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CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2019; 29(02): 219-222
DOI: 10.4103/ijri.IJRI_461_18
Case Report

Role of interventional radiology in the diagnosis and management of congenital extrahepatic portosystemic shunts: Two case reports

Sheetal V Mathai
Department of Radiology, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Karnataka, India
,
Victor Kondray
Department of Radiology, University Hospitals, Case Western Reserve University School of Medicine, 11100 Euclid Ave, Cleveland, OH 44106, USA
,
Elias Salloum
Department of Radiology, Massachusetts General Hospital, 55 Fruit St, Boston, MA 02114, USA
,
Kamlesh Kukreja
Department of Radiology, Texas Children’s Hospital, 6701 Fannin Street, Suite 470, Houston, TX 77030, USA
,
Sidhartha Tavri
Department of Radiology, University Hospitals, Case Western Reserve University School of Medicine, 11100 Euclid Ave, Cleveland, OH 44106, USA
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Abstract

Congenital extrahepatic portosystemic shunt (CEPS) is a rare splanchnic venous malformation, wherein the portal venous outflow drains into the systemic venous circulation via a pathologic shunt. CEPS exhibits heterogeneous clinical behavior and angiography is the gold standard for evaluation of the portomesenteric communication to systemic vasculature. The potential severity of complications necessitates shunt closure. Here, we present two cases of CEPS. The first patient presented with an asymptomatic hyperammonemia and was found to have a Type 1 CEPS with absence of intrahepatic portal system. The second patient was asymptomatic and was incidentally found to have a Type 2 CEPS on imaging with normal intrahepatic portal system. Both patients were successfully treated with endovascular occlusion of the CEPS.

Keywords

Congenital extrahepatic portosystemic shunt - endovascular closure - focal nodular hyperplasia - hyperammonemia - portal hypertension


Publication History

Article published online:
22 July 2021

© 2019. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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  • References

  • 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: 273-87
    Thieme ConnectPubMedSearch in Google Scholar
  • 2 Srinivasa RN, Beecham Chick JF, Chen N, Gemmete JJ, Saad WA, Dasika NL. et al. Pediatric portal interventions. SeminIntervent Radiol 2018; 35: 143-50
    Search in Google Scholar
  • 3 Alonso-Gamarra E, Parrón M, Pérez A, Prieto C, Hierro L, López-Santamaría M. Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: A comprehensive review. Radiographics 2011; 31: 707-72
    CrossrefPubMedSearch in Google Scholar
  • 4 Shah A, Aziz A, Awwad A, Ramjas G, Higashi Y. Incidental radiological diagnosis of asymptomatic Abernethy malformations—two case reports. BJR Case Reports 2017; 3: 20150496
    CrossrefPubMedSearch in Google Scholar
  • 5 Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatr Radiol 2003; 33: 614-20
    CrossrefPubMedSearch in Google Scholar
  • 6 Timpanaro T, Passanisi S, Sauna A, Trombatore C, Pennisi M, Petrillo G. et al. Congenital portosystemic shunt: Our experience. Case Rep Pediatr 2015; 2015: 691618
    CrossrefPubMedSearch in Google Scholar
  • 7 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: 675-81
    CrossrefPubMedSearch in Google Scholar
  • 8 Yamagami T, Yoshimatsu R, Matsumoto T, Terayama K, Nishiumra A, Maeda Y. et al. Successful embolization using interlocking detachable coils for a congenital extrahepatic portosystemic venous shunt in a child. J Pediatr Surg 2007; 42: 1949-52
    CrossrefPubMedSearch in Google Scholar
  • 9 Elnekave E, Belenky E, Van der Veer L. Noncirrhotic extrahepatic portosystemic shunt causing adult-onset encephalopathy treated with endovascular closure. Case Rep Radiol Case Rep Radiol 2015; 2015: 852853
    CrossrefPubMedSearch in Google Scholar