Va s c u l a r a n d I n t e r ve n t i o n a l R a d i o l o g y • R ev i ew

Ferral et al.
Budd-Chiari Syndrome

Vascular and Interventional Radiology

Review
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FOCUS ON:

Budd-Chiari Syndrome
Hector Ferral1 OBJECTIVE. Budd-Chiari syndrome (BCS) is an uncommon condition characterized
George Behrens 2 by obstruction of the hepatic venous outflow tract. Presentation may vary from a completely
Jorge Lopera3 asymptomatic condition to fulminant liver failure. BCS is an example of postsinusoidal portal
hypertension. The management can be divided into three main categories: medical, surgical,
Ferral H, Behrens G, Lopera J and endovascular. The purpose of this article is to present an overall perspective of the prob-
lem, diagnosis, and management.
CONCLUSION. BCS requires accurate, prompt diagnosis and aggressive therapy.
Treatment will vary depending on the clinical presentation, cause, and anatomic location of
the problem. Patients with BCS are probably best treated in tertiary care centers where liver
transplantation is available.

Keywords: Budd-Chiari syndrome, stents, thrombolysis,
transjugular intrahepatic portosystemic shunts
DOI:10.2214/AJR.12.9098
Received April 16, 2012; accepted without revision
April 23, 2012.
1
Department of Radiology, Section of Interventional
Radiology, NorthShore University HealthSystem, 2650
Ridge Ave, Evanston, IL 60201. Address correspondence
to H. Ferral (hectorferral@gmail.com).
2
Department of Radiology, Section of Interventional
Radiology, Rush University Medical Center, Chicago, IL.
3 struction to hepatic venous outflow is related
Department of Radiology, Section of Interventional
Radiology, University of Texas Health Science Center,
San Antonio, TX.
AJR 2012; 199:737–745
0361–803X/12/1994–737
© American Roentgen Ray Society
B
udd-Chiari syndrome (BCS) is an
uncommon condition character-
ized by obstruction of the hepatic
venous outflow tract; it has been
described to occur in 1 in 100,000 of the popu-
lation worldwide [1, 2]. The term “Budd-
Chiari” was coined in the late 1800s after the
work of George Budd, an internist, who de-
scribed three cases of hepatic vein thrombosis
in 1845 and Hans Chiari, an Austrian pathol-
ogist, who reported the first pathologic de-
scription of “obliterating endophlebitis of the
hepatic veins” in 1899 [1]. The current defi-
nition of BCS encompasses a number of con-
ditions that cause obstruction of the hepatic
outflow tract from the small hepatic veins to
the junction of the inferior vena cava (IVC)
and right atrium [3–5]; this condition is a clas-
sic example of postsinusoidal portal hyperten-
sion [6, 7]. This term does not include cardiac
or pericardial causes of hepatic outflow ob-
struction or the sinusoidal problems related to
toxic substance exposure [3, 8].
BCS is classified as primary when the ob-
to a primary venous problem, such as throm-
bosis, stenosis, or webs [1, 9], and as second-
ary when it is related to extrinsic compres-
sion, such as that caused by abscess, tumor,
cyst, or hyperplastic nodules [2, 3, 10–12].
The presentation and cause of BCS may vary
depending on the geographic area of presen-
tation [3]. Interestingly, obstruction of the
IVC with or without involvement of the he-
patic veins is predominant in Asia [3, 13],
and pure hepatic vein obstruction predomi-
nates in Western countries.
Clinical presentation may vary from a
completely asymptomatic condition to ful-
minant liver failure [13]. The management
of BCS can be divided into three main cat-
egories: medical, surgical, and endovascu-
lar [14–17]. The purpose of this article is to
present an overview of the most important
aspects of this uncommon clinical entity.
Cause and Risk Factors
Common causes of BCS include inherited
and acquired hypercoagulable states [18]. In-
herited hypercoagulable states such as factor
V Leiden mutation, protein C deficiency, pro-
tein S deficiency, the prothrombin G20210A
mutation, and antithrombin III deficiency are
common causes of hepatic vein thrombosis re-
sulting in BCS. Acquired prothrombotic states
such as myeloproliferative disorders (e.g.,
polycythemia vera, paroxysmal nocturnal he-
moglobinuria, essential thrombocytosis, agno-
genic myeloid metaplasia, and myelofibrosis)
account for more than 50% of BCS cases [3,
4, 18]. Other prothrombotic conditions, such
as antiphospholipid syndrome, have also been
mentioned. Other conditions have been pro-
posed as risk factors for the development of

AJR:199, October 2012 737


BCS, including Behçet disease, hypereosino-
philic syndrome, and ulcerative colitis [3] and
pregnancy, malnutrition, and the use of oral
contraceptives [4, 19].
Pathophysiology
Once the hepatic veins occlude, the liver
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venous outflow is compromised, the sinusoi-
dal and portal pressures increase, and the por-
tal flow decreases; if this process continues, it
leads to hepatic congestion; formation of asci-
tes; and, in certain cases, portal vein thrombo-
sis [18]. Hepatocytes undergo hypoxic damage
that eventually evolves into noninflammatory
centrilobular cell necrosis [1, 20]. If this hepa-
tocellular damage is massive, the patient will
present with a fulminant form of BCS, which
is a potentially fatal condition [21]. Otherwise,
the process evolves slowly and allows the pa-
tient to develop portal hypertension and as-
cites; histologically, the liver develops fibro-
sis and, in later stages, cirrhosis [1]. If BCS
is not treated, the natural course is that of a
progressive and fatal condition. Previous re-
ports, when no specific therapy was available,
showed that 90% of patients with BCS had
died by 3 years [3]. The most common causes
of death are intractable ascites, gastrointestinal
bleeding, and liver failure.
Clinical Presentation
Clinical presentation of BCS may be ful-
minant (5%), acute (20%), and subacute or
chronic (60%) [13]. BCS may be asympto-
matic in approximately 15–20% of cases. A
lack of symptoms is associated to the fact that
only a single hepatic vein is thrombosed or
that venous outflow is carried by large hepat-
ic vein collaterals [3, 22, 23]. These clinical
manifestations may be directly related to the
speed of the underlying obstructive process
[3, 4]. Approximately 75–80% of patients
with BCS will have clinical manifestations;
the most common include fever, abdominal
pain, abdominal distention, ascites, liver fail-
ure, lower extremity edema, gastrointestinal
bleeding, and encephalopathy [3, 13].
Fulminant Budd-Chiari Syndrome
Fulminant BCS develops in a few days;
patients present with severe liver failure with
elevation of hepatic enzymes, hyperbiliru-
binemia, encephalopathy, and coagulopa-
thy [24]. The liver is massively enlarged and
painful [18, 20, 23]. Ascites and renal failure
are constant [20]. Renal failure may be sec-
ondary to severe compression of the hepatic
vein with compromise of the renal outflow.
738 AJR:199, October 2012
Ferral et al.
Acute Budd-Chiari Syndrome
Acute BCS develops usually within 1
month and is characterized by intractable as-
cites, abdominal pain, liver enlargement, re-
nal failure, elevation of hepatic enzymes, and
coagulopathy [20]. Histologically, hepatic
congestion and necrosis are present.
Subacute Budd-Chiari Syndrome
Subacute BCS is the most common clinical
type. This form of BCS has an insidious onset
and it may take as long as 3 months to become
asymptomatic. Biopsy shows minimal hepatic
necrosis and trace or no ascites. The develop-
ment of the anatomic problem is slow and al-
lows time for decompressive collaterals to de-
velop [18]. Patients with subacute BCS do not
have esophageal bleeding.
Chronic Budd-Chiari Syndrome
The chronic form of BCS is characterized by
the development of portal hypertension. Histo-
logically, patients with chronic BCS have con-
gestive cirrhosis [23]. There is progressive ab-
dominal distention due to ascites. The liver
function tests may be minimally affected or nor-
mal [20]. Renal failure is seen in 50% of patients
with chronic BCS [20]. Esophageal bleeding is
seen in 5–15% of these patients, and splenomeg-
aly is common in the chronic from [20].
Diagnosis and Morphologic
Classification
The diagnosis of BCS should be clinical-
ly suspected in patients who present with any
one of the following findings: fulminant liver
failure with abrupt onset of ascites and hepa-
tomegaly; massive ascites with relatively pre-
served liver function; unexplained chronic liv-
er disease; or liver disease and an associated
thrombogenic disorder [1]. BCS may be clas-
sified into three types depending on the type of
existing venous occlusion [8]. Type I is limit-
ed to the IVC. In type II BCS, lesions are with-
in the hepatic veins. If lesions are short-seg-
ment occlusions (< 4 cm), type IIa BCS is the
diagnosis. BCS type III is the mixed type with
involvement of the IVC and hepatic veins.
Imaging studies play an important role in
confirming the diagnosis of BCS by show-
ing the venous abnormalities. Probably the
most useful imaging methods include con-
ventional and Doppler ultrasound, CT, MRI,
and catheter venography [8].
Ultrasound
An enlarged caudate lobe, hepatomegaly,
lack of visualization of the hepatic veins, a
compressed IVC, enlarged intrahepatic col-
laterals, splenomegaly, and ascites are con-
ventional sonographic findings in patients
with BCS [8, 25, 26]. In some instances, an
enlarged caudate lobe vein (> 3 mm) can be
seen draining directly into the IVC, a spider-
web appearance of hepatic veins or replace-
ment of the hepatic vein by a fibrous, echo-
genic cord [3, 8, 27–30]. Ultrasound may
also show that the stenotic IVC, especially
in the intrahepatic segment, is associated
with an enlarged caudate lobe [8]. In some
chronic cases, large regenerative nodules
that may simulate carcinoma may be pres-
ent [30, 31]. On Doppler evaluation, patients
with BCS may present with enlarged hepat-
ic veins with no flow signal or with reversed
flow. The identification of collateral vessels
with drainage into the subcapsular or inter-
costal veins is a highly sensitive and specific
feature for the diagnosis of BCS [20]
CT
CT findings in acute BCS may include nor-
mal liver morphology, patchy enhancement,
an enlarged caudate lobe, a compressed IVC,
the absence of hepatic veins, and ascites [25,
27, 32] (Figs. 1–3). The patchy enhancement
seen on CT scans of patients with BCS is re-
lated to the stasis in the sinusoids and por-
tal vein and is associated with increased en-
hancement of the central portion of the liver
parenchyma [29, 32, 33]. Acute BCS may
present with acute thrombosis of the IVC,
iliac veins, and femoral veins (Fig. 4); this
presentation of acute BCS is rare but may be
seen in young patients taking oral contracep-
tives. Findings in subacute BCS depend on
the type of venous involvement and may in-
clude the presence of portosystemic and in-
trahepatic collaterals as well as hepatic artery
enlargement. Typically, the IVC is com-
pressed but may be thrombosed; patients may
also present with portal vein thrombosis that
is secondary to the profound flow disturbance
[25, 34]. Chronic BCS is associated with an
alteration in the morphology of the liver and
regenerative nodule formation. These nod-
ules typically show hyperattenuation in the
arterial phase and remain hyperattenuating
in the portal phase; patchy enhancement may
also be seen in this stage of the disease [25].
MRI
MRI is a useful imaging tool in the diagnosis
of BCS. Its main advantage is the lack of ioniz-
ing radiation. The anatomic findings identified
by ultrasound and CT are also depicted on MRI

including enlarged liver, inhomogeneous signal
intensity of the parenchyma on spin-echo imag-
es (Fig. 5), intrahepatic collaterals, caudate lobe
enlargement, regenerative nodules, and ascites
[25]. BCS is identified on MRI by the absence
of blood flow in the occluded veins. Detection
of thrombus causing an absence of flow may be
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difficult on spin-echo images because thrombus
may manifest as different signal intensities de-
pending on its age and composition [25].
MR angiography is useful in the diagnosis
of BCS because it shows the vascular chang-
es seen in this disease entity. The most use-
ful technique is 3D contrast-enhanced MR
angiography because it shows the vascu-
lar anatomy in multiple projections, includ-
ing the presence of intrahepatic collaterals.
This technique may also show the presence
of thrombus within the hepatic veins, portal
vein, and IVC [35].
Catheter Venography
Patients with BCS are usually referred to
an interventional radiologist to confirm the
diagnosis and for therapeutic interventions.
Catheter venography is considered the refer-
ence standard for the diagnosis of this con-
dition [8]. If properly performed, catheter
venography provides anatomic information
by precisely depicting the venous problem,
hemodynamic information through pressure
measurements, and histologic information
by obtaining a transjugular liver biopsy and
allows the possibility of endovascular man-
agement of the condition. Access into the he-
patic venous system may be achieved using
a transvenous approach via either an inter-
nal jugular vein or the common femoral vein
(Fig. 6). In cases in which retrograde access
into the hepatic veins is difficult or impos-
sible, a transhepatic approach may be used
to visualize these veins (Fig. 7). Diagnostic
venography in patients with BCS may show
severe stenotic areas in the hepatic veins
very close to the junction with the IVC (Fig.
6), complete occlusion of the hepatic veins
with the formation of large intraparenchy-
mal collaterals (Fig. 7), occlusion of the he-
patic veins associated with a spiderweb ap-
pearance of the hepatic parenchyma, (Fig. 8)
and severe stenosis of the IVC secondary to
hypertrophy of the caudate lobe [4] (Fig. 9).
Management
Medical Management
Medical management of BCS is focused
on the treatment of the underlying cause: con-
trol of portal hypertension, ascites control, sys-
AJR:199, October 2012 739
Budd-Chiari Syndrome
temic or catheter-directed thrombolysis, and
anticoagulation [1, 13, 17, 18, 36]. Medical
management is usually insufficient and most
patients will require either surgical or en-
dovascular intervention [17]. Most patients
who undergo endovascular or surgical man-
agement will need long-term anticoagulation
because most have an underlying prothrom-
botic disorder [4, 18, 37].
Surgical Management
Surgical management includes membrane
resection, IVC reconstruction with a pericar-
dial patch, portosystemic shunts, mesoatrial
shunts, portoatrial shunts, and liver trans-
plant [15, 38–41]. Surgical portosystem-
ic shunts (portocaval or mesocaval shunt)
placed below the diaphragm will fail if the
IVC is thrombosed or severely compressed
because the system will not be properly de-
compressed [13]; in these cases, combined
endovascular IVC stenting followed by sur-
gical portosystemic shunting is an option for
optimal system decompression [42]. Dang
and coworkers [39] documented clinical suc-
cess in 87.7% of patients with BCS who were
treated with membrane resection, making it
a feasible option for patients with this con-
dition. Inafuku and coworkers [15] evaluat-
ed 53 patients with IVC reconstruction with
the creation of a pericardial patch. The intra-
operative mortality was 3.7% and the 5- and
10-year survival was 89.8% and 70.7%, re-
spectively [15].
Liver transplant is another reported ther-
apeutic approach for BCS. Most investiga-
tors agree that patients with fulminant BCS
should be immediately listed for a liver trans-
plant and undergo emergency liver transplant
as soon as feasible [17]. The first liver trans-
plant for BCS was performed in 1974 [17],
and the reported survival rates of patients
with BCS 1 and 3 years after liver transplan-
tation are 70% and 45%, respectively [17,
43]. Results from a European registry re-
corded between 1988 and 1999 reported 1-,
5-, and 10-year survival rates of 248 patients
with BCS treated with liver transplant were
75%, 71%, and 68% [17, 44]. Most authors
agree that not all patients with BCS should
undergo liver transplant and that this thera-
peutic option should probably be used exclu-
sively in patients with fulminant BCS or in
patients with chronic cirrhosis [17, 18].
Endovascular Management
Endovascular management of BCS will
depend on the type of venous abnormality to
be treated and involves application of vari-
ous techniques including balloon angioplasty,
stent placement, catheter-directed thromboly-
sis, thrombus maceration, and endovascular
portosystemic shunt creation (transjugular in-
trahepatic portosystemic shunt [TIPS] proce-
dure) [37, 45–50]. Isolated hepatic vein or IVC
webs, stenoses, or occlusions may be success-
fully treated with balloon angioplasty or stent
placement only with good technical outcome
(Figs. 10 and 11); however, the long-term re-
sults of these different therapeutic techniques
are not entirely proven because most studies
have included a small number of patients or
are isolated case reports. If an isolated hepatic
vein or IVC intervention fails, the next endo-
vascular step is TIPS creation [18]. The ma-
jor advantages of the percutaneous approach
are that it is minimally invasive and that it cre-
ates a portosystemic shunt directly into the
suprahepatic IVC, which essentially func-
tions as a portoatrial shunt; if TIPS is prop-
erly created, it will effectively decompress
the portal vein and IVC with a single proce-
dure. The results of TIPS procedures in pa-
tients with BCS have been encouraging, with
a more than 90% technical success rate and a
more than 75% clinical success rate report-
ed [37, 48, 51]. The most common technical
challenge is the recanalization of the occluded
hepatic vein. TIPS creation with bare metal-
lic stents has the disadvantage of reduced pa-
tency rates [48], whereas a TIPS created with
covered stents have shown better patency rates
[14, 37, 48, 52]. Gandini and coworkers [48]
found much higher patency rates in patients
who underwent TIPS with expanded poly-
tetrafluoroethylene covered stents. The mean
patency duration was 4.4 months for patients
treated with bare stents compared with 22.2
months for patients treated with stent-grafts.
The 6- and 12-month patency rates were 100%
and 85.7%, respectively, for stent-grafts com-
pared with 16.7% and 0% for bare stents (p <
0.001) [48]. TIPS is minimally invasive and, if
performed by experienced operators, it has a
high technical and clinical success rate. Long-
term shunt patency is one of the most common
problems and most patients with BCS require
long-term anticoagulation.
Conclusion
BCS is an uncommon clinical condition
that requires accurate, prompt diagnosis and
aggressive therapy. Treatment will vary de-
pending on the clinical presentation, cause,
and anatomic location of the problem. Med-
ical, surgical, and endovascular therapeutic

options are available for the management of
this condition. Patients with BCS are proba-
bly best treated in tertiary care centers where
liver transplant is available.
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ology 2006; 241:298–305
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intrahepatic portosystemic shunt patency and bectomy device and transjugular liver biopsy can- for primary transjugular intrahepatic portosys-
clinical outcome in patients with Budd-Chiari nula for mechanical thrombectomy of the inferior temic shunts? Semin Intervent Radiol 2005; 22:
syndrome: covered versus uncovered stents. Radi- vena cava in a patient with Budd-Chiari syndrome. 287–299

Fig. 1—Axial contrast-enhanced CT scan of abdomen of 30-year-old woman with Fig. 2—Axial contrast-enhanced CT scan of abdomen of 30-year-old woman with
Budd-Chiari syndrome. Note patchy enhancement of liver (arrow) and absence of Budd-Chiari syndrome. Note massive enlargement of caudate lobe (CL) and patchy
hepatic veins. Asterisk = presence of ascites. enhancement of liver (arrow). Asterisk = presence of ascites, arrowhead = inferior
vena cava.

Fig. 3—Contrast-enhanced CT scan of abdomen of 19-year-old woman with

subacute Budd-Chiari syndrome. Note absence of hepatic veins, enlargement
of liver, compression of inferior vena cava, and presence of ascites (asterisks).
Arrowhead = inferior vena cava.

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A B
Fig. 4—15-year-old girl with acute Budd-Chiari syndrome who presented with acute inferior vena cava (IVC) thrombosis.
A, Contrast-enhanced CT scan shows enlarged caudate lobe (CL) and lack of opacification of IVC; these findings indicate presence of acute thrombosis.
Arrowhead = thrombosed IVC.
B, Contrast-enhanced CT scan obtained at lower level than A shows extension of IVC thrombosis down to level of renal veins. Renal veins are also thrombosed
(arrowheads).

Fig. 5—Axial gadolinium-enhanced MR image

of 32-year-old man with Budd-Chiari syndrome.
Note hyperintensity of periphery of liver (arrows).
Arrowhead = compressed inferior vena cava.

Fig. 6—Intraparenchymal injection of CO2 in

24-year-old woman with Budd-Chiari syndrome.
Image was obtained during transjugular intrahepatic
portosystemic shunt (TIPS) procedure. Lower
accessory hepatic vein is opacified and there is
severe stenosis (arrow) of hepatic vein at junction
with inferior vena cava. 

Fig. 7—Transhepatic hepatic venogram shows

occlusion of middle hepatic vein (arrow) in 24-year-old
woman with Budd-Chiari syndrome. Note fugal flow
of contrast material into large intrahepatic collaterals
and no opacification of inferior vena cava. 

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Fig. 8—Contrast injection into liver parenchyma
in 24-year-old man with Budd-Chiari syndrome
(BCS). Image was obtained during transjugular
liver biopsy procedure. Biopsy needle has been
advanced into parenchyma and small amount of
contrast material has been injected. Image shows
spiderweb appearance typical of BCS. Hepatic vein
is not opacified, indicating thrombosis of that vessel.
Arrow = spiderweb deformity of veins.
Fig. 9—Inferior venacavogram of 30-year-old woman
with Budd-Chiari syndrome (BCS). Note severe
stenosis of intrahepatic inferior vena cava (IVC). This
is classic finding in patients with BCS. There is also
opacification of hemiazygos system (arrow). RA =
right atrium.

A B
Fig. 10—44-year-old woman with Budd-Chiari syndrome.
A and B, Axial contrast-enhanced CT scans of abdomen show enlarged liver (arrow) with heterogeneous enhancement with central hyperattenuation. Note caudate lobe
(CL) enlargement, large gastric varices (arrowhead, B), and lack of visualization of hepatic veins and inferior vena cava (IVC).
(Fig. 10 continues on next page)

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C D

E F

Fig. 10 (continued)—44-year-old woman with Budd-Chiari syndrome.

C, Spot film obtained during catheter venography shows selective catheterization of right hepatic vein (HV).
Catheter has been advanced from femoral venous approach. There is complete occlusion of IVC (arrow) just
caudad to right atrium. RA = right atrium.
D, Spot film obtained during catheter venography corresponds to selective left hepatic venogram. Note small,
irregular hepatic vein (HV); opacification of IVC with caudal retrograde flow; and occlusion of IVC (arrow)
immediately caudad to right atrium (RA). This case is example of BCS caused by IVC web or occlusion.
E, Spot film obtained during balloon angioplasty of IVC web. In this particular case, 14-mm high-pressure balloon
was used. IVC occlusion was successfully crossed with wire. Arrow = angioplasty balloon, RA = right atrium.
F, IVC venogram obtained after angioplasty shows treatment of IVC occlusion was successful. RA = right atrium.

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A B

C D

Fig. 11—33-year-old woman with Budd-Chiari syndrome managed with transjugular intrahepatic
portosystemic shunt (TIPS) procedure. RA = right atrium.
A, Spot film obtained during direct portography immediately after gaining transhepatic access into portal vein
shows fugal flow into small left gastric, splenic, and inferior mesenteric veins. Also, there is faint opacification
of intrahepatic inferior vena cava (IVC), which is severely narrowed. Arrow = transparenchymal tract.
B, Spot film obtained during direct portography after TIPS completion shows complete diversion of flow from
main portal vein directly into RA. Arrow = transparenchymal tract.
C, Spot film obtained during IVC venography shows severe stenosis (arrow) of intrahepatic IVC. Note
opacification of hemiazygos vein draining into azygos vein at level of diaphragm.
D, Spot film obtained during IVC venography shows IVC immediately after TIPS. Note that intrahepatic IVC has
decompressed, lumen of vein is now wide, and flow into hemiazygos system is less prominent. Arrow = less
prominent azygos vein.

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