Transjugular intrahepatic portosystemic shunt with expanded-polytetrafuoroethylene-covered stents in non-cirrhotic patients with portal cavernoma
Article Outline
- Abstract
- 1. Introduction
- 2. Patients and methods
- 3. Results
- 4. Discussion
- Conflict of interest statement
- References
- Copyright
Abstract
Aims
To evaluate the feasibility and efficacy of Transjugular intrahepatic portosystemic shunt (TIPS) in non-cirrhotic patients with symptomatic portal hypertension secondary to portal cavernoma.
Methods
Our cohort includes 13 consecutive patients. Eleven were considered for Transjugular intrahepatic portosystemic shunt placement for complications not manageable by medical/endoscopic treatment and two because of the need of oral anticoagulation in presence of high-risk varices. Expanded-polytetrafluoroethylene-covered stents were used in all.
Results
One of the 13 patients was excluded because of a thrombosis of the superior cava and jugular veins. In 10 patients, Transjugular intrahepatic portosystemic shunt was successfully implanted [83.3%; 95% confidence interval: 52–98%]. One patient had an early shunt dysfunction with recurrence of variceal bleeding which required an emergency surgical shunt. Late shunt dysfunction occurred in two patients, successfully treated with angioplasty and re-stenting. Two patients experienced an episode of encephalopathy.
Conclusions
Transjugular intrahepatic portosystemic shunt is feasible in most of the patients with portal cavernoma and should be considered in those with severe complications uncontrolled by conventional therapy. The use of Transjugular intrahepatic portosystemic shunt to achieve a lifelong anticoagulation therapy in selected patients with high-risk varices may be another possible indication. These patients should be referred to selected Units with large experience in Transjugular intrahepatic portosystemic shunt placement.
Keywords: Chronic thrombosis of portal vein, Covered stents, Portal cavernoma, Portal hypertension, Transjugular intrahepatic portosystemic shunt
1. Introduction
Chronic portal vein thrombosis is an infrequent cause of portal hypertension [1], [2], [3], [4], [5], [6] which, despite well-preserved liver function, may result in serious complications including variceal bleeding, ascites, intestinal ischaemia and jaundice [6], [7], [8], [9], [10]. Transjugular intrahepatic portosystemic shunt (TIPS) has became a well-established therapeutic tool in cirrhotic patients with portal hypertension and variceal bleeding or ascites refractory to standard treatment [11], [12], [13], [14], [15], [16], [17], [18]. However, TIPS placement may be technically difficult in patients with a cavernous transformation of the portal vein and, until some years ago, was considered contraindicated in this condition [19]. More recently, after several unsuccessful reports [20], [21], [22], some authors [23], [24], [25], [26] presented their first satisfactory results by showing that TIPS may be possible in patients with portal cavernoma, although with a lower feasibility [27]. Until now, the reports available in literature are limited to small groups of patients and have assorted cases with and without cirrhosis and even patients with portal vein thrombosis of neoplastic origin. Moreover, the new expanded-polytetrafluoroethylene (ePTFE)-covered stents, recently introduced as a technical tool with a significant advantage for the maintenance of TIPS patency [28], [29], [30], [31], were used only occasionally in patients with portal hypertension due to portal cavernoma [32].
The aim of the present study was to evaluate the feasibility, safety and efficacy of TIPS created with ePTFE-covered stents in non-cirrhotic patients with symptomatic portal hypertension secondary to chronic portal vein occlusion and cavernoma.
2. Patients and methods
2.1. Clinical data
This study includes 13 consecutive cases (9 men and 4 women with a mean age of 44.8
±
13.5 years) without cancer or liver cirrhosis, selected among the patients referred and followed at our Unit for the evaluation and treatment of complications of cavernous transformation of the portal vein. Cavernous transformation of the portal vein was defined as the presence of numerous venous collaterals of the hilum in the presence of extra-hepatic portal vein thrombosis on the basis of imaging studies. Cancer—except for myeloproliferative diseases—was excluded on the basis of imaging studies and negative tests for serum tumour markers. Cirrhosis was excluded on the basis of the absence of causes for chronic liver disease, normal results of liver function tests, and liver biopsy findings when the former two criteria were not fulfilled. At entry, the presence of oesophageal and/or gastric varices was evaluated by means of endoscopy in all patients. Variceal treatment, when indicated, was pharmacological (i.e., beta-blockers) or endoscopic (i.e., band ligation). When the patients developed severe complications—such as variceal bleeding, intestinal ischaemia, refractory ascites or large varices at high-risk of bleeding with a concomitant need of lifelong anticoagulation—despite the medical and/or endoscopic treatment, they were considered for TIPS placement.
2.2. Pre-TIPS evaluation
A detailed explanation, including the description of early and late procedural complications, was given to the patients considered for TIPS. All of them were informed that the experience with TIPS in cases with portal cavernoma is very limited worldwide and that there is no possibility of predicting the success of the procedure based on preliminary imaging studies. A written informed consent was then obtained from each patient.
Doppler sonography (US), computed tomography (CT), or magnetic resonance (MR) imaging, both in axial and multiplanar reconstructions, were used to confirm the diagnosis of portal cavernoma and to assess the extension of the thrombus to the splenic and/or superior mesenteric vein.
2.3. TIPS technique
All TIPS were created with ePTFE-covered stent-grafts (Viatorr, W.L. Gore & Associates, Flagstaff, AZ, USA). All TIPS procedures were carried out by the same senior interventional radiologists (FF and FMS). In the presence of portal cavernoma, the initial step of the procedure is performed according to the standard technique, with puncture of the (right or left) internal jugular vein and introduction of a TIPS sheath into the selected hepatic vein. In case of Budd–Chiari syndrome, if the hepatic veins are obstructed, a transcaval puncture may be attempted. In our series, however, in the two patients with Budd–Chiari syndrome, the right hepatic veins were not completely occluded, thus permitting their catheterisation. All procedures were performed only through a transjugular (either right or left) access, using a thin (21 G) TIPS-set needle (AngioDynamics Inc., Queensbury, NY, USA). Several authors [33], [34] suggest a trans-hepatic or a trans-splenic access to localize the portal vein during the TIPS procedure, or to perform a ricanalisation of the portal vein before the transjugular approach. In our cases, we were however able to localise and cross the portal vein with a blind puncture under fluoroscopic guidance (Philips Integris 5000, Philips Healthcare, Best, The Netherlands). We did not use the US guiding technique to avoid long X-ray exposure of the hand of the operator and for the difficulty in identifying the remnant thrombosed portal vein. However, in some cases, we found helpful to perform a superior mesenteric arteriography to identify, in the venous phase, the cavernoma and the liver hilum and to re-assess the patency of the mesenteric and/or splenic veins. Moreover, the use of 21-G needles reduces puncture-related complications, thus allowing a safer direct search of the portal system through repeated punctures. Once the periportal space is entered, a low-pressure contrast medium injection permits the identification of the portal contour and provides a guide for its puncture. Sometimes, the visualisation of the biliary ducts may be of great help in directing the subsequent puncture: the portal vein is posterior to the confluence of the main biliary ducts, therefore a posteriorly oriented puncture may allow to enter the thrombosed portal vein. When the needle is presumably positioned into the thrombus, a 5-F catheter is advanced on it and the needle withdrawn, a hydrophilic curved guidewire (Terumo Corp., Tokyo, Japan) is then advanced in an attempt to cross the thrombosed segment. When the guidewire is inside the thrombosed vein, it will follow the path of the main portal vein and will advance through the thrombus negotiating the obstacle opposed by the thrombus until it reaches the true lumen of the vessel. If the wire does not follow the portal path, the puncture is repeated. Once the catheter is advanced into a patent portion of the main portal vein or in the superior mesenteric vein, a contrast is injected to visualize the venous anatomy. Portal pressure gradient, as the difference between the pressure in the superior mesenteric vein and in the right atrium is then measured, by means of a pressure transducer. The procedure is continued with dilation of the thrombosed segment with a balloon-catheter (Wanda Balloon of 6
mm diameter, Boston Scientific, Natick, MA, USA) and one or two ePTFE stent-grafts (Viatorr, W.L. Gore & Associates, Flagstaff, AZ, USA) are then deployed with their proximal end placed at the hepato-caval junction and their distal end in a non-thrombosed portion of the portal system: the spleno-mesenteric junction or the superior mesenteric vein. If a fresh thrombus was to be removed, we used a manual aspiration technique with an 8-Fr guiding catheter (Boston Scientific, Natick, MA, USA). In those patients treated with oral anticoagulation before TIPS, the procedure was performed after oral therapy withdrawal was replaced by subcutaneous low-molecular-weight heparin. Heparin was administered to all the patients during and following the procedure. The portal pressure is then re-measured as the difference between the pressures measured distally to the stent and the right atrium. The duration of the recanalisation procedure ranged from 60 to 180
min.
2.4. Follow-up
After TIPS placement, all patients were monitored for shunt patency according to the follow-up schedule adopted by our Institution for cirrhotic patients treated with TIPS created with PTFE-covered stents, as previously described [29], [31]. In detail, ultrasonography with colour and/or power-Doppler was performed 1 and 4 weeks after TIPS, at three and 6 months post-TIPS, and at 6-month intervals thereafter, or whenever clinically necessary. Patients underwent upper gastrointestinal endoscopy 1 week and 1 month after TIPS placement and then at 6-month intervals, or whenever clinically necessary. Venography and portosystemic pressure gradient measurement were performed only if the patients had clinical evidence of shunt dysfunction (recurrence of gastrointestinal bleeding). Venography was also performed in case of recurrences or enlargement of varices in addition to one of the following ultrasonography findings: (1) absent flow in the shunt; and (2) presence of a significant increase of flow velocity in the stent or an increase of the splenic longitudinal diameter. Finally, venography was also performed in case of clinical and/or CT evidence of recurrence of intestinal ischaemia.
Oral anticoagulation was prescribed to all patients after the TIPS construction.
3. Results
3.1. Clinical data
Thirteen patients (9 males; mean age, 44.8
±
13.5 years) with portal cavernoma were included. At entry, a concomitant involvement of the superior mesenteric vein was recorded in three patients (in 2 out of 3 the thrombosis was complete) and of splenic vein (complete) in one patient. Further three patients had both splenic and mesenteric vein complete thrombosis. Mean serum bilirubin was 1.5
±
0.9
mg/dl, mean serum albumin 3.7
±
0.6
g/dl, mean serum creatinine 0.8
±
0.2
mg/dl. The cause of venous obstruction was identified in all cases. Chronic myeloproliferative disorders were found in seven patients, one or more prothrombotic coagulation disorders in seven and paroxysmal nocturnal haemoglobinuria in one. Prothrombotic risk factors were present in three cases: one patient was receiving oral contraceptives and two had hyperhomocysteinaemia. In only one of our patients, a local factor alone was the cause of portal thrombosis.
3.2. TIPS: indications and follow-up
The indications for a TIPS procedure are listed in Table 1. Eight patients were candidates for TIPS placement because of bleeding related to portal hypertension uncontrolled by conventional treatment. Two patients had symptoms of intestinal ischaemia due to acute superior mesenteric vein thrombosis despite oral anticoagulation. In one patient with concomitant Budd–Chiari syndrome, TIPS was indicated for ascites refractory to the diuretic therapy. In the other two patients, TIPS was planned because of the need of a lifelong oral anticoagulation therapy in presence of large oesophageal varices at high-risk of bleeding associated with gastric varices.
Table 1. Indications for TIPS procedure and technical results in 13 non-cirrhotic patients with portal cavernoma.
| Patients | TIPS indications | Time from diagnosis of cavernoma | Shunt created (no. of stents) | Post-TIPS PSG (mm | Amelioration of varices after TIPS |
|---|---|---|---|---|---|
| 1 | Recurrent variceal bleeding refractory to medical/endoscopic therapy | 7.6 years | Yes (1) | 10 | Yes |
| 2 | Recurrent variceal bleeding refractory to medical/endoscopic therapy | 22.2 years | Yes (1) | 5.6 | Yes |
| 3 | Recurrent variceal bleeding refractory to medical/endoscopic therapy | 5.9 months | Yes (3) | 6.3 | Yes |
| 4 | Recurrent bleeding from gastric varices refractory to medical/endoscopic therapy | 6.3 years | Yes (1) | 4.2 | No |
| 5 | Recurrent variceal bleeding refractory to endoscopic therapy | 5.5 months | Yes (2) | 7 | Yes |
| 6 | Recurrent variceal bleeding refractory to endoscopic therapy | 1.2 years | Yes (1) | 6 | Yes |
| 7 | Recurrent bleeding from portal hypertensive gastropathy | 34 years | Yes (2) | 7 | Yes |
| 8 | Recurrent bleeding from ectopic varices | 10.7 years | No | – | – |
| 9 | Intestinal ischaemia due to acute superior mesenteric vein thrombosis | 1.7 years | Yes (1) | 11.9 | No varices before TIPS |
| 10 | Intestinal ischaemia due to acute superior mesenteric vein thrombosis | 3 months | Yes (2) | 12 | Yes |
| 11 | Varices at high-risk of bleeding need of anticoagulation therapy | 1 month | Yes (1) | 9.8 | Yes |
| 12 | Varices at high-risk of bleeding need of anticoagulation therapy | 8.7 years | No | – | – |
| 13 | Refractory ascites | 9.9 years | Excluded (occlusion of the superior cava and the jugular veins) | – | – |
The patient with concomitant Budd–Chiari syndrome—evaluated as a possible candidate for TIPS because of a refractory ascites—was excluded for the complete occlusion of the jugular veins bilaterally and that of the superior cava vein. Other approaches (through the inferior vena cava) were considered too risky, since the patient's ascites was controllable with monthly repeated paracenteses. The remaining 12 patients underwent a TIPS procedure. In 10 (83.3%; 95% confidence interval: 52–98%), TIPS was successfully implanted with a significant reduction of the portosystemic pressure gradient from 22.9
±
6 to 8
±
2.7
mm
Hg (Table 1). In 2/12 patients, TIPS placement failed because catheterisation of the extra-hepatic portion of the thrombosed/sclerotic portal vein was not achieved (Table 1).
As shown in Table 2, 2 of the 10 patients in whom TIPS was successful developed an early complication: Patient 1 had a post-procedural intraperitoneal bleeding with anaemia and a scrotal haematoma that did not require any blood transfusion and resolved spontaneously; Patient 4 had a very early shunt dysfunction within 24
h after TIPS, with recurrence of bleeding from gastric varices which required an emergency spleno-renal shunt after failure of several endoscopic attempts to stop the haemorrhage. This patient (still living) was our first case and a closer revision of the images highlighted that the stent was deployed into a collateral vessel with an insufficient blood flow to maintain the stent patency.
Table 2. Early and late complications after TIPS placement and outcomes at follow-up.
| Patients | TIPS complications | Shunt dysfunction | Relapsed complications | Encephalopathy | Outcome (months after TIPS) |
|---|---|---|---|---|---|
| 1 | Haemoperitoneum | No | No | No | Death due to sepsis (6.3) |
| 2 | No | No | No | Yes | Alive |
| 3 | No | No | No | No | Alive |
| 4 | No | Early shunt dysfunction (24 | Bleeding from gastric varices | No | Alive (surgical shunt) |
| 5 | No | Yes | Persistence of large varices | No | Alive |
| 6 | No | No | No | No | Alive |
| 7 | No | No | No | Yes | Death for ischaemic stroke |
| 8 | – | – | – | – | Alive (β-blockers) |
| 9 | No | Yes (angioplasty/re-stenting) | Abdominal pain and signs of intestinal ischaemia | No | Alive |
| 10 | No | No | No | No | Death for neoplasm (9.3) |
| 11 | No | No | No | No | Alive |
| 12 | – | – | – | – | Alive (β-blockers) |
| 13 | – | – | – | – | Alive (repeated paracenteses) |
In four patients, late post-procedure complications occurred (Table 2): In Patient 5, TIPS was revised 1 week after construction because the persistence of large varices and was successfully treated with re-stenting; Patient 9 had two episodes of shunt dysfunction with recurrence of signs of intestinal ischaemia at 3 and 10 months after TIPS creation. In both cases, luminal stenosis was evident in the uncovered portion of the ePTFE-covered stent (Fig. 1A). The first episode was treated with mechanical thrombolysis and angioplasty (Fig. 1B), while in the second one a re-stenting was performed (Fig. 1C). To date, the patient is asymptomatic and the shunt is still patent. Finally, two patients (Patients 2 and 7) experienced an episode of encephalopathy during follow-up. Due to the infrequent occurrence of hepatic encephalopathy in patients without significant liver damage, both patients were submitted to extensive diagnostic investigations, including EEG and cerebral magnetic resonance. Moreover, following encephalopathy resolution, both patients underwent a liver biopsy that excluded the presence of cirrhosis. In particular, Patient 7 experienced an episode of encephalopathy that occurred in the course of a severe infection. This episode was characterised by loss of consciousness associated with tonic-clonic seizure requiring endotracheal intubation. Elevated values of glycaemia (>500
mg/dl) and venous ammonia (260
μg/dl) were present at the same time, while the neurological assessment (including cerebral magnetic resonance) did not identify any alteration. Further neurological alterations were not recorded and psychometric performance was normal (Trail Making Test-A, Trail Making Test-B and digit symbol test) at subsequent periodic examinations. In Patient 2, the diagnostic work-up concluded for a typical hepatic encephalopathy, graded as mental state 3 according to West Haven criteria. Venous ammonia level was 189
μg/dl. The patient was successfully treated with oral and rectal lactulose.

Fig. 1.
Patient 9: Evidence of luminal stenosis at the level of the non-covered portion of the stent, successfully treated with angioplasty and re-stenting.
No significant alterations of hepatic and renal functions (i.e., serum bilirubin, albumin and creatinine values) were recorded at post-TIPS follow-up. No complications were observed in patients in which TIPS construction was unsuccessful. In particular, despite several attempts to reach the portal system, no bleeding episodes occurred.
No patients died peri-procedurally or within 30 days post-procedurally. The mean follow-up after TIPS placement was 17.4
±
14.7 months (range: 3.3–40.1).
As shown in Table 2, three cases of late mortality occurred: Patient 1, who had an autoimmune hemolytic anaemia and was treated with immuno-suppressive agents, died 6 months after TIPS from acute sepsis; Patient 7 died from ischaemic stroke 24 months after TIPS placement; Patient 10 died from a neoplasm 9 months after the procedure. Their shunts were patent and no recurrence of symptoms was observed. The remaining seven patients who were successfully stented are alive and asymptomatic. The two patients in whom TIPS failed are both alive and are continuing their β-blockers and anticoagulation therapy. The last patient—evaluated as a candidate for TIPS because of a refractory ascites but excluded for the occlusion of the superior cava vein and the jugular veins—is alive and undergoes repeated monthly paracenteses.
4. Discussion
Non-cirrhotic portal hypertension due to chronic portal vein thrombosis is uncommon and frequently associated with an underlying prothrombotic disorder. The treatment is complicated by the wide range of clinical manifestations of the disease and the need of a lifelong anticoagulation therapy. Moreover, the infrequency of the disease has discouraged the possibility of carrying on randomized controlled trials with a sufficient number of patients. Thus, the approach to this type of portal hypertension is mostly based on observational studies or on treatments shown to be effective in cirrhotic patients, such as non-selective β-blockers or band ligation [35].
Theoretically, TIPS may be useful in patients with non-cirrhotic portal hypertension both as a rescue therapy for acute variceal bleeding (also for those with ectopic varices) and in the prophylaxis of variceal re-bleeding. In addition, TIPS-induced acceleration of the portal blood flow may prevent the extension of thrombosis into the portal system or may be of help in presence of intestinal ischaemia due to the extension of thrombosis to the superior mesenteric vein. Moreover, lifelong anticoagulation therapy, which, when indicated, has been associated with a reduction of further thrombotic events [36], may be carried on with difficulty in individuals with large varices at high-risk of bleeding or in those who have already bled. TIPS creation, by definitely eliminating the presence of varices, may therefore facilitate the use of anticoagulation therapy. This potential indication has been recently suggested [37].
The present study described our initial experience with TIPS in a group of non-cirrhotic patients with symptomatic portal hypertension due to cavernomatous transformation of the portal vein and showed that the procedure was feasible, although not in all patients. If performed correctly, TIPS is able to reduce the portal pressure gradient to clinically non-significant levels (Table 1) and, as a consequence, varices disappear. In the two patients with acute superior mesenteric vein thrombosis associated with portal cavernoma, TIPS was able to treat the signs of intestinal ischaemia; in one patient, in particular, the abdominal pain recurred as a consequence of a shunt dysfunction and disappeared again after restoration of the shunt patency.
Although this is not the first report on TIPS placement in patients with portal cavernoma, it describes the first series including only non-cirrhotic cases all treated with ePTFE-covered stents. Our results are essentially comparable with those reported by Senzolo et al. [26] (who obtained a TIPS placement success rate of 67% in a subgroup of nine patients with portal cavernoma), and with those presented by Van Ha et al. [24] and by Perarnau et al. [27] who report a success rate of 75% and 63%, respectively.
Similarly to previous reports [26], we were unable to accurately predict TIPS success or failure on the basis of pre-operative imaging studies. After this experience, our opinion is that the main factor contributing to the success of TIPS placement is the possibility of crossing the thrombosed/sclerotic portal vein with the guidewire thus reaching a patent tract of the portal system with a sufficient flow (Fig. 2). In case of thrombus extension in the splenic and mesenteric veins, we have to differentiate the presence of a complete or partial thrombosis: the complete thrombosis of both vessels is a contraindication to the procedure. Partial splenic or mesenteric vein thrombosis as well as the involvement of only one vessel increases the risk of failure but does contraindicate shunt creation. In the two cases in which TIPS failed, it was impossible to negotiate the wire through the thrombus into the main portal vein (Fig. 3). This was mainly due to thrombus hardness or the impossibility to find a way between the thrombus and the portal vein wall (features which are not detectable by any pre-operative imaging studies). Thrombus age, which can in theory be considered an important factor, was, in our experience, not determinant. In fact, a successful TIPS procedure was achieved in one of our patients with a 30-year-old portal vein thrombosis and extensive calcifications of the portal system (Fig. 4). The possibility of deploying the stent into the main trunk of the portal vein—and not into a collateral vessel where the blood flow can be insufficient to maintain the shunt patency—is another technical element of vital importance for a successful procedure. In our first case (Patient 4), the stent was placed into a collateral vessel and, after an initial decrease of the portal pressure, shunt dysfunction with variceal re-bleeding occurred immediately post-procedure.

Fig. 2.
Patient 11: Successful TIPS placement in a 30-year old woman with essential thrombocythaemia and portal cavernoma. (A) Guidewire advancing from the left portal branch through the main portal vein. (B) Portal venogram performed after the portal vein occlusion was crossed, showing a patent superior mesenteric vein (SMV) and the occluded main portal vein with cavernous transformation. (C) Venogram performed after TIPS placement with recanalisation of the portal system.

Fig. 3.
Patient 12: Attempt for TIPS creation in a 41-year old man with idiopathic myelofibrosis and portal cavernoma. (A) Venogram after crossing the thrombosed remnant portal vein showing the lack of visualisation of a valid communication with the other splanchnic veins. (B) The only visualised venous tract was a small coronary vein feeding the oesophageal varices, probably not sufficient to keep the stent open.

Fig. 4.
Patient 7: Successful TIPS placement in a 37–year old man with portal cavernoma since childhood. (A) CT scan showing the occlusion of the portal vein at the hilum with large pericholecystic collateral veins supplying the intrahepatic portal veins. Extensive venous calcification and presence of a spontaneous spleno-renal shunt. (B) Venogram showing an occluded superior mesenteric vein. (C) Venogram performed after portal vein reconstruction and TIPS placement with the distal end of the stent into the patent splenic vein.
Although non-cirrhotic patients with portal vein thrombosis may exhibit neuropsychological and brain magnetic resonance changes consistent with a minimal hepatic encephalopathy [38], episodes of overt encephalopathy are unusual [19]. Following TIPS, two patients experienced an episode of encephalopathy although in one it occurred in the course of a severe bacterial infection and was accompanied with concomitant severe hyperglycaemia.
A further important point is that the shunt dysfunction rate may be increased in patients with portal vein thrombosis due to a prothrombotic disease. Therefore, the timing of the radiological, endoscopic and clinical follow-up for the maintenance of TIPS patency may be different from that used in cirrhotic patients. The role of anticoagulation therapy after TIPS also needs to be established. The limited number of these patients treated with TIPS did not allow any firm conclusion on these issues. Our patients were followed up according to a protocol established for cirrhotic patients undergoing TIPS with covered stents and all were orally anticoagulated after TIPS. With this regimen, only two cases developed a shunt dysfunction. The use of ePTFE-covered stents in all our patients might also explain the good results achieved in our series. However, more data are necessary to establish the optimal management and follow-up to prevent TIPS dysfunction.
In conclusion, according to our experience and our success rate of 83.3%, portal vein cavernomas should not be considered a contraindication to TIPS, which when technically feasible, is effective. Given the unpredictability of procedure success and the potential risk to which the patient is exposed, TIPS should be considered only in cases with severe complications of portal hypertension not responding to medical and/or endoscopic treatment or in patients with complications due to an extension of thrombosis to the superior mesenteric vein associated to portal cavernoma. Other possible indications, although less documented and accepted, may be the use of TIPS to permit lifelong anticoagulation therapy in selected patients with varices at a very high-risk of bleeding and the relief of biliary tract obstructions in cases with severe symptoms of cholestasis [39]. These patients should be referred to selected Units with a large experience in TIPS procedures. Although more data are needed to fully establish the potential role of TIPS, we believe that this procedure should be incorporated in the algorithm for the management of patients with portal cavernoma.
Conflict of interest statement
None declared.
References
- Risk factors for thrombophilia in extrahepatic portal vein obstruction. Hepatology. 2005;41:603–608
- . Thrombosis and anticoagulation in liver disease. Hepatology. 2008;47:1384–1393
- Cause of portal and hepatic venous thrombosis in adults: the role of multiple concurrent factors. Hepatology. 2000;31:587–591
- . Portal vein thrombosis in adults: pathophysiology, pathogenesis and management. J Hepatol. 2000;32:865–871
- Extrahepatic portal vein thrombosis: aetiology and determinants of survival. Gut. 2001;49:720–724
- . Extrahepatic portal vein obstruction. Semin Liver Dis. 2002;22:43–58
- . Portal vein thrombosis: a concise review. Am J Gastroenterol. 2002;97:535–541
- . Portal hypertension due to portal venous thrombosis: etiology and clinical outcome. World J Gastroenterol. 2007;13:2535–2540
- . Nonmalignant portal vein thrombosis in adults. Nat Clin Pract Gastroenterol Hepatol. 2006;3:505–515
- Prognostic factors in noncirrhotic patients with splanchnic vein thromboses. Am J Gastroenterol. 2007;102:1–7
- Transjugular intrahepatic portosystemic shunts for patients with active variceal hemorrhage unresponsive to sclerotheraphy. Gastroenterology. 1996;111:138–146
- Randomised trial of transjugular-intrahepatic-portosystemic shunt versus endoscopy plus propanolol for prevention of variceal rebleeding. Lancet. 1997;349:1043–1049
- Transjugular intrahepatic portosystemic shunt versus endoscopic sclerotheraphy for the prevention of variceal bleeding in cirrhosis: a randomized multicenter trial. Hepatology. 1998;27:40–45
- Transjugular intrahepatic portosystemic shunts: comparison with paracentesis in patient with cirrhosis and refractory ascites: a randomized trial. J Hepatol. 1996;25:135–144
- A comparison of paracentesis and transjugular intrahepatic portosystemic shunting in patients with ascites. N Engl J Med. 2000;342:1701–1707
- Transjugular intrahepatic portosystemic shunt versus paracentesis plus albumin for refractory ascites in cirrhosis. Gastroenterology. 2002;123:1839–1847
- The North American study for the treatment of refractory ascites. Gastroenterology. 2003;124:634–641
- Randomized controlled study of TIPS versus paracentesis plus albumin in cirrhosis with severe ascites. Hepatology. 2004;40:629–635
- Consensus on extra-hepatic portal vein obstruction. Liver Int. 2006;26:512–519
- Transjugular intrahepatic portosystemic shunt in patients with portal vein occlusion. Radiology. 1993;186:523–527
- Portal venous thrombosis: percutaneous therapy and outcome. J Vasc Interv Radiol. 1998;9:119–127
- Transjugular intrahepatic portosystemic shunt for palliative treatment of portal hypertension secondary to portal vein tumor thrombosis. World J Gastroenterol. 2004;10:1881–1884
- Transjugular intrahepatic portosystemic shunt in a patient with cavernomatous portal vein occlusion. Cardiovasc Intervent Radiol. 2000;23:145–149
- Transjugular intrahepatic portosystemic shunt placement in patients with cirrhosis and concomitant portal vein thrombosis. Cardiovasc Intervent Radiol. 2006;29:785–790
- The role of TIPS for portal vein patency in liver transplant patients with portal vein thrombosis. Liver Transpl. 2006;12:1544–1551
- Transjugular intrahepatic portosystemic shunt for portal vein thrombosis with and without cavernous transformation. Aliment Pharmacol Ther. 2006;23:767–775
- Feasibility and long-term evolution of TIPS in cirrhotic patients with portal thrombosis. Eur J Gastroenterol Hepatol. 2010;[Epub ahead of print]
- Patency of stents covered with polytetrafluoroethylene in patients treated by transjugular intrahepatic portosystemic shunts: long-term results of a randomized multicentre study. Liver Int. 2007;27:742–747
- Polytetrafluorethylene-covered stent-graft for TIPS procedure: 1-year patency and clinical results. Am J Gastroenterol. 2004;99:280–285
- Polytetrafluoroethylene-covered nitinol stent-graft for transjugular intrahepatic portosystemic shunt creation: 3-year experience. Radiology. 2004;231:820–830
- Incidence, natural history and risk factors of hepatic encephalopathy after transjugular intrahepatic portosystemic shunt with polytetrafluoroethylene-covered stent grafts. Am J Gastroenterol. 2008;103:1–9
- Transjugular intrahepatic portosystemic shunt in patients with chronic portal vein occlusion and cavernous transformation. J Clin Gastroenterol. 2009;10:982–984
- . Interventional radiological management of prehepatic obstruction the splanchnic venous system. Cardiovasc Intervent Radiol. 2007;30:688–695
- Transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of venous symptomatic chronic portal thrombosis in non-cirrhotic patients. Cardiovasc Intervent Radiol. 2004;27:474–480
- . Evolving consensus in portal hypertension. Report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension. J Hepatol. 2005;43:167–176
- Current outcome of portal vein thrombosis in adults: risk and benefit of anticoagulant therapy. Gastroenterology. 2001;120:490–497
- Interventional treatment should be incorporated in the algorithm for the management of patients with portal vein thrombosis. Hepatology. 2008;48:1352–1353
- Non-cirrhotic portal vein thrombosis exhibits neuropsychological and MR changes consistent with minimal hepatic encephalopathy. Hepatology. 2006;43:707–714
- Relief of biliary obstruction due to portal vein cavernoma using a transjugular intrahepatic portosystemic shunt (TIPS) without the need for long-term stenting. Endoscopy. 2006;38:760
PII: S1590-8658(10)00206-9
doi:10.1016/j.dld.2010.06.001
© 2010 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Inc All rights reserved.
