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2019, Volume 3
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Volume 3, Issue 1, June 2019, Page: 23-26
Management of Hepatopulmonary Syndrome in a Child Due to a Large Congenital Intrahepatic Porto-Systemic Shunt
Chandan Kumar Kedarisetty, Department of Hepatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, India
Rajeev Kamble, Department of Interventional Radiology, Sri Ramachandra Institute of Higher Education and Research, Chennai, India
Santhosh Joseph, Department of Interventional Radiology, Sri Ramachandra Institute of Higher Education and Research, Chennai, India
Jayanthi Venkataraman, Department of Hepatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, India
Received: Aug. 17, 2019;       Accepted: Sep. 10, 2019;       Published: Sep. 24, 2019
DOI: 10.11648/j.ijg.20190301.14      View  20      Downloads  6
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.
Keywords
Clinical Decision Making, Computer Tomography, Development Genes, Hepatic Encephalopathy, Dyspnea
To cite this article
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, International Journal of Gastroenterology. Vol. 3, No. 1, 2019, pp. 23-26. doi: 10.11648/j.ijg.20190301.14
Copyright
Copyright © 2019 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Reference
[1]
Bernard O, Franchi-Abella S, Branchereau S, Pariente D, Gauthier F, Jacquemin E. Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis 2012; 32 (4): 273–287.
[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.
[15]
Kuo MD, Miller FJ, Lavine JE, Peterson M, Finch M. Exploiting phenotypic plasticity for treatment of hepatopulmonary syndrome in Abernethy malformation. J Vasc Interv Radiol 2010; 21: 917-922.
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