Radiology

Other Cases

Case Report :

A 39-year-man presented with history of recurrent hemetemesis (4-5 episodes) of three months’ duration for which he had required repeated blood transfusions.

Ultrasound & colour Doppler examinations revealed hepatosplenomegaly with liver parenchymal disease & chronic thrombosis of all three hepatic veins with veno-venous collaterals (Fig 1). CECT confirmed the findings with typical enlargement of the caudate lobe - consistent with Budd-Chiari syndrome (BCS) (Figs 2, 3, 4).

Fig 1	Fig 2
Fig 3	Fig 4

The patient’s Child-Pugh score was 9 (Child B group) and MELD score was 10. Hematological investigations revealed deficiencies of Prot C - 64.6 ( N=70-140)& Prot S - 50 (N=60-130). Antithrombin3, lupin anticoagulant, homocysteine, ACLA IgG, ACLA IgM were unremarkable.

A direct intrahepatic porto-systemic shunt was planned as the hemetemesis did not respond to conservative and endoscopic management. None of hepatic veins could be cannulated through the right transjugular route (Fig 5). The liver was punctured through the intra-hepatic segment of the IVC under fluoroscopic guidance using a RTPS needle (Cook Medical) and the needle was further advanced into the portal vein (Fig 6 ).The pressure in the main portal vein was 35 mm of Hg. The length of the intraheaptic tract to be covered with the stent-graft was measured at 12cms using a marker catheter (Cook Medical) .The intra-hepatic tract was then dilated using 5 mm x 40 mm and 8 mm x 40mm balloon angioplasty catheters (Opta Pro, Cordis) (Fig 7).The tract was covered using a Viatorr device 10 mm x 80mm (W L Gore Technologies) (Fig 8 ) and a 10mm x 60mm stentgraft (Fluency, Bard) (Fig.9 ) with a 2 cm overlap. This was followed by stentgraft plasty using a 10mm x 40 mm balloon angioplasty catheter (Opta Pro,Cordis)(Fig 10).Post stent-graft plasty shuntogram revealed a widely patent DIPSS shunt with good antegrade flow into the right atrium (Fig 11). The post DIPSS the pressure in the main portal vein was 9 mm of Hg. The patient was kept on anticoagulation in the form of heparin for next 36 hours and warfarin (life-long) with a maintenance of INR between 2-3. A colour Doppler examination the showed shunt to be patent at 1 week and patient was symptom free at one month follow up.

Fig 5	Fig 6

 

Fig 7	Fig 8

 

Fig 9	Fig 10
Fig 11

DISCUSSION:

BCS may be present with a wide range of clinical manifestations from asymptomatic to fulminant liver failure. In patients with Budd-Chiari syndrome, abdominal pain, hepatomegaly, and ascites are usually present. Esophageal varices and splenomegaly are observed in the chronic form, and jaundice is usually present in the fulminant and acute forms. Treatment of this heterogeneous disease may require a multidisciplinary approach including gastroenterologists & hepatologists, hematologists, interventional radiologists and surgeons.

The medical treatment of Budd-Chiari syndrome includes anticoagulation therapy, maintenance of the international normalized ratio between 2.0 and 2.5, and treatment of ascites. Thrombolysis with urokinase or tissue plasminogen activator is used in patients who have the fulminant form. This approach should be attempted in early diagnosed cases, especially in those detected in the initial 24 to 72 hours, although it is worth trying in patients with hepatic vein thrombosis less than 2 weeks old.. Medical treatment, including therapy of the frequent underlying hematologic disease, anticoagulation, and diuretics have for a long time been considered completely ineffective and associated with very poor long-term results. Indeed, a 2-year survival lower than 10% was reported in medically treated patients. Recognition of these facts and endovascular stenting of a clinically significant short-length hepatic vein stenosis allows the decompression of the congested liver, which preserves liver perfusion and prevents the need to perform a procedure that would deprive the liver from portal blood flow. Surgical options are portal decompressive therapy in the form of mesocaval or mesoatrial shunts as the only possible approach except from liver transplantation. Surgical portosystemic shunt has several disadvantages such as a high rate of thrombosis and a high operative morbidity-mortality which may overcome the benefit of surgical shunts.

In patients in whom long-segment h epatic vein obstruction (> 3 cm) involving all three Hepatic veins is present & in whom hepatic vein revascularization is not feasible, the DIPSS procedure creates a portacaval shunt through the liver parenchyma. It does not require laparotomy, is less invasive, and subsequently has a lower morbidity-mortality than surgical treatments, even in critically ill patients. In addition, DIPSS will drain the portal venous system to the suprahepatic part of the IVC avoiding any deleterious effects of vena cava obstruction or compression of the IVC by the enlarged, congested liver. Both of these factors suggest a potential advantage of DIPSS over surgery in the treatment of BCS patients uncontrolled by medical therapy. DIPSS provided a survival rate of 85% at four years in one series and 74% at five years in another series.Covered DIPSS created by using expanded PTFE covered stents has advantage of over bare stents - of the higher patency rates (85.7% vs 67% at 1 year) and the lower number of revisions in one recent series . The problem of TIPS dysfunction necessitates reintervention .Covered DIPSS in BCS has shown lower TIPS dysfunction rate (33% vs 87% in uncovered group) at 1-year in another recent series . However, liver decompression by TIPS allowed the patient to gain time for the development of collaterals that, later on, will suffice to maintain the liver decompression despite DIPSS stenosis or occlusion. Supporting this concept, it has been shown that patients with asymptomatic forms of BCS are more likely to have larger intraheptic or extrahepatic collaterals than those with symptomatic forms.

There are no randomized controlled clinical trials comparing DIPSS against surgery, so it is not possible to make a definitive statement about the best approach in BCS patients. However, in experienced hands, due to the lower procedure related morbidity-mortality rates, its efficiency even in the presence of IVC obstruction and fewer disturbances of the portal anatomy or hepatic hilum, DIPSS should be the initial approach in patients needing definitive therapy. In cases of DIPSS failure, surgical shunt therapy is still available as a rescue treatment. In cases of fulminant BCS, liver transplantation is considered to be the therapeutic option of choice. In these cases, TIPS may be a bridge to liver transplantation and in some cases may help to avoid it.

In conclusion, BCS patients should be managed by a multidisciplinary team. If technically feasible, patients should first be treated by angioplasty or stenting of a clinically significant short-length hepatic vein stenosis .In cases with no hepatic vein revascularization possible, DIPSS is a safe and highly efficacious endovascular treatment in most patients with BCS uncontrolled by medical therapy.