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

Sheetal V Mathai; Victor Kondray; Elias Salloum; Kamlesh Kukreja; Sidhartha Tavri

doi:10.4103/ijri.IJRI_461_18

Indian Journal of Radiology and Imaging, Inhaltsverzeichnis

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

Autor*innen

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

Verantwortlicher Herausgeber dieser Rubrik:

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

Volltext

Referenzen

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
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
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
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
5 Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatr Radiol 2003; 33: 614-20
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
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
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
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