Current diagnosis and management of post-transjugular intrahepatic portosystemic shunt refractory hepatic encephalopathy.

Transjugular intrahepatic portosystemic shunt has evolved into an important option for management of complications of portal hypertension. The use of polytetrafluoroethylene covered stents enhances shunt patency. Hepatic encephalopathy (HE) remains a significant problem after TIPS placement. The approach to management of patients with refractory hepatic encephalopathy typically requires collaboration between different specialties. Patient selection for TIPS requires careful evaluation of risk factors for HE. TIPS procedure-related technical factors like stent size, attention to portosystemic pressure gradient reduction and use of adjunctive variceal embolization maybe important. Conservative medical therapy in combination with endovascular therapies often results in resolution or substantial reduction of symptoms. Liver transplantation is, however, the ultimate treatment.

Transjugular intrahepatic porto-systemic stent-shunt for therapy of bleeding esophageal varices due to extramedullary hematopoiesis in primary myelofibrosis: a case report.

BACKGROUND: Primary myelofibrosis belongs to the group of myeloproliferative syndromes. Extramedullary hematopoiesis in the liver can lead to portal hypertension. PATIENT AND METHODS: We report a case of a patient with life-threatening, endoscopically not treatable bleeding from esophageal varices due to extramedullary hematopoiesis of the liver that was successfully treated with placement of a transjugular intrahepatic porto-systemic stent-shunt (TIPS). RESULTS: Therapy of variceal bleeding by TIPS insertion was successful. During a 29-month follow-up, no hepatic failure, hepatic encephalopathy, or further variceal bleeding episode occurred. CONCLUSION: TIPS placement is a well-established procedure for the treatment of complications due to portal hypertension mainly due to liver cirrhosis. This report illustrates that TIPS placement can also be a promising treatment option in patients with primary myelofibrosis and portal hypertension due to extramedullary hematopoiesis.

Custom-made covered transjugular intrahepatic portosystemic shunt (TIPS) in an infant with trisomy 22 and biliary atresia.

We report an 8-month-old girl with portal hypertension secondary to biliary atresia. The decision to treat with TIPS was made at the age of 8 months due to recurrent variceal bleeding. The procedure was carried out with a 6-mm bare stent due to her small size. Radiological follow-up with Doppler US showed gradual stenosis and finally occlusion of the stent 80 days after implantation. Revision was performed with placement of an additional 6-mm expanded polytetrafluoroethylene (e-PTFE) stent-graft that had remained patent for 9 months, proving that in small children with a portal vein diameter less than 8 mm, the combination of a bare stent and stent-graft can provide excellent results.

Endoscopic treatment of esophageal varices in patients with liver cirrhosis.

Variceal bleeding is a life-threatening complication of portal hypertension with a six-week mortality rate of approximately 20%. Patients with medium- or large-sized varices can be treated for primary prophylaxis of variceal bleeding using two strategies: non-selective beta-blockers (NSBBs) or endoscopic variceal ligation (EVL). Both treatments are equally effective. Patients with acute variceal bleeding are critically ill patients. The available data suggest that vasoactive drugs, combined with endoscopic therapy and antibiotics, are the best treatment strategy with EVL being the endoscopic procedure of choice. In cases of uncontrolled bleeding, transjugular intrahepatic portosystemic shunt (TIPS) with polytetrafluoroethylene (PTFE)-covered stents are recommended. Approximately 60% of the patients experience rebleeding, with a mortality rate of 30%. Secondary prophylaxis should start on day six following the initial bleeding episode. The combination of NSBBs and EVL is the recommended management, whereas TIPS with PTFE-covered stents are the preferred option in patients who fail endoscopic and pharmacologic treatment. Apart from injection sclerotherapy and EVL, other endoscopic procedures, including tissue adhesives, endoloops, endoscopic clipping and argon plasma coagulation, have been used in the management of esophageal varices. However, their efficacy and safety, compared to standard endoscopic treatment, remain to be further elucidated. There are safety issues accompanying endoscopic techniques with aspiration pneumonia occurring at a rate of approximately 2.5%. In conclusion, future research is needed to improve treatment strategies, including novel endoscopic techniques with better efficacy, lower cost, and fewer adverse events.

Transjugular intrahepatic portosystemic shunt with expanded-polytetrafuoroethylene-covered stents in non-cirrhotic patients with portal cavernoma.

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.

Transjugular intrahepatic portosystemic shunt for acute variceal hemorrhage.

Acute variceal hemorrhage is life-threatening and requires a multidisciplinary approach for effective therapy. Transfusion of blood products, systemic therapy with vasopressin, octreotide, and selective beta-blockers and early endoscopic therapy are often effective; however, uncontrollable variceal hemorrhage is best treated with transjugular intrahepatic porto-systemic shunt (TIPS) creation. This procedure involves establishment of a direct pathway between the hepatic veins and the portal veins to decompress the portal venous hypertension that is the source of the patient's hemorrhage. The procedure is technically challenging, especially in critically ill patients, and has a mortality of 30%-50% in the emergency setting, but has greater than 90% effectiveness in controlling bleeding from gastroesophageal varices. In this review, we discuss the technical aspects of TIPS and briefly examine the results of TIPS in managing acute variceal hemorrhage.

Polytetrafluoroethylene-covered nitinol stent-graft for transjugular intrahepatic portosystemic shunt creation: 3-year experience.

PURPOSE: To prospectively evaluate the use of a recently developed expanded polytetrafluoroethylene (PTFE)-covered nitinol stent-graft in preventing the need for repeated intervention after transjugular intrahepatic portosystemic shunt (TIPS) creation. MATERIALS AND METHODS: Fifty-three consecutive patients underwent TIPS procedures between January 2000 and February 2002. Minimum patient follow-up was 9 months (mean, 16.3 months). Fifty-six stent-grafts were implanted in 53 patients; eight of the devices were 8 mm in diameter and 48 were 10 mm in diameter. The stent length varied from 4 to 7 cm. Indications for the procedure included recurrence of bleeding after sclerotherapy (28 patients with cirrhosis, one patient without), refractory ascites or hydrothorax (21 patients with cirrhosis, one patient without), and Budd-Chiari syndrome (two patients). RESULTS: A technical success rate of 100% was obtained, with an early clinical success rate of 96.2%. During the follow-up period, the recurrence rate was 3.4% (one of 29 patients) for bleeding and 9.0% (two of 22 patients) for ascites. Shunt malfunction occurred in nine of 53 patients (16.9%); in one of these nine patients, shunt occlusion was evident after revision, and a parallel shunt was created. The 1-year primary and secondary patency rates were 83.8% and 98.1%, respectively. In this series, the incidence of encephalopathy (included even as a single short-lived episode) was 47.1% (25 of 53 patients). The 30-day mortality rate was 3.8% (two of 53), and the late mortality rate was 17.3% (eight of 46), excluding seven patients who underwent transplantation. CONCLUSION: The new PTFE-covered nitinol stent-graft used appears to be excellent in preventing the need for repeated interventions. A primary patency rate of 83.8% and a secondary patency rate of 98.1% were achieved.

[Ultrasound guided puncture of the portal vein in transjugular intrahepatic portasystemic stent shunt (TIPSS)]. / Sonographisch gesteuerte Punktion der Pfortader beim transjugulären intrahepatischen portosystemischen Stentshunt (TIPSS).

Successful completion of a transjugular intrahepatic portosystemic stentshunt (TIPSS) relies on knowledge of the individual hepatic vascular anatomy. The patients referred for TIPSS have an increased risk of contrast-medium-induced renal failure, and therefore the potential for a complete angiographic work-up during the procedure is limited. The same patient population also carries an increased risk of bleeding, which necessitates a rather accurate guiding technique for portal punctures. We have established transcutaneous sonographic guidance as a standard technique for transjugular portal puncture, reducing complication rates and intervention time. Competing imaging modalities for guidance are discussed.