Budd Chiari Syndrome: Symptoms, Diagnosis, Treatment And Prognosis

BCS is a heterogeneous disorder related to the obstruction of the hepatic venous outflow tract, where secondary involvement of the portal venous system plays a major aggravating role. Most affected patients carry underlying thrombophilias, whether acquired or inherited.

Increased awareness of clinicians and radiologists on the multiple aspects of this disease should allow early recognition using the highly effective noninvasive imaging that currently is available. Current outcome is improved, likely as a result.

The so-called membranous obstruction of the inferior vena cava (MOVC), in effect, also blocks blood flow from the hepatic veins, and is included in BCS. Although MOVC has been reported from the West, it occurs much more frequently in developing countries such as South Africa and India.

What Causes of budd chiari syndrome

Budd Chiari syndrome

The role of thrombosis in causing primary BCS is still a matter of debate. Primary endophlebitis and congenital malformation have been proposed as alternative explanations. Indeed, explanted livers. Rather, fibrous subendothelial thickening is found, involving various length of the veins, with or without superimposed thrombosis. Short-length stenosis can take the aspect of a membrane, suggesting a congenital anomaly.

However, short-length stenoses are now observations: the transformation of recent thrombi into short-length stenoses; the presence of venous wall remnants within the stenosed segment; the similarly high prevalence of underlying thrombophilias in patients with and without short-length stenoses; and presentation of most patients with short-length stenoses in adulthood, arguing strongly against a congenital malformation. Short-length stenoses are found in about 25% of patients with inferior vena cava (IVC) obstruction.

Indeed, IVC thrombosis is generally associated hepatic veins, or may follow hepatic vein thrombosis.

Moreover, comprehensive investigations show similar underlying thrombophilias for both sites.  BCS is preferably used for both entities, although the level of obstruction in the hepatic venous. Portal hypertension arises.

What remains to be elucidated is why the IVC is involved so frequently in the far East and relatively spared in the West. Consistent with current views on deep vein thrombosis, has become increasingly clear that several thrombophilias are commonly combined in BCS patients.

The differences can be explained by heterogeneous diagnostic criteria, different background ethnicity, and small patient samples.

Symptoms

Budd Chiari illustration

The manifestations are thought to result from 3 interrelated, and variously associated, phenomena: (1) acute phlebitis producing pain and fever; (2) increased sinusoidal pressure upstream. Presentation varies from a fulminant picture to an asymptomatic condition recognized fortuitously. Several classifications into acute, subacute, and chronic presentation

Presentation appears to depend both on the extent and on the speed of the obstructive process. Thus, obstruction of only one major hepatic vein usually develops without symptoms; slow obstruction of 2 or 3 major veins produces a chronic presentation or, when accompanied with extensive collaterals, no symptoms at all; both rapid obstruction of at least 2 major veins, and a fresh thrombus superimposing on a long-standing but partial obstruction, give rise to an acute presentation.

Extrahepatic portal vein thrombosis is found in up to 20% of patients with BCS. Two factors may precipitate portal venous thromboses in BCS patients: the underlying thrombophilia and stagnant portal flow caused by outflow.  Large regenerative nodules have been the presenting manifestation in some BCS patients. Bleeding from, or malignant transformation of, these large regenerative nodules has not been reported.

Depending on whether only hepatic veins or both hepatic veins and portal veins are obstructed, bridging fibrosis can be found in a venovenous or in a portovenous or portoportal disposition, respectively.

A frequent, and odd, feature of long-standing BCS is the development of multiple large regenerative nodules, some of them resembling focal nodular hyperplasia. These nodules can be viewed as a response to a focal loss of portal perfusion and hyperarterialization in areas with preserved hepatic venous outflow.

Diagnosis of budd-chiari syndrome

Color-Doppler imaging combined with pulsed Doppler as first-line testing, and magnetic resonance as second-line testing, can be recommended.

In a minority of patients, mostly individuals with cirrhosis, in whom uncertainty persists, the third-line investigation can be liver biopsy as it is expected to provide information for important differential diagnoses: sinusoidal obstruction syndrome, cirrhosis of other origins, and diffuse spreading of malignant cells within the microcirculation. Direct venography is no longer considered necessary for establishing the diagnosis. In planning treatment, however, direct venography remains the gold standard, permitting a precise delineation of outflow obstruction, which is facilitated by prior noninvasive imaging.

Therefore, venography can be reserved for patients in whom interventional therapy is deemed necessary, while being ready perform a decompressive intervention in the same session.

In the absence of extrahepatic involvement or marked changes in peripheral blood counts, indications for the specific treatment of myeloproliferative disorders, paroxysmal nocturnal hemoglobinuria, or primary antiphospholipid syndrome are unclear. Moreover, the use of screening asymptomatic relatives for inherited disorders is controversial.

However, a consensus has emerged in which the well-established causes of BCS should be investigated at the time of initial diagnosis because awareness will permit better monitoring, earlier recognition of subsequent complications of the underlying disease, and counseling relatives with inherited thrombophilia.

Prognosis

budd chiari syndrome

Recently, multivariate analyses have permitted adjustment for treatment variables. in dependent younger age and a lower Child-Pugh score. The independent value of impaired renal function (including refractory ascites) and evidence for ischemic injury (i.e., increased aminotransferase levels) have been found to be less predictable. The significance of histopathologic findings has been debated. In recent large cohort studies, the extent of fibrosis, congestion, or necrosis had no independent value after adjustment for age and Child-Pugh score whereas they were associated significantly with a poor outcome at univariate analyse.

These lesions can be regarded as indices of severity, but age and Child-Pugh score perform better to predict outcome. Other features require additional assessment. Portal vein thrombosis is associated with a poor outcome. Moreover, intrahepatic or extrahepatic portal vein obstruction, are found consistently at the terminal stage. Therefore, be checked in multivariate analyses. Differentiating chronic outflow obstruction from that without acute presentation also may be of prognostic significance.

For portal hypertension and ascites in cirrhotic patients also apply to BCS patients, albeit with some caution. Anticoagulant therapy increases the risk for bleeding from paracentesis. During active bleeding from esophageal varices, the reduction in splanchnic blood flow induced by exogenous vasoconstrictors might precipitate portal venous thrombosis. Anticoagulation therapy may make endoscopic therapy for esophageal varices highly problematic.

Can Budd Chiari syndrome be cured?

Liver Decompression. Decompression aims at decreasing sinusoidal pressure by restoring the outflow of hepatic blood via recanalization of the obstructed venous outflow, or side-to-side portacaval shunting. Recanalization can be attempted using thrombolytic therapy for recent thrombosis, or percutaneous angioplasty, or surgery. thrombolysis combined with angioplasty for a pre-existing stenosis appears superior to thrombolysis delayed patchy enhancement and prolonged retention of in their collaterals, and in the intrahepatic or extrahepatic the contrast medium in the periphery of the liver. This portal venous system aims at preventing deterioration, perfision is compromised.

Liver Transplantation

The year survival rate is about is below 55% in patients with severe BCS in

recent studies. Further cohort studies including transplant and nontransplant patients are needed to evaluate the results according to baseline prognostic factors. The risk for recurrence is acceptably low when anticoagulant therapy is instituted early.

The apparently low risk for exacerbating malignant transformation of an underlying myeloproliferative disorder requires further assessment

First, treatment for the underlying condition and life-long anticoagulation therapy should be initiated without delay. Second, patients in a stable condition without symptoms should not have any interventional therapy proposed. Third, portosystemic shunting or transplantation should not be proposed to patients whose condition improves rapidly on medical therapy, as judged from recovery in liver function and easy control of ascites on low-salt diets and diuretics. In these patients, finding a short-length stenosis in the IVC or a large hepatic vein may prompt angioplasty with or without stenting.

Reasonably, in patients with an acute presentation and severe liver disease, the healing  should be a matter of days, whereas in patients with a chronic presentation it could be a matter of weeks. Fourth, patients that do not improve steadily, or whose symptoms recur on medical therapy, should be considered for decompression.

The order, or the combination, in which the various options for decompression should be proposed remains matter of debate. Still, there appears to be some further areas of consensus. First, the possible need for prompt transplantation should be kept in mind because deterioration in the patient’s condition can be rapid. Stent placement should be in a position that it will not hamper subsequent transplantation if needed. Preservation of good renal function deserves attention so that optimal immune suppression regimens can be used early post transplant.

Patients are better managed at, or in close connection with, centres where all possible treatment options, including transplant, are readily available.