Gaucher Disease

Objective. Our large tertiary clinic for patients with Gaucher disease has used sonography as the preferred modality to monitor hepatosplenomegaly in hundreds of patients for more than 18 years. With the advent of specific enzyme replacement therapy (ERT), sonographic monitoring of changes in both hepatomegaly and the echogenicity of the hepatic tissue may highlight features that are amenable to ERT. Methods. All patients (500) seen at presentation and at annual or semiannual routine visits have undergone sonographic examinations by a single senior radiologist (I.H.‐H.). Results. Thirty‐nine patients (7.8%) had sonographic evidence of hepatic disease (21 male and 18 female; age range, 18–90 years); 26 (66.7%) of these received ERT, and 10 (25.6%) were splenectomized. Conclusions. Liver findings are relatively rare. Among 500 patients, there was no instance of computed tomographic findings that had not been previously shown by sonography. Radiologists should be acquainted with the variable sonographic spectrum of the Gaucher liver. If hepatic lesions are small, hyperechoic, and slowly evolving, one may surmise that they are due to Gaucher cell accumulation. However, special attention should be paid to progressive deterioration and irregularities in liver texture because other metabolic processes and cancers must be ruled out.     

Sonographic findings of the hepatobiliary-pancreatic system in adult patients with cystic fibrosis.

OBJECTIVE: Sonography of the liver, biliary system, and pancreas in adult patients with cystic fibrosis is by far less systematically documented than in pediatric patients with cystic fibrosis. In this prospective study, duplex sonographic findings of the liver, biliary system, and pancreas in adult patients with cystic fibrosis were compared with those of healthy control subjects. METHODS: Seventy-two consecutive patients with cystic fibrosis and 60 healthy control subjects were examined by high-resolution sonography. The incidence of perihepatic lymphadenopathy, the hepatic echo pattern, the detection rate of liver tumors, the flow patterns in the hepatic and portal veins, and pathologic gallbladder and pancreas findings were recorded. Additionally, cholestasis-indicating enzyme levels (gamma-glutamyl transpeptidase and alkaline phosphatase), liver function test results (alanine aminotransferase and aspartate aminotransferase levels), and amylase and lipase levels were recorded as well. RESULTS: Patients with cystic fibrosis, when compared with healthy subjects on sonographic examination, had a higher incidence of microgallbladder (25% versus 0%) and cystic lesions of the pancreas (18% versus 0%). The number of abnormal echo patterns of the liver was increased (46% versus 15%), with a higher incidence of a nontriphasic flow pattern in the right hepatic vein. The differences proved to be statistically significant (P < .05). CONCLUSIONS: Typical sonographic findings in adult patients with cystic fibrosis are a microgallbladder and small cystic lesions of the pancreas. Pathologic findings of the liver can be shown by B-mode and duplex sonography, but the resulting patterns are less characteristic.     

Visualization of blood flow in hepatic vessels and hepatocellular carcinoma using B-flow sonography

PURPOSE: A B-flow sonographic technique was recently developed to provide direct visualization of blood flow with gray-scale sonography. Compared with color Doppler sonography, B-flow imaging has wideband resolution and a high frame rate. The purpose of this study was to evaluate the usefulness of B-flow sonography for visualizing blood flow in hepatic vessels and tumor vascularity in patients with liver cirrhosis or hepatocellular carcinoma (HCC). METHODS: Twenty-five patients with liver cirrhosis, including 15 with HCC, were studied by B-flow and color Doppler sonography. Blood-flow detection rates in portal veins and hepatic arteries and tumor vascularity in HCC were analyzed, and the 2 methods were compared. RESULTS: Using B-flow, blood flow was visualized in the portal vein in 23 (92%) of 25 patients and was visualized in the hepatic artery separately from the portal vein in 9 (36%) of 25 patients. The blood-flow signals were visualized only within vessels, never "bleeding" outside the vessel's lumen. Blood flow in the portal vein was observed with color Doppler sonography in all 25 patients, but the hepatic artery was never clearly separated from the portal vein. Vascularity within the HCC tumor was detected in 9 (60%) of 15 nodules with B-flow imaging, and fine arteries flowing into the tumor were observed in 6 nodules. Color Doppler sonography detected blood flow in 13 (87%) of the 15 HCC nodules. CONCLUSIONS: Blood flow in hepatic vessels and tumor vessels of HCC were visualized with B-flow sonography. B-flow sonography is a potentially useful technique for the evaluation of liver vascularity and intratumoral vessels.     

超声波评分系统对代偿性肝硬化的诊断价值

目的探讨超声波评分系统对代偿性肝硬化的诊断价值。方法选取55例慢性肝病患者行经皮肝脏穿刺活检术进行病理组织学检查,同时行空腹肝脏B型超声波观察肝硬化声像改变并对肝实质回声、肝表面、肝脏边缘、肝静脉、脾脏面积等7项参数综合评分。结果超声波评分系统对早期肝硬化的诊断灵敏度为93．75％,特异度为87．18％。结论超声波评分系统可作为筛检代偿性肝硬化较灵敏的诊断系统。     

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.     

Sonographic findings in bone marrow transplant patients with symptomatic hepatic venoocclusive disease.

Sonographic findings were retrospectively compared between 19 patients with hepatic venoocclusive disease and 23 patients with other common causes of symptomatic liver dysfunction after bone marrow transplantation (14 grafts versus host disease and nine hepatitis). Doppler sonographic examination was available in all patients with venoocclusive disease, in nine of the patients with graft versus host disease, and in three of the patients with hepatitis. The hepatic artery resistive index and the overall flow direction, peak forward and retrograde velocities, and time-averaged mean velocities in the hepatic veins and main portal vein were compared. The portal vein waveform was arbitrarily considered abnormal in the presence of any of the following: highly pulsatile waveform, very low mean velocity, biphasic flow, or flow reversal. Ascites was the most predictive gray scale sonographic finding for venoocclusive disease. Doppler sonographic findings of potential value in the diagnosis of hepatic venoocclusive disease include an abnormal portal vein waveform, resistive index of greater than 0.75, and marked thickening and edema of the gallbladder wall. However, the study is limited by its retrospective nature and reliance primarily on clinical criteria for the diagnosis of venoocclusive disease. Therefore, our findings will need to be verified in a large prospective study.     

胎儿肾脏微囊性病变的产前超声表现及病理学观察

目的 探讨胎儿肾脏微囊性病变产前超声特征及肾脏的病理改变.方法 对产前超声检查、临床随访(家族史调查证实常染色体显性遗传性多囊肾1例)或引产后病理检查(5例)证实的6例孕22～32周肾脏微囊性病变胎儿的产前超声表现、分类诊断特征及引产后肾脏病理表现进行总结分析.结果 产前超声显示6例胎儿双侧肾脏对称均匀性增大,实质回声弥漫性增强;肝门脉区回声增粗(2例)呈纤维网格样变(2例),CDFI显示6例肾血管充盈良好,频谱呈高阻力指数血流,羊水指数低于正常或在正常范围内.引产后病理标本显示5例胎儿双侧肾脏弥漫性增大,失去胎儿肾脏特有的"桑葚"状表现,肾皮质及髓质见多发微小囊肿,集合系统发育不良;镜下显示5例胎儿肾实质内见弥漫分布大小不等的囊肿,并见肾小管囊性扩张,1例肾小球Bowman囊扩张.5例引产儿尸检病理诊断:胎儿肾小管囊性变4例,肾小球囊性变1例;1例经临床追踪随访后诊断胎儿肾脏微囊性病变.结论 胎儿肾脏微囊性病变产前超声表现为肾脏增大,弥漫性回声增强,病理表现为肾小管或肾小球的弥漫性微囊性扩张.     

Caroli病的超声检查

Ｃａｒｏｌｉ病是一种少见的先天性肝内胆管非梗阻性扩张性疾病，同时伴有婴儿型多囊肾和先天性肝纤维化，常在儿童期发现，我们应用超声诊断８例，声像图上表现为肝脏增大，实质内散在多发的不规则无回声区且与胆管相通；肾脏增大，回声明显增强，皮髓之间界线不清，具有特征性声像图表现可提示诊断。从而不再依靠经皮肝穿刺活检、逆行胆道造影等侵害性技术，同时能及时发现肝脓肿、肾结石等合并症，观察病变的发展，超声检查具有很大的优势     

Association between duplex Doppler sonographic flow pattern in right hepatic vein and various liver diseases

PURPOSE: The aim of this study was to evaluate the association between the Doppler sonographic waveforms in the right hepatic vein and various liver diseases. METHODS: We performed Doppler sonography of the right hepatic vein in 225 individuals (189 patients with liver disease and 36 control subjects). Patients were categorized on the basis of their histologic diagnosis: cirrhosis (n = 122), fibrosis (n = 23), fatty liver disease (n = 11), metastatic liver disease (n = 8), and noncirrhotic liver disease with neither fatty infiltration nor metastases (n = 25). The waveforms of the hepatic veins were categorized as type 1, triphasic; type 2, biphasic; or type 3, flat. RESULTS: Type 1 waveforms were found in 100% of the control subjects and in patients with each of the various liver diseases, including 40% of those with cirrhosis. Type 2 waveforms were not found in control subjects or in patients with either fatty infiltration or metastatic liver disease. Type 2 waveforms were, however, found in 16% of patients with cirrhosis, 13% of patients with fibrosis, and 4% of patients with other noncirrhotic liver diseases. Type 3 waveforms were found in 43% of patients with liver cirrhosis, 13% of patients with liver fibrosis, 27% of patients with fatty liver disease, and 50% of patients with metastatic liver disease. In contrast, type 3 waveforms were not found in any control subjects or in patients with other noncirrhotic liver diseases without fatty liver or metastases. CONCLUSIONS: Type 3 waveforms in the right hepatic vein are suggestive of liver cirrhosis, although they may also occur in patients with fibrosis, fatty liver disease, or metastatic liver disease.Copyright 2000 John Wiley & Sons, Inc.     

肝内“假平行管征”的超声研究

目的：研究肝内“假平行管征”的超声表现及其临床意义。方法：检查正常成人214例,观察肝右后下静脉（IRHV）的超声表现,统计其显示率,并对10例肝动脉分支扩张的患者进行彩色多普勒和频谱多普勒超声检查。结果：IRHV的显示率为24％,在第一肝门水平注入下腔静脉,和门静脉右后叶支相伴而行,形成“平等管征”。肝内扩张的肝动脉分支在二维超声上与伴行门静脉分支亦构成“平行管征”,但彩色多普勒显示双管内均有血流信号,频谱多普勒分别探测动脉血流频谱和静脉血流频谱。结论：认识IRHV和肝内扩张的肝动脉分支有助于鉴别真假“平行管征”,有助于阻塞性黄疸的鉴别诊断。     

Gallbladder cancer: can ultrasonography evaluate extent of disease?

This study reviews the spectrum of sonographic findings in patients with gallbladder cancer, attempts to determine if sonography can identify patients with potentially resectable disease, and emphasizes the limitations of ultrasonography in the evaluation of -gallbladder cancer. Thirty-five consecutive patients with histologically proven gallbladder carcinoma who had preoperative abdominal ultrasonography and surgery were identified. Involvement of the gallbladder and gallbladder fossa, metastases, bile ducts, portal vein, and adjacent lymph nodes was assessed sonographically. The extent of disease and staging as revealed by sonography was compared to operative and surgical pathologic findings. Masses in the gallbladder or gallbladder fossa were present at surgery in 26 patients; 22 (85%) of these masses were shown by sonography. Sonography identified six (67%) of nine cases of pathologically confirmed liver metastases, 11 (79%) of 14 cases of bile duct involvement, and two (67%) of three cases of portal venous involvement by tumor. Sonography revealed lymph node metastases in only five (36%) of 14 patients. None of the 12 cases with peritoneal metastases was identified sonographically. By surgical staging 16 (46%) patients had potentially resectable disease (stage III or less), and 19 (54%) patients had unresectable stage IV disease. Sonography correctly identified 15 (94%) of 16 patients with potentially resectable disease and seven (37%) of 19 patients with advanced disease. Twelve patients with advanced disease were under-staged: nine had peritoneal metastases, two had liver metastases, and one had celiac adenopathy, which was not shown by sonography. In conclusion, sonography is reliable in the detection of a primary gallbladder mass or of local extension of tumor into the liver. However, sonographic findings do not accurately reflect the full extent of disease, and sonography is particularly limited in the diagnoses of metastases to the peritoneum and lymph nodes.     

Sonography of acute pancreatitis: prevalence of findings and pictorial essay

The purpose of this article is to describe the sonographic findings of acute pancreatitis, establish their individual prevalence, and illustrate the sonographic findings in acute pancreatitis. Prevalence of findings was determined by reviewing abdominal sonograms in 48 adult patients, derived from 71 consecutive patients who had been scanned using our standard abdominal protocol, which routinely evaluates the pancreas and peripancreatic regions. The mean anteroposterior measurement of the pancreatic body at the level of the superior mesenteric artery was 21.1 mm +/- 6.4 mm with a range of 12 to 45. Decreased pancreatic echogenicity compared with the liver was noted in 21 patients (44%). A heterogeneous echo pattern was detected in 27 patients (56%). Focal intrapancreatic regions of abnormal echogenicity were seen in 11 of 48 patients (23%). Focal masses were seen in 8 of 48 patients (17%). Hypoechoic peripancreatic areas of inflammation were seen in 29 of 48 patients (60%) and acute peripancreatic fluid collections were seen in 10 of 48 patients (21%).In this study, sonography revealed abnormalities in 45 of 48 patients (91.7%). Diffuse decreased echogenicity, focal contour, and focal echogenicity changes within the pancreas are associated with extrapancreatic disease.     

Hepatic Vein Morphology

Objective. The purpose of this study was to evaluate the accuracy of a new sonographic marker for the diagnosis of cirrhosis using hepatic vein wall changes. Methods. A prospective pilot study evaluating 88 patients, 38 with cirrhosis and 50 with no evidence of liver disease, was undertaken. Hard copy sonograms of the hepatic veins were obtained and reviewed in a blinded fashion by 2 radiologists. The hepatic vein morphology was assessed by 3 parameters: hepatic vein wall straightness, uniformity of hepatic vein wall echogenicity, and visualization of a complete 1‐cm hepatic vein segment. The 3 parameters were compared to evaluate sensitivity and specificity for the diagnosis of cirrhosis. Interobserver and intraobserver errors for each parameter were also calculated with κ statistics to assess reproducibility. Results. There was a strong correlation between altered straightness and nonuniformity of hepatic vein wall echogenicity and cirrhosis. The straightness parameter had superior sensitivity of 97% (95% confidence interval [CI], 85%–100%) and specificity of 91% (95% CI, 78%–97%) for diagnosis of cirrhosis. Uniformity of hepatic vein wall echogenicity was the next most useful parameter, with sensitivity of 88% (95% CI, 73%–97%) and specificity of 86% (95% CI, 72%–95%). The continuous 1‐cm segment of the hepatic vein had sensitivity of 68% (95% CI, 49%–83%) and specificity of 91% (95% CI, 78%–97%). Hepatic vein evaluation was found to show both good intraobserver and interobserver error. Conclusions. Hepatic vein morphology on sonography, in particular, changes in the straightness and uniformity of hepatic vein wall echogenicity, is a new sign of cirrhosis, which may increase the overall accuracy of sonographic diagnosis of cirrhosis and which appears to have a moderately high degree of reproducibility.     

The small spleen: sonographic patterns of functional hyposplenia or asplenia

PURPOSE: Functional hyposplenia or asplenia (FAS) can be associated with potential fatal infections. The diagnosis of FAS is traditionally made on liver-spleen scintigraphy and finding Howell-Jolly bodies within erythrocytes. In this retrospective study, our goal was to identify any characteristic sonographic findings of the spleen in patients with FAS in an attempt to determine whether the diagnosis of FAS can be made sonographically. METHODS: In a review of all medical and sonographic records from the period of January 1, 1985, through December 31, 2001, we identified 24 patients (11 men, 13 women) in whom FAS had been diagnosed by liver-spleen scintigraphy (n = 13) or the finding of Howell-Jolly bodies (n = 11). The following sonographic parameters were determined: size of spleen (small, normal, or large), echotexture of the spleen (homogeneous versus inhomogeneous), echogenicity (isoechoic versus hyperechoic), presence of focal splenic lesions, and patterns of splenic vascularization as determined by color Doppler sonography (absent flow, hilar flow, or parenchymal flow). RESULTS: The spleen was small in 20 patients (83%) and normal in the other 4 (17%). Echotexture was homogeneous in 13 patients (54%) and inhomogeneous in 11 (46%). The spleen was isoechoic in 18 cases (75%) and hyperechoic in 6 (25%). Six patients (25%) had focal lesions. Color Doppler sonography showed absent flow in 4 patients (17%), hilar flow in 17 (71%), and hilar and parenchymal vascularization in 3 (12%). CONCLUSIONS: Sonographic findings in the spleen of patients with FAS are characterized predominantly by a small spleen with absence of parenchymal vascularization on color Doppler sonography in most cases. Future prospective studies will be necessary to confirm these findings and to determine whether FAS can be diagnosed reliably with sonography.     

肝淀粉样变性与肝窦阻塞综合征临床及影像特征的鉴别分析

目的 肝淀粉样变性与肝窦阻塞综合征的病理发病部位同为肝窦周隙,影像学表现相似,临床表现又缺乏特异性,临床对其认识不足,容易误诊,本研究通过总结肝淀粉样变性及肝窦阻塞综合征的临床及影像特点,提高对两种疾病的诊断水平.材料与方法 回顾北京佑安医院2009-2019年经病理诊断的11例肝淀粉样变性和20例肝窦阻塞综合征患者的...     

Sonographic diagnosis and hemodynamic correlation in veno-occlusive disease of the liver

This study evaluated the role of duplex Doppler sonography in the diagnosis of VOD of the liver after bone marrow transplantation. Sixteen patients with clinical criteria of VOD were studied. The final diagnosis was achieved by transjugular liver biopsy, and the hepatic venous pressure gradient was measured during the procedure to estimate portal pressure. Nine patients (56.2%) had histologically proved VOD. Gallbladder wall thickening was present in 75% of patients with VOD, but in none of those without it (P = 0.01). Gallbladder wall thickening and ascites were present only in patients with portal hypertension. Nine patients (five with VOD and four without it) also were evaluated with duplex Doppler sonography, and no obvious flow abnormalities were detected in the portal vein and hepatic veins. These results suggest that sonography is useful in detecting early signs of portal hypertension pointing to the diagnosis of VOD in patients with bone marrow transplantation.     

Blunt hepatic trauma: evaluation with contrast-enhanced sonography: sonographic findings and clinical application.

OBJECTIVE: The purpose of this study was to report our initial experience in the assessment of liver trauma with real-time contrast-enhanced sonography (CES). METHODS: From January 2000 to December 2003, there were 431 hemodynamically stable patients evaluated with sonography for blunt abdominal trauma. Among these patients, 87 were selected to undergo second-level imaging, consisting of CES and computed tomographic (CT) evaluation. Indications for further assessment were baseline sonographic findings positive for liver injury, baseline sonographic findings positive for injury to other abdominal parenchyma, baseline sonographic findings positive for free fluid only, baseline sonographic findings indeterminate, and baseline sonographic findings negative with persistent clinical or laboratory suspicion. RESULTS: There were 23 hepatic lesions shown by CT in 21 patients. Peritoneal or retroperitoneal fluid was identified in 19 of 21 positive cases by all 3 imaging modalities. Liver injury was found in 15 patients on sonography and in 19 on CES. Contrast-enhanced sonography compared better than unenhanced sonography with the criterion standard for related injury conspicuity, injury size, completeness of injury extension, and involvement of the liver capsule. Both CES and CT showed intrahepatic contrast material pooling in 2 cases. All patients with false-negative sonographic or CES findings recovered uneventfully. CONCLUSIONS: Contrast-enhanced sonography is an effective tool in the evaluation of blunt hepatic trauma, being more sensitive than baseline sonography and correlating better than baseline sonography with CT findings. In institutions where sonography is regarded as the initial procedure to screen patients with trauma, this technique may increase its effectiveness. In addition, CES may be valuable in the follow-up of patients with conservatively treated liver trauma.     

Doppler sonographic findings in children with idiopathic portal vein cavernous deformity and variceal hemorrhage.

OBJECTIVE: To describe the flow patterns in the portal vascular territory in children with portal vein cavernous deformity. METHODS: The study included 12 children (age 4-10 years) with hematemesis, melena, or both in whom B-mode gray scale sonography revealed small anechoic spaces replacing the site of the portal vein. The portal vein cavernous deformity was present either alone (in 8 patients) or with congenital hepatic fibrosis (in 4). Doppler sonography (color and spectral) was performed to assess the flow in the portal vascular territory, splenic vein, intrasplenic veins, and abdominal collaterals. RESULTS: Doppler sonography confirmed the venous flow waveform in the cavernous portal vein in all children with normal flow direction in the few intrahepatic portal vein branches and also in the intrahepatic veins. Splenomegaly was present in all. The intrasplenic veins were dilated in all but had normal flow direction except in 2 with spontaneous trans-splenic shunts. Gallbladder varices were shown in 4 patients, and perisplenic collaterals were shown in 3. CONCLUSIONS: Doppler sonography is a valuable noninvasive imaging technique for assessment of the portal hemodynamic profile in patients with portal vein cavernous deformity, which can affect subsequent treatment decision making. Trans-splenic shunts are uncommon, but this Doppler sonographic report documents such shunts in children with portal hypertension.     

Increased renal cortical echogenicity in pediatric renal disease: histopathologic correlations

Retrospective comparisons between sonographic renal cortical echogenicity and the results of renal biopsies were made for 65 pediatric patients ranging in age from neonate to 18 years. There was a positive correlation between an increase in renal cortical echoes and interstitial infiltration as well as with glomerular obsolescence, tubular atrophy, and vascular changes. Since the sonographic changes were heterogeneous in origin, they are not specific. The sonographic findings also correlated positively with the clinical severity of disease. However, the heterogeneous origins of the sonographic finding and the absence of strong correlations indicate that gray-scale sonography cannot act as a prognostic index of the type or severity of disease in pediatric patients.     

Portal vein hemodynamics in patients with non-alcoholic fatty liver disease

Paralleling the rise in the incidence of obesity and diabetes worldwide, nonalcoholic fatty liver disease (NAFLD) is being increasingly recognized as one of the major causes of chronic liver disease. Doppler sonography is used as a diagnostic method in the non-invasive assessment of the hemodynamics of hepatic vascular flow in liver diseases. We investigated the effects of fatty infiltration in the liver on the Doppler flow hemodynamics of the portal vein. Doppler sonography of the liver and portal vein was performed in 60 subjects with NAFLD and 20 healthy volunteers (control). The patients were grouped into mild (grade 1), moderate (grade 2), and severe (grade 3) according to sonographic appearance of hepatosteatosis (n = 20 for each group). The vein pulsatility index (VPI), mean flow velocity (MFV), peak maximum velocity (V(max)), and peak minimum velocity (V(min)) of the portal vein were significantly lower in patients with NAFLD than those of the controls (p < 0.001). The VPI was 0.20 in the patients and 0.31 in the control. The MFV was 12.3 cm/sec in the patients and 16.5 cm/sec in the control group. The portal vein flow was found to be decreased as the grade of fatty infiltration increased for VPI (r = -0.946, p < 0.001), MFV (r = -0.951, p < 0.001). The alteration in Doppler waveform pattern of portal vein with fatty liver population suggests reduced vascular compliance in the liver.