Klaus Haag

The Transjugular Intrahepatic Portosystemic Stent-Shunt Procedure for Variceal Bleeding

BACKGROUND Transjugular placement of an intrahepatic stent is a new technique to establish a portosystemic shunt for treatment of portal hypertension. A puncture needle is advanced in a catheter through the inferior vena cava into a hepatic vein; then an intrahepatic branch of the portal vein is punctured and an expandable stent of metallic mesh is implanted to establish the shunt. METHODS We attempted the stent-shunt procedure in 100 of 112 consecutive patients with variceal bleeding due to cirrhosis, who were then followed for a mean (+/- SD) of 12 +/- 6 months. Of the 100 patients, 22 had Child-Pugh class C cirrhosis, 10 were treated on an emergency basis, and 68 had alcoholic cirrhosis. The shunt was established with use of Palmaz stents expanded to 8 to 12 mm in diameter. RESULTS Technical success was achieved in 93 percent of the patients. The mean (+/- SD) time for the procedure was 1.2 +/- 0.3 hours. The shunt reduced the portal venous pressure gradient by 57 percent. Major complications were hemorrhage (intraabdominal bleeding in six patients, biliary bleeding in four, and bleeding in the liver capsule in three) and migration of the stent into the pulmonary artery (in two patients). At follow-up, stenosis of the shunt was evident in 21 patients and occlusion in 10 patients; 10 of these 31 patients had variceal rebleeding. Stenoses and occlusions of the shunt were all treated successfully by redilation, thrombolysis, or implantation of an additional stent. Hepatic encephalopathy (stages I to III) developed in 25 percent of the patients. The proportion of patients with shunts who remained free of variceal rebleeding was 92 percent at six months and 82 percent at one year. The 30-day mortality was 3 percent. The cumulative one-year survival was 85 percent. CONCLUSIONS These results suggest that the transjugular placement of an intrahepatic portosystemic stent is an effective and safe treatment for variceal hemorrhage in patients with portal hypertension due to cirrhosis.

The transjugular intrahepatic portosystemic stent - shunt procedure for refractory ascites

BACKGROUND Previous studies have suggested that the transjugular placement of an intrahepatic stent to establish a portosystemic shunt is an effective treatment of uncomplicated ascites accompanying variceal bleeding. We studied the stent shunt for use in patients with liver cirrhosis and ascites refractory to medical treatment. METHODS Fifty of 62 consecutive patients with cirrhosis and refractory ascites (18 with Child-Pugh class B liver disease and 32 with class C) were treated with the stent shunt--an expandable stent of metallic mesh placed between a major branch of the portal vein and one of the hepatic veins. Patients were followed for a mean (+/- SD) of 426 +/- 333 days. Those with advanced cancer, severe heart failure, or severe liver failure were excluded. RESULTS The stent shunt was successfully placed in all patients and reduced the pressure gradient between the portal vein and the inferior vena cava by an average of 63 percent. Thirty-seven patients (74 percent) had complete responses (total remission of ascites within three months), and nine patients (18 percent) had partial responses (ascites detected by ultrasound but with no need for paracentesis). Four patients did not respond, including two who died within two weeks of shunt placement. After the procedure, 25 patients had hepatic encephalopathy, as compared with 20 patients before the procedure; although encephalopathy improved in 3 patients, new encephalopathy developed in 8 patients. In the 28 of the 33 patients followed for more than six months who were evaluated, the mean serum creatinine concentration was 1.5 +/- 0.09 mg per deciliter (133 +/- 8 mumol per liter) before placement of the stent shunt, 1.5 +/- 1.6 mg per deciliter (133 +/- 141 mumol per liter) one week after the procedure, and 0.9 +/- 0.3 mg per deciliter (80 +/- 27 mumol per liter) after six months (P = 0.008 for the comparison of concentrations before and six months after the procedure). Renal function did not improve in the six patients with organic kidney disease. Procedure-related complications developed in 16 patients, including intraabdominal bleeding requiring blood transfusions in 2 patients. Thrombotic occlusion of the stent shunt occurred within two weeks in 5 patients, and later insufficiency of the shunt occurred in 16 patients, including 12 with recurrence of ascites after complete remission. During followup, an additional 29 patients died--10 of progressive liver disease and 19 of other causes. Survival for at least one year was associated with a patients being under 60 years of age, having a serum bilirubin level before placement of the stent shunt of less than 1.3 mg per deciliter (22 mumol per liter), and having a complete response. CONCLUSIONS Our findings in an uncontrolled prospective study suggest that the transjugular intrahepatic porto-systemic stent-shunt procedure was an effective treatment for many patients with liver cirrhosis and refractory ascites, but mortality from underlying diseases was substantial.

Pancreatic lesions in the von Hippel-Lindau syndrome

Common manifestations of the von Hippel-Lindau syndrome, an autosomally dominant inherited cancer-prone disorder, include retinal angiomatosis, hemangioblastoma of the central nervous system, renal cysts, renal cancer, pheochromocytoma, and epididymal cystadenoma. Multiple cysts and microcystic (serous) cystadenomas of the pancreas have also been reported occasionally in patients afflicted with this syndrome. In the large Freiburg study of the von Hippel-Lindau syndrome composed of 66 affected individuals, pancreatic lesions were systematically studied. Fifty-five living individuals were examined by abdominal ultrasound imaging. Abnormal findings were confirmed by computed tomographic scan and/or magnetic resonance imaging. For an additional 11 decreased patients autopsy data were available. Cystic lesions of the pancreas were found in 10 patients (15%). One of these patients presented with multiple pancreatic cysts as the only manifestation of the syndrome. In one patient, a malignant islet-cell tumor was found at autopsy. Because multiple pancreatic cysts did not cause major clinical symptoms and because follow-up examinations over an average period of 5 years did not show significant progression of the lesions, it is concluded that these patients usually do not require surgical treatment. Abdominal ultrasound screening is recommended for patients at risk as a tool to identify potential von Hippel-Lindau syndrome gene carriers with pancreatic manifestations. In all patients with multiple pancreatic cysts, the von Hippel-Lindau syndrome should be included in the differential diagnosis.

Transjugular intrahepatic portosystemic stent-shunt (TIPS) in the treatment of Budd-Chiari syndrome

Budd-Chiari syndrome is characterized by splanchnic congestion due to obstruction of the hepatic venous outflow. A variety of treatment modalities have limited applicability due to their invasive nature, complications or low effectivity. The transjugular intrahepatic portosystemic stent-shunt (TIPS) offers a new treatment by creating an intraparenchymal duct between a main branch of the portal vein and hepatic vein i.e. the intrahepatic part of the inferior vena cava. This paper describes the treatment of two patients with fulminant and subacute Budd-Chiari syndrome treated 2 days and 2 months after the onset of clinical symptoms. It demonstrates that TIPS is a feasible treatment of Budd-Chiari syndrome that restores splanchnic blood flow, reduces collateral circulation and ascites and provides sufficient time to allow for elective liver transplantation, if indicated. Further studies are required to evaluate the effect of TIPS on liver function and survival.

How much reduction in portal pressure is necessary to prevent variceal rebleeding? A longitudinal study in 225 patients with transjugular intrahepatic portosystemic shunts.

OBJECTIVES:This longitudinal study determines the risk of rebleeding in relation to the reduction of the portosystemic pressure gradient in patients with a transjugular intrahepatic portosystemic shunt (TIPS) for variceal bleeding.METHODS:The study included 225 patients in whom a TIPS revision was indicated by the endoscopic finding of varices with a high risk for rebleeding (n = 167) or a recent variceal rebleed (n = 58). The portosystemic pressure gradient was determined before and after TIPS placement and at revision performed after a mean of 10 ± 15 months.RESULTS:The portosystemic pressure gradient at revision approached the index pressure gradient before TIPS implantation (23.1 ± 5.5 mm Hg) by 8.4 ± 31%. Rebleeding was inversely correlated with the reduction in index pressure gradient found at revision. Thus, 80% of rebleedings occurred with pressure gradients close to the index pressure gradient (<25% reduction) or with gradients equal to or greater than the index pressure gradient. In contrast, only one patient (0.4%) and three patients (1.3%) rebled with a pressure gradient of <12 mm Hg or a reduction of the index pressure gradient by >50%, respectively. Kaplan-Meier analysis of rebleeding, which included the 225 patients at risk, showed a probability of rebleeding of 18%, 7%, and 1% for a reduction of the index pressure gradient by 0%, 25–50%, and >50%, respectively.CONCLUSIONS:Most rebleedings occurred with pressure gradients similar to the index-pressure gradient measured at first bleeding. Accordingly, a graded reduction by 25–50% sufficiently prevents rebleeding. It can be assumed that, in comparison with the widely used threshold value of 12 mm Hg, a reduction by 25–50% may have a favorable benefit-to-risk ratio with respect to shunt-induced hepatic encephalopathy and liver failure. It should therefore be a goal in the decompressive treatment of portal hypertension and maintained during follow-up of patients with variceal bleeding.

Acute hemoperitoneum after large-volume paracentesis.

Hemoperitoneum resulting from rupture of mesenteric varices is a rare complication of portal hypertension with a high mortality of up to 70%. This case report describes the symptoms, clinical course, and treatment of 4 patients with acute hemoperitoneum caused by mesenteric variceal bleeding after large-volume paracentesis. Abdominal pain and/or hemorrhagic shock developed in 4 patients (age, 48-68 years), admitted for refractory ascites, 3 hours to 4 days after 1-4 large-volume paracenteses (> 4000 mL). Duplex sonography, performed in 3 of the 4 patients before onset of bleeding, showed retrograde flow in the mesenteric veins, suggesting large-caliber mesenteric collateralization. Treatment consisted of surgical ligation followed by transjugular intrahepatic portosystemic shunt (TIPS) (2 patients) and emergency TIPS with embolization of the bleeding vessel (1 patient). One patient died before any intervention could be initiated. In these 4 patients, the concurrence of large-volume paracentesis and hemoperitoneum suggests their causal relationship. The mechanism may be a sudden reduction in intraperitoneal pressure increasing the pressure gradient across the wall of the mesenteric varices, resulting in rupture and bleeding. The awareness of this complication may accelerate the diagnostic process and treatment.

Morphologic and clinical results of the transjugular intrahepatic portosystemic stent-shunt (TIPSS)

The concept of transjugular intrahepatic portosystemic stent-assisted shunt (TIPSS) using the Palmaz iliac stent has been successfully accomplished in 18 of 24 patients representing a technical success rate of 75%. Fourteen were male, 4 female with a mean age of 60 years (range 34–84 years). According to classification of Child’s and Turcotte, 6 were in stage A, 6 in stage B, and 6 in stage C. Five patients were treated on an emergency basis because of massive active bleeding. In 10 patients the portosystemic tract was created between the middle hepatic vein and the right main stem of the portal vein in 8, and the left main stem in 2 patients. In 8 patients, the shunt was established between the right hepatic vein and the right main branch of the portal vein. The portosystemic gradient in 18 patients was 29.9±6 mm Hg and dropped to an average of 16.9±4 mm Hg after shunt establishment. Within the early postprocedural period of 30 days, 1 patient died of direct complications of the procedure. Because of catheter dislocation, embolization of the percutaneous transhepatic approach to the portal vein after successful shunt “creation” could not be done and was followed by intraabdominal exsanguination. One patient died of an ARDS after TIPSS. A third developed pulmonary infection. In 13 patients, because of hematomas at the puncture site of the transhepatic approach, only the transjugular approach was elected for establishing TIPSS. The mean portosystemic gradient in 18 patients prior to TIPSS was 29±6 mm Hg (range 19–41 mm Hg), dropped to an average of 16.9±4 mm Hg (range 7–21 mm Hg), and showed no significant change 6 months after TIPSS with a pressure of 16±1.8 mm Hg. The 1-year survival rate was 75% (8/12); the 2-year rate was 50% (3/6).

The transjugular intrahepatic portosystemic stent-shunt: A review of the literature and own experiences

The transjugular intrahepatic portosystemic stent‐shunt (TIPS) technique consists of a transhepatic puncture of the portal vein and stenting of the parenchymal tract between the hepatic and portal veins. Complications of both puncture and stenting are observed in approximately 5% of procedures. Most of the complications are without clinical consequences and the procedural mortality is very low in experienced hands (1%). During a 1 year follow up, 35% of patients were seen to develop stenosis and 15% developed occlusion of the stent‐shunt. However, in spite of the considerable incidence of stenosis/occlusion, the rate of variceal rebleeding is rare when patients are followed up carefully by duplex sonography, which allows accurate and early detection of shunt insufficiency. One of the major long‐term clinical problems of TIPS is the induction or worsening of hepatic encephalopathy. Although most patients respond to medical treatment, some develop debilitating encephalopathy or progressive liver failure. In these patients, reduction of shunt flow by the implantation of a reducing stent, or its occlusion with a balloon catheter, may be indicated. In conclusion, in spite of many complications, TIPS is relatively safe and efficient and hepatic encephalopathy is manageable in most cases.

Transjugular Intrahepatic Portosystemic Shunt (Tips) Thrombogenicity in stents and its effect on shunt patency

Purpose: To compare the thrombogenicity and patency of the Palmaz stent and the Wallstent, and to evaluate the effect of periprocedural heparin therapy in cirrhotic patients with maintained coagulation capacity who receive a transjugular intrahepatic portosystemic shunt (TIPS). Material and Methods: Twenty-four patients were randomized into 4 groups of 6 patients. Each received a Palmaz-stent or Wallstent TIPS with or without periprocedural heparin therapy. The groups receiving periprocedural heparin were given 24 U/ kg b.w. just before stent placement, followed by 24 h therapeutic i.v. heparin. After 24 hours, all patients received i.v. heparin for 1 week followed by subcutaneous treatment with low-molecular-weight heparin (0.3 ml/day) for another 4 weeks. Stent thrombogenicity was determined scintigraphically after i.v. injection of 120-290 mBq of 99mTc-labeled platelets at the time of stent placement and expressed as the stent/heart ratio. Shunt patency was assessed by duplex sonography and confirmed radiologically. Results: The aggregation ratio was highest 90 min after stent implantation. Wall-stents showed a significantly higher ratio than Palmaz stents. Heparin reduced the ratio in patients with a Wallstent (-41%) but had no effect on Palmaz stents. Patients with a Wallstent without heparin had a higher rate of early shunt insufficiency (66.6%) than the other patients (0-16.6%). Primary assisted long-term patency was similar in the 4 groups. Conclusion: Wallstents were more thrombogenic than Palmaz stents and gave a significantly higher risk of early shunt insufficiency in cirrhotic patients with maintained coagulation capacity. Periprocedural heparin was effective in the prevention of shunt insufficiency and is therefore indicated in such patients.

Late Manifestation of Autosomal-Recessive Polycystic Kidney Disease in Two Sisters

We report on 2 siblings with autosomal-recessive polycystic kidney disease, diagnosed at the ages of 14 and 18 years, respectively. Clinical findings and differential diagnosis, especially for autosomal-dominant polycystic kidney disease, are given. The consequences for genetic counselling are discussed.