Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 10  |  Issue : 6  |  Page : 596-598  

Multimodality imaging in budd-chiari syndrome


Department of Radiology, St. John's Medical College, Bengaluru, Karnataka, India

Date of Submission03-Oct-2016
Date of Acceptance16-Jan-2017
Date of Web Publication17-Jan-2018

Correspondence Address:
Dr. Reddy Ravikanth
St. Johnís Medical College, Bengaluru - 560 034, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.223362

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  Abstract 


Budd-Chiari syndrome refers to the clinical picture that occurs when there is obstruction of the hepatic veins. The etiology is mixed and varied. The majority of cases result from thrombosis within the hepatic veins. However, 25% result from external compression that results in obstruction. Here, we present the multimodal imaging findings in a case of Budd-Chiari syndrome.

Keywords: Anticoagulation, ascites, Budd-Chiari syndrome, imaging, thrombosis


How to cite this article:
Ravikanth R. Multimodality imaging in budd-chiari syndrome. Med J DY Patil Univ 2017;10:596-8

How to cite this URL:
Ravikanth R. Multimodality imaging in budd-chiari syndrome. Med J DY Patil Univ [serial online] 2017 [cited 2020 Sep 20];10:596-8. Available from: http://www.mjdrdypu.org/text.asp?2017/10/6/596/223362




  Introduction Top


 Budd-Chiari syndrome More Details is a manifestation of hepatic venous outflow obstruction that was first described by Budd in 1845 and then expounded on by Chiari, who presented 13 cases in 1899.[1] 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. Early diagnosis of Budd-Chiari syndrome is important for establishing an appropriate treatment. Because of inhomogeneous distribution of disease in the liver, normal biopsy findings do not exclude this entity. Therefore, imaging studies combined with clinical information are often essential for reaching a definitive diagnosis.


  Case Report Top


A 40-year-old young man with a history of nausea, vomiting, jaundice, and abdominal distension for 3 weeks presented to the emergency department. On examination, vitals were stable. Liver function tests were deranged with elevated bilirubin, aspartate aminotransferase, alanine aminotransferase, and alkaline phosphatase levels. Ascitic fluid analysis revealed elevated albumin, more than 2.5 g/dl. On examination, right hypochondrial tenderness with organomegaly was noted. No hepatojugular-reflux was elicited. Ultrasound and contrast-enhanced computed tomography (CECT) abdomen examinations were requested.

Imaging revealed gross hepatomegaly, thrombosis of the hepatic veins, splenomegaly, moderate ascites, moderate right-sided pleural effusion with the consolidation of the right posterobasal and posterolateral segments of the right lower lung lobe. Caudate lobe hypertrophy suggestive of volume redistribution with filling defects in the hepatic veins on the portal venous phase of CECT abdomen [Figure 1], [Figure 2], [Figure 3], [Figure 4]. A diagnosis of Budd-Chiari syndrome was made, and the patient started on anticoagulation treatment.
Figure 1: Ultrasound color Doppler image showing absent color uptake of the middle and right hepatic veins with patchy uptake in the left hepatic vein. Right pleural effusion is seen

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Figure 2: Delayed phase contrast-enhanced computed tomography image at the level of the intrahepatic inferior vena cava showing its compression by the enlarged caudate lobe of liver with nonvisualization of the hepatic veins. Right-sided pleural effusion seen. Schematic diagrammatic representation of portal venous and hepatic venous circulation. Occlusion of the hepatic veins and inferior vena cava is denoted by arrows

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Figure 3: Reformatted coronal contrast-enhanced computed tomography image of the abdomen showing organomegaly and ascites. Thrombosed left hepatic vein and inferior vena cava are denoted by arrows

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Figure 4: Schematic diagrammatic representation of portal venous and hepatic venous circulation. Occlusion of the hepatic veins and inferior vena cava is denoted by arrows

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  Discussion Top


Abdominal ultrasonography is usually the first step in imaging evaluation of patients with Budd-Chiari syndrome. Typically, patients with Budd-Chiari manifest stigmata of portal hypertension, such as splenic enlargement, abdominal varices, and dilatation of the splenoportal venous axis even as the liver remains normal in size, contour, and echotexture. The portal vein axis displays mural thickening (0.3 mm) with increased echogenicity and thickening of the larger portal tracts, changes suggesting periportal fibrosis.[2] At times, this periportal hyperechogenicity alternates with hypoechoic stripes resulting in a “layered” appearance of the larger portal tracts. The intrahepatic portal vein radicles show smooth and regular tapering with a sudden cutoff of the segmental and/or subsegmental branches, popularly termed as the “withered-tree” appearance.[3] Apart from substantiating portal hypertension, Doppler ultrasound plays an important role in assessing the patency of the splenoportal axis and hepatic veins that are pivotal to exclude other causes such as extrahepatic portal venous obstruction (EHPVO) or Budd-Chiari syndrome. EHPVO is an important cause of noncirrhotic portal hypertension which is characterized by the complete cavernomatous transformation of portal vein resulting in prehepatic portal hypertension. Cavernomatous transformation of the portal vein can be readily depicted on Doppler ultrasound that has an overall sensitivity and specificity of 95%.

Transient elastography when available ultrasonography assessment of liver stiffness using transient elastography (FibroScan ®; Echosens, Paris, France) can be a vital tool that can be employed for differentiating obliterative portal venopathy (OPV) and cirrhosis. As opposed to liver cirrhosis, patients with OPV exhibit a relatively soft liver and a hard spleen, and consequently, the liver stiffness is low (mean, 5.9 kPa) and the spleen/liver stiffness ratio increased.[4]

Computed tomography (CT) imaging features of Budd-Chiari on CT and magnetic resonance imaging (MRI) include a nonnodular liver with enlarged caudate lobe 6 atrophic right lobe and preserved liver volume amidst features of portal hypertension.[5] The spleen in Budd-Chiari gets massively enlarged at portal pressures comparable to other disorders of portal hypertension. Larged spleen may display multiple Gamna–Gandy bodies on CT or MRI. Gamna–Gandy bodies are fibrosiderotic splenic nodules impregnated with iron and calcium – signs of long-standing portal hypertension.

MRI in patients with Budd-Chiari is also conspicuous in detecting stigmata of portal hypertension in the form of splenic enlargement abdominal varices and dilated portal venous axis. As with CT, MRI findings in nonadvanced disease include relatively enlarged caudate lobe amid the absence of liver nodularity and preserved liver volumes. However, during advanced stages, liver contour changes and decreased liver volume make differentiation from cirrhosis unfeasible. These changes on MRI correspond to the periportal changes that can be identified on sonography and have been attributed to periportal fibrosis.[6] In addition, this periportal hyperintensity on T2-weighted sequence may also signify aberrant neovascular proliferation adjoining the portal vein radicals. In addition, benign focal nodular hyperplasia-like liver nodules are also better discernible on contrast-enhanced MRI.

The thrombosed hepatic veins are hypoattenuating, and the inferior vena cava is compressed by the enlarged caudate lobe. Ascites and splenomegaly are usually present. Sometimes the only clue to the diagnosis of Budd-Chiari syndrome is large intrahepatic collateral vessels with no ascites or major morphologic changes.[7] The therapeutic approach to Budd-Chiari syndrome should be adapted to disease severity. Careful follow-up is needed for patients with signs of portal hypertensionor of liver failure do not respond to anticoagulation or nutritional therapy.


  Conclusion Top


Budd-Chiari syndrome is a rare clinical entity characterized by hepatic venous outflow obstruction. A hematologic abnormality can be identified that predisposes the patient to the occurrence of Budd-Chiari syndrome. Awareness of its imaging findings is important for early diagnosis and appropriate treatment. In acute Budd-Chiari syndrome, the morphologic features of the liver usually are normal, and occlusion of the hepatic veins with severe ascites is the typical finding. Evaluation of occlusion of the hepatic veins and inferior vena cava, caudate lobe enlargement, inhomogeneous liver enhancement, and presence of intrahepatic collateral vessels and hypervascular nodules is the most important role of imaging examinations of patients with Budd-Chiari syndrome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Okuda K, Kage M, Shrestha SM. Proposal of a new nomenclature for Budd-Chiari syndrome: Hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion. Hepatology 1998;28:1191-8.  Back to cited text no. 1
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2.
Mathieu D, Vasile N, Menu Y, Van Beers B, Lorphelin JM, Pringot J. Budd-Chiari syndrome: Dynamic CT. Radiology 1987;165:409-13.  Back to cited text no. 2
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3.
Mukund A, Gamanagatti S. Imaging and interventions in Budd-Chiari syndrome. World J Radiol 2011;3:169-77.  Back to cited text no. 3
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4.
Ruh J, Malagó M, Busch Y, Lang H, Paul A, Verhagen R, et al. Management of Budd-Chiari syndrome. Dig Dis Sci 2005;50:540-6.  Back to cited text no. 4
    
5.
Boozari B, Bahr MJ, Kubicka S, Klempnauer J, Manns MP, Gebel M. Ultrasonography in patients with Budd-Chiari syndrome: Diagnostic signs and prognostic implications. J Hepatol 2008;49:572-80.  Back to cited text no. 5
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6.
Janssen HL, Garcia-Pagan JC, Elias E, Mentha G, Hadengue A, Valla DC; European Group for the Study of Vascular Disorders of the Liver. Budd-Chiari syndrome: A review by an expert panel. J Hepatol 2003;38:364-71.  Back to cited text no. 6
[PUBMED]    
7.
Faraoun SA, Boudjella Mel A, Debzi N, Afredj N, Guerrache Y, Benidir N, et al. Budd-Chiari syndrome: A prospective analysis of hepatic vein obstruction on ultrasonography, multidetector-row computed tomography and MR imaging. Abdom Imaging 2015;40:1500-9.  Back to cited text no. 7
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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