Guidelines for TIPS Placement in Pediatric Patients
TIPS should be considered in pediatric patients with recurrence of portal hypertension-related bleeding despite standard prophylaxis (band ligation + beta-blockers), refractory bleeding, or refractory ascites, but is not indicated in children with cirrhosis without portal hypertension-related complications. 1
Indications for TIPS in Pediatric Patients
TIPS placement in children should be considered in the following scenarios:
- Recurrent portal hypertension-related bleeding despite standard prophylaxis (band ligation + beta-blockers)
- Refractory bleeding from portal hypertension
- Refractory ascites 1, 2
TIPS is specifically not recommended for:
Technical Considerations and Patient Selection
Age and Size Requirements
- TIPS has been successfully performed in children as young as 4 months with a minimum weight of 6 kg 1
- Special attention to equipment selection is necessary for children <2 years or <10 kg 1, 2
Anatomical Considerations
The main causes of TIPS placement failure in children include:
- Extrahepatic portal vein thrombosis with portal cavernoma
- Portal trunk hypoplasia
- Anatomical peculiarities (especially in syndromic biliary atresia with azygos continuation of the inferior vena cava) 1
Pre-Procedure Assessment
- Thorough assessment of vascular anatomy using cross-sectional imaging is essential 1
- CT scan allows examination of vascular relationships and vessel patency 1
- Cardiovascular assessment should be performed to rule out severe left ventricular dysfunction or pulmonary hypertension 1
- Nutritional and functional assessment is recommended 1
Procedural Recommendations
Stent Selection and Placement
- Covered stents are strongly recommended with a diameter of 8-10 mm and average final dilation of 7-8 mm 1
- Covered stents are associated with significantly reduced risk of variceal bleeding recurrence compared to uncovered stents (p=.01) 1
Technical Goals
- The portal pressure gradient should be reduced to <12 mmHg or by ≥20% of baseline 1, 2
- The procedure must be performed by an expert interventional radiologist with experience in pediatric patients 1, 2
Procedural Preparation
- General anesthesia or deep sedation is recommended 1
- Prophylactic antibiotics are not routinely recommended except for TIPS for variceal bleeding, complex procedures, or where there is previous biliary instrumentation 1
- Coagulopathy correction should be based on thromboelastography rather than INR 1
- Platelet transfusion can be considered if platelet count <50×10^5/L 1
Outcomes and Follow-Up
Success Rates
- Technical feasibility: 93-95% of cases
- Hemodynamic success: 89% of cases
- Clinical success: 93% of cases 1, 2
- Resolution of bleeding related to portal hypertension: 99.5% of cases
- Improvement in ascites: 96% of cases 1
Complications
- Risk of hepatic encephalopathy appears lower in children than in adults 1
- Shunt dysfunction may occur and requires monitoring
Post-Procedure Monitoring
- Regular follow-up with Doppler ultrasonography is recommended at 3 months post-TIPS and every 6 months thereafter 2
- Nutritional status assessment (weight change, hand grip strength, and frailty assessment) should be performed at each follow-up visit 1
Special Considerations
Post-Liver Transplantation
- TIPS is feasible after liver transplantation but should be decided in a multidisciplinary approach with hepatologist, radiologist, and surgeons 1
- A complete assessment (including abdominal CT scan, cardiovascular examination, splanchnic hemodynamic study, and liver biopsy) is needed in case of portal hypertension relapse after liver transplantation 1
Technical Innovations
- Adjustable diameter TIPS may be considered in pediatric patients to accommodate future growth and prevent the need for additional procedures 3
TIPS placement in pediatric patients requires specialized expertise and should be performed at centers with experience in both pediatric liver disease and interventional radiology procedures.