Single-center experience of interventional therapy for congenital portal-systemic shunt in children

Xiangfeng Guo1, Qi Di1, Gang Shen1

  • 1Vascular Anomalies and Vascular Interventional Center, Capital Center for Children's Health, Capital Medical University, Capital Institute of Pediatrics, Beijing, China.

Insights

Interventional therapy for congenital portosystemic shunt (CPSS) in children is safe and effective, showing significant short-term benefits. This approach offers a promising first-line treatment option for pediatric CPSS patients.

Area of Science:

  • Pediatric Interventional Radiology
  • Vascular Surgery
  • Hepatology

Background:

  • Congenital portosystemic shunt (CPSS) is a rare vascular anomaly requiring effective treatment strategies.
  • Interventional therapy offers a less invasive approach compared to traditional surgical methods for CPSS.
  • Understanding the safety and efficacy across different CPSS subtypes is crucial for clinical decision-making.

Purpose of the Study:

  • To evaluate the safety and short-term efficacy of interventional therapy for pediatric congenital portosystemic shunt (CPSS).
  • To provide empirical data from a single center to guide clinical practice for CPSS treatment.
  • To analyze outcomes based on different anatomical subtypes of CPSS.

Main Methods:

  • Retrospective analysis of 26 pediatric patients with CPSS treated between January 2022 and August 2025.
  • Categorization into intrahepatic portal-systemic shunt (18 cases) and Abernethy malformation (8 cases) groups.
  • Utilized coil embolization and Amplatzer occluder embolization, with 1-12 months follow-up.

Main Results:

  • Overall technical success rate was 73% (19/26), with higher rates for intrahepatic shunts (94.4%) versus Abernethy malformations (25%).
  • A low incidence of mild complications (3.8%) was observed, with no severe adverse events.
  • Successful treatments showed significant improvements: 63% liver function normalization, 73.7% bile acid improvement, and 100% blood ammonia normalization.

Conclusions:

  • Individualized interventional embolization is safe and effective for pediatric CPSS.
  • The strategy demonstrates definite short-term efficacy across different anatomical subtypes.
  • Interventional therapy can be considered a first-line treatment for children with CPSS.
Abstract