A Congenital Portosystemic Shunt in a Child With Heterotaxy, Situs Inversus, Polysplenia, and Interrupted Inferior Vena Cava With Azygous Continuation

Congenital portosystemic shunts are rare vascular malformations in which portal venous blood from the intestines and spleen bypasses the liver and diverts directly into the systemic circulation through abnormal vessels. We report a case of a 4-year-old girl with heterotaxy syndrome, polysplenia, and...

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Autores principales: Carvajal, Victoria, Reddy, Saigopala, Gopalareddy, Vani, Bean, Adrienne, Wallis, Gonzalo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer 2023
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10697625/
http://dx.doi.org/10.14309/crj.0000000000001201
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author Carvajal, Victoria
Reddy, Saigopala
Gopalareddy, Vani
Bean, Adrienne
Wallis, Gonzalo
author_facet Carvajal, Victoria
Reddy, Saigopala
Gopalareddy, Vani
Bean, Adrienne
Wallis, Gonzalo
author_sort Carvajal, Victoria
collection PubMed
description Congenital portosystemic shunts are rare vascular malformations in which portal venous blood from the intestines and spleen bypasses the liver and diverts directly into the systemic circulation through abnormal vessels. We report a case of a 4-year-old girl with heterotaxy syndrome, polysplenia, and situs inversus presenting with persistent hypoxemia who was found to have pulmonary arteriovenous malformations (PAVMs) and hypoxemia secondary to a congenital portosystemic shunt. Management of this patient's PAVMs involved endovascular occlusion of the portosystemic shunt with subsequent resolution of hypoxemia. PAVMs secondary to extrahepatic portosystemic shunt should be explored as a cause of progressive cyanosis in children with heterotaxy, polysplenia, and interrupted inferior vena cava with azygous continuation.
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spelling pubmed-106976252023-12-06 A Congenital Portosystemic Shunt in a Child With Heterotaxy, Situs Inversus, Polysplenia, and Interrupted Inferior Vena Cava With Azygous Continuation Carvajal, Victoria Reddy, Saigopala Gopalareddy, Vani Bean, Adrienne Wallis, Gonzalo ACG Case Rep J Case Report Congenital portosystemic shunts are rare vascular malformations in which portal venous blood from the intestines and spleen bypasses the liver and diverts directly into the systemic circulation through abnormal vessels. We report a case of a 4-year-old girl with heterotaxy syndrome, polysplenia, and situs inversus presenting with persistent hypoxemia who was found to have pulmonary arteriovenous malformations (PAVMs) and hypoxemia secondary to a congenital portosystemic shunt. Management of this patient's PAVMs involved endovascular occlusion of the portosystemic shunt with subsequent resolution of hypoxemia. PAVMs secondary to extrahepatic portosystemic shunt should be explored as a cause of progressive cyanosis in children with heterotaxy, polysplenia, and interrupted inferior vena cava with azygous continuation. Wolters Kluwer 2023-12-04 /pmc/articles/PMC10697625/ http://dx.doi.org/10.14309/crj.0000000000001201 Text en © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0/) , where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
spellingShingle Case Report
Carvajal, Victoria
Reddy, Saigopala
Gopalareddy, Vani
Bean, Adrienne
Wallis, Gonzalo
A Congenital Portosystemic Shunt in a Child With Heterotaxy, Situs Inversus, Polysplenia, and Interrupted Inferior Vena Cava With Azygous Continuation
title A Congenital Portosystemic Shunt in a Child With Heterotaxy, Situs Inversus, Polysplenia, and Interrupted Inferior Vena Cava With Azygous Continuation
title_full A Congenital Portosystemic Shunt in a Child With Heterotaxy, Situs Inversus, Polysplenia, and Interrupted Inferior Vena Cava With Azygous Continuation
title_fullStr A Congenital Portosystemic Shunt in a Child With Heterotaxy, Situs Inversus, Polysplenia, and Interrupted Inferior Vena Cava With Azygous Continuation
title_full_unstemmed A Congenital Portosystemic Shunt in a Child With Heterotaxy, Situs Inversus, Polysplenia, and Interrupted Inferior Vena Cava With Azygous Continuation
title_short A Congenital Portosystemic Shunt in a Child With Heterotaxy, Situs Inversus, Polysplenia, and Interrupted Inferior Vena Cava With Azygous Continuation
title_sort congenital portosystemic shunt in a child with heterotaxy, situs inversus, polysplenia, and interrupted inferior vena cava with azygous continuation
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10697625/
http://dx.doi.org/10.14309/crj.0000000000001201
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