What are the technique, results, and outcomes of Transjugular Intrahepatic Portosystemic Shunt (TIPS) in pediatric patients?

Transjugular Intrahepatic Portosystemic Shunt (TIPS) in Pediatric Patients: Technique, Results, and Outcomes

TIPS is a technically feasible and clinically effective procedure in pediatric patients with portal hypertension, with technical success rates of 93-95% and clinical success rates of 93%, though it should be reserved for specific indications including recurrent portal hypertension-related bleeding despite standard prophylaxis or refractory ascites. 1

Indications

TIPS in pediatric patients should be considered in the following scenarios:

• Recurrence of portal hypertension-related bleeding despite standard prophylaxis (band ligation + beta-blockers) 1
• Refractory bleeding from portal hypertension 1
• Refractory ascites 1, 2
• As a bridge to liver transplantation 2

Unlike in adults, TIPS is generally not indicated in children with cirrhosis who don't have portal hypertension-related complications 1. It is also not recommended for hypersplenism alone 1.

Technical Considerations

Patient Selection

• Age and size: Successfully performed in children as young as 4 months with minimum weight of 6 kg 1
• Special attention to equipment selection for children <2 years or <10 kg 1

Anatomical Considerations

The main causes of TIPS placement failure in children include:

• Extrahepatic portal vein thrombosis with portal cavernoma 1
• Portal trunk hypoplasia 1
• Anatomical peculiarities (especially in syndromic biliary atresia with azygos continuation of the inferior vena cava) 1

Stent Selection and Placement

• Covered stents are recommended with 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 1
• The procedure must be performed by an expert interventional radiologist 1
• The goal is to achieve a portal pressure gradient <12 mmHg or a 50-60% decrease from initial pressure 3

Results and Outcomes

Technical and Clinical Success

• Technical success rates: 93-95% 1, 4, 5
• Hemodynamic success rates: 89-91% 4, 5
• Clinical success rates: 93% 1, 4
• Portosystemic gradient reduction: from average 21.5 mmHg pre-TIPS to 8.3 mmHg post-TIPS 4

Clinical Efficacy

• Resolution of variceal bleeding: 99.5% of cases 5
• Improvement of refractory ascites: 96% of cases 5
• Control of hard-to-control portal hypertension complications 2

Shunt Patency and Revision

• Primary patency rates: 84% 4
• Shunt revision rates: 27-45% 5, 6
• Secondary patency rates: nearly 100% after revision 4
• Surveillance with Doppler ultrasonography recommended at 3 months post-TIPS and every 6 months thereafter 3

Complications

• Hepatic encephalopathy: 10.6% of pediatric patients, with 85.7% resolving with medical management 5
• This rate is lower than observed in adults 1
• Post-TIPS bleeding rate: 14% 5
• No significant increase in ammonia levels reported in some studies 2

Long-term Outcomes

• 88% of patients were alive or successfully received liver transplantation 5
• TIPS can serve as an effective bridge to liver transplantation 2
• Some patients maintain long-term shunt patency without needing transplantation 7

Special Considerations

TIPS vs. Other Interventions

• For Budd-Chiari syndrome, TIPS should be considered in patients not responsive to medical therapy 1
• For non-cirrhotic portal hypertension, TIPS is an option when endoscopic control fails 1
• The meso-Rex bypass may be a better option if the child has an accessible intrahepatic left portal vein 1

TIPS in Specific Etiologies

TIPS has been successfully used in pediatric patients with various etiologies including:

• Biliary atresia
• Cryptogenic cirrhosis
• Portal or hepatic vein thrombosis
• Autosomal-recessive polycystic kidney disease
• Primary sclerosing cholangitis 6

Practical Recommendations

1. Thorough assessment of vascular anatomy before considering TIPS is crucial 1
2. TIPS insertion should be performed by an expert physician with experience in pediatric interventional radiology 1
3. The procedure and device must be adapted to each case 1
4. Concurrent obliteration of varices during TIPS creation is recommended 3
5. Regular surveillance is essential to detect and address shunt dysfunction 3

TIPS represents an important tool in the management of portal hypertension complications in selected pediatric patients, particularly as a bridge to liver transplantation or when conventional therapies fail.