Portal vein (PV) thrombosis with superior mesenteric vein (SMV) thrombosis happens in patients with liver cirrhosis as well as liver transplantation (LT) recipients with impaired graft function [1]. The underlying causes of such SMV thrombosis include decreased splanchnic blood flow leading to blood stagnation. Sudden-onset high-grade PV thrombosis can result in massive ascites like prehepatic portal hypertension because the development of sufficient collateral veins takes a long time. Such deprivation of portal blood inflow and massive ascites can lead to deterioration of liver function, resulting in liver failure. To solve such a situation, transjugular intrahepatic portosystemic shunt (TIPS) can be considered as a bridge or rescue therapy for the restoration of portal blood flow and the control of ascites [2,3]. We herein present a case of an LT patient who underwent TIPS as a rescue therapy for the control of PV thrombosis-associated refractory ascites.

A 47-year-old female underwent living-donor liver transplantation (LDLT) for primary biliary cirrhosis (Fig. 1A, B). The patient received a modified right liver graft from her daughter. The patient had been doing well for the first 10 years after LDLT (Fig. 1C, D). However, thereafter, liver graft function was progressively deteriorated, showing overt evidence of hepatic fibrosis (Fig. 2). After the administration of combination therapy with tacrolimus and everolimus, such deterioration of liver graft function was stationary. At the age of 62 years (posttransplant 15 years), massive ascites suddenly developed. Work-up studies revealed that the cause of refractory ascites was sudden-onset total occlusion of the main PV by non-tumorous thrombus was (Fig. 3). This situation appeared to indicate TIPS to achieve PV recanalization, thus TIPS was performed.

Figure 1. Peritransplant computed tomography scans taken before transplantation (A, B) and one year after transplantation (C, D).

Figure 2. Computed tomography taken at 14 years after transplantation. (A) Some morphological changes were visible at the right posterior section area. (B–D) Inflow portal vein and outflow right hepatic vein appeared to be patent.

Figure 3. Computed tomography taken at 15 years after transplantation. (A) Massive ascites was identified. (B–D) Complete occlusion of the main portal vein by thrombus was found (arrows).

First, a pigtail was percutaneously inserted to drain the ascites. Second, TIPS procedure was initiated by trans-splenic splenic vein puncture. SMV venogram showed total thrombotic occlusion of the main PV with the development of collaterals, in which the pressure gradient between the SMV (38 mmHg) and right hepatic vein (9 mmHg) was 29 mmHg. A puncture was performed from the right hepatic vein to the right posterior PV under balloon catheter guidance via a trans-splenic access. Pre-TIPS dilation using 8-mm balloon was performed. TIPS stenting was performed using two partially covered stents of each 10 mm×8 cm and 10 mm×6 cm. Post-TIPS dilation using 8-mm balloon was performed. Post-TIPS splenic vein venogram showed remaining thrombus in the main portal vein-splenic vein junction. Balloon percutaneous transluminal angioplasty at the splenic vein and subsequent kissing balloon percutaneous transluminal angioplasty for the remaining thrombus were performed, but thrombus still remained. Due to the remaining thrombus, additional bare metal stenting of 10 mm×6 cm was thus performed at the SMV-main PV. Completion SMV venogram showed patent stent flow without thrombus. The final pressure gradient was 6 mmHg between the SMV (16 mmHg) and right hepatic vein (10 mmHg). Finally, trans-splenic tract embolization was performed with one coil and N-butyl cyanoacrylate (Fig. 4).

Figure 4. Procedures of transjugular intrahepatic portosystemic shunt. (A, B) Superior mesenteric vein venogram showed total thrombotic occlusion of main portal vein with the development of collaterals. (C) A puncture was performed from the right hepatic vein to the right posterior portal vein. (D) Balloon dilation was performed. (E) Stenting was performed using two covered stents. Balloon angioplasty was performed and additional bare metal stent was inserted. (F) Completion superior mesenteric vein venogram showed patent stent flow without thrombus.

Soon after TIPS, the amount of ascites decreased rapidly, and the abdominal pigtail catheter was removed. The patient was discharged at 3 weeks after TIPS. Anticoagulation was maintained to prevent thrombus formation. Liver function was maintained well after TIPS, but the patient suffered from several episodes of hepatic encephalopathy. After 3 months of TIPS, hepatic encephalopathy did not occur again for 3 months. At 6 months after TIPS, stent flow was well maintained with stable liver function (Fig. 5).

Figure 5. Computed tomography taken at 4 months after transjugular intrahepatic portosystemic shunt. Stenting was patent at the right hepatic vein (A, B) and at the portal vein (C, D).

The underlying cause of PV thrombosis was fibrosis of the liver graft, which is compatible with liver cirrhosis. Because the efficacy and patency duration of TIPS would be limited, the patient is eligibly indicated for LT. Considering low model for end-stage liver disease score of 12, the possibility of allocation for deceased donor LT is very low in the current Korean setting. Thus, the patient is going to undergo repeat LDLT. A potential living donor has been losing body weight to alleviate hepatic steatosis for liver donation.

TIPS has been performed for the control of refractory ascites in liver cirrhosis patients awaiting LT as a bridge therapy. Portal hypertension is responsible for most severe complications of patients with decompensated liver cirrhosis, such as variceal bleeding or refractory ascites, spontaneous bacterial peritonitis, and hepatorenal syndrome. Insertion of TIPS is an effective option both for the prevention of variceal bleeding and for control of refractory ascites [4-6]

TIPS leads to the creation of a low-resistance connection between the hepatic vein and the intrahepatic portion of the portal vein and can be used as effective treatment for refractory ascites and variceal bleeding in these patients [7,8]. Nowadays, only covered stents are used, since they are associated with higher patency and superior outcomes as compared with bare metal stents [9]. TIPS implantation itself has a reported 30-day mortality between 4% and 45% [10], and benefits as well as potential complications such as hepatic encephalopathy should be carefully evaluated.

A few studies have considered pretransplant TIPS because it can work as a bridge therapy to LT [3,11]. Meanwhile, no clinical study was available on posttransplant TIPS probably because retransplantation would be the treatment of choice in LT recipients requiring TIPS. TIPS was also performed for the control of post-hepatectomy refractory ascites [12,13].

The established TIPS indications are persistent bleeding despite combined pharmacological and endoscopic therapy and rebleeding during the first five days. A new indication in the European recommendations is early TIPS placement within 72 hours, ideally within 24 hours, in patients bleeding from esophageal or gastroesophageal varices at high risk of treatment failure (e. g. Child-Pugh class C<14 points or Child-Pugh class B with active bleeding) after initial pharmacological and endoscopic therapy. For the prevention of recurrent variceal hemorrhage in the recommendations, covered TIPS placement is the treatment of choice only after failed first-line therapy, although numerous TIPS studies show a prolonged time to rebleeding and a reduction of mortality. Similarly for secondary prophylaxis in patients with refractory ascites, covered TIPS placement may be considered only if the patient continues to be intolerant to non-selective beta-blockers and is an appropriate TIPS candidate even though studies show that the TIPS procedure controls ascites, and improves survival and renal function better than paracentesis. Potential indications for TIPS implantation are Budd-Chiari syndrome, acute portal vein thromboses, hydrothorax, hepatopulmonary and hepatorenal syndrome, portal hypertensive gastropathy and prophylaxis of complications of abdominal surgery, very rarely bleeding in ectopic varices or in patients with chylothorax or chylous ascites. TIPS placement is an established procedure with a new indication as &quot;early TIPS&quot;. In the European recommendations it is only the second-line therapy for the prevention of recurrent variceal hemorrhage and for secondary prophylaxis in patients with refractory ascites although several studies showed a clear benefit of the TIPS procedure compared to ligation and non-selective beta-blockers [14].

In conclusion, the experience with the present case suggests that TIPS can be used as a rescue therapy for refractory ascites in LT recipients with graft failure-associated portal vein thrombosis.

There was no funding related to this study.

All authors have no conflicts of interest to declare.

Conceptualization: SH. Data curation: All. Formal analysis: All. Methodology: All. Visualization: SH, GYK. Writing - original draft: All. Writing - review & editing: GY, SH.