Budd-Chiari syndrome

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Management conference
Middle age man with
nephrotic syndrome,
ascitis and edema
Raika Jamali MD
Digestive Disease Research Center
Tehran University of Medical Sciences
• A 49 years old man with progressive
bilateral pedal edema and ascitis from 1
month ago.
• History of DM for 4 years.
• Three months ago during the evaluation
for excessive proteinuria inappropriate for
diabetic nephropathy ,prolongation of PT
was detected before kidney biopsy.
• Viral markers requested and was referred
for liver function evaluation.
EXAM
• Vital signs were stable. No fever.
• Mild anemia. Ichterus in sclera.
• Parallel collaterals in chest and upper
abdomen which filled upward.
• Tense ascitis. liver span 14 cm.
• Moderate splenomegaly .
• No signs of chronic liver disease.
• Bilateral pedal edema.
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WBC=6500
HB=10
PLT=245000
MCV=85
• FBS=180
• TG=200
• AFP =60,92
AST=52
ALT=43
Bili T=5
Bili D=1.3
ALP=508
PT(INR)=2.6
PTT=38
Albumin=2.2
Protein=5.2
• BUN=15
• Cr=0.9
• Uric Acid=4
• U/A: 3+ protein
• 24 h urine protein: 7 gr /day
• HCV Ab=suspicious
• HBs Ag=Neg
• HBs Ab=positive
• HBc Ab=positive
• HBV DNA and HCV RNA Titer :
undetectable
Ascitic fluid
• RBC=20
• WBC=70
• Albumin=0.5
• Cytology=negative for malignancy
Sonography
• Liver was enlarged with hetrogenous echo
pattern.
• PV diameter 10 mm.
• Severe ascitis.
• Moderate splenomrgaly.
Color Doppler sonography
• IVC and suprahepatic veins were
occluded.
• Portal vein was occluded with collaterals in
hilum.
• Renal veins were thrombosed.
• Splenic vein was patent.
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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
Endoscopy
• 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).
Follow Up
• 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.
Budd-Chiari syndrome
• 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).
Major causes of the Budd-Chiari
syndrome
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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%) :
(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 months
with evidence of cirrhosis)
Acute
• 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
Subacute and chronic disease
• 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
• encephalopathy is infrequent
• Hepatopulmonary syndrome (28%)
• liver biochemical tests are usually mildly
abnormal
DIAGNOSIS
• 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 BuddChiari 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
• 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
Doppler ultrasonography
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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
CT scan
• 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
Magnetic resonance imaging
• typical distorted "comma-shaped" intrahepatic
collaterals
• 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
Liver biopsy
• 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.
• 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
TREATMENT
• Prevent the propagation of the clot
• Decompress the congested liver
• Prevent complications (malnutrition, portal
hypertension)
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• Medical treatment (supportive care,
anticoagulation, thrombolysis),
• Radiologic procedures (angioplasty,
TIPS,)
• Surgical intervention (shunting procedures
, transplantation).
Medical therapy
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Diuretics and a low sodium diet
large-volume paracenteses
Improve nutritional status
Underlying cause should be investigated
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.
• 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
• Patients receiving only medical therapy
should be monitored closely for disease
progression (liver biopsies annually )and
portal hypertension complications (looking
for varices)
Thrombolytic therapy
• 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.
Radiologic treatment
• Angioplasty
• Stenting
• Transjugular intrahepatic portosystemic
shunt
Surgical therapy
• 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.
Liver transplantation
• 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
Budd-Chiari syndrome during
nephrotic relapse in a patient
with resistance to activated
protein C clotting inhibitor
• 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.
• 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
Budd-Chiari syndrome and
inferior vena cava thrombosis
in a nephrotic child.
• 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.
• 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.
• 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 reestablishment 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.
Thromboembolic
complications in children with
nephrotic syndrome in
Bulgaria (1974-1996).
• Pediatr Nephrol.
• 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).
Etiology based prevalence of
Budd-Chiari syndrome in
eastern India
• J Assoc Physicians 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.
Budd-Chiari syndrome--a
case report
• 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.
• 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.
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