Placement of Transjugular Intrahepatic Portosystemic Shunts in Children
Section snippets
Indications
Expert consensus has recommended acceptable indications for TIPS in adults5 but not in children. While causes for end-stage liver disease and portal hypertension in children undergoing TIPS differ from those of adults (Table 2), indications for TIPS in children are similar to adults with complications of portal hypertension. In children, TIPS has been employed to bypass sinusoidal2, 3, 4 and postsinusoidal7 obstruction of portal venous flow to alleviate symptoms of refractory ascites and
Preparation
In addition to the typical considerations of anatomical variations, indications, and contraindications considered before adult TIPS, many considerations should be emphasized before pediatric TIPS.
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In transplant candidates, discuss acceptable limits of stent extension with the transplant surgeon. Transplant surgeons have variable tolerance for stent encroachment into the main portal vein (Fig. 1), which is more difficult to control in children with short parenchymal tracts.
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Coordinate the timing
TIPS Set
In the early days of TIPS at The University of Chicago and other centers,3, 4 TIPS sets created for express use in pediatric patients were evaluated, and a hard lesson was learned. Cirrhotic livers in children are every bit as rock-hard as cirrhotic livers in adults. Smaller sheaths (<9 Fr) and smaller puncture systems (>16 G) seemed to increase the difficulty and length of the procedure. In particular, biliary atresia is the most common cause of cirrhosis in children and the most common
Tract Creation
Puncture of the portal vein is the most time-consuming portion of the procedure. If blind punctures fail after about 10-15 attempts, we consider maneuvers for visualizing the portal vein, all of which carry inherent risks.
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Transhepatic placement of a wire into the portal vein using sonographic guidance of a 21-G needle and placement of a 0.018-in. wire. At the University of Chicago, sonographically guided transhepatic portal venous and biliary interventions in children are common, fast, and
Stent Selection and Anticipation of Future Growth
Stent selection requires greater preprocedure planning for children compared to adults. Cross-sectional imaging is available in most children by the time TIPS is considered and provides valuable information regarding anticipated tract length, portal venous patency, and the diameter of the hepatic veins, portal veins, and inferior vena cava (IVC). Unlike adults for whom Viatorr stents (WL Gore, Flagstaff, AZ) are usually preferable and these anatomical parameters are relatively consistent,
Gradient Reduction
In earlier studies of pediatric patients,2 a post-TIPS portosystemic gradient of 15 mm Hg was targeted because the incidence of hepatic encephalopathy was unknown. Experience remains limited, but later reports suggest that the incidence is comparable to adult studies (20%-30%) when the upper threshold for a final gradient is set at 12 mm Hg.2, 3, 6 Moreover, most cases are medically treatable. Therefore, more recent studies tend to resolve the portosystemic gradient to below 12 mm Hg.
Clinical Follow-Up and Imaging Surveillance
We consider heparinization after pediatric TIPS for indications other than variceal bleeding, and for small diameter stents (8 mm or less). When bare stents are used for TIPS creation, diligent clinical and imaging surveillance is required to detect and treat early thrombosis and delayed stenosis. Ultrasound evaluation and clinical evaluation is performed 1 day, 1 week, 3 months, and then at 6-month intervals after TIPS. Recurrent symptoms or abnormal ultrasound findings should prompt
Post Transplantation
Before attempting TIPS in any transplant recipient, patency of the hepatic artery should be documented in addition to the portal vein. TIPS after hepatic artery thrombosis is a recipe for acute ischemic graft failure. TIPS in a left lateral segment transplant recipient is technically feasible but very challenging.2 Careful review of cross-sectional imaging before the procedure is a must to puncture medial enough to access the intrahepatic left portal vein and lateral enough to avoid puncture of
A Final Word of Caution
Despite excellent technical success rates reported, evidence that TIPS is more difficult in children is reflected in longer procedure times,3, 4 typically ranging from 2 to 6 hours and resulting from an increased difficulty puncturing small portal veins. In adults, the heavy-handed approach to TIPS creation—“muscling them in” with punctures into the peritoneum, gallbladder, and biliary system before accessing the portal vein—still results in high technical success and low complication rates. In
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Cited by (28)
Transjugular Intrahepatic Portosystemic Shunts in Pediatric Portal Hypertension: A Systematic Review and Meta-Analysis
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2022, Journal of HepatologyCitation Excerpt :Also, there is no evidence to show that it is superior to the conventional endoscopic technique of glue injection.123 In patients in whom pharmacotherapy and endotherapy have failed, transjugular intrahepatic portosystemic shunt (TIPS) should be considered to control variceal bleeding, especially in Budd-Chiari syndrome and congenital hepatic fibrosis.124–130 In patients with cirrhosis, TIPS usage could lead to encephalopathy.124
Transjugular intrahepatic portosystemic shunt placement in an infant weighing less than 22 pounds
2020, Diagnostic and Interventional ImagingPortal Hypertension
2020, Pediatric Gastrointestinal and Liver Disease, Sixth Edition