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Management of Hepatopulmonary Syndrome in a Child Due to a Large Congenital Intrahepatic Porto-Systemic Shunt

Received: 17 August 2019     Accepted: 10 September 2019     Published: 24 September 2019
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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.

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

Keywords

Clinical Decision Making, Computer Tomography, Development Genes, Hepatic Encephalopathy, Dyspnea

References
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[2] Alvarez A E, Ribeiro A F, Hessel G, Baracat J, Ribeiro J D. Abernethy malformation: One of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol 2002; 34: 391-4.
[3] Sokollik C, Bandsma RH, Gana JC, van den Heuvel M, Ling SC. Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr 2013; 56: 675–681.
[4] Park JH, Cha SH, Han JK, Han MC. Intrahepatic portosystemic venous shunt. Am J Roentgenol 1990; 155: 527-528.
[5] Ghuman SS, Gupta S, Buxi TBS, Rawat KS, Yadav A, Mehta N, Sud S. The Abernethy malformation – myriad imaging manifestations of a single entity. Indian J Radiol Imaging 2016: 26: 364-72.
[6] Senocak E, Oğuz B, Edgüer T, Cila A. Congenital intrahepatic portosystemic shunt with variant inferior right hepatic vein. Diagn Interv Radiol 2008; 14: 97-99.
[7] Zhang J and Fallon MB. Hepatopulmonary syndrome: update on pathogenesis and clinical features. Nat Rev Gastroenterol Hepatol 2012; 9: 539-549.
[8] Yoshimatsu R, Takeuchi Y, Morishita H, Iida N, Okabe H, Yamagami T, Nishimura T. Successful embolization of intrahepatic portosystemic venous shunt using coils and n-butyl cyanoacrylate through two approach routes. Br J Radiol 2006; 79 (947): e162-5.
[9] Power AH, Bjarnason H. Large spontaneous intrahepatic portal- systemic venous shunt treated with coil and Amplatzer vascular plug embolization. Perspect Vasc Surg Endovasc Ther 2012; 24 (2): 90–94.
[10] Matsuura T, Takahashi Y, Yanagi Y, et al. Surgical strategy according to the anatomical types of congenital portosystemic shunts in children. J Pediatr Surg 2016; 51 (12): 2099-2104.
[11] Sanada Y, Urahashi T, Ihara Y, Wakiya T, Okada N, Yamada N et al (2012 Mar) The role of operative intervention in management of con- genital extrahepatic portosystemic shunt. Surgery 151 (3): 404-411.
[12] Gordon-Burroughs S, Balogh J, Weiner MA et al. Liver transplantation in an adult with adenomatosis and congenital absence of the portal vein: a case report. Transplant Proc 2014; 46 (7): 2418-2421.
[13] Shiomi S, Kuroki T, Ueda T et al. Clinical usefulness of evaluation of portal circulation by per-rectal portal scintigraphy with technetium-99m pertechnetate. Am J Gastroenterol 1995; 90: 460-5.
[14] O'Leary JG, Rees CR, Klintmalm GB, Davis GL. Inferior vena cava stent resolves hepatopulmonary syndrome in an adult with spontaneous inferior vena cava-portal vein shunt. Liver Transplantation 2009; 15: 1897-1900.
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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

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    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

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    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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  • @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}
    }
    

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  • 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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Author Information
  • Department of Hepatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, India

  • Department of Interventional Radiology, Sri Ramachandra Institute of Higher Education and Research, Chennai, India

  • Department of Interventional Radiology, Sri Ramachandra Institute of Higher Education and Research, Chennai, India

  • Department of Hepatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, India

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