Anterograde recanalisation of hepatic vein in Transjugular Intrahepatic Porto-systemic Shunt (TIPSS) in a patient with Budd-Chiari syndrome

Clinical History

A 17-year-old female patient without any known medical history presented with fatigue and subtle jaundice. Exploratory laboratory test showed the following: AST=66 IU/L (reference range: 0-31 IU/L), ALT=50 IU/L (reference range: 0-34 IU/L), LDH=258 IU/L (reference range: 0-247 IU/L) and a total bilirubin=48 IU/L (reference range: 0-17 IU/L).

Imaging Findings

Abdominal ultrasound (US) showed ascites, hepatomegaly and occlusion of the hepatic veins. Multi-phase contrast-enhanced CT confirmed these findings but also showed inhomogeneous enhancing liver with an enhancement of the perivascular regions on the arterial phase alternated with an enhancement of peripheral regions on the venous phase (Fig. 1). These findings were consistent with Budd-Chiari Syndrome (BCS).

The patient was referred to undergo Transjugular Intrahepatic Porto-systemic Shunt (TIPSS). A right portal vein (PV) branch was approached percutaneously under US guidance with a 22G Chiba needle and a 6F Neff set (Neff Percutaneous Access Set, Cook Medical) was introduced. Venography showed hepatofugal flow in the PV with formation of multiple gastric and oesophageal varices (Fig. 2). Attempts to recanalize the right hepatic vein (RHV) from a retrograde approach failed.

Discussion

BCS is a rare condition which affects 1 in 100,000 individuals and it is due to obstruction of hepatic venous outflow, primarily caused by hypercoagulable states in about 75% or due to secondary causes such as web, tumour or abscess [1-5]. The clinical presentation depends on the degree of obstruction [6]. Approximately 80% of the patients with BCS will eventually develop symptoms varying from non-specific abdominal complains to severe portal hypertension and liver failure [3, 6].

The management of BCS depends on the cause and the clinical presentation. Medical, surgical and endovascular therapies are available and mostly directed at management of the symptoms pending a possible liver transplantation. Especially in BCS with severe portal hypertension and ascites, TIPSS procedures showed a promising success rate of over 90% and a clinical success rate of over 70% [7, 8, 9]. However, to ensure this, anti-coagulation therapy and routine ultrasound examination are required.

In our case, an initial retrograde recanalization of the RHV failed. For an anterograde recanalisation, the RHV was punctured with a 22G Chiba needle under US guidance and a 6F Neff set was introduced. A 0.035 Terumo guidewire and 4F Berenstein catheter were inserted through the Neffset and manipulated through the occluded RHV and into the inferior caval vein (ICV) (Fig. 3). The guidewire was then brought into the 10F sheath and externalized at the puncture site in the neck. Next, a TIPSS puncture set (Rosch-Uchiba Transjugular Liver Access Set, Cook Medical) was advanced over the guidewire into the recanalized RHV. From the RHV, a needle was advanced toward the marked tip of the Neffset in the PV. After confirming that the needle tip was in the PV, a guidewire was advanced through the TIPSS sheath and into the superior mesenteric vein (SMV). An 8mm balloon was advanced over stiff guidewire and the intraperenchymal tract was pre-dilated. Then, 2 overlaying 10mm partially covered Viattor stents with a covered length of 6 cm and 7 cm were placed covering the tract from PV up to the ICV (Fig. 4).

Recanalisation of HV is a common technical challenge encountered during TIPSS procedures in BCS. This challenge can be overcome using alternative techniques such as anterograde recanalisation. Also, since development of covered stents, a higher long-term TIPSS patency rate is achieved [8]. In conclusion, TIPSS is a minimally invasive procedure with high success rate in management of patients with BCS.

Differential Diagnosis List

Final Diagnosis

Transjugular porto-systemic shunt in a patient with Budd-Chiari syndrome

URL:	https://www.eurorad.org/case/11658
DOI:	10.1594/EURORAD/CASE.11658
ISSN:	1563-4086