Portal vein was occluded with collaterals in hilum.
Renal veins were thrombosed.
Splenic vein was patent.
Activated protein C resistance: 221(120)
Activated protein C resistance: 221(120)
B2 micro globulin : 10 (0-3)
Anti cardiolipin Ab: normal
Anti phospholipids Ab: normal
Pr C: reduced
Pr S: reduced
Anti thrombin 3: normal
homocysteine: normal
Ham, sucrose test: normal
CD 55,59: normal
Fundal and esophageal varices were seen.
Fundal and esophageal varices were seen.
Snake skin appearance in fundus and body.
mottled appearance to the underperfused liver with collapsed portal veins,
ascites (small arrows)
extensive retroperitoneal varices (large arrow).
enlarged caudate lobe of the liver (large arrowhead)
the collapsed small IVC (small arrowhead).
The patient was treated with diuretic and concomitant albumin.
The patient was treated with diuretic and concomitant albumin.
Several abdominal paracentesis were performed.
Heparin started and switched to warfarin.
Proteinuria decreased during F/U.
Ascitis and edema is partially controlled with diuretic.
Hypercoagulability states were checked again which showed normal results.
more common in women
more common in women
third or fourth decade
most common symptoms is ascites (84%) and hepatomegaly (76%)
obstruction was in the hepatic veins (62%) inferior vena cava (7%)
portal vein thrombosis (14%)
myeloproliferative disorder was present in 23% (polycythemia vera).
Myeloproliferative diseases
Malignancy (Hepatocellular carcinoma)
Infections and benign lesions of the liver
Oral contraceptives
Pregnancy
Hypercoagulable
Behcet's disease
Membranous webs of IVC
Idiopathic
Acute (20%) :
Acute (20%) :
(2% with fulminant hepatic failure)
Subacute (40%):
(having signs or symptoms for < 6 months and no evidence of cirrhosis)
Chronic (40%):
(having signs or symptoms for > 6 monthswith evidence of cirrhosis)
most commonly in women (during pregnancy )
most commonly in women (during pregnancy )
pain and hepatomegaly
Jaundice and ascites develop rapidly
Liver function can deteriorate quickly, leading to hepatic encephalopathy
DDx: ischemic, viral, malignant/infiltrative, and toxic hepatitis
clinical manifestations depend upon the extent of occlusion, and the recruitment of collateral circulation.
clinical manifestations depend upon the extent of occlusion, and the recruitment of collateral circulation.
Chronic occlusion of the hepatic veins may be associated with hypertrophy of the caudate lobe.
This cause compression of the intrahepatic portion of the IVC, leading to lower extremity edema
cirrhosis may develop in the chronically congested liver, resulting in portal hypertension
cirrhosis may develop in the chronically congested liver, resulting in portal hypertension
encephalopathy is infrequent
Hepatopulmonary syndrome (28%)
liver biochemical tests are usually mildly abnormal
Chronic or subacute Budd-Chiari syndrome should be considered in unexplained liver dysfunction, particularly if ascites is a principal feature, or if risk factors for Budd-Chiari syndrome exist.
Chronic or subacute Budd-Chiari syndrome should be considered in unexplained liver dysfunction, particularly if ascites is a principal feature, or if risk factors for Budd-Chiari syndrome exist.
Clinical:
Splenomegaly, venous collaterals
Edema of the lower extremities suggests occlusion of the inferior vena cava
Signs of right-sided congestive heart failure (such as jugular venous distension)
Acute : hepatomegaly, RUQ pain, ascites
Acute : hepatomegaly, RUQ pain, ascites
Accuracy of noninvasive imaging modalities depends upon: duration of disease, location of the clot.
Portal vein thrombosis limits therapeutic options and has a poor prognosis
Screening test
Screening test
hepatomegaly,
splenomegaly,
ascites,
intraabdominal collaterals,
caudate lobe hypertrophy,
atrophy of other hepatic lobes,
compression of IVC
Thickening, irregularity, stenosis, or dilation of the walls of the hepatic veins
Abnormal flow in the major hepatic veins or IVC
Delayed or absent filling of the three major hepatic veins
Delayed or absent filling of the three major hepatic veins
Patchy flea-bitten appearance of the liver
Rapid clearance of dye from the caudate lobe
Narrowing and/or lack of opacification of the inferior vena cava
unremarkable ultrasound examination but in whom the suspicion is high
Venography
Gold standard for diagnosis
plan therapeutic interventions .
Determine pressure gradient above and below the entrance of the hepatic veins into the inferior vena cava
Accurately define the extent or characteristics of the hepatic venous flow
Compression of the intrahepatic IVC, leads to sluggish flow in hepatic veins. As a result, the hepatic veins can be undetectable during ultrasound Doppler studies, although they may be patent and amenable to therapy
Can be diagnostic in the acute or subacute form
Can be diagnostic in the acute or subacute form
Features include centrizonal congestion, necrosis, and hemorrhage
Cirrhosis may be present in the chronic form
Determine prognosis and guide therapy
Cirrhotics are less likely to benefit from revascularization procedures
thrombotic process in Budd-Chiari syndrome may not involve all the hepatic veins.
thrombotic process in Budd-Chiari syndrome may not involve all the hepatic veins.
Thus, the distribution of the typical pathologic findings may be focal or patchy. As a result, some patients require biopsy of both the right and the left lobes of the liver.
laparoscopic approach may be better suited
Perfom Bx when there is confusion regarding the diagnosis and plan treatment accordingly
Myeloproliferative disorder may benefit from treatment with aspirin and hydroxyurea
Anticoagulation alone is unlikely to lead to sufficient recanalization of occluded vessels to avoid the progression of liver disease.
Anticoagulation alone is unlikely to lead to sufficient recanalization of occluded vessels to avoid the progression of liver disease.
A trend for a benefit of anticoagulation on survival in less severe disease.
Medical therapy :
1) Chronic or subacute Budd-Chiari syndrome with well compensated liver disease at the time of presentation.
2) When other types of therapy are not feasible
Risk of anticoagulation should also be considered, especially in patients who present with bleeding complications
Risk of anticoagulation should also be considered, especially in patients who present with bleeding complications
Patients receiving only medical therapy should be monitored closely for disease progression (liver biopsies annually )and portal hypertension complications (looking for varices)
In acute form which blood clots are younger than three to four weeks
In acute form which blood clots are younger than three to four weeks
Do not use thrombolytic agents in:
patients who have extensive clot involving the IVC
or a clot of unknown age.
Angioplasty
Angioplasty
Stenting
Transjugular intrahepatic portosystemic shunt
Restore hepatic venous drainage using shunt surgery
Restore hepatic venous drainage using shunt surgery
Because of the availability of TIPS, few vascular surgeons routinely perform shunt surgery.
Underlying cause of the thrombotic diathesis should be identified and treated prior to considering shunt surgery.
Unlikely to be beneficial in patients who have cirrhosis, Such patients are best managed with liver transplantation.
survival following shunt surgery depends upon the extent of liver damage prior to surgery, and the continued patency of the shunt
Maintenance of shunt patency often requires anticoagulation
deterioration in patients following shunt surgery should be investigated by angiography to determine whether the shunt has thrombosed, which may be corrected by angioplasty.
who are not candidates for radiologic or surgical decompression
who are not candidates for radiologic or surgical decompression
or who have decompensated cirrhosis
protein S, protein C, or antithrombin III deficiency may also be cured of their clotting tendency by liver transplantation,
Survival following OLT depends upon the underlying cause of the Budd-Chiari syndrome and the patients condition at the time of the transplant
Am J Kidney Dis.
Am J Kidney Dis.
It has long been known that patients with nephrotic syndrome have a hypercoagulable state, which explains the association between nephrotic syndrome, renal vein thrombosis, and thromboembolism.
It has long been known that patients with nephrotic syndrome have a hypercoagulable state, which explains the association between nephrotic syndrome, renal vein thrombosis, and thromboembolism.
However, the Budd-Chiari syndrome has never been reported in nephrotic patients.
This is the first report of such an association that, most likely, depended on a primary resistance to activated protein C
Pediatr Nephrol.
Pediatr Nephrol.
We observed Budd-Chiari syndrome in a boy aged 2 years 6 months with nephrotic syndrome due to hepatic vein and inferior vena cava thrombosis, confirmed by Doppler imaging.
We observed Budd-Chiari syndrome in a boy aged 2 years 6 months with nephrotic syndrome due to hepatic vein and inferior vena cava thrombosis, confirmed by Doppler imaging.
Normal values of the routine hemostatic parameters proved that they are of little predictive value for the thrombotic state.
Immediate heparin infusion was initiated. High doses of heparin up to 59 IU/kg per hour were required for efficient anticoagulation.
Immediate heparin infusion was initiated. High doses of heparin up to 59 IU/kg per hour were required for efficient anticoagulation.
A remission of the nephrotic syndrome was achieved with vincristine.
Oral anticoagulation with a vitamin K antagonist was continued for 6 months.
Doppler imaging then indicated full re-establishment of the blood flow through the affected vessels.
The favorable outcome was due to the immediate heparin infusion and prompt remission of the nephrotic syndrome.
Doppler imaging was an important tool for non-invasive diagnosis and follow-up.
Pediatr Nephrol.
Pediatr Nephrol.
Over a period of 22 years, 447 children with nephrotic syndrome (NS) have been retrospectively studied for clinically apparent thromboembolic complications (TEC).
Over a period of 22 years, 447 children with nephrotic syndrome (NS) have been retrospectively studied for clinically apparent thromboembolic complications (TEC).
The incidence of TEC is 2% (9/447).
TEC were predominantly venous (81% venous vs. 19% arterial).
The most commonly affected vessels were deep leg veins, IVC, SVC, mesenteric artery, and hepatic veins (Budd-Chiari syndrome).
J Assoc Physicians India.
J Assoc Physicians India.
Idiopathic membranous obstruction and stricture of IVC are the commonest cause of BCS in the eastern part of India.
Idiopathic membranous obstruction and stricture of IVC are the commonest cause of BCS in the eastern part of India.
Hepatocellular carcinoma is also a common cause, presenting in the fulminant form.
Ultrasonography may be a helpful screening test for BCS,
IVC and hepatic vein catheterisation is essential for a complete work up of these patients.
Nepal Med Coll J.
Nepal Med Coll J.
A 21year old male presented with abdominal pain for 2 months and abdominal distension and swelling of lower limbs for 1 month.
A 21year old male presented with abdominal pain for 2 months and abdominal distension and swelling of lower limbs for 1 month.
US showed coarse echotexture of liver and intraluminal filling defect of IVC
Confirmation of diagnosis was done by inferior venacavography.
The patient had nephrotic syndrome as the risk factor for thrombosis.
The patient underwent portocaval shunt with significant symptomatic relief.
