Imaging findings in congenital hepatic fibrosis

Congenital hepatic fibrosis (CHF) is a rare congenital multisystemic disorder, mostly inherited in autosomal recessive fashion, primarily affecting renal and hepatobiliary systems. Main underlying process of the disease is the malformation of the ductal plate, the embryological precursor of the biliary system, and secondary biliary strictures and periportal fibrosis ultimately leading to portal hypertension. The natural course of the disease is highly variable ranging from minimally symptomatic disease to true cirrhosis of the liver. However, in most patients the most common manifestations of the diseases that are related to portal hypertension, particularly splenomegaly and bleeding varices. Many other disease processes may co-exist with the disease including Caroli's disease, choledochal cysts and autosomal recessive polycystic kidney disease (ARPKD) reflecting the mulstisystemic nature of the disease. The associating biliary ductal disease led the authors to think that all these entities are a continuum and different reflections of the same underlying pathophysiological process. Although, conventional method of diagnosis of CHF is the liver biopsy the advent of imaging technologies and modalities, today, may permit the correct diagnosis in a non-invasive manner. Characteristic imaging features are generally present and recognition of these findings may obviate liver biopsy while preserving the diagnostic accuracy. In this article, it is aimed to increase the awareness of the practising radiologists to the imaging findings of this uncommon clinical disorder and trail the blaze for future articles relating to this issue.     

Cirrhosis: CT and MR imaging evaluation

In this article, we present the CT and MR imaging characteristics of the cirrhotic liver. We describe the altered liver morphology in different forms of viral, alcoholic and autoimmune end-stage liver disease. We present the spectrum of imaging findings in portal hypertension, such as splenomegaly, ascites and varices. We describe the patchy and lacelike patterns of fibrosis, along with the focal confluent form. The process of hepatocarcinogenesis is detailed, from regenerative to dysplastic nodules to overt hepatocellular carcinoma. Different types of non-neoplastic focal liver lesions occurring in the cirrhotic liver are discussed, including arterially enhancing nodules, hemangiomas and peribiliary cysts. We show different conditions causing liver morphology changes that can mimic cirrhosis, such as congenital hepatic fibrosis, "pseudo-cirrhosis" due to breast metastases treated with chemotherapy, Budd-Chiari syndrome, sarcoidosis and cavernous transformation of the portal vein.     

Primary biliary cirrhosis: clinical, pathologic, and helical CT findings in 53 patients

PURPOSE: To evaluate and compare clinical, pathologic, and helical computed tomographic (CT) findings of primary biliary cirrhosis (PBC). MATERIALS AND METHODS: The authors reviewed the medical records and CT scans of 53 patients who underwent evaluation, treatment, and orthotopic liver transplantation (OLT) at their institution. All patients underwent helical multiphase CT (total, 98 abdominal CT scans; range, one to five scans per patient). Multiple epidemiologic, clinical, and morphologic criteria were evaluated. Advanced disease was defined as hepatic insufficiency leading to OLT within the subsequent 2 years. Clinical and morphologic features were evaluated and compared in the advanced and less advanced cases of PBC. RESULTS: Common and characteristic findings included the following: 45 (85%) of the 53 patients were women with the onset of disease (diagnosis) in middle age (mean, 50.7 years; range, 26-71 years). The average time from diagnosis to OLT was 6.1 years (range, 1.5-20.0 years). CT findings in advanced PBC often resembled those seen in other forms of cirrhosis, with a small heterogeneously attenuating liver, varices, and splenomegaly. The liver in less advanced disease was usually enlarged or normal in size, with a smooth contour, little atrophy, and lacelike fibrosis and regenerative nodules in nearly one-third of the livers. Patients with less advanced disease frequently had varices (n = 33 [62%]) and ascites (n = 13 [24%]). Lymphadenopathy was seen in 47 (88%) patients. Hepatocellular carcinoma was found in four (8%) patients, two of whom also had chronic hepatitis C. During a follow-up period of 5-72 months (median, 46 months; mean, 42 months) after OLT, only two patients experienced recurrence of PBC. CONCLUSION: PBC is an important cause of liver failure, with distinctive clinical and CT findings that may assist diagnosis and allow adequate treatment. CT can demonstrate varices and ascites before frank cirrhosis is evident and can help evaluate the progression of the disease.     

Postoperative assessment of splenorenal shunts with MRI: preliminary investigation

Magnetic resonance imaging (MRI) was performed on nine patients with hepatic cirrhosis and portal hypertension after selective distal splenorenal shunt for decompression of bleeding esophageal varices. MRI demonstrated the splenorenal anastomosis and patency of the shunt, splenic vein, and left renal vein in each case. Imaging in the transverse plane provided optimal visualization of the shunts. The authors conclude that MRI is a safe, noninvasive technique for the postoperative evaluation of selective distal splenorenal shunts.     

Pitfalls of transhepatic portal venography and therapeutic coronary vein occlusion.

Coronary vein occlusions via transhepatic portography for bleeding esophageal varices was attempted in 24 patients. Problems occurred that either prevented the attempt or resulted in less than adequate occlusion. These included blood flow in the left gastric (coronary) vein toward the liver due to occluded or stenotic splenorenal shunts, spontaneous left gastric vein to inferior vena cava shunts, and failure of powdered Gelfoam and heat-treated autogenous clot to cause permanent occlusion. Of 89 total transhepatic portographies, 65 for diagnosis and 24 for occlusion, major complications occurred in two.     

自发性脾-肾分流16排螺旋CT门静脉三维成像表现

目的探讨自发性脾-肾静脉分流16排螺旋CT门静脉三维成像表现及其在胃静脉曲张临床处理中的作用。资料与方法对211例疑有门静脉高压的患者行腹部门静脉期增强扫描,在后处理工作站进行三维重建。结果共检出63例自发性脾-肾静脉分流患者,其中,脾静脉主干或其大分支直接同左肾静脉分流14例,胃短静脉和／或胃后静脉在胃底后下方与左肾静脉交通9例,胃左静脉经左膈下、胰腺后小区与左肾静脉分流63例,其他类型分流2例。结论16排螺旋CT门静脉三维成像技术可多方位、多角度显示脾-肾静脉分流。根据自发性脾,肾静脉分流的血流路径可分成直接脾．肾分流、直接胃-肾分流、间接胃-肾分流和其他4种类型,从而为选择患者、优化治疗方案及评估疗效提供客观影像学依据。     

Selective embolization of the splenic vein in patients with hepatic encephalopathy and splenorenal shunt

Obliteration of portal-systemic shunts is effective for portosystemic encephalopathy but is often associated with complications such as retention of ascites and worsening of esophageal varices. Selective embolization of the splenic vein was performed on six patients with hepatic encephalopathy and splenorenal shunts. Hepatic encephalopathy was not observed in four patients after the procedure. Neither retention of ascites nor rupture of esophageal varices was observed because postoperative elevation of portal venous pressure was not as great as that seen when shunt obliteration is performed. This procedure can be an effective and safe treatment option for hepatic encephalopathy with a splenorenal shunt.     

Radiographic evaluation of patients with schistosomiasis.

Plain films of the abdomen in advanced states of schistosomiasis reveal massive splenomegaly without evidence of splenic calcification. The liver may be enlarged in the early stages but ultimately decreases and becomes small. Ascites is evident in far advanced disease. Chest films are negative in the early stages, but in advanced disease, dilated pulmonary arteries, right ventricular enlargement, and dilatation of the azygous vein may be recognized. Granulomata are seen as multiple small rounded densities scattered throughout both lung fields. The routine barium swallow will reveal unsuspected esophageal varices. Nine patients were studied preoperatively by panhepatic angiography and 14 post-operatively following splenorenal shunt. Thrombosis of the shunt and hepatic encephalopathy were common postoperative complications.     

Post-TIPS hepatic encephalopathy treated by occlusion balloon-assisted retrograde embolization of a coexisting spontaneous splenorenal shunt

A 51-year-old man with posthepatitis cirrhosis underwent a transjugular intrahepatic portosystemic shunt (TIPS) for bleeding of recurrent esophageal varices. The patient had a coexisting, spontaneous, splenorenal shunt. He subsequently developed hepatic encephalopathy, presumably due to excessive portosystemic shunting. Since medical management resulted in no significant improvement, the splenorenal shunt was embolized from the jugular vein approach via renal vein access during temporary balloon occlusion. Within a few days, the patient's hepatic encephalopathy resolved. Twelve months later the patient showed no recurrence of encephalopathy and had maintained a patent TIPS.     

肝硬化患者CT门静脉血管成像中门静脉侧支血管的表现

目的 分析肝硬化患者在16排螺旋CT门静脉血管成像(CTPV)上门静脉侧支血管的表现.方法 对36例经临床、肝功能和影像学检查诊断为肝硬化门静脉高压的患者行腹部CTPV检查,经图像后处理,获得门静脉系统及侧支血管三维重建图像.结果 CTPV可以直观地显示门静脉系统及整个门静脉侧支循环系统.36例病例中显示胃左静脉曲张29例(80.6%),食管下段静脉曲张18例(50.0%),胃短/W后静脉曲张15例(41.7%),食管旁静脉曲张9例(25.0%),胃-肾/脾-肾分流8例(22.2%),门静脉海绵样变7例(19.4%),脐静脉与腹壁静脉曲张6例(16.7%),椎旁静脉分流4例(11.1%).结论 CTPV能很好地显示肝硬化患者门脉高压侧支循环的开放部位、范围及严重程度,对判断病情、选择治疗方案及估计预后有重要的临床应用价值.     

Imaging features of ductal plate malformations in adults

Ductal plate malformations, also known as fibrocystic liver diseases, are a group of congenital disorders resulting from abnormal embryogenesis of the biliary ductal system. The abnormalities include choledochal cyst, Caroli's disease and Caroli's syndrome, adult autosomal dominant polycystic liver disease, and biliary hamartoma. The hepatic lesions can be associated with renal anomalies such as autosomal recessive polycystic kidney disease (ARPKD), medullary sponge kidney, and nephronophthisis. A clear knowledge of the embryology and pathogenesis of the ductal plate is central to the understanding of the characteristic imaging appearances of these complex disorders. Accurate diagnosis of ductal plate malformations is important to direct appropriate clinical management and prevent misdiagnosis.     

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.     

MSCTA容积再现对门静脉高压侧支循环检出的价值

目的 评价多层螺旋CT血管造影（MSCTA）容积再现（VR）重建对门静脉高压侧支循环检出的价值。方法 对40例门静脉高压侧支循环形成患者行MSCTA门静脉成像，工作站上采用VR进行血管重建。结果 40例中胃底-食道静脉曲张35例，脐静脉再通7例，脾-肾静脉交通11例，发生率分别为87．5％，17．5％，27．5％。其中，多发侧支循环：胃底-食道静脉曲张合并脐静脉再通、附脐静脉曲张1例，合并脾-肾静脉交通10例。脐静脉再通合并脾-肾静脉交通及胸廓静脉分流各1例。结论 MSCTAVR重建能清晰完整显示门静脉高压（PH）侧支循环的详细情况。     

门静脉高压脾静脉与左肾静脉自发交通CT表现

目的：探讨门脉高压脾静脉与左肾静脉自发交通的ＣＴ表现及其在诊断中的应用价值。材料与方法：回顾性分析９８０例肝硬化患者,门脉高压导致脾静脉与左肾静脉自发交通及其他侧支循环开放的ＣＴ增强表现特征。结果：ＣＴ增强扫描发现１８例脾静脉与左肾静脉自发交通,表现为脾门与左肾静脉之间柱状、团块状均匀强化影,边缘光整、锐利。脾静脉与左肾静脉明显增粗、扭曲。结论：ＣＴ增强扫描能明确诊断门脉高压时有无脾静脉与左肾静脉自发交通存在,并与左肾上腺肿瘤和肿大淋巴结鉴别,在诊断与鉴别诊断中具有重要的应用价值     

Embolization of portal-systemic shunts in cirrhotic patients with chronic recurrent hepatic encephalopathy

Purpose To evaluate the efficacy of embolization of portal-systemic shunts in cirrhotic patients with chronic recurrent hepatic encephalopathy (CRHE). Methods Seven cirrhotic patients with CRHE refractory to medical treatment (3 men and 4 women, mean age 66 years) were studied. Five patients had splenorenal shunts, 1 had a gastrorenal shunt, and 1 had an intrahepatic portal vein-hepatic vein shunt. Shunt embolization was performed using stainless steel coils, with a percutaneous transhepatic portal vein approach in 4 patients and a transrenal vein approach in 3 patients. Results After embolization, the shunt disappeared in 4 patients on either ultrasound pulsed Doppler monitoring or portography. Complications observed in the 7 patients were fever, transient pleural effusion, ascites, and mild esophageal varices. For 3–6 months after embolization, the 4 patients whose shunts disappeared showed minimal or no reappearance of a shunt, and had no recurrence of encephalopathy. The serum ammonia levels decreased and electroencephalograms also improved. One of the 4 patients, who developed mild esophageal varices, required no treatment. Treatment was effective in 3 of the 4 patients (75%) who underwent embolization via a transhepatic portal vein. Conclusion Transvascular embolization of shunts improved the outcome in 4 of 7 patients. The most effective embolization was achieved via the percutaneous transhepatic portal vein approach.     

Percutaneous transhepatic obliteration of gastroesophageal varices: some technical aspects.

Two cases of bleeding from gastroesophageal varices treated by variceal obliteration illustrate multiple routes of variceal supply, and alert embolizing angiographers to the presence of spontaneous portosystemic shunts which should be kept open. In one case, variceal obliteration required embolization of the left gastric vein and a transhepatic collateral originating from the left hepatic portal branch and contributing substantially to variceal filling. A second patient with a relatively large spontaneous splenorenal shunt had recurrent variceal bleeding two months after a successful embolization of the coronary vein and a short gastric vein. A repeat study revealed the recurrent varices were supplied by enlarged right gastric and gastroepiploic veins. Superior mesenteric venography was necessary for their visualization.     

MRI for evaluating congenital bile duct abnormalities

Congenital bile duct diseases consist of ductal plate development abnormalities and are genetically determined. These biliary abnormalities are encountered mainly in congenital fibrocystic diseases, represented by congenital hepatic fibrosis and different forms of Caroli disease. On the other hand, polycystic hepatic diseases also present cystic abnormalities, which could be confused with biliary dilatations, especially in the perihilar area. Further, intricate forms between Caroli and polycystic hepatic diseases are possible. In congenital bile duct paucity, which is extremely rare, the biliary tree, located on the opposite, is not visible. MRI modalities for the analysis of the biliary tree are mainly represented by T -weighted sequence, also known as MR cholangiography (MRCP), and T gadolinium-enhanced sequences. Familiarity with the most common appearances of congenital bile duct dilations, its variants, and related complex diseases facilitates accurate diagnosis and allows and helps avoid misinterpretation.     

Congenital Cystic Lesions of the Bile Ducts: Imaging-Based Diagnosis

Congenital cystic lesions of the bile ducts represent a spectrum of liver and biliary system lesions, resulting from abnormal embryologic development of the ductal plate. These disorders include Caroli disease, choledochal cysts, autosomal dominant polycystic liver disease, congenital hepatic fibrosis, and biliary hamartomas. Each disorder carries a peculiar clinical presentation, prognosis, and risk of complications. Knowledge of radiological findings of fibropolycystic liver diseases is crucial for their appropriate detection and for differential diagnosis with other similar hepatic cystic lesions, in order to avoid relevant misdiagnosis. The aim of this review is to provide an illustrative summary of the most relevant imaging findings of these conditions as encountered on ultrasound, computed tomography, and magnetic resonance imaging, and provide pearls for imaging-based differential diagnosis.     

Portal hypertension with intra- and extrasplenic arterial aneurysms and large venous varices

Imaging studies of two women with portal hypertension demonstrated multiple splenic artery aneurysms, splenomegaly, and large splenic vein varices with a spontaneous splenorenal shunt. In each case, a large splenic hilar, as well as intrasplenic aneurysms were found along with portal vein pressures of about 15 mmHg. Portal hypertension with splenomegaly might be an important factor in the pathogenesis of intra- and extraparenchymal splenic artery aneurysms.     

Relationship between <Superscript>99m</Superscript>Tc-GSA scintigraphic indices of liver function reserve and portal circulation in patients with chronic liver disease

Objectives In order to predict the prognosis or complications of portal hypertension in patients with chronic liver disease, it is important to evaluate both hepatic functional reserve and portal circulation. On ^99mTc-galactosyl human serum albumin (^99mTc-GSA) scintigraphy, the index of blood clearance (HH15) and receptor index (LHL15) have been widely used to evaluate the hepatic functional reserve. However, the relationship between these indices and portal circulation is unknown. The purpose of this study was to examine the relationship between HH15 and LHL15 and portosystemic shunts evaluated with arteriographic portography or esophagogastroduodenoscopy. Methods A total of 82 patients with chronic liver disease (mean age, 66.7 years) who underwent ^99mTc-GSA scintigraphy, arteriographic portography, and esophagogastroduodenoscopy were enrolled. HH15 and LHL15 were obtained from dynamic ^99mTc-GSA scintigraphy. The patients were divided into three groups according to the arteriographic portography findings: group 1, no portal collateral circulation; group 2, mild collateral development; and group 3, moderate to severe collateral development. They were also divided into three groups based on the esophagogastroduodenoscopic findings: group A, no varices; group B, small-caliber varices; and group C, enlarged varices. The Kruskal–Wallis test was used to compare each index among these groups. Receiver operating characteristic (ROC) analysis was used to determine whether each index was an indicator for the presence of portosystemic shunts. Results Both HH15 and LHL15 differed significantly between groups 1 and 2 and between groups 1 and 3. However, only HH15 differed significantly between groups A and B and between groups A and C. On the basis of the ROC analysis, the HH15 threshold value of 0.62 yielded both excellent sensitivity (83.9%) and specificity (84.6%) for the presence of portosystemic shunts, as evaluated with arteriographic portography. The HH15 threshold value of 0.64 yielded both good sensitivity (66.1%) and specificity (69.2%) for the presence of portosystemic shunts, as evaluated with esophagogastroduodenoscopy, whereas no adequate threshold value of LHL15 was found for the presence of portosystemic shunts. Conclusions HH15 is a potent indicator of the presence of portosystemic shunts in chronic liver disease.