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    Prof. of Hepatology & Gastroenterology, Cairo University.

    Consultant of Hepatology,Gastroenterology and Endoscopy

    Management Positions: •

    Chief of Hepatology unit El Manial University Hospital (1994-1998).

    • Chief of Gastroentero ICU in Cairo university hospital (1997-2000)

    • President of the board of AlfaScope GI Specialized center (2004-2014).

    • Head of Endoscopy Unit in Cairo University Hospitals (2005-2010).       

     

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    استاذ الكبد و الجهاز الهضمى بكلية الطب جامعة القاهرة

    استشارى الكبد و الجهاز الهضمى و المناظير

    دكتوراه امراض الكبد و الجهاز الهضمى من كلية الطب جامعة القاهرة

    الرئيس السابق لقسم الامراض الباطنية بكلية الطب جامعة ٦ اكتوبر

    الرئيس السابق لوحدة مناظير الجهاز الهضمى و مركز الكبد و الرعاية المركزة بقصر العينى

     

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Tuesday, Aug 20th

Last update01:52:33 AM

        

Budd Chiari Syndrome

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Budd-Chiari syndrome

Definition:

Occlusion (Thrombosis) of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava.

Etiology:

An underlying disorder can be identified in over 80% of patients. Many of these disorders are characterized by a hypercoagulable state, which is important to consider when treating the patient.

1. Myeloproliferative disorders:

Up to 50% of cases of Budd-Chiari syndrome may be due to an underlying myeloproliferative disorder (as polycythemia vera, essential thrombocythemia & chronic idiopathic myelofibrosis) associated with a hypercoagulable state. Peripheral blood evidence for myeloproliferation may not be present if portal hypertension, and hypersplenism accompany Budd-Chiari syndrome (occult Myeloproliferation), but BM examination may diagnose these cases.

JAK2 mutations (present in patients with polycythemia vera,  essential thrombocythemia & chronic idiopathic myelofibrosis) have been described in more than 26% of patients with Budd-Chiari syndrome, many of whom had negative results from standard testing for myeloproliferative disorders.

2. Oral contraceptives & pregnancy:

Nearly 20% of cases of Budd-Chiari syndrome occur in women who have been on oral contraceptives, are pregnant, or have recently delivered. This is probably due to a hypercoagulable state.

3. Malignancy:

Malignancies account for nearly 10% of cases of Budd-Chiari syndrome. Compression, invasion or thrombosis, are responsible. HCC are found most often, followed by adrenal or renal tumors, and tumors of the lung, pancreas & stomach.

4. Infections & benign lesions of the liver:

Infections or benign space-occupying lesions of the liver can cause thrombosis or compression of blood vessels. They account for nearly 10% of cases of Budd-Chiari syndrome.

5. Hypercoagulable states:

  • Factor V (Leiden) mutation, was found in 25% of cases.
  • Antiphospholipid syndrome.
  • Antithrombin deficiency.
  • Protein C and Protein S deficiencies.

6. Membranous webs:

Membranous obstruction of the inferior vena cava (MOVC) and/or the hepatic veins is an unusual but potentially treatable cause of the Budd-Chiari syndrome.  

7. Other causes:

Behcet's disease, SLE, MCTD, Sjögren's syndrome, IBD and  hypereosinophilic syndrome. 

8. Idiopathic:

 Up to 20% of cases of Budd-Chiari syndrome are idiopathic.

A 2009 guideline from AASLD recommends the following approach

 for investigating causes of Budd-Chiari Syndrome:

  • Search for space occupying lesions or malignant tumors compressing or invading the hepatic venous outflow tract with sonography, CT scan, or MRI.
  • Seek evidence for UC, celiac disease, and systemic diseases.
  • Evaluate for multiple, concurrent risk factors for thrombosis.

 

Clinical picture:

It is more common in women, and usually presents in the third or fourth decade, although it may occur in children or the elderly. Presentation can be acute, subacute, or chronic.

Acute disease:

Patients usually present with severe right upper quadrant pain and hepatomegaly. Jaundice and ascites may not be apparent initially, but often develop rapidly. Variceal bleeding may occur.

Aminotransferases are elevated, reflecting liver congestion and resulting ischemic hepatocellular damage.

Serum bilirubin is usually less than 7 mg/dL at presentation, but may continue to rise.

Liver functions can deteriorate quickly, leading to hepatic encephalopathy and liver failure.

Subacute and chronic disease:

Patients with incomplete or partial occlusion of the hepatic veins, may remain asymptomatic or minimally symptomatic until the disease progresses. Patients may have vague discomfort related to the mid epigastrium or right upper quadrant.

Chronic occlusion of the hepatic veins may be associated with hypertrophy of the caudate lobe of the liver, which receives a separate blood supply from the rest of the liver. The hypertrophied caudate lobe can eventually cause compression of the intra-hepatic part of the inferior vena cava, leading to further outflow obstruction, accompanied by the development of ascites and lower limb edema. Cirrhosis may develop in the chronically congested liver, leading to portal hypertension and varices. Patients may then develop ascites, which may be massive. Hepatomegaly and abdominal pain are common. Hepato-pulmonary syndrome may occur in up to 28% of cases. Jaundice & encephalopathy are rare. Splenomegaly is uncommon, but may be found on physical examination in up to 30 percent of patients, and venous collaterals may be seen on the anterior abdominal wall. Edema of the lower extremities and the presence of venous collaterals on the back suggest occlusion of the inferior vena cava.

Investigations:

Liver functions:

Aminotransferases are normal or mildly elevated. Bilirubin, albumin and prothrombin are mildly to moderately affected.

Ascitic fluid:

 protein concentration may be greater than 3.0 g/L, with a serum-ascites albumin concentration gradient greater than 1.1 g/dL.

Doppler ultrasonography:

This is the most useful noninvasive test to screen for the presence of Budd-Chiari syndrome.

Nonspecific findings include hepatomegaly, splenomegaly, ascites, intra-abdominal collaterals, caudate lobe hypertrophy, atrophy of other hepatic lobes, and compression or narrowing of IVC.

More specific findings include:

  • Inability to visualize the junction of the major hepatic veins with the inferior vena cava
  • Thickening, irregularity, stenosis, or dilation of the walls of the hepatic veins
  • Abnormal flow in the major hepatic veins or IVC.

CT scan:

May reveal the same nonspecific abnormalities seen during ultrasonography. More specific findings on CT scan that suggest Budd-Chiari syndrome include:

  • Delayed or absent filling of the three major hepatic veins.
  • Patchy flea-bitten appearance of the liver due to increased central relative to peripheral contrast enhancement. This pattern reverses and produces inhomogeneous parenchymal opacification
  • Rapid clearance of dye from the caudate lobe
  • Narrowing or lack of opacification of IVC.

Magnetic resonance imaging:

MRI may be of great use in the diagnosis of Budd-Chiari syndrome. In addition to the nonspecific findings described above, the absence or reduction in caliber of hepatic veins and the typical distorted "comma-shaped" intrahepatic collaterals are easily demonstrable.

Three-dimensional contrast-enhanced MR venography:

Is proving to be helpful in diagnosis of Budd-Chiari syndrome. However, it is not yet widely available.

Sulfur-colloid scintigraphy:

Has been replaced by other imaging modalities.

Venography:

This is the gold standard for diagnosis. It is performed by accessing the hepatic venous circulation percutaneously, either via the internal jugular vein, cephalic vein, or femoral vein. Venography should be performed if noninvasive tests are negative but there is strong clinical suspicion for the disease.

Venography may show the "spider web" pattern characteristic of Budd-Chiari syndrome. This pattern occurs due to the formation of  venous collaterals to bypass the occluded hepatic veins.

Venography can be critical for deciding upon optimal therapy. As noninvasive studies may not accurately define the extent or characteristics of the hepatic venous flow.

Arteriography:

This is mandatory when planning a surgical approach to decompress the congested liver. The hepatic arteries are usually stretched, arched, and attenuated, which may possibly influence the surgical approach. In addition, the patency of the portal, splenic, and mesenteric veins can be determined and tumors involving the liver, inferior vena cava, or the hepatic and portal veins can be identified.

Liver biopsy:

A liver biopsy can be diagnostic of Budd-Chiari syndrome. Histologic features include centrizonal congestion, necrosis, and hemorrhage. Large regenerative nodules and obstructive portal venopathy may also be found. Cirrhosis may be present in the chronic form of the disease.

A 2009 guideline from AASLD recommends performing a liver biopsy only when an obstructed hepatic venous outflow tract has not been demonstrated with noninvasive imaging.

Treatment:

I. Medical treatment:

 1. Symptomatic treatment:

Diuretics and a low sodium diet.

Repeated large-volume paracenteses, for resistant cases.

Treatment of the underlying cause, if possible.

2. Anticoagulation:

Although anticoagulation is unlikely to lead to recanalization of occluded vessels, or development of adequate collateral circulation to avoid the progression of liver disease, there are reports of good long-term survival with only medical therapy.

The use of anticoagulation and diuretics alone as therapy for  Budd-Chiari syndrome should be reserved only for those who have chronic or subacute Budd-Chiari syndrome with well compensated liver disease at the time of presentation (however, additional measures to decompress the liver should be considered in such patients in an attempt to delay progression of the liver disease), and for those in whom other types of therapy are not feasible.

3. Thrombolytic therapy:

Thrombolytic treatment can be considered in patients with the acute or subacute form of Budd-Chiari syndrome, within 3-4 weeks of thrombosis. In most cases, treatment has limited efficacy, and exposes the patient to the risks of thrombolytic therapy.

Thrombolytic agents should not be used in the treatment of the chronic form of Budd-Chiari syndrome, as thrombi in such patients are unlikely to be recanalized by thrombolytic agents and because such patients may have developed portal hypertension, treatment can be associated with catastrophic bleeding complications.

II. Radiologic intervention:

These interventional procedures are assuming a major role in the management of patients with recent onset Budd-Chiari syndrome

1. Angioplasty:

The anatomy of the hepatic venous circulation in some patients with Budd-Chiari syndrome may reveal a focal abnormality that is amenable to balloon angioplasty.

2. Stenting:

Placement of an expandable metal stent in the occluded vessel following angioplasty may help to maintain patency. Experience with this technique is limited to small numbers of patients. Long-term survival with improvement in liver function and decrease of portal hypertension has been reported.

Placement of a stent, should be coordinated with a liver transplant team in patients who would otherwise be eligible for liver transplantation, as stent may complicate or prevent transplant.

3. TIPS:

TIPS is used to decompress congested segments in the liver by creation of an alternative venous outflow tract.

Several reports suggest that TIPS can be successfully performed in patients with Budd-Chiari syndrome. Five-year survival after TIPS (84%) was similar to five-year survival of liver transplantation for Budd-Chiari syndrome.

However, TIPS may not be technically feasible in many patients, may only drain a small portion of the liver, and is associated with a high rate of occlusion.

As a result of these considerations, TIPS is reserved for patients who do not respond to dilation of a hepatic venous outflow stricture or in whom a dilatable lesion cannot be found.

III. Surgical treatment:

1. Surgical shunts:

 These drain the portal or mesenteric venous system into IVC or another systemic vein. This allows blood entering the liver to drain out of the liver. Severe acute hepatocellular necrosis caused by pressure-induced atrophy of hepatocytes will often regress following the creation of decompressive shunts.

Surgical decompression is unlikely to be beneficial in patients who have cirrhosis or advanced liver dysfunction. Such patients are best managed with liver transplantation.

The decision to perform shunt surgery should be coordinated with a liver transplant team, since shunt surgery may not preclude the need for subsequent liver transplantation. The underlying cause of the thrombotic diathesis should be identified and treated prior to considering shunt surgery.

Five-year survival as high as 90% has been reported in patients who underwent shunt surgery prior to the development of cirrhosis, and whose shunts remained patent.

2. Liver transplantation:

This may be the only option for patients with Budd-Chiari syndrome who are not candidates for radiologic or surgical decompression or who have decompensated cirrhosis. Patients who developed the Budd-Chiari syndrome as a result of protein S, protein C, or antithrombin III deficiency may also be cured of their clotting tendency by liver transplantation, since the transplanted liver produces normal amounts of these enzymes.

Survival following OLT depends upon the underlying cause of the Budd-Chiari syndrome and the patient's condition at the time of the transplant. Large studies reported five-year survival to be 71-85%.

AASLD guideline for treatment (2009):

         Correct the underlying risk factor(s) for venous thrombosis.

  • Initiate anticoagulation therapy immediately. Use low molecular weight heparin, targeting anti-Xa activity to 0.5-0.8 IU/mL. Change to an oral anticoagulation agent when clinically appropriate, targeting the INR between 2 and 3.
  • Maintain permanent anticoagulation therapy unless a major contraindication is present or complication occurs.
  • Treat complications of portal hypertension as recommended for other types of liver disease.
  • Check for a venous obstruction amenable to percutaneous angioplasty/stenting in all symptomatic patients and treat accordingly.
  • In patients without ongoing improvement on anticoagulation therapy (with or without angioplasty), consider TIPS.
  • Consider liver transplantation if TIPS insertion fails or does not improve the patient's condition and in those with fulminant hepatic failure.
  • Coordinate care with a transplant center.
  • Monitor patients with longstanding, well controlled Budd-Chiari syndrome for the late development of hepatocellular cancer and transformation of underlying myeloproliferative disease.

Last Updated on Sunday, 18 November 2012 19:55

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  • عنوان: 98 شارع التحرير , ميدان الدقي, القاهرة , مصر
     
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