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Radiology

Case of the Month


Case No. : 58
Month : October
Year : 2003
Contributor : Dr. Yogini Panchal

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Discussion


CLINICAL PROFILE :

A 58-year-old male with history of mild to moderate fever since five months, generalised distension of abdomen since four months and edema of feet since two months presented with upper GI bleed.

Clinical examination showed distension of abdomen with dilated veins over the abdominal wall. There was moderate edema of feet.


IMAGING FINDINGS:

On abdominial ultrasonography ( Figs.1-7) , there was an echogenic linear lesion in the intrahepatic-subdiaphragmatic IVC with high velocity flow in this region suggestive of a membranous obstruction. There was a hypoechoic lesion in the intrahepatic IVC distal to the membrane suggestive of a thrombus. The flow was along the periphery of the IVC. There were retroperitoneal collaterals. Splenomegaly with ascites was noted.

Fig.1
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Fig.1
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Fig.7


A contrast enhanced CT scan (Fig. 8 ) of the abdomen showed the thrombus as a hypodense lesion with flow at the periphery. There was caudate lobe hypertrophy with the surface of the liver beinh nodular. There were lumbar collaterals. There was ascites with splenomegaly.

Fig.8
Fig.8

Gradient echo coronal reconstruction of MRI (Fig. 9) showed the hyperintense free flowing blood at the periphery of the IVC with the hypointense thrombus.

Fig.9
Fig.9

An IVCgram (Figs. 10,11) showed flow only at the periphery of the IVC with a short segment, high grade narrowing of the subdiaphragmatic-intrahepatic IVC due to partial a membrane. The common iliac veins were dilated.

Fig.10
Fig.11
Fig.10
Fig.11

This narrowing was negotiated with a glide wire and then dilated with a four cm long 16mm balloon catheter. This procedure dramatically relieved the patients symptoms.


DISCUSSION:

Three types of membranous obstruction have been described:

In Type 1, the IVC is obstructed by a thin membrane at the level of the entrance to the right atrium. This membrane may be complete or partial with a central hole.

In Type 2, a segment of the IVC (of varying lengths) is absent. In these cases, the IVCgram shows a characteristic conical narrowing at the level of obstruction.

In Type 3, there is complete obstruction of the IVC secondary to thrombosis.

Budd Chiari syndrome is a relatively rare disorder characterised by occlusion of the lumina of the hepatic veins with or without occlusion of the lumen of the IVC.

The causes of the Budd Chiari Syndrome are coagulation abnormalities such as polycythemia rubra vera, chronic leukemia and paroxysmal nocturnal hemoglobinuria, trauma, tumour extension from primary hepatocellular carcinoma, renal carcinoma and adrenal cortical carcinoma. Pregnancy, congenital abnormalities and obstructing membranes are also other causes.

The caudate lobe is often spared in Budd Chiari syndrome because the emissary veins drain directly in the IVC at a lower level than the involved the main hepatic veins. Increased blood flow through caudate lobe leads to relative caudate lobe enlargement.

Ultrasonographic findings of Budd Chiari syndrome are:
Partial or complete inability to see the hepatic veins, stenosis with proximal dilatation, intraluminal echogenicity, thickened walls, thrombosis and intrahepatic collaterals.

CT findings of Budd Chiari syndrome are :
Patchy hepatic parenchymal enhancement with poor visualisation of the hepatic veins classically described as nutmeg liver. Intravascular thrombi are identified as hypoattenuating masses within the hepatic veins or the IVC. Ascites and hepatic infarcts are also seen.

MRI findings of Budd Chiari syndrome are:
Hepatomagaly, reduction in caliber or lack of visualisation of the hepatic veins and ascites.




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