Role of transjugular intrahepatic portosystemic shunt in the treatment of portal hypertension in pediatric patients.

Abstract

The transjugular intrahepatic portosystemic shunt (TIPS) procedure has evolved over the past 10 years to become an established therapy for the treatment of portal hypertension and its complications. Since the first attempt at placement of a TIPS in 1982 (1), advances in technology have allowed extension of the procedure into smaller patients who have more complications. Knowledge of its technical aspects is essential in the process of applying the TIPS procedure in clinical practice. This article reviews the current state of the art of the TIPS procedure, focusing on the technique itself and consequent clinical considerations, with special reference to its role in the treatment of portal hypertension in pediatric patients. CONSIDERATIONS IN THE TREATMENT OF PORTAL HYPERTENSION: WHEN IS THE TIPS PROCEDURE INDICATED? Increased portal pressure can result from prehepatic, intrahepatic, or portal system disease. The two major complications of portal hypertension are bleeding from varices and ascites. Varices develop when pressure is increased anywhere within this system. In contrast, ascites develops when resistance to hepatic flow originates from within the hepatic sinusoids. Decompression of portal hypertension does not always result in reduction of intrahepatic blood flow. Thus, although the risk of variceal bleeding can be effectively reduced by any shunt procedure that decreases portal pressure, ascites is only ameliorated by shunts that relieve sinusoidal obstruction. Examples of shunts that do not effectively treat ascites include the portacaval end-to-side shunt and the distal splenorenal shunt. Side-to-side shunts that maintain continuity of the intrahepatic and portal venous circulation help to decompress the sinusoids and include the mesocaval shunt, the portacaval H-graft shunt, and the TIPS (2). The tradeoff is increased risk for hepatic encephalopathy. Liver transplantation is the ultimate treatment when alternatives are ineffective. Indications and contraindications for the TIPS procedure are listed in Tables 1 and 2. In general, TIPS placement is indicated when portal decompression is necessary to treat complications of portal hypertension. The most frequent indication is uncontrolled bleeding from gastroesophageal varices.TABLE 1: Indications for TIPS placementTABLE 2: Contraindications to TIPSThe role of TIPS in the control of esophageal variceal bleeding is controversial. The therapy for variceal hemorrhage is outlined in Table 3. Medical management for acute hemorrhage begins with endoscopic control of the hemorrhage, either with sclerotherapy or band ligation. Pharmacologic therapy with vasoactive drugs is begun concomitantly, using either vasopressin, vasopressin plus nitroglycerin, somatostatin, octreotide, or terlipressin. A TIPS is an effective therapy for patients with acute bleeding unresponsive or not amenable (e.g., nonesophageal variceal bleeding) to endoscopic therapy or pharmacotherapy (3). Although several studies document the efficacy of TIPS compared with sclerotherapy or band ligation in adults (4), there are still no studies comparing TIPS with surgical shunts. A preliminary and controversial report suggests that in adults small-bore prosthetic H-graft portacaval shunts are safer and more successful than TIPS (5). A National Institutes of Health-sponsored study is underway to compare TIPS and the splenorenal shunt. For prevention of recurrent bleeding, therapy is initiated with endoscopic and/or pharmacologic methods. For patients unresponsive to medical management, remaining options are shunts or liver transplantation. Studies show that TIPSs, like surgical shunts, yield better results compared with sclerotherapy or banding (less rebleeding and thus fewer hospital admissions and similar rates of survival), although a TIPS (again, similar to surgical shunts) is also associated with a higher incidence (about twofold) of hepatic encephalopathy, which is treatable with medical regimens. A TIPS avoids the local complications (esophageal ulceration and stricture) of endoscopic procedures.TABLE 3: Control of variceal hemorrhageThe advantage of the TIPS procedure compared with surgical shunts is the lower procedural morbidity and mortality (noted earlier) compared with surgical procedures. Quality-of-life studies have not been published, but reports of comparison of costs per patient or per day survived indicate that TIPS is at least equivalent to if not less expensive than endoscopic methods (4,6). At this time, TIPS appears to be best reserved for those patients who are unresponsive to endoscopic therapy (bleeding that persists or that recurs despite endoscopic therapy) or who are at high risk for recurrent variceal hemorrhage and are awaiting a donor for orthotopic liver transplantation. Evidence is inadequate to support a prophylactic role for endoscopic and shunt procedures to prevent a first variceal bleed (3,7). Ascites unresponsive to medical management is the other major complication of portal hypertension that can be treated effectively with a TIPS (Fig. 1). However, many patients, particularly children, respond to standard therapy and should not undergo more invasive alternatives until fluid and salt management becomes ineffective or is impeded by complications of the therapy. Large-volume paracentesis with colloid replacement may be effective for acute relief, but chronic implementation results in significant protein depletion and hypovolemia and does not resolve the ascites. A peritoneovenous shunt can provide temporary reduction of ascites but often, especially in children, becomes occluded, infected, or leads to coagulopathy (8). Studies are needed to determine which therapy has a better outcome. A TIPS has the capability to diminish or in many cases resolve ascites because of its effectiveness in reducing intrahepatic sinusoidal pressure and hepatic lymph production (9). Urinary sodium excretion, free water clearance, and the glomerular filtration rate are increased after a TIPS is placed (10). A TIPS has also been reported to be beneficial in patients with hydrothorax, an occasional complication of ascites (11). If symptoms or signs of ascites return after TIPS placement, TIPS stenosis or occlusion should be suspected. Additionally, patients with ascites and some renal insufficiency appear to be at higher risk (up to 15%) for contrast-induced renal failure after TIPS placement. Overall, TIPS appears to be indicated for patients with ascites who are unresponsive to medical management, often requiring frequent large-volume paracentesis or patients awaiting liver transplantation.FIG. 1: Treatment of ascites.WHAT IS THE TIPS PROCEDURE? The goal of the TIPS procedure is the creation of a conduit for blood to flow freely from the portal vein to the hepatic vein (Fig. 2), thus decompressing the portal venous system. Although the technique may differ among various centers, the basic principles applied by the interventional radiologists are similar.FIG. 2: Graphic illustration of placement of a transjugular intrahepatic portosystemic shunt (TIPS). The Wallstent (Schneider) extends from the hepatic vein (HV) to the portal vein (PV). Reprinted, with permission, from Heyman MB, LaBerge JM, Somberg KA, et al. Transjugular intrahepatic portosystemic shunts (TIPS) in children.J Pediatr 1997;131:914-9 .Vascular Considerations Application of this technique requires a thorough knowledge of the vascular anatomy of the hepatoportal vasculature (Fig. 3). In adults, the three main hepatic veins flow into the inferior vena cava about 1 cm below the diaphragm and 2 cm below the right atrium. Other, primarily smaller, veins also drain into the lower intrahepatic vena cava from the right and caudate lobes of the liver (12). The right hepatic vein, most commonly cannulated for TIPS, is approximately 1 cm in diameter in adults. The middle and left hepatic veins are smaller and have more anatomic variation than the right hepatic vein. About 30% of patients have variations in the course and number of hepatic veins, making the procedure more complicated. In particular, the hepatic veins may have multiple branches, accessory venous tributaries may be present, or, as is found in 3% of cases, an inferior right hepatic vein may be the dominant vein draining blood from the right lobe in place of a small or absent right hepatic vein.FIG. 3: Normal hepatic vascularity. (A) Superior hepatic veins. The right hepatic vein is the largest of the three superior hepatic veins. The middle hepatic vein forms a common trunk with the left hepatic vein before joining the inferior vena cava. (B) Portal veins. The main right portal vein divides into anterior and posterior segmental branches. The transverse segment of the left portal vein gives off branches to the lateral and then medial segments of the left lobe. (C) Normal relationship of the hepatic and portal veins. Note that the peripheral right hepatic vein divides the anterior and posterior branches of the right portal vein. The peripheral middle hepatic vein is ventral to the middle portal vein. Reprinted, with permission, from LaBerge JM. Anatomy relevant to TIPS. Tech Vasc Interv Radiol 1998;1:51-67 .44The main portal vein (approximately 8-10 mm in diameter in adults) originates at the juncture of the superior mesenteric and splenic veins and enters the liver at the porta hepatis, where it divides into right and left portal vessels. Within the liver, the right portal vein bifurcates into anterior and posterior segments. Anatomy is altered by variability of these vessels, seen in approximately 20% of patients, and by pathologic situations, particularly thrombosis. Patients with cirrhosis or other liver diseases are at increased risk for thrombosis of the portal veins. The radiologist must understand the complex anatomic relationships of the hepatic and portal venous systems. If the right hepatic vein is used, the portal vein lies to the left and ventral to the needle. In contrast, if the middle hepatic vein must be used as an alternative, the portal vein lies dorsal to the needle (Fig. 3). A limiting aspect of the procedure is the narrowest diameter of the veins in the course of the shunt and often relates to the size of the balloon used to dilate the intrahepatic portion of the tract. Procedural Considerations Preoperative assessment is essential to confirm a diagnosis (Table 4). Because uncontrolled upper gastrointestinal bleeding due to variceal hemorrhage is the most common indication, upper endoscopy must be performed to exclude other causes of bleeding that would indicate an alternate therapeutic approach. Abdominal sonography including Doppler imaging of the portal and hepatic vessels is essential to determining the size and patency of the portal veins, patency of hepatic veins, presence or absence of hepatic lesions, hepatic size and orientation, and the amount and location of ascites, if present. Transarterial portography is only necessary when the ultrasonographer is uncertain of portal vein patency. Before TIPS placement, laboratory assessment of hemodynamic and coagulation status and baseline chemistries must be documented. Some centers perform paracentesis for tense ascites before TIPS placement.TABLE 4: Preprocedure assessmentAt the time of the procedure, a prophylactic antibiotic (e.g., cefazolin or vancomycin) is routinely administered. Any coagulopathies and thrombocytopenia should be corrected as indicated. The TIPS procedure can be performed in adults with conscious sedation alone. In children, general anesthesia has been used to provide adequate sedation and analgesia. The procedure is initiated with the introduction of a guide wire from the right internal jugular vein into the inferior vena cava (13). A long, curved needle is then passed over the guide wire into the hepatic vein. The selection of the hepatic vein is critical in determining the next step, because the direction of the needle is influenced by the choice of the hepatic vein that is cannulated. To visualize the portal vein's position, a wedged hepatic venogram is obtained. This procedure is now facilitated by the use of CO2 and digital subtraction images (14). After identifying the appropriate venous structures, a needle system to puncture the hepatic-to-portal vein is inserted over a guide wire. Two needles are currently available: the Colapinto needle, a 16-gauge metal needle with a 9-French outer diameter, and the Rosch-Uchida system, which is more flexible and involves a 14-gauge, 10-French outer diameter, metal cannula (15). The needle is advanced through the liver parenchyma into a branch of the portal vein. The outer sheath is advanced far into the portal vein, the needle is withdrawn, and a 5-French catheter is advanced over the guide wire into the spleen. Portal pressures and a portal venogram are obtained. An angioplasty balloon catheter is then used to dilate the resultant hepatic vein-to-portal vein tract, including the portal vein entry site and the hepatic vein exit site (to reduce the risk of stricture at these points). An expandable metallic stent (Wallstent, Schneider [U.S.A.], Inc., Plymouth, MN) is deployed across the tract to support the shunt channel (Fig. 4). The stent should not extend to the splenic vein-superior mesenteric vein confluence or proximally to the right atrium, because this may interfere with the subsequent feasibility of liver transplantation. Portal venography is then performed to assess the extent of portal decompression. If residual varices or portal hypertension (portosystemic pressure gradient ≥12 mm Hg) are identified, the shunt is dilated with a larger balloon, another parallel shunt may be placed, and/or the residual varices are embolized with coils.FIG. 4: Venogram on completion of transjugular intrahepatic portosystemic shunt (TIPS) with Wallstent (Schneider) shunt deployed appropriately. Injection of contrast in the splenic vein shows flow through the TIPS (arrow). Persistent hepatopetal flow is also noted in the right and left intrahepatic portal veins (arrowheads).After placement of the TIPS, the patient is observed overnight for the development of complications, and shunt patency is documented the following morning by Doppler sonography. Pediatric Modifications The diminutive size of the liver and venous structures requires special consideration for placement of TIPS in pediatric patients. Additionally, the amount of contrast that can be injected is limited compared with that used in adults. After placement, growth will certainly alter the long-term outcome of an indwelling shunt. Data regarding this issue are not available. In addition to the use of general anesthesia, several technical modifications have been introduced to facilitate TIPS placement in children. The transjugular needle has been modified. At our center, a 55-cm, 16-gauge (1.6 mm, 5 French) Colapinto needle (Ring TIPS set, Cook Inc., Bloomington, IN, U.S.A.) is used in adults. A similar but smaller needle that is 40 cm long and 18 gauge (1.3 mm, 4.1 French) has been designed for use in children. The modified needle can be introduced through a long 7-French sheath. Of note, however, is that the larger, adult-sized Colapinto needle set is preferable because of its superior torque control. In adults, a 68-mm Wallstent is used to line the shunt from the hepatic vein to the portal vein. In children, because of the smaller size of the liver, a shorter, 42-mm Wallstent has been used. In adults, the shunt tract is dilated with an angioplasty balloon to a diameter ranging from 8 to 12 mm, and then a metallic stent with a maximal unconstrained diameter of 10 or 12 mm is inserted. For children, balloon diameters of 6 to 10 mm are often selected. Wallstents with a maximal unconstrained diameter of 10 mm were used in initial pediatric patients. New, smaller stent (8-mm diameter, 40-mm length) subsequently became available and have been used more recently (16). Special Problematic Vascular Situations The TIPS procedure may be modified for patients with various underlying disorders that alter the vascular anatomy. In the setting of a Budd-Chiari syndrome, when the superior hepatic veins are occluded, the intrahepatic portion of the inferior vena cava may be used to accomplish a successful TIPS. The length of the intrahepatic portion of the inferior vena cava may approximate 5 to 6 cm in adults but is not precisely known in normal or cirrhotic adults or in children. A percutaneous transhepatic approach can be used to place a guide wire into the inferior vena cava, where it can be snared by instruments placed through a jugular approach. An angioplasty balloon can then be used to dilate a tract in the occluded venous segment, and a TIPS can then be placed (15). Although a relative contraindication, portal vein thrombosis is sometimes amenable to placement of a TIPS. In an acute setting, placing the TIPS is not difficult if the thrombosis is nonocclusive. Fibrinolytic therapy can be used in conjunction with TIPS placement (17). However, if the portal vein is completely occluded, successful TIPS placement is uncommon, and in this setting, a surgical shunt is preferable. If the confluence of the superior mesenteric vein and splenic is involved, the success of either procedure is low. WHAT COMPLICATIONS ARE ASSOCIATED WITH THE TIPS PROCEDURE? Complications are an inherent risk for any shunt procedure (Table 5). However, in adults, the risks associated with TIPS placement are significantly lower than the comparable side-to-side portacaval surgical shunt. The 30-day mortality risk of surgical shunts performed emergently ranges from 40% to 100% and electively from 4% to 20%. The overall risk for a TIPS procedure is reported to be 7% to 45%. Procedure-related mortality is low (<2%) (18). Morbidity and mortality associated with the TIPS procedure are related to the severity of the illness in patients, with most deaths occurring in those most critically ill.TABLE 5: Complications associated with the TIPS procedureThe principle risks of TIPS are shunt occlusion and stenoses and increased encephalopathy. Exclusive of these, the overall risk of complications associated with the TIPS procedure is less than 10% (18). Stenosis remains a major complication of TIPS, with estimates varying from 25% to 75%, depending on the definition of the problem (19-21). Patency also declines with the length of time the shunt is in place. The stenosis is most commonly caused by intimal hyperplasia, although occasionally a technical error such as leaving a portion of the parenchymal tract unstented is the cause (22). Monitoring the patency of the TIPS is most readily performed by ultrasound technique. Sonography is actually useful at several stages of treating with a TIPS. Initially, it can be used to screen patients for intrahepatic lesions and vascular abnormalities that will affect the placement of the TIPS. For examples, alternative approaches to the patient is necessary if the ultrasonographer uncovers an unsuspected liver mass, hepatic vein thrombosis, anomalous connections to the inferior vena cava, or a portal vein thrombosis. After TIPS placement, periodic sonography every 3 to 6 months is used to monitor shunt patency and to determine the presence of an occlusion or stenosis. Surveillance for shunt obstruction is important, because its first clinical manifestation may be life-threatening variceal hemorrhage (21). If an obstruction is found, treatment can usually be successfully instituted by either dilating the stent or by restenting. Encephalopathy after TIPS placement is reported to range from 5% to 35%, similar to the rate with surgical side-to-side portacaval shunts, and is most common among patients who have encephalopathy before TIPS. Patients usually respond to simple methods, including protein restriction and lactulose. Fewer than 5% have refractory encephalopathy after TIPS placement. This complication is minimized by keeping the shunt size as small as possible, allowing decompression of the portal venous system without allowing too much shunting of blood flow from the liver. Rarely (3-7%), worsening liver failure develops because of inadequate blood flow to the liver (23). Patients at highest risk of development of liver failure have advanced liver disease with a prothrombin time of more than 17.5 seconds (24) and have progressive jaundice, coagulopathy, and encephalopathy. Intentionally occluding the TIPS or treating iatrogenically induced stenosis of the celiac or hepatic artery with angioplasty may be required. Other consequences of the altered circulation are also rare and include pulmonary hypertension, pulmonary edema, and congestive cardiac failure. Acquiring venous access is relatively safe, although a hematoma rarely develops. Care to assure adequate coagulation parameters before the procedure will help to minimize this risk. Tracheal puncture has been reported in a pediatric patient (18). Cardiac arrhythmias may be precipitated by buckling of the catheter as it passes through the right atrium. All structures in the path of the needle used to create the TIPS are at risk for injury. Extracapsular puncture of the portal vein can lead to serious intraabdominal bleeding. Extending the needle in the anterior direction to cannulate the portal vein from the middle hepatic artery is a common error leading to perforation of the hepatic capsule and intraperitoneal bleeding. Because of the close relationship of the bile ducts and hepatic artery to the portal vein, either may be injured by the needle during TIPS placement. A necropsy study revealed that almost half of the livers studied had a bile duct or artery in the path between the right hepatic and portal veins (25). An interesting observation in the same study was that a bile duct or artery was in the path of a needle from the middle hepatic vein to left portal vein in only 4% (1 of 25) of cases. Other complications include puncture of the right kidney, the hepatic flexure of the colon or the gallbladder (leading to bile peritonitis). Passage of the needle through the hepatic capsule is estimated to happen in approximately 30% of cases (18). With adequate coagulation parameters, this corrects itself and does not require blood products or surgical intervention. The risk of this adverse event diminishes with the increasing experience of the operator. Hemorrhage from inadvertent injury to the inferior vena cava or the portal vein in the process of canulating these vessels can lead to life-threatening bleeding. For example, an important consideration is the use of the bifurcation of the portal vein into the right and left portal veins. Although this may appear to be a preferable target, the bifurcation is in an extrahepatic position in 40% to 50% of patients studied (25,26). The risk of intraperitoneal bleeding caused by puncture of extrahepatic veins may be minimized by fibrous peritoneal tissue in this area. Injury to the hepatic artery is usually not serious, although significant hemorrhage has been reported (27). Extension of the TIPS stent above the diaphragm into the superior vena cava can lead to serious complications during stent implantation subsequently and particularly during liver transplantation when the stent is removed and the vessels reanastomosed. A death has been reported caused by right atrial rupture (28). Rarely, the actual placement of the stent or its subsequent occlusion may lead directly to formation of a thrombosis of the portal system. This may render liver transplantation no longer feasible. Hemolysis has been reported in as many as 10% of patients after TIPS placement and is usually self-resolving (within 12-15 weeks). The hemolysis is possibly a result of microtrauma from the wire mesh of the stent (29). Procedure-related complications also include reactions to the contrast media, including allergic reactions and contrast-related nephropathy. Patients may also be exposed to significant radiation, although this can be minimized with increased operator experience and use of pulsed fluoroscopy. WHAT IS THE OUTCOME FOR PATIENTS WHO HAVE UNDERGONE THE TIPS PROCEDURE? By the end of 1995, it was estimated that more than 2000 patients had been treated with a TIPS (30). With resolution of many technical difficulties that were encountered as the technique evolved, the TIPS procedure can be successfully completed in most (>95%) patients. However, long-term problems remain to be solved. Approximately 30% of patients experience new-onset or worsening of encephalopathy after TIPS placement (31,32). This problem is usually controlled with protein restriction and lactulose, although in approximately 5% of patients, further intervention may be necessary. Options include partial or complete occlusion of the shunt, although recurrence of the portal hypertension and its complications is problematic. The risk of bleeding from varices is minimal when the hepatic venous pressure gradient is less than 12 mmHg. However, a major drawback with the TIPS procedure is the high rate of stenosis or occlusion, resulting in a significant risk for rebleeding. Large studies report a rebleeding rate after TIPS placement in the range of 7% to 31%. With careful screening and monitoring of the portal pressures, possibly by angiographic assessment at 6-month intervals (33), this risk may be minimized. WHAT IS THE PEDIATRIC EXPERIENCE WITH THE TIPS PROCEDURE? Table 6 summarizes the current experience with TIPS reported in pediatric patients. Although only approximately 40 pediatric patients have been reported to date (in contrast to more than 2000 adults), the data suggest that success in pediatric patients is comparable with that in adults, ranging from 75% to 90% (16,34-36). As in adult patients, the TIPS procedure appears to be an efficacious treatment for children with variceal bleeding and intractable ascites and is most frequently used as a bridge to gain time before liver transplantation. Severe hypersplenism has also been treated with TIPS placement before liver transplantation, although TIPS as a therapy for this condition is controversial, because consistent improvement in cell and platelet counts remains to be demonstrated. It is also important to note that subsequent liver transplantation including some of its inherent and potential technical complications is not compromised by a TIPS, because the shunt is intrahepatic (37).TABLE 6A: Transjugular intrahepatic portosystemic shunts in pediatricsTABLE 6B: Results of transjugular intrahepatic portosystemic shunts in pediatricsHackworth et al., (34) reported 13 TIPS procedures in 12 pediatric patients without major complications or morbidity. Indications were similar to those in adults: 11 patients had TIPS placed for active or recurring variceal hemorrhage without recurrence after TIPS placement, and 2 had TIPS for massive ascites, also with subsequent clinical improvement. Postprocedural encephalopathy developed in one patient who was treated with lactulose until liver transplantation. Mean portal pressure declined from 20.4 to 8.6 mm Hg. Shunt stenosis was documented 186 days after placement in one patient with recurrence of ascites. The duration of TIPS placement ranged from 9 to 357 days. For the 10 patients who underwent liver transplantation, the median was 53 days (range, 9-127 days), and the 2 patients who did not undergo liver transplantation had the longest duration (301 and 357 days). Schweizer et al., (36) reported successful placement of TIPS in seven children (age range, 3-13 years) for bleeding esophageal varices. The TIPS prevented rebleeding in all patients. Splenic enlargement and hypersplenism decreased in five. One patient sustained an intrahepatic puncture without sequela. No long-term complications were reported. Heyman et al. (16) described results of 12 attempted TIPS placements in 9 patients (5-15 years), 7 with bleeding varices and 2 with hypersplenism with thrombocytopenia. A TIPS could not be placed in two because of vascular anomalies precluding creation of the hepatic-to-portal vein tract. One patient with congenital hepatic fibrosis has had a functioning TIPS for more than 2 years. In four patients, the shunt permitted time for an appropriate liver transplant donor to be found. Overall complication rates among the pediatric patients are also comparable with the adult data. Of note is that encephalopathy appears to be less problematic in children treated with TIPS compared with incidence in adults. The reason for this difference is unclear but could relate to altered and more favorable circulatory or central nervous system adaptation to the changes that are incurred by the procedure. Alternatively, encephalopathy in these patients may be underreported because of difficulty with its detection. A TIPS does not have a significant deleterious effect on liver function in pediatric patients. The size of the vascular structures and the liver itself predispose the smaller child in particular to technical complications, the most common being extrahepatic puncture. At least two of the reported patients had intraperitoneal bleeding from extrusion of the needle through the hepatic capsule (16,34). Pediatric disorders may have a greater chance for anomalous vascularity in the liver and in the connections to the systemic circulation, making the procedure in this age group more challenging and sometimes technically unfeasible (16). Shunt stenosis or occlusion continues to be vexing. Growth may inherently lead to potential problems in patients requiring prolonged portal decompression with a TIPS, because the shunt must extend into both the hepatic and portal veins to maintain patency. Shunts may have to be dilated to larger diameters as the pediatric patient grows. This issue awaits further study. In some patients, growth delay caused by certain liver disorders may be reversed by the TIPS, as noted in a case of a hepatic angiodysplasia (34). CONCLUSION The TIPS procedure is a valuable tool in the armamentarium for the treatment of portal hypertension complicated by variceal hemorrhage and possibly by intractable ascites. The TIPS functions as a viable side-to-side shunt with preliminary results that already show it to be a safe and effective means for treating these problems. Certainly, it functions as an excellent bridge before more definitive therapy, particularly while a child is waiting for a donor for liver transplantation. Although reports document prolonged success, long-term use is still controversial and is especially limited by the development of intimal deposits with subsequent stenosis or occlusion. Optimally, the TIPS should be performed in a setting where pediatric hepatologists, radiologists, and liver transplantation surgeons are available to provide a comprehensive treatment plan and to manage serious complications that may arise.