CASE 14246 Published on 16.12.2016

Budd-Chiari Syndrome in a Patient with Chronic Alcoholic Cirrhosis

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

Authors: Maxwell Norris, Zain Badar M.D.; Jason Chu M.D.; Sajel Patel M.D

Upstate University Hospital ,Upstate Medical University ; 750 East Adams Street 13210 Syracuse ; Email:norrism@upstate.edu

Patient

46 years, female

Categories

Area of Interest Abdomen ; Imaging Technique Catheter venography, Ultrasound, Ultrasound-Colour Doppler, CT

Procedure Diagnostic procedure, Imaging sequences ; Special Focus Cirrhosis, Haemodynamics / Flow dynamics, Obstruction / Occlusion ;

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Clinical History

A 46-year-old female with a past medical history of alcoholic cirrhosis presented with 3 months of jaundice, right upper quadrant pain, nausea, vomiting, diarrhoea, dark urine, and abdominal distension. Blood work showed elevated liver enzymes, PT and INR. Hepatitis panel was negative. Imaging studies and biopsy confirmed Budd-Chiari Syndrome (BCS).

Imaging Findings

Abdominal ultrasound demonstrated a nodular liver contour suggestive of cirrhosis. The hepatic veins demonstrated stenosis and absent flow. Doppler imaging showed reversal of flow in the portal vein. CT abdomen with IV contrast showed an enlarged liver with heterogeneously diminished attenuation, stenosis of the hepatic veins with early enhancement of the central region and hypo-attenuation of the periphery, hypertrophied caudate lobe, and atrophy of the remaining segments suggestive of budd chiari syndrome [9]. Additionally, the CT demonstrated enlarged and tortuous variceal vessels communicating with the superior mesenteric vein in addition to ascites [10]. A venacavogram was done for further confirmation and showed a stenotic hepatic vein. Intrahepatic IVC stenosis caused intrahepatic hypertension with an intrahepatic-extrahepatic IVC pressure gradient of 13mmHg. Portal-capillary wedge pressure measured 20mmHg indicating portal hypertension.

Discussion

Background:
BCS results from vascular occlusion of the hepatic outflow tract anywhere from the junction of the IVC and the right atrium to the small hepatic veins (HV) with an incidence of 1/2.5 million per year [1]. The pathophysiology of BCS involves vascular obstruction leading to hepatic sinusoidal congestion and portal hypertension. Primary BCS can be associated with an underlying prothrombotic disorder such as myeloproliferative disorder [2]. Secondary BCS occurs when lesions that originates outside of the vein such as malignancies of adjacent organs, polycystic kidneys, or parasitic infections [8] compress or invade the HVs or the IVC.

Clinical Perspective:
An initial high index of suspicion plays a major role. Severe right upper quadrant pain is a common symptom of acute disease. Patients can present with ascites and hepatosplenomegaly. Paracentesis demonstrates findings consistent with portal hypertension [3].

Imaging Perspective:
In acute disease, US can be used to show distended HVs and partial or complete thrombosis of HVs/IVC. Color doppler may show intrahepatic collateralization with opposing flow in adjacent veins, and hepatofugal flow in the portal vein [9]. CT shows hyperdense thrombus within the vessel with hypoattenuation of an enlarged liver. Contrast enhancement is stronger in regions surrounding the IVC and caudate lobe [9]. MRI demonstrates hypointensity on T1 images, and heterogeneous hyperintensity on T2 images because the congested liver has a high water content predominantly in the periphery of the hepatic lobes [4]. T1 post-contrast sequences shows occlusion of the hepatic veins and ICV [5].

In chronic BCS, US reveals caudate hypertrophy, and stenosis/thrombosis of the HVs [9]. MRI enhancement is variable with decreased T1 and T2 signal intensity with delayed enhancement on post-contrast images [6]. T1 sequences are optimal for demonstrating hyperintense regenerative nodules which show venous phase enhancement without washout distinguishing them from HCC [9].

Venogram demonstrates a spider web pattern of HV collaterals and long segmental compression or stenosis of the HVs/IVC.

Outcome:
Anticoagulation is the cornerstone of treatment. Vitamin K antagonist with a goal INR of 2-3⁷is standard. Anticoagulation is often insufficient and treatment typically requires a multimodal approach. Invasive treatments include local balloon angioplasty or stenting of the IVC and hepatic veins, TIPS and liver transplantation [2]. 5-year survival is about 90% [1].

Take Home:
BCS is rare and caused by occlusion of the hepatic outflow tract. US, MRI and CT have good sensitivity and specificity for BCS. Venography is the gold standard. Treatment is usually multimodal depending on the patient’s clinical picture.

Differential Diagnosis List

Budd-Chiari Syndrome

Hepatocellular Carcinoma

Alcoholic cirrhosis

Non-alcoholic fatty liver disease

Hemochromatosis

Drug induced hepatitis

Hepatic venous congestion secondary to right-sided heart failure

Other Malignancy

Final Diagnosis

Budd-Chiari Syndrome

References

[1] Darwish MS, Plessier A, Hernandez-Guerra M, et al. (2009) Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med 151: 167–175. (PMID: 19652186)

[2] Martins P, Nevens F (2015) Budd-Chiari Syndrome. United European Gastroenterol J Dec; 3(6): 489–500 (PMID: 26668741)

[3] Chung RT, Iafrate AJ, Amrein PC, et al. (2006) Case records of the Massachusetts General Hospital. Case 15-2006. A 46-year-old woman with sudden onset of abdominal distention. N Engl J Med 354: 2166–2175. (PMID: 16707754)

[4] Soyer P, Rabenandrasana A, Barge J, et al. (1994) MRI of Budd-Chiari syndrome. Abdom Imaging 19(4): 325–329. (PMID: 8075555)

[5] Cura, M., Haskal, Z., & Lopera, J. (2009) Diagnostic and Interventional Radiology for Budd-Chiari Syndrome. Radiographyics 29(3), 669-698. (PMID: 19448109)

[6] Noone TC, Semelka RC, Siegelman ES, et al. (2000) Budd-Chiari syndrome: spectrum of appearances of acute, subacute, and chronic disease with magnetic resonance imaging. J Magn Reson Imaging 11 (1): 44–50. (PMID: 10676619)

[7] DeLeve LD, Valla DC, Garcia-Tsao G, et al. (2009) Vascular disorders of the liver. Hepatology 49: 1729–1764. (PMID: 19399912)

[8] Musa A, Bayraktar Y (2007) Budd-Chiari syndrome: Etiology, pathogenesis and diagnosis. World J Gastroenterol 13(19): 2693–2696 (PMID: 4147117)

[9] Brancatelli G et al (2007) Budd-Chiari Syndrome: Spectrum of Imaging Findings. AJR Am J Roentgenol Feb;188(2):W168-76. (PMID: 17242224)

[10] Valla DC (2003) The diagnosis and management of the Budd-Chiari syndrome: consensus and controversies. Hepatology 38:793–803 (PMID: 14512865)

Case information

URL:	https://www.eurorad.org/case/14246
DOI:	10.1594/EURORAD/CASE.14246
ISSN:	1563-4086

License

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Figure 1

Ultrasound

Ultrasound images demonstrate stenosed hepatic veins and ascites with a nodular liver contour, suggestive of cirrhosis.

Gray scale ultrasound images demonstrate stenosed hepatic veins.

Hepatofugal flow in portal vein is demonstrated on these colour Doppler images of the abdomen.

US of the liver shows hepatofugal flow in the portal vein, which is indicative of continuous flow with reduced velocity. This a sign that can be visualized in the acute phase of budd chiari syndrome.

colour Doppler images demonstrate no flow in the hepatic veins

colour Doppler images demonstrate stenosed hepatic veins.

Figure 2

CT Abdomen

Axial CT demonstrates stenosed hepatic veins. Ascites and capsular retraction is identified.

Axial CT shows obliteration of the hepatic veins, with early enhancement of the central portion around the IVC, with decreased peripheral liver enhancement.

Axial CT shows hypertrophied caudate lobe, atrophy of the remaining liver segments with collateralization are findings that can be seen in the chronic stage of budd chiari syndrome.