病例31例的C T资料,病因包括多种原因所致的急性梗阻性化脓性胆管炎和胆道逆行感染。所有病例分别从肝脓肿征象、胆源性征象及反映两者间相关性的征象进行观察。结果 31例中均可见肝脓肿CT表现特征（31／31,100％）。胆源性CT表现包括各种梗阻病因的特征性 CT表现和其他胆道异常表现,包括胆管扩张（29／31,93.5％）、扩张的胆管与脓肿相通（5／31,16.1％）或相紧邻（8／31,25.8％）及胆道积气（10／31,32.2％）等。前两者间相关的CT表现,包括肝脓肿上与梗阻点及其近侧扩张胆管相一致（15／31,48.4％）,肝脓肿发生于无（7／31,22.6％）或有（４／３１,１２.９％）肝内胆管积气的肝叶、肝段等。结论 ＩＢＤＡ的ＣＴ表现包括肝脓肿征象、胆源性征象,并能反映两者间相关的具有一定特征的ＣＴ征象。     

胆道阻塞的CT诊断—与直接胆管造影的比较研究

本文对８２例手术病理证实的阻塞性黄疸作前瞻性ＣＴ诊断，并与直接胆道造影比较研究。发现胆管逐渐尖削，明显的肝外胆管壁环状增厚是良性阻塞可靠且特异的ＣＴ征象。肝内外胆管不一致扩张（内轻外重），扩张胆管腔内ＣＴ值大于２０ＨＵ对良性阻塞的判断有一定帮助。管突断伴或不伴肿块，胆管壁局根不规则增厚是恶性阻塞相对可靠且特异的ＣＴ征象。ＣＴ能清楚显示肝内胆管扩张及它们汇合处解部剖及变异。比较直接胆管造影，ＣＴ对肝     

Malignant papillary neoplasms of the intrahepatic bile ducts: CT and histopathologic features

OBJECTIVE. We describe the CT and pathologic features of malignant papillary neoplasms of the intrahepatic bile ducts in 15 patients. CONCLUSION. CT is a useful technique for revealing intraductal lesions, although the findings are nonspecific and variable. When intraductal masses or nodules are seen with localized dilatation of the intrahepatic bile ducts on CT scans, malignant papillary neoplasms of the intrahepatic bile ducts should be included in the differential diagnosis.     

CT findings of clonorchiasis

Clonorchiasis is a snail-transmitted trematodiasis caused by Chinese liver fluke, Clonorchis sinensis. Forty-two patients with the disease were examined with CT. Seventeen patients had clonorchiasis alone, and 25 patients had clonorchiasis with hepatobiliary malignancies (20 cholangiocarcinomas, four hepatocellular carcinomas, one carcinoma of the ampulla of Vater). In three of the 17 patients with clonorchiasis alone, the CT scans were normal. In 14 patients (82%), CT showed diffuse, minimal, or mild dilatation of the intrahepatic bile ducts. None of the patients had marked dilatation. The extrahepatic biliary tree was normal in all 17 patients. All 25 patients with clonorchiasis and hepatobiliary malignancies had diffuse dilatation of the intrahepatic bile ducts on CT, including 18 patients with minimal or mild dilatation and seven patients with marked dilatation. All seven patients with marked dilatation had extrahepatic biliary malignancies. Clonorchis sinensis per se or thickening of the bile duct wall could not be recognized on CT scans. Additional abnormalities evident on CT included pyogenic liver abscesses in two patients and gallstones in five patients. The diagnosis of clonorchiasis can be suspected when CT shows diffuse, uniform, and minimal or mild dilatation of the intrahepatic bile ducts, particularly in the periphery of the liver, without evidence of extrahepatic biliary dilatation.     

The stilette sign: the appearance of dilated bile ducts in the fatty liver

The appearances of hepatic steatosis and of dilated intrahepatic bile ducts occurring independently are well described. When both conditions occur simultaneously a major sign of biliary obstruction, a double lumen with echogenic walls (the double-barrelled shotgun sign), is modified by the abnormally echogenic liver. The combined appearance is of a tubular structure with no echogenic interface with the liver, containing an echogenic structure at its centre representing echoes from the adjacent bile duct and portal vein walls. We have named this appearance the 'stilette' sign because of its resemblance to a fine needle within a tube. Its importance lies in the fact that it makes dilated intrahepatic bile ducts difficult to recognise by ultrasound.     

Oriental cholangiohepatitis: pathologic, clinical, and radiologic features

Oriental cholangiohepatitis, an endemic disease in Southeast Asia, is characterized by recurrent attacks of abdominal pain, fever, and jaundice. Pathologically, the intra- and extrahepatic ducts are dilated and contain soft, pigmented stone and pus. There is proliferation of bile ducts and infiltration of inflammatory cells along the periportal spaces and hepatic parenchyma. Localized intrahepatic segmental ductal stenosis may be present, especially in the lateral segment of the left lobe or posterior segment of the right hepatic lobe. The cause of the disease is not known, but associations with clonorchiasis, ascariasis, and nutritional deficiency have been suggested. Sonographic and CT findings include intra- or extrahepatic duct stones, dilatation of the extrahepatic duct with relatively mild or no dilatation of the intrahepatic ducts, localized dilatation of the lobar or segmental bile ducts, increased periportal echogenicity, segmental hepatic atrophy, and gallstones. Cholangiographic findings include bile duct stones; disproportionately severe dilatation of the extrahepatic ducts with mild or no dilatation of the intrahepatic ducts; and focal strictures, acute peripheral tapering, straightening, rigidity, decreased arborization, and an increased branching angle of the intrahepatic bile ducts.     

CT and MR cholangiography: advantages and pitfalls in perioperative evaluation of biliary tree

Recent developments in imaging technology have enabled CT and MR cholangiopancreatography (MRCP) to provide minimally invasive alternatives to endoscopic retrograde cholangiopancreatography for the pre- and post-operative assessment of biliary disease. This article describes anatomical variants of the biliary tree with surgical significance, followed by comparison of CT and MR cholangiographies. Drip infusion cholangiography with CT (DIC-CT) enables high-resolution three-dimensional anatomical representation of very small bile ducts (e.g. aberrant branches, the caudate branch and the cystic duct), which are potential causes of surgical complications. The disadvantages of DIC-CT include the possibility of adverse reactions to biliary contrast media and insufficient depiction of bile ducts caused by liver dysfunction or obstructive jaundice. Conventional MRCP is a standard, non-invasive method for evaluating the biliary tree. MRCP provides useful information, especially regarding the extrahepatic bile ducts and dilated intrahepatic bile ducts. Gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid-enhanced MRCP may facilitate the evaluation of biliary structure and excretory function. Understanding the characteristics of each type of cholangiography is important to ensure sufficient perioperative evaluation of the biliary system.     

Autosomal recessive polycystic kidney disease: radiologic-pathologic correlation.

Autosomal recessive polycystic kidney disease is a heritable but phenotypically variable disorder characterized by varying degrees of nonobstructive renal collecting duct ectasia, hepatic biliary duct ectasia and malformation, and fibrosis of both liver and kidneys. In the kidney, the dilated collecting ducts and interstitial fibrosis, when severe, may significantly impair renal function and result in hypertension and renal failure. Imaging typically shows large but reniform kidneys, diffusely increased renal parenchymal echogenicity at ultrasonography, and a striated nephrogram after contrast material administration. In the liver, periportal fibrosis accompanies the malformed and dilated bile ducts; this may result in portal hypertension. The liver may appear normal or may show intrahepatic biliary dilatation; once portal hypertension develops, splenomegaly and varices are usually evident. The relative degrees of kidney and liver involvement tend to be inverse: Children with severe renal disease usually have milder hepatic disease, and those with severe hepatic disease tend to evidence mild renal impairment. Presently, treatment consists of supportive management and control of hypertension. Replacement therapy for renal failure (dialysis or kidney transplantation) and control of portal hypertension (portal circulatory diversion or liver transplantation) may be necessary.     

胆管癌误诊和漏诊的影像学分析

目的：分析胆管癌误诊和漏诊的原因，评价不同检查方法对肝门区胆管癌诊断的价值，提高胆管癌的影像学诊断和鉴别诊断水平。方法：收集31例临床和病理诊断为胆管癌的病例(ERCP检查31例，B超检查31例，25例CT检查，11例行MRCP检查)，回顾性分析不同检查方法的影像学表现。结果：31例中发生在肝门区的胆管癌25例。2例初次ERCP显示胆管内出血，再次行鼻胆管造影后诊断为胆管癌，4例合并有总胆管结石。6例为总胆管中下段癌。初次诊断准确性为90．3％。11例MRCP中9例显示病变，2例显示不满意，7例显示胆管或胆囊结石。25例CT中23例显示肝内胆管扩张，9例显示肝门区肿块，2例见后腹膜转移淋巴结，1例见肝内多发性转移瘤。12例初次诊断正确(48％)。B超检查23例提示肝内胆管扩张，6例提示总胆管结石，18例诊断为胆囊炎、胆囊结石，8例提示胆管占位(25．8％)。结论：良好的直接胆管造影是诊断胆管癌的金标准，MRCP可以在一定程度上准确显示病变的范围，是ERCP的良好补充，很大程度上取代了诊断性ERCP；CT诊断的关键在于显示扩张的胆管和梗阻的定位，肝门区梗阻多为胆管癌所致。多种影像检查方法的结合明显提高了诊断的准确性，对胆管癌治疗方案的选择有重要价值。     

MR imaging of the dilated biliary tract

The magnetic resonance (MR) examinations of 18 patients with dilated bile ducts were reviewed retrospectively to determine the capability of MR to demonstrate biliary dilatation, assess MR appearance of the dilated biliary tract using spin-echo techniques, and define the optimal MR imaging parameters (repetition time [TR] and echo time [TE]) for its demonstration. On images with short TR (0.5 sec) and TE (28 msec), the dilated intrahepatic and intrapancreatic bile ducts usually had lower signal intensity compared with the surrounding liver or pancreas; on images with long TR (2.0 sec) and TE (56 msec), they had higher signal intensity. Because of the observed variation in percentage of contrast between dilated bile ducts and surrounding liver and pancreas, two imaging sequences are recommended to obtain reliable demonstration of dilated intrahepatic and intrapancreatic bile ducts. The dilated common bile duct at the level of the hepatic hilus is best seen with a short TR and TE.     

胆管内生长型肝内胆管细胞癌的CT诊断

目的:探讨胆管内生长型肝内胆管细胞癌(IICC)的CT表现特征。方法:对手术及病理证实的11例IICC的临床及CT资料进行回顾性分析总结。结果:11例ICIG中4例为周围型,5例为肝门型,2例同时侵润肝门及肝内胆管(下称混合型)。肝叶或肝段肝内胆管扩张,伴有或不伴有胆管内乳头状肿块、无定形态结构或只有轻度的扩张胆管密度增高是IICC的CT特点,管内肿块的大小决定了其是否在CT表现出来。结论:IICC的CT表现具有一定的特征性,对提高本病的术前CT诊断的准确性有一定的价值。