Case 0599_02 -- Budd-Chiari Syndrome Presenting with Right Hydrothorax: Treatment with TIPS

Fig. 1. CT of the abdomen with contrast enhancement shows right hydrothorax, mild ascites, inhomogeneous liver with patchy enhancement, enlarged caudate lobe and compression of the intrahepatic IVC.

Fig. 3. Hepatic venography from a hepatic vein remnant shows a "spider web" appearance representing intrahepatic venous collaterals.

Case Reference No. CC-0599-02

A 68-year-old woman presented with shortness of breath, right chest pain and feeling of unwellness for a few months. She also complained of leg swelling and decreased appetite. Her liver function tests were mildly elevated and her total bilirubin was 2.5. CT of the abdomen (Fig. 1) showed right hydrothorax, mild ascites and inhomogeneous appearance of the liver. The IVC appeared compressed (arrowhead). Based on these findings, a diagnosis of Budd-Chiari syndrome was suspected and hepatic venography was performed. The IVC was first visualized (Fig. 2) showing narrowing of its intrahepatic portion (arrow). The pressure gradient across this lesion was 8 mm Hg. Cannulation of the hepatic veins was attempted, however, normal hepatic veins were not found. The catheter was wedged into a nubbin and venography demonstrates a "spider web" appearance (Fig. 3) representing intrahepatic venous collaterals. A right pleural drain was introduced to improve the respiratory status of the patient.

A discussion followed as to the best management of this patient including a surgical porto-caval shunt, TIPS and observation with anticoagulation. A decision was made to attempt TIPS. The portal puncture was made through what looked like a middle-left hepatic vein trunk and the needle was directed posteriorly. Portal venography (Fig. 4) shows varices and reflux into the IMV with a portal-systemic gradient of 20 mm Hg. TIPS was created using two 10X68 mm Wallstents with reduction of the portal-systemic gradient to 8 mm Hg. The patient was started on heparin with a plan to be converted to coumadin as an outpatient. Doppler ultrasonography performed the next day showed a patent TIPS but a filling defect was identified in the mid stent. Repeat transjugular portal venography (Fig. 5) showed a filling defect in the mid-stent. A 10 mm wide angioplasty balloon was used to macerate and eliminate the lesion, however, an additional stent was needed to create a good result.

The patient had an additional Doppler ultrasound a few days later showing recurrence of a lesion in a similar position which was nearly occlusive. Repeat TIPS venogram showed a circumferential lesion in the mid stent with recurrent portal hypertension (Fig. 6). It was considered that the most likely reason for the recurrent lesion was a biliary fistula. As a last resort (the patient was scheduled for porto-caval shunt surgery) it was decided to deploy a stentgraft and seal the fistula. A quadruple-barrel 10X60 mm Gianturco stent sewn to predilated 5 mm PTFE graft was deployed through a 12F sheath and reinforced underneath with an additional 10X68 mm Wallstent and dilated to 12 mm. This arrangement resulted in excellent flow through the shunt (Fig. 7). The patient was discharged on coumadin after the chest tube was pulled. At six months of follow up her TIPS remains patent and has no recurrence of hydrothorax. Her general well being and appetite have improved and she is doing well.

Fig. 2. Inferior venacavagram shows narrowing of the intrahepatic IVC (arrow).

Fig. 4. Portal venography shows signs of portal hypertension with varices and reflux into the IMV. The portal-systemic gradient was 20 mm Hg.

Fig. 5.TIPS venogram showing a non-occlusive filling defect (arrow) in the mid stent likely representing clot.

Fig. 6. Repeat TIPS venogram showing a circumferential lesion in the mid stent with recurrent portal hypertension. The portal-systemic gradient was 16 mm Hg.

Fig. 7. Following deployment of a quadruple-barrel 10X60 mm Gianturco stent sewn to predilated 5 mm PTFE graft reinforced underneath with an additional 10X68 mm Wallstent and dilated to 12 mm there is excellent flow through the shunt with elimination of portal hypertension.

Questions:
1) How frequently does Budd-Chiari syndrome present primarily with hydrothorax?

2) Is the recurrent mid-stent lesion due to hypercoagulable state or a biliary fistula?

3) Is TIPS a good solution for Budd-Chiari syndrome?

4) How long should the patient be kept on anticoagulation?

Related Cases:

Case 0498_09 --Hepatic vein occlusion

Case 597_10-- Fine needle TIPS for Budd-Chiari Syndrome

MMVI_06 TIPS-Mismanagement of Budd-Chiari Syndrome

Case 0599_03--TIPS for Budd-Chiari: Late Follow Up and Management

Fig. 1. CT of the abdomen with contrast enhancement shows right hydrothorax, mild ascites, inhomogeneous liver with patchy enhancement, enlarged caudate lobe and compression of the intrahepatic IVC. Fig. 3. Hepatic venography from a hepatic vein remnant shows a "spider web" appearance representing intrahepatic venous collaterals. Case Reference No. CC-0599-02 A 68-year-old woman presented with shortness of breath, right chest pain and feeling of unwellness for a few months. She also complained of leg swelling and decreased appetite. Her liver function tests were mildly elevated and her total bilirubin was 2.5. CT of the abdomen (Fig. 1) showed right hydrothorax, mild ascites and inhomogeneous appearance of the liver. The IVC appeared compressed (arrowhead). Based on these findings, a diagnosis of Budd-Chiari syndrome was suspected and hepatic venography was performed. The IVC was first visualized (Fig. 2) showing narrowing of its intrahepatic portion (arrow). The pressure gradient across this lesion was 8 mm Hg. Cannulation of the hepatic veins was attempted, however, normal hepatic veins were not found. The catheter was wedged into a nubbin and venography demonstrates a "spider web" appearance (Fig. 3) representing intrahepatic venous collaterals. A right pleural drain was introduced to improve the respiratory status of the patient. A discussion followed as to the best management of this patient including a surgical porto-caval shunt, TIPS and observation with anticoagulation. A decision was made to attempt TIPS. The portal puncture was made through what looked like a middle-left hepatic vein trunk and the needle was directed posteriorly. Portal venography (Fig. 4) shows varices and reflux into the IMV with a portal-systemic gradient of 20 mm Hg. TIPS was created using two 10X68 mm Wallstents with reduction of the portal-systemic gradient to 8 mm Hg. The patient was started on heparin with a plan to be converted to coumadin as an outpatient. Doppler ultrasonography performed the next day showed a patent TIPS but a filling defect was identified in the mid stent. Repeat transjugular portal venography (Fig. 5) showed a filling defect in the mid-stent. A 10 mm wide angioplasty balloon was used to macerate and eliminate the lesion, however, an additional stent was needed to create a good result. The patient had an additional Doppler ultrasound a few days later showing recurrence of a lesion in a similar position which was nearly occlusive. Repeat TIPS venogram showed a circumferential lesion in the mid stent with recurrent portal hypertension (Fig. 6). It was considered that the most likely reason for the recurrent lesion was a biliary fistula. As a last resort (the patient was scheduled for porto-caval shunt surgery) it was decided to deploy a stentgraft and seal the fistula. A quadruple-barrel 10X60 mm Gianturco stent sewn to predilated 5 mm PTFE graft was deployed through a 12F sheath and reinforced underneath with an additional 10X68 mm Wallstent and dilated to 12 mm. This arrangement resulted in excellent flow through the shunt (Fig. 7). The patient was discharged on coumadin after the chest tube was pulled. At six months of follow up her TIPS remains patent and has no recurrence of hydrothorax. Her general well being and appetite have improved and she is doing well.	Fig. 2. Inferior venacavagram shows narrowing of the intrahepatic IVC (arrow).
	Fig. 4. Portal venography shows signs of portal hypertension with varices and reflux into the IMV. The portal-systemic gradient was 20 mm Hg.
	Fig. 5.TIPS venogram showing a non-occlusive filling defect (arrow) in the mid stent likely representing clot.
	Fig. 6. Repeat TIPS venogram showing a circumferential lesion in the mid stent with recurrent portal hypertension. The portal-systemic gradient was 16 mm Hg.
	Fig. 7. Following deployment of a quadruple-barrel 10X60 mm Gianturco stent sewn to predilated 5 mm PTFE graft reinforced underneath with an additional 10X68 mm Wallstent and dilated to 12 mm there is excellent flow through the shunt with elimination of portal hypertension.
Questions: 1) How frequently does Budd-Chiari syndrome present primarily with hydrothorax? 2) Is the recurrent mid-stent lesion due to hypercoagulable state or a biliary fistula? 3) Is TIPS a good solution for Budd-Chiari syndrome? 4) How long should the patient be kept on anticoagulation? Related Cases: Case 0498_09 --Hepatic vein occlusion Case 597_10-- Fine needle TIPS for Budd-Chiari Syndrome MMVI_06 TIPS-Mismanagement of Budd-Chiari Syndrome Case 0599_03--TIPS for Budd-Chiari: Late Follow Up and Management