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Radiology
Hepatic Vein Stenting in Budd Chiari Syndrome
Case 37: Contributed by Dr. Nikhil Karnik
A 25-year-old male presented with history of dull aching pain in the right hypochondrium and abdominal distension developing over a period of two months. There was no history of fever or jaundice. On examination, there was moderate ascites and hepatomegaly. Few distended veins were seen on the anterior abdominal wall. The routine blood parameters were within normal limits. A color Doppler examination of the abdomen revealed mild hepatomegaly with coarse liver parenchymal echotexture, moderate ascites, right pleural effusion, normal IVC, with no demonstrable flow in the middle and right hepatic veins and multiple veno-venous collaterals. Hence, a working diagnosis of Budd Chiari syndrome due to hepatic vein obstruction was made. An MRI scan confirmed the diagnosis and showed a dilated left hepatic vein which was not seen draining in to the IVC. (Fig 1, 2, 3)
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The patient was referred for an IVC gram and dynamic hepatic venogram. IVC gram showed the IVC to be normal. The right and the middle hepatic veins could not be cannulated. A dynamic hepatic venogram was not performed at this sitting as the patient had ascites.
With the diagnosis of Budd Chiari syndrome, endovascular management was planned for the patient. An Inferior vena cava gram was obtained via a 5 Fr right transfemoral venous access. Then via the 5 Fr right transjugular access, the dilated left hepatic vein was cannulated using a 0.035” Terumo guide wire. A 4 Fr Head Hunter catheter was advanced into the left hepatic vein over the wire. A hepatic venogram revealed a short segment high grade stenosis at the ostium of the left hepatic vein (fig. 4,5).
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Balloon angioplasty of this stenotic lesion was done using a 5 X 40 mm (CORDIS) balloon angioplasty catheter (fig. 6,7)
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following which a 8 X 39 mm sized CORDIS GENESIS balloon expandable nitinol stent was deployed across the lesion over a Amplatz Ultrastiff wire. (fig.8, 9, 10).
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Post stenting venogram revealed no significant residual stenosis, good stent position and good antegrade flow of contrast into the Inferior vena cava and right atrium (fig 11).
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Fig 11 |
Follow-up of the patient at one week revealed significant resolution of symptoms with weight loss of 9kg (76.5kg one week after the procedure compared to 87kg prior), significant increase in the 24hour urine output (2550 ml/24hrs one week after the procedure compared to 1500ml/24hrs prior), reduction in the abdominal girth from 102cm to 94cm and significant reduction in the ascites and right pleural effusion on follow up ultrasound.
DISCUSSION:
Budd Chiari syndrome is a manifestation of hepatic venous outflow obstruction. The obstruction involves the hepatic veins and/or IVC between the liver and the heart. Primary Budd Chiari syndrome occurs due to diseases or thrombosis of the veins themselves while secondary Budd Chiari syndrome occurs due to tumor invasion or extrinsic compression by tumors or other masses. Based on the location of the obstruction it has been classified as:
Type A : Inferior vena cava obstruction
Type B: Pure hepatic vein obstruction with short segment stenosis
Type C: Combined short segment hepatic vein and IVC occlusion
Type D: Extensive intrahepatic occlusion of hepatic veins with no identifiable main hepatic vein.
Whatever be the cause, obstruction of hepatic venous outflow in BCS leads to venous congestion and reduced portal inflow, with resultant ischemia and centrilobular necrosis. When the necrosis is extensive and develops rapidly, acute liver failure may ensue. In the milder and more chronic form, there is a sequence of liver damage, regeneration and fibrosis that progresses to cirrhosis at a later stage.
An acute syndrome is usually managed conservatively with anticoagulation whereas Chronic Budd Chiari syndrome which occurs due to fibrosis of the veins warrants endovascular/ surgical treatment. In all cases, investigations are first required to discover the underlying cause of the thrombophilia or hematological disorder.
Endovascular management has a significant role in the management of Chronic Budd Chiari syndrome and has shown very good results. Management should be aimed at restoring physiological blood flow to the liver wherever possible. If there is a possibility of restoring hepatic venous outflow in one of the major hepatic veins by balloon dilatation, recanalisation, or stent insertion, then that is the procedure of choice. In cases where the hepatic venous blood flow cannot be restored or in cases where the first method fails in producing relief of symptoms, a Transjugular Intrahepatic Portosystemic shunt ( with a patent right hepatic vein ) or a Direct Intrahepatic Portosystemic shunt is used as decompressive non-surgical shunt.
Short segment hepatic vein stenoses are best managed by angioplasty which may be supplemented with stenting. Restoration of good outflow in one of the three main hepatic veins is usually sufficient to relieve the symptoms. The pre procedure imaging usually allows the best possible hepatic vein to be selected for the recanalisation. Hepatic veins replaced by collateral ‘‘spider webs’’ or with obstruction inside the liver are usually unsuitable for dilatation. In a recent study Zhang et al reported on 115 patients with Budd Chiari syndrome of whom 30 patients were treated with hepatic vein stenting and showed good long term results (follow up for 45 months). They showed a success rate of 87% related to hepatic vein stenting. Failed stenting was seen due to long occlusions in hepatic veins >3cm. The long term results of stenting were better than angioplasty alone which showed high incidence of restenosis. Another study on Indian population on 49 patients with Budd Chiari syndrome with Hepatic vein stenosis as a predominant cause, endovascular management showed good long term results.
In conclusion Budd-Chiari syndrome should be diagnosed and treated aggressively, as it is one of the few potentially reversible chronic liver diseases. Diagnosis depends on clinical suspicion and imaging studies. Interventional radiological techniques are rapidly being considered as the treatment of choice in the management of acute and chronic cases refractory to medical management. Recanalisation of the hepatic vein and provides excellent long-term restoration of physiological circulation through the liver.