CASE OF THE MONTH ( JULY 2007)
A 18 year old female presented with pain in abdomen in left hypochondriac
and epigastric region, jaundice, abdominal distension, oliguria and bilateral pedal oedema
since one week.
Hematological investigations revealed raised bilirubin (total 2.16 mg% conjugated 1.19 mg%)
and anemia (HB 8.8 gm%). HBsAg , HCV, HIV were negative.
Ultrasound abdomen showed hepatosplenomegaly, ascites and minimal right pleural effusion.
Patient was advised CT scan of the abdomen to rule out hepatic venous outflow tract obstruction.
CT scan of the abdomen (plain and post contrast triphasic scanning) was performed using
multidetector ultrafast 64 slice CT scanner. Reconstructions were performed in sagittal and
coronal planes.
There was poor visualization of the hepatic veins which showed hypodensity within them
even on delayed scans suggestive of thrombus within them. There was significant luminal
narrowing of 60-70% of the retrohepatic IVC without any thrombus within it. The liver
showed heterogenous enhancement with only the caudate lobe showing normal
size and enhancement. Few relatively poorly enhancing foci were noted especially in the
posterior subcapsular region of segment II and III; possibly hepatic infarcts. There was
gross ascites with bilateral moderate pleural effusion.
These imaging findings were suggestive of Hepatic veno-occlusive disease (acute
Budd Chiari syndrome)

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Fig 1 Thrombosed hepatic veins with
sparing of the caudate lobe |
Fig 2 Enlarged heterogenously enhancing liver with compressed IVC
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| Fig 3 Sagittal reconstruction showinghetrogenously enhancing liver and compressed retrohepatic IVC |
Fig 4 Coronal reconstruction clearly showing thrombosed hepatic veins in the left lobe
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Patient was shifted to higher institution for further management. Subsequently patient
developed portal vein thrombosis and surgery was deferred. At present patient is on
anticoagulants and requires therapeutic ascitic tap once a week.
DISCUSSION
The Budd-Chiari syndrome is a rare disorder of hepatic-vein or inferior-venacaval
occlusion. In some instances the obstruction occurs at the venule level.
In most instances obstruction to the hepatic veins and/or cava is partial. Because
of this hepatic venous outflow obstruction, patients with this syndrome may present with
variable degrees of hepatic enlargement, pain, tenderness, and ascites.
Jaundice, portal hypertension, and variceal bleeding may also be present. Much
less common is the acute fulminant presentation with massive liver failure and
shock. In comparison with the frequency in the United States, the frequency of
this syndrome is higher in northern India, South Africa, and the Orient. Budd-
Chiari may be associated with polycythemia rubra vera, chronic leukemia, oral
contraceptives, neoplasms, pregnancy, trauma, and congenital abnormalities. However, the
exact etiology cannot be determined in almost two-thirds of cases.
The Budd-Chiari syndrome may be classified as primary or secondary depending
on its pathophysiology. The primary type refers to congenital obstruction of the
hepatic veins or the hepatic portion of the inferior vena cava by webs or diaphragms.
The secondary type refers to hepatic-vein or inferior-vena-cava obstruction caused by
tumor, thrombosis, or trauma. Thus, the radiographic manifestations of the syndrome may
differ significantly.
DIAGNOSTIC PROCEDURES
1} ULTRASONOGRAPHY WITH DOPPLER — For screening patients with suspected
Budd Chiari syndrome.
2} CT SCAN – PLAIN AND CONTRAST ENHANCED- for confirming the diagnosis of
hepatic veno-occlusive disease and exact delineation of the extent and level of hepatic
veins and IVC obstruction.
3} ANGIOGRAPHY –1) Inferior vena cavography for confirmation of any thrombus
within it and severity of compression with pressure gradient measurements across the
stenotic segment. 2) Hepatic wedge cavography which typically show the ‘spider web’
pattern of collateral intrahepatic veins, 3) celiac / SMA arteriography shows diffuse narrowing ,
pruning and stretching of the hepatic arteries.
6} PERCUTANEOUS BIOPSY – using large bore 14 – 16 gauge trucut needles.
Demonstration of organic thrombi within the main intrahepatic veins is diagnostic.
Cirrhosis may be seen in chronic cases.
TREATMENT
1} MEDICAL Rx – Diuretics and Anticoagulants
2} SURGICAL – 1) Percutaneous transluminal angioplasty
2) Portocaval or mesocaval shunts
3) Ballooon angioplasty
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