Teaching Case

A 20-year-old female patient, case of Budd-Chiari syndrome with thrombosis of portal, hepatic, inferior mesenteric and splenic veins, status post TIPS and porto-mesenteric stenting done in our hospital. She came back with abdominal pain.

Doppler study revealed absence of flow in TIPS and porto-mesenteric stents suggesting acute thrombotic occlusion (Fig. 1, 2). Patient was urgently shifted to operation theater equipped with digital subtraction angiography, where TIPS venogram revealed complete occlusion of the stent extending from the IVC to SMV (Fig. 3). Using catheter guide wire combination, TIPS stent was negotiated and balloon angioplasty and chemical thrombolysis done (Fig. 4, 5). Repeat Doppler revealed good flow across both the stents (Fig. 6, 7, 8). Patient was kept on alteplase infusion. Next day she had severe abdominal pain with a significant drop of haemoglobin. USG revealed haemoperitoneum. Her B.P decreased significantly requiring multiple transfusions and she had to be ventilated for a day. Repeat Doppler showed patent stent with good flows. She was later discharged on warfarin with advice to follow up regularly with Doppler. Subsequent Doppler showed patent stents and patient has been doing well clinically.

Budd-Chiari syndrome is characterised by hepatic venous outflow obstruction at the level of hepatic veins, IVC or right atrium. Manifestations range from mild symptoms to fulminant acute liver failure to chronic liver disease. Since manifestations are non-specific, imaging is necessary for diagnosis and assessment of extent of disease. Duplex ultrasonography is a useful tool as it allows easy assessment of hepatic venous flow and detection of hepatic parenchymal heterogeneity. If not treated promptly, the outcome is dismal. Goal of treatment is to relieve hepatic congestion, thereby improving hepatocyte function and allowing resolution of portal hypertension. Various medical, endovascular, and surgical treatment options are available. Transjugular intrahepatic portosystemic shunt (TIPS) is an effective means of decompressing the portal system in patients unresponsive to traditional medical therapy [1]. TIPS can become dysfunctional if stenosis/thrombosis develops in the stent. Trans-shunt venography is the gold standard technique in TIPS evaluation, but it is expensive and invasive. Doppler ultrasound is most commonly used non-invasive tool in checking TIPS patency. Ultrasound screening allows detection of stent occlusion before complications occur and warrants its timely management. First examination should be performed within 1 week of placement for Wallstents and 1 month after placement for covered stents. Surveillance schedule consists of an examination 3 months after the first examination, with a follow-up every 6 months thereafter [2]. Each examination consists of looking for shunt patency and flow characteristics. Each stent is assessed at hepatic vein end, mid portion and portal vein end. Optimally functioning stent shows pulsatile turbulent flow and PSV of at least 50–60 cm/s (range 90–120 cm/s); portal vein velocity at least 30 cm/s (range 20–50 cm/s) [3]. Increased echogenicity within the stent lumen and absence of colour flow is seen in occluded stents. Abnormal indices are localised high velocity (>220 cm/s) with post-stenotic turbulent flow, change in velocity of >100 cm/s across the stent, diffuse low velocity throughout the stent (<50 cm/s) or velocity <30 cm/sec in the main portal vein. Development of hepatofugal main portal or splenic vein flow or hepatopetal flow in the intrahepatic portal vein branches [3], development or recurrence of collaterals, new, recurrent, or worsening ascites are signs of failure of shunt [2]. Timing of dysfunction of TIPS cannot be predicted; hence routine surveillance is required to ensure its patency. Timely anticoagulant therapy or shunt revision can significantly improve the outcome.
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