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Congenital portosystemic shunts: diagnosis and treatment

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Abstract

Congenital portosystemic shunts (CPSS) are rare vascular malformations that create an abnormal connection between portal and systemic veins resulting in complete or partial diversion of the portal flow away from the liver to the systemic venous system. Different anatomic types exist and several classifications have been proposed. They can be associated with other malformations especially cardiac and heterotaxia. The main complications include hepatic encephalopathy, liver tumors, portopulmonary hypertension, and pulmonary arteriovenous shunts. Diagnosis relies on imaging, and prenatal diagnosis is possible. Spontaneous closure of the CPSS is possible in some anatomic forms during the first year of life. When the CPSS remains patent, radiologic or surgical closure of the CPSS may prevent, resolve, or stabilize complications. Interventional radiology plays a key role for both the preoperative evaluation with occlusion test to assess the exact anatomy and to measure portal pressure after occlusion of the CPSS. Endovascular closure is the first option for treatment when possible.

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Acknowledgements

We would like to thank Professor Olivier Bernard and Professor Frédéric Gauthier for their major valuable contribution to the knowledge of portosystemic shunts.

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Authors and Affiliations

  1. Pediatric Radiology Department, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, 78 rue du Général Leclerc, 94278, Le Kremlin-Bicêtre, France

    Stéphanie Franchi-Abella & Danièle Pariente

  2. Pediatric Hepatology, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, National Centre for Biliary Atresia, Le Kremlin-Bicêtre, France

    Emmanuel Gonzales & Oanez Ackermann

  3. Pediatric Surgery Department, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, Le Kremlin-Bicêtre, France

    Sophie Branchereau & Florent Guérin

  4. Pediatric Liver Transplantation Unit, National Reference Centre for Rare Pediatric Liver Diseases and Filfoie, Paris, France

    Stéphanie Franchi-Abella, Emmanuel Gonzales, Oanez Ackermann, Sophie Branchereau, Danièle Pariente & Florent Guérin

  5. Hepatinov, University Paris –Sud, Orsay, France

    Stéphanie Franchi-Abella, Emmanuel Gonzales, Oanez Ackermann, Sophie Branchereau, Danièle Pariente & Florent Guérin

  6. IR4 M-UMR 8081- University Paris –Sud, Orsay, France

    Stéphanie Franchi-Abella

  7. INSERM UMR-S1174, Orsay, France

    Emmanuel Gonzales

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Franchi-Abella, S., Gonzales, E., Ackermann, O. et al. Congenital portosystemic shunts: diagnosis and treatment. Abdom Radiol 43, 2023–2036 (2018). https://doi.org/10.1007/s00261-018-1619-8

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