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[Idiopathic intrahepatic portosytemic shunts in 4 children]
F Rouveyrol1, M Meyer, J R Lusson
1Service de pédiatrie, Hôtel-Dieu, CHU de Clermont-Ferrand, France.
Insights
This study details four cases of intrahepatic portosystemic shunts, rare liver vascular anomalies. Diagnosis and outcomes varied, with some cases showing spontaneous resolution or remaining asymptomatic.
Area of Science:
- Vascular Surgery
- Pediatric Radiology
- Hepatology
Background:
- Intrahepatic portosystemic shunts are abnormal connections between the portal vein and systemic circulation within the liver.
- These shunts are rare vascular anomalies with diverse clinical presentations and diagnostic challenges.
Observation:
- Four new cases of type II intrahepatic portosystemic shunts are presented.
- Diagnosis occurred antenatally via ultrasound in two cases, with spontaneous involution observed.
- One case was incidentally found at 3 weeks, with the child remaining asymptomatic at 6 years.
Findings:
- Antenatal diagnosis is feasible for some intrahepatic portosystemic shunts.
- Spontaneous involution can occur in pediatric cases.
- Diagnosis can be incidental, and patients may remain asymptomatic.
Implications:
- Early diagnosis, including antenatal detection, is crucial for managing these rare anomalies.
- Understanding the spectrum of presentation aids in clinical decision-making.
- Further research into the natural history and management of these shunts is warranted.
Abstract:
Intrahepatic portosystemic anastomoses are macroscopic communications between the venous portal system and the systemic circulation and located partly in the liver. We report 4 new cases of type II shunts, which illustrate the circumstances of the diagnosis of these exceptional anomalies. For 2 children, the diagnosis was done antenataly by US and spontaneous involution in a few months was observed. In the third case the malformation was evidenced fortuitously at 3 weeks of life, and this 6-year-old child remains completely asymptomatic so far. Then, in the fourth case, a cerebral venous thrombosis was fortuitously and antenatally evidenced in an otherwise uneventful pregnancy and portosystemic shunt was demonstrated postnataly in the extensive work up of the neonate.
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