BUDD CHIARI 2010

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Budd-Chiari Syndrome

Michael A. Zimmerman, MD,Andrew M. Cameron, MD, PhD,

R. Mark Ghobrial, MD, PhD*

Division of Liver and Pancreas Transplantation, Department of Surgery,The Pfleger Liver Institute, The Dumont-UCLA Transplant Center,

The David Geffen School of Medicine at the University of California Los Angeles,

10833 Le Conte Avenue, Los Angeles, CA 90095, USA

Budd-Chiari syndrome (BCS) represents a spectrum of disease states re-

sulting in hepatic venous outflow occlusion. Obstruction can occur at any

level from the hepatic venules to the right atrium of the heart. Anatomic ab-

normalities including vascular webs or strictures may be present and possi-

bly predispose to venous thrombosis. Hypercoagulable states often arepresent, and the incidence of concomitant disorders is increasing (Box 1)

[1]. Untreated, BCS has a mortality rate close to 80% [2]. The most common

causes of BCS in the Western world are myeloproliferative disorders, includ-

ing polycythemia vera and essential thrombocytosis [3]. Pregnancy, use of

oral contraceptives, and cancer are also documented causes. An evolving

pathologic concept advocates the presence of a genetic mutation in one or

more genes leading to a hypercoagulable predisposition. Coupled with an

acquired thrombogenic stimulus (ie, cancer), the result is hepatic venous

thrombosis and outflow occlusion. Spontaneous resolution has been re-ported [4], and up to 25% of patients remain asymptomatic [5]. Most pa-

tients, however, require an aggressive multidisciplinary approach,

including invasive radiologic procedures and surgery, to control symptoms

and prevent disease progression. Liver transplantation recently has emerged

as the preferred therapy for patients who have fulminant liver failure or cir-

rhosis [6]. Although the cadaveric organ pool is limited, portosystemic

shunts remain a therapeutic alternative in patients who have preserved liver

function. This article outlines the approach to clinical diagnosis and sup-

portive medical therapy in patients who have BCS and reviews the clinical

* Corresponding author. The Dumont-UCLA Transplant Center, 77-120 CHS, Box

957054, 10833 Le Conte Avenue, Los Angeles, CA 90095.

E-mail address: [email protected] (R.M. Ghobrial).

1089-3261/06/$ - see front matter Ó 2006 Elsevier Inc. All rights reserved.

doi:10.1016/j.cld.2006.05.005 liver.theclinics.com

Clin Liver Dis 10 (2006) 259–273

mailto:[email protected]

mailto:[email protected]



data supporting surgical shunting and liver transplantation as viable treat-

ment options in this patient population.

Diagnosis

Clinical manifestations of BCS classically include hepatomegaly, right

upper quadrant pain, and abdominal ascites. These findings are present in

the majority of patients [7]. The degree of symptomatology corresponds di-

rectly with the rapidity with which hepatic outflow occlusion occurs. Fulmi-

nant and acute forms of BCS are characterized by rapidly worsening

encephalopathy and accumulation of abdominal ascites. Menon and col-

leagues [8] note that subacute BCS is the most common form and presentswith minimal ascites and hepatic necrosis because of sinusoidal decompres-

sion through the collateral venous circulation. Patients who have the

chronic form of BCS present with the usual manifestations of end-stage liver

disease and portal hypertension.

The initial study of choice for documenting hepatic outflow obstruction is

Doppler ultrasound. With an overall accuracy of approximately 70% [9],

Box 1. Causes and associated disorders of Budd-Chiari

syndromeProthrombotic conditionsFactor V Leiden mutation

Antiphospholipid syndrome

Antithrombin III deficiency

Protein C, S deficiencyHyperhomocysteinemia

Myeloproliferative disorders

Polycythemia vera

Essential thrombocytosisParoxysmal nocturnal hemoglobinuria

Oral contraceptive use

Pregnancy

CancerAnatomic webs (inferior vena cava, hepatic veins)

Behcet’s syndrome

Hepatic abscess

Hepatitis

TraumaInflammatory bowel disease

Polycystic liver disease

Idiopathic

260 ZIMMERMAN et al



ultrasound is relatively inexpensive and is readily available at most institu-

tions. A second line of investigation includes either CT scan or MRI. Each

imaging modality has specific advantages. CT scanning allows the evalua-tion of hepatic vein patency, parenchymal abnormalities, and the degree

of abdominal ascites. The presence of caudate lobe hypertrophy also may

be assessed (Fig. 1) [10]. A well-documented compensatory mechanism fol-

lowing hepatic venous outflow obstruction, significant caudate lobe enlarge-

ment, has serious implications for surgical intervention. The role of MRI

continues to evolve as technology is enhanced. Noone and colleagues [11]

suggest that MRI may be valuable in differentiating acute from chronic dis-

ease. More importantly, it may allow further definition of the vascular anat-

omy, including patency of the supra- and infrahepatic vena cava, as welldistinguishing benign from malignant disease [12]. Although not absolutely

essential, hepatic venography still is considered the criterion standard imag-

ing technique. Precise definition of vascular abnormalities may directly in-

fluence therapeutic interventions. Further, infra- and suprahepatic caval

Fig. 1. Portosystemic shunt with caudate lobe hypertrophy. (A) The caudate lobe is enlarged on

both sides of the inferior vena cava (IVC) and portal vein. (B) A side-to-side portacaval shunt is

in place and patent.

261BUDD-CHIARI SYNDROME



pressures can be measured, and a liver biopsy can be obtained to assess the

degree of inflammation or fibrosis [13].

Pathology

Hepatic venous outflow occlusion provides an excellent clinicopathologic

model of hepatocellular injury and regeneration. The continuum of histo-

logic alteration ranging from severe sinusoidal congestion and inflammation

to fibrosis and ultimately cirrhosis is well documented in this patient popu-

lation [14]. Fortunately, there is no clinical correlation between the histo-

logic presence of congestion or fibrosis and overall patient survival [15].

Hepatic explants following liver transplantation in patients who have BCS

contain regenerative hyperplastic nodules [16]. These neoplastic foci repre-

sent a spectrum of cellular changes from hyperplasia to adenomas. Al-

though the relationship once was considered controversial, hepatocellular

carcinoma also is associated with BCS by two mechanisms. Hepatocellular

carcinoma can cause hepatic vein or inferior vena cava (IVC) obstruction by

direct vascular invasion of the tumor, resulting in secondary outflow occlu-

sion. Alternatively, hepatocellular carcinoma can develop de novo in pa-

tients who have BCS and cirrhosis [17].

An important study by Tanaka and colleagues [18] describes the ‘‘two-

hit’’ vascular hypothesis for the development of regenerative nodules char-

acteristic of BCS. Examining 15 explants following liver transplantation,

they define several different forms of cirrhosis in patients who have chronic

disease. This study identified a definitive link between the presence or ab-

sence of portal vein thrombosis and the specific pattern of cirrhosis. Patients

who had severe portal vein obliteration developed veno-portal cirrhosis,

a form of fibrosis in which the septa bridge the portal tracts and hepatic

veins. Specimens without portal vein occlusion develop fibrosis that includes

only the hepatic vein. Further, the authors propose a vascular mechanismfor the formation of regenerative nodules initiated by ischemia and inflam-

mation subsequent to outflow obstruction. A concomitant decrease in portal

perfusion leads to portal vein thrombosis with a compensatory increase in

arterial perfusion. Nodular regeneration is histologically related to these

areas of parenchymal hyperemia. Other groups have confirmed these obser-

vations [19–22]. Whether these regenerative lesions undergo malignant

transformation is unknown.

Medical management

Medical management of BCS centers on diagnosis and aggressive treat-

ment of the underlying cause as well as controlling abdominal pain and as-

cites. All patients should undergo a hypercoagulable work-up to identify

any predisposition to venous thrombosis. Anticoagulation therapy with

262 ZIMMERMAN et al



successful. Primarily, the thrombus must be recent and incompletely occlu-

sive. Total occlusion of the vessel portends a worse prognosis. To achieve

high concentrations of thrombolytic agent, the drug must be delivered eitherimmediately proximal or within the thrombus. They suggest that tissue plas-

minogen activator is the preferred agent because it has a very short half-life

(5 minutes), and that systemic therapy is of little value.

Balloon angioplasty and stent placement in the IVC and hepatic veins are

useful adjuncts to medical therapy. Stent placement is recommended be-

cause reocclusion occurs frequently after angioplasty [37]. A recent series

of 115 patients who had BCS reported successful stent placement in the

IVC and hepatic veins of 94% and 87%, respectively [38]. Stent patency

with a mean follow-up of more than 45 months was greater than 90%. Clin-ically, almost all patients who had patent stents improved symptomatically.

Seventeen patients had combined IVC and hepatic vein occlusion. Angio-

plasty and stent placement were undertaken first in the IVC, with the hepatic

vein stent placed 1 week later. During long-term follow-up only one patient

developed hepatic vein occlusion (possibly reocclusion), and all IVC stents

were open. A caveat of this report is that all patients who had hepatic

vein occlusion had obstruction of all three veins. Only one vein was stented

in each case, with a universally successful result. Thus, the entire liver can be

decompressed by intrahepatic venous collaterals.Although thrombolytic therapy, angioplasty, and stent placement can be

effective in the acute setting, most patients present weeks to months after he-

patic vein occlusion. Thus, formation of an intrahepatic shunt between the

hepatic veins and main branch of the portal vein, transjugular intrahepatic

portosystemic shunt (TIPS), is an extremely effective method of splanchnic

decompression [39]. Indications for the TIPS procedure have been extended

to select patients who have BCS in both elective and emergent situations

[40,41]. Several authors have documented rapid improvement in symptoms

and liver function after successful shunt placement [42,43]. Similar to angio-plasty and stenting, TIPS does not require surgical exploration and can be

applied in high-risk patients. TIPS also can be employed to manage patients

who have concomitant hepatic outflow obstruction and portal vein throm-

bosis [44].

Few large series exist documenting TIPS placement in patients who have

BCS. Attwell and colleagues [45] at the University of Colorado recently re-

ported their experience in 17 patients who had BCS and undergoing TIPS

placement. With 100% success with stent placement, 82% of patients expe-

rienced improved symptoms or liver function in the short term. Unfortu-nately, at 3-years follow-up only 47% of patients were clinically well.

Twenty-nine percent of the patients in this series ultimately required liver

transplantation. Because three of these patients presented with fulminant

hepatic failure and were acutely stabilized using TIPS, the authors conclude

that TIPS may serve as a bridge to definitive therapy. Almost one in four

patients experienced a complication, however. Overall, seven patients

264 ZIMMERMAN et al



constructed between the superior mesenteric vein and the right atrium. Portal

pressures were reduced by 20 cm H2O. A recent surgical experience in more

than 1300 patients, including both mesocaval and mesoatrial shunts, reports

an overall complication rate of 15% and a mortality rate of only 3% [58].Orloff and colleagues [60] recently reported a prospective series of 60 pa-

tients who had BCS over a 27-year period, treated with a portosystemic

shunt or liver transplantation. Overall, 50 patients underwent a shunt oper-

ation, and 10 were referred for liver transplantation. The authors make sev-

eral important observations. This is the single largest reported series of

patients who had BCS with isolated hepatic vein occlusion treated by

a side-to-side portacaval shunt (n ¼ 32). With a mean follow-up of almost

14 years, 30-day and overall survival rates are 97% and 94%, respectively.

Only 3% of patients in this group have developed ascites or hepatic enceph-alopathy or have required diuretics in this time interval. Additionally, only

10% of these patients have abnormal liver function tests. Ninety-seven per-

cent had no evidence of parenchymal congestion or hepatocellular necrosis

on biopsy.

A second group of patients had combined hepatic vein obstruction and

IVC occlusion/stenosis (Fig. 3). Over the course of this study the treatment

Table 1

Surgical therapy for Budd-Chiari syndrome: portosystemic shunt versus orthotopic liver

transplantation

Author

[Reference] # in Study Shunt (#)

Liver

Transplant (#) Comments

Ringe [59] 50 12 43 10-year survival after transplant was

69%. Success rate in shunt group

was only 29%.

Mahmoud [4] 44 16 10 37% of shunt patients died within

30 days of surgery. 90% of patients

died after OLT.

Zeitoun [26] 82 82 0 Overall 10-year survival 57%. Shunt

surgery had no impact on survival

versus medical therapy alone.

Orloff [60] 60 50 7 Overall survival rate of 94% after

porto-caval shunt at a mean follow-

up of 13.6 years. 5-year survival rate

after OLT was 30%.

Slakey [56] 43 43 6 5-year survival rate at least 75% for

both shunt and OLT when

treatment was directed at portal

hypertension versus liver failure.

Xu [58] 1360 1360 0 89% success rate at a mean follow-up

of 6.8 years when type of shunt

procedure was tailored to specific

anatomic defect. Recurrence rate

almost 7%.

Abbreviation: OLT, orthotopic liver transplantation.

266 ZIMMERMAN et al



strategy for IVC occlusion changed dramatically. Eight patients were

treated with a mesoatrial shunt (Fig. 4). Unfortunately, five of these patientsdeveloped shunt thrombosis and died. With a 5-year survival of 38%, mes-

oatrial shunting was abandoned in favor of a side-to-side portacaval shunt

combined with a cavoatrial Gore-Tex stent graft. Ten patients treated with

the combined approach have 30-day and overall survival rates of 100% with

mean follow-up of 9 years. Finally, the side-to-side portacaval shunt had an

overall thrombosis rate of 0.2%. The authors conclude that, once the diag-

nosis of BCS is made, there is no reason to delay portal decompression in

the absence of end-stage liver disease. When performed early, portacaval

shunting can stop or reverse ongoing parenchymal injury.

Orthotopic liver transplantation

Liver transplantation is the preferred treatment for patients who have

BCS and acute fulminant failure or cirrhosis and decompensated disease.

Since the first successful transplantation in a patient who had BCS was

Fig. 2. Mesocaval shunt. Synthetic Gore-Tex H-graft shunt between the superior mesenteric

vein and the vena cava.




reported in 1976 [61], several small studies have documented acceptable

long-term survival rates [6,56,62]. In a series of 43 patients who had BCS,Ringe and colleagues [59] reported 10-year survival of nearly 70% following

transplantation. The authors’ experience at UCLA reveals an actuarial sur-

vival rate of 76% at 3 years following liver transplantation [54]. Long-term

laboratory data suggest a slow progression of hepatic disease in shunted pa-

tients. Conversely, patients receiving a transplant maintain higher serum al-

bumin levels and improved synthetic function. Although a cadaveric organ

shortage exists in most countries around the world, many groups are refin-

ing a treatment algorithm that begins with conservative management. Med-

ical therapy and interventional techniques are used early in the diseaseprocess before the patient develops end-stage decompensation. A recent se-

ries notes successful conservative therapy for up to 8 years before transplan-

tation [63].

Unfortunately, lifelong anticoagulation therapy is recommended after

transplantation because BCS does recur [64]. Re-emergence of outflow ob-

struction has been observed from 4 months to 7 years after transplantation

[6]. A series of 23 patients from the University of Pittsburgh receiving trans-

plants for BCS notes three recurrences [65]. All three patients died. The re-

currence rate of outflow occlusion has been reported to be as high as 10%and may require retransplantation [13]. Technical concerns at the time of

transplantation center around prior shunt placement and caudate lobe hy-

pertrophy. Pre-existing portacaval shunts can make the hilar dissection par-

ticularly challenging. At UCLA, all patients who have significant portal

hypertension and prior shunt surgery are placed on venovenous bypass

(both peripheral and portal). This bypass allows portal decompression

Fig. 3. Magnetic resonance venogram with IVC stenosis. (A) Inferior vena cava obstruction

(arrow) is shown at the level of the caudate lobe. (B) Caudate lobe hypertrophy (arrow) is noted

inferior to segment 5.

268 ZIMMERMAN et al



during the dissection, thus reducing blood loss and preserving hemodynamic

stability. Caudate lobe enlargement may make mobilization of the liver ex-

tremely difficult. Optimal exposure is mandatory.

Comments

BCS is a spectrum of disease processes characterized by hepatic venous

outflow occlusion, acute hepatic parenchymal congestion and inflammation,

and ultimately liver dysfunction and cirrhosis. Untreated, BCS is an ex-tremely morbid and lethal condition. Early experience indicates that medical

therapy alone is unsuccessful in most patients. Liver transplantation is the

preferred treatment in the setting of decompensated disease. With a limited

cadaveric organ pool, however, alternate interventions including TIPS and

portosystemic shunts have been employed increasingly. These procedures

may serve as a bridge to transplantation when they are instituted in the acute

Fig. 4. Mesoatrial shunt. Gore-Tex graft from the superior mesenteric vein to the right atrium

of the heart.




setting, in patients who have preserved hepatocellular function. Patients

should be listed for transplantation when cirrhosis and decompensation are

documented. Currently, living-donor liver transplantation is becominga viable treatment option for relatively healthy patients who have end-stage

liver disease. Unfortunately, anatomic abnormalities including caval stenosis

may require complete resection of the IVC at the time of transplantation.

Vascular reconstruction of the hepatic veins and IVC may render living-

donor liver transplantation a practical alternative to cadaveric transplanta-

tion for BCS in the future.

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