Large-caliber congenital portocaval shunt and hepatopulmonary syndrome: Limitations of closure and the role of liver transplantation.

Authors

Emilie Balent Aguirre, José G Cervantes, Lucía Navarro, Rosana Pérez Carusi, Patricia D'Alia, Anahí Reartes, Carlos Luque, Miguel Palmeiro, Fabián Salgueiro, Fernando R García Maida, Marcelo E Lenz Virreira, Fernando Cairo, Francisco J Mattera, Emilio G Quiñonez

Published in

Archivos argentinos de pediatria. Pages e202611066. Sep 24, 2026. Epub Sep 24, 2026.

Abstract

Congenital portosystemic shunts (CPS) are rare vascular malformations associated with hepatic and extrahepatic complications, including hepatopulmonary syndrome (HPS). We present a 16-year-old adolescent with no relevant medical history who presented with episodes of abdominal pain, jaundice, and dark urine. Ultrasound revealed a heterogeneous liver with multiple hyperechoic nodules and no visualization of the intrahepatic portal vein. Angiography demonstrated an extrahepatic portocaval shunt at the level of the portal trunk, with no opacification of the intrahepatic portal tree and low portal pressures; liver biopsy revealed regenerative nodular hyperplasia. Subsequently, he developed hypoxemia with orthodeoxygenation and required home oxygen therapy. He was placed on the waiting list and received an orthotopic liver transplant from a deceased donor. This case highlights the need for timely diagnosis, proper anatomical characterization, and individualized treatment planning in CPS associated with HPS.

PMID:
42770774
Bibliographic data and abstract were imported from PubMed on 22 Sep 2026.

Read full publication at:
Please sign in to see all details.

Stats

1-terrible, 9-excellent. How would you rate this publication? Sign in in to submit your rating.

Post a comment

You need to be signed in to post comments. You can sign in here.

Comments

There are no comments yet.