Background: Abernethy malformations are rare vascular anomalies of the portal system which present as extra-hepatic congenital portosystemic venous shunts (CPSS). Sometimes they can be intra-hepatic anomalies. There is scarcity of literature on management of these rare anomalies especially intra-hepatic shunts. Case: A five years old child came with the complaints of progressive breathlessness on exertion with effort-intolerance for the past two years. There was no history suggestive of underlying cardiopulmonary illness. On examination, there was cyanosis and clubbing. On evaluation, the imaging showed a large congenital intra-hepatic portosystemic shunt from the left portal vein draining directly into intrahepatic inferior vena cava (IVC) and a hypoplastic right branch of the portal vein leading to a clinical presentation of hepatopulmonary syndrome. Result: The shunt was occluded by placing a covered stent in the IVC across the shunt opening, making sure the openings of hepatic veins and renal vein also were not being covered. There was a significant improvement in oxygenation post procedure with complete disappearance of cyanosis. Conclusion: Covered IVC stent placement is a novel technique for large fusiform dilated intra-hepatic CPSS by closing the shunt flow into the IVC, thereby restoring the physiological flow in the liver.
| Published in | International Journal of Gastroenterology (Volume 3, Issue 1) |
| DOI | 10.11648/j.ijg.20190301.14 |
| Page(s) | 23-26 |
| Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
| Copyright |
Copyright © The Author(s), 2019. Published by Science Publishing Group |
Clinical Decision Making, Computer Tomography, Development Genes, Hepatic Encephalopathy, Dyspnea
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APA Style
Chandan Kumar Kedarisetty, Rajeev Kamble, Santhosh Joseph, Jayanthi Venkataraman. (2019). Management of Hepatopulmonary Syndrome in a Child Due to a Large Congenital Intrahepatic Porto-Systemic Shunt. International Journal of Gastroenterology, 3(1), 23-26. https://doi.org/10.11648/j.ijg.20190301.14
ACS Style
Chandan Kumar Kedarisetty; Rajeev Kamble; Santhosh Joseph; Jayanthi Venkataraman. Management of Hepatopulmonary Syndrome in a Child Due to a Large Congenital Intrahepatic Porto-Systemic Shunt. Int. J. Gastroenterol. 2019, 3(1), 23-26. doi: 10.11648/j.ijg.20190301.14
AMA Style
Chandan Kumar Kedarisetty, Rajeev Kamble, Santhosh Joseph, Jayanthi Venkataraman. Management of Hepatopulmonary Syndrome in a Child Due to a Large Congenital Intrahepatic Porto-Systemic Shunt. Int J Gastroenterol. 2019;3(1):23-26. doi: 10.11648/j.ijg.20190301.14
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Download@article{10.11648/j.ijg.20190301.14,
author = {Chandan Kumar Kedarisetty and Rajeev Kamble and Santhosh Joseph and Jayanthi Venkataraman},
title = {Management of Hepatopulmonary Syndrome in a Child Due to a Large Congenital Intrahepatic Porto-Systemic Shunt},
journal = {International Journal of Gastroenterology},
volume = {3},
number = {1},
pages = {23-26},
doi = {10.11648/j.ijg.20190301.14},
url = {https://doi.org/10.11648/j.ijg.20190301.14},
eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijg.20190301.14},
abstract = {Background: Abernethy malformations are rare vascular anomalies of the portal system which present as extra-hepatic congenital portosystemic venous shunts (CPSS). Sometimes they can be intra-hepatic anomalies. There is scarcity of literature on management of these rare anomalies especially intra-hepatic shunts. Case: A five years old child came with the complaints of progressive breathlessness on exertion with effort-intolerance for the past two years. There was no history suggestive of underlying cardiopulmonary illness. On examination, there was cyanosis and clubbing. On evaluation, the imaging showed a large congenital intra-hepatic portosystemic shunt from the left portal vein draining directly into intrahepatic inferior vena cava (IVC) and a hypoplastic right branch of the portal vein leading to a clinical presentation of hepatopulmonary syndrome. Result: The shunt was occluded by placing a covered stent in the IVC across the shunt opening, making sure the openings of hepatic veins and renal vein also were not being covered. There was a significant improvement in oxygenation post procedure with complete disappearance of cyanosis. Conclusion: Covered IVC stent placement is a novel technique for large fusiform dilated intra-hepatic CPSS by closing the shunt flow into the IVC, thereby restoring the physiological flow in the liver.},
year = {2019}
}
TY - JOUR T1 - Management of Hepatopulmonary Syndrome in a Child Due to a Large Congenital Intrahepatic Porto-Systemic Shunt AU - Chandan Kumar Kedarisetty AU - Rajeev Kamble AU - Santhosh Joseph AU - Jayanthi Venkataraman Y1 - 2019/09/24 PY - 2019 N1 - https://doi.org/10.11648/j.ijg.20190301.14 DO - 10.11648/j.ijg.20190301.14 T2 - International Journal of Gastroenterology JF - International Journal of Gastroenterology JO - International Journal of Gastroenterology SP - 23 EP - 26 PB - Science Publishing Group SN - 2640-169X UR - https://doi.org/10.11648/j.ijg.20190301.14 AB - Background: Abernethy malformations are rare vascular anomalies of the portal system which present as extra-hepatic congenital portosystemic venous shunts (CPSS). Sometimes they can be intra-hepatic anomalies. There is scarcity of literature on management of these rare anomalies especially intra-hepatic shunts. Case: A five years old child came with the complaints of progressive breathlessness on exertion with effort-intolerance for the past two years. There was no history suggestive of underlying cardiopulmonary illness. On examination, there was cyanosis and clubbing. On evaluation, the imaging showed a large congenital intra-hepatic portosystemic shunt from the left portal vein draining directly into intrahepatic inferior vena cava (IVC) and a hypoplastic right branch of the portal vein leading to a clinical presentation of hepatopulmonary syndrome. Result: The shunt was occluded by placing a covered stent in the IVC across the shunt opening, making sure the openings of hepatic veins and renal vein also were not being covered. There was a significant improvement in oxygenation post procedure with complete disappearance of cyanosis. Conclusion: Covered IVC stent placement is a novel technique for large fusiform dilated intra-hepatic CPSS by closing the shunt flow into the IVC, thereby restoring the physiological flow in the liver. VL - 3 IS - 1 ER -
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