Dr. Pradeepta Kumar Sethy (Sr. Consultant Gastroenterology).
Hepatic venous outflow obstruction in Budd Chiary syndrome can be at various level causing symptoms due to hepatic congestion. Effective treatment strategy to relieve the obstruction by combination of medical therapy, transcatheter angioplasty and stent placement has good clinical outcome.
Keywords: Inferior vena cava stenting, Budd-Chiari syndrome
Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction at any level from small hepatic veins to junction of inferior vena cava (IVC) with right atrium. It causes increased hepatic sinusoidal pressure resulting in portal hypertension, liver congestion, and decreased liver perfusion. Relief of the obstruction is helpful in relieving symptoms. We report a case of IVC rehabilitation in a young lady with Budd Chiari Syndrome.
A thirty year old lady presented with complaints of pain abdomen and anorexia.
She had pallor, no jaundice, mild ascites, hepatomegaly andsplenomegaly.
Hemogram showed hypochromic microcytic anemia (Hemoglobin 7 gm%). She had mildly elevated total bilirubin (1.4 mg%), direct bilirubin 0.5 mg%, normal hepatic enzyme except mildly elevated alkaline phosphatase. Ultrasonography (USG) of abdomen showed enlarged liver and spleen along with hepatopetal flow in portal vein; normal hepatofugal flow of hepatic vein shows; interrupted flow in inferior Vena cava (IVC). Contrast enhanced computed tomography (CECT) of the abdomen revealed enlarged liver with multiple nodules, features of congestive liver disease as well as non-enhancement of hepatic veins; thinned out portal vein with multiple collaterals; significant splenomegaly (Fig. 1).
The CECT scan features were suggestive of Budd Chiarisyndrome with a very tight stenosis in the supra-hepatic IVC. She was taken for catheterization and venoplasty. The basal IVC angiogram in antero-posterior view showed IVC interruption in the supra-hepatic region, approximately one cm discontinuation. There was large filling defect in IVC suggestive of thrombus. (Fig. 2)
In view of thrombus presence in the IVC the plan was to make a small opening in the obstructed IVC channel (not opening fully for risk of pulmonary thromboembolism) and keep the patient on anti-coagulation. Small thrombus found in the suction. (Fig. 3)
The interrupted segment of IVC was perforated with help of 5Fmultipurpose angiographic catheter (MPA) and hard end of 0.035 Terumo wire followed by conquest pro wire (Abott Vascular,Northern California). Once wire entered in the RA, the pathway was sequentially dilated with 2.5, 4.5 and 6 mm balloon. (Fig. 4)
There was a good communication between IVC- right atrium (RA) measuring approximately 5 mm. The pulmonary artery (PA) pressure was 23/10 (mean 16 mm hg) and RA mean pressure was5 mmhg. The mean IVC pressure was 18 mm hg (pre and post procedure). She was given anti coagulation heparin with warfarin with target INR of 2. Her symptoms improved in next few days and splenic size reduced. She was discharged on Aspirin, warfarin and was suggested re-intervention after 3 months. Re-evaluation after three months had not showed any IVC flow in the CT scan.
IVC angiogram showed IVC interruption in the hepatic region ofIVC with multiple small collateral flow to right atrium.
The interrupted segment of IVC was entered with help of 5F MPAand Conquest pro wire followed by dilatation of the obstructed segment with 2.5 × 10 mm balloon. The MPA catheter was negotiated to superior vena cava. A 0.035 mm Amplatz stiff wire (St Jude Medical, Minnesota) was parked in innominate vein. The IVC was dilated with 10 × 40 mm Mustang balloon (Boston Scientific Marlborough, Massachusetts). Repeat check angiogram showed good path way from IVC to RA. A self-expanding 18 × 60 mm Wall stent (Boston Scientific Marlborough, Massachusetts) was deployed across the narrow segment while keeping upper end in IVC RA junction. The stent attained stable position with good flow across the stent.(Fig. 5)
The patient was discharged anti-coagulation and doing well in interim follow up.
Budd Chiari Syndrome is a rare clinical syndrome characterized by occlusion of the hepatic venous outflow from small hepatic veinto cavo-atrial junction at any level by thrombosis or its fibrous squeal. Based on the type of obstruction BCS is classified as IVC type where the obstruction is in hepatic or suprahepatic segment of inferior vena cava with at least one patent hepatic vein. In the hepatic vein variety three main hepatic veins are occluded. In the third mixed variety both inferior vena and major three hepatic veins are obstructed. The IVC thrombosis reported in up to 5% of the cases and is more common in Asian population. Our patient will fall in the first category of IVC occlusion.
The treatment strategy for Budd Chiari Syndrome is determined by level of obstruction and clinical presentation. Different reported therapies are medical therapy with anticoagulation, thrombolysis, transcatheter angioplasty or surgical porto-systemic shunting. The primary goal of treatment is to alleviate hepatic congestion and liver injury and its sequel. In a well-established obstruction the initial preferred intervention is recanalization of narrowed or occluded hepatic veins or IVC to restore physiologic hepatic venous
drainage by doing angioplasty with or without stent placement. Rathod et al. described endovascular treatment strategy based on the property of thrombus. Thrombolysis done for fresh thrombus followed by recanalization, for old thrombus balloon dilatation with or without stenting done. In the mixed thrombus group after an initial small balloon dilatation of IVC done followed by thrombolysis and then large balloon dilatation. We also adopted partially the same strategy of initial balloon dilatation and anticoagulation and repeating the procedure after three months. Balloon dilatation of the obstruction relieves obstruction and was found effective in membranous type of IVC obstruction in BCS. The balloon angioplasty has recurrence and may require re-intervention. Stent placement for relieving the obstruction is well established treatment modality for recurrent lesion or as primary modality with good long term outcome.
The congestive symptoms in Budd Chiari syndrome due to obstruction in hepatic vein and or IVC can be effectively treated with well-planned management protocol based on the nature of obstruction. Medical therapy along with transcatheter angioplasty with or without placement of stent is found to be effective.
Conflict of interests
Appendix A. Supplementary data
Supplementary data associated with this article can be found, in the online version, at https://doi.org/10.1016/j.ihjccr.2017.09.008
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