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CardioVascular and Interventional Radiology

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01-07-2018 | Case Report

Catheter Embolization of Type II Congenital Extrahepatic Portosystemic Shunt with Hematochezia: A Case Series and Review of the Literature

Authors: Bo Zhang, Ke-Tong Wu, Lin Li, Hai-Yang Lai

Published in: CardioVascular and Interventional Radiology | Issue 7/2018

Abstract

Hematochezia is a rare clinical presentation of congenital extrahepatic portosystemic shunt (CEPS). We describe a series of three patients with type II CEPS presenting as hematochezia that were treated by catheter embolization, followed by a brief review of published articles. Hematochezia of the patients was due to the giant inferior mesenteric vein, superior rectal vein and colonic varices. The catheter embolization was successfully accomplished in all of the patients. After a mean follow-up of 27 months, no serious adverse effects were observed. For unexplained massive hematochezia, CEPS needs to be considered as a differential diagnosis. Based on our present results and the review of the literature, transcatheter permanent embolization of the giant inferior mesenteric vein might be an effective and safe treatment for type II CEPS.

Level of Evidence Level 4, case series.

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(3):707–22.CrossRefPubMed

Kobayashi N, Niwa T, Kirikoshi H, Fujita K, Yoneda M, Saito S, Nakajima A. Clinical classification of congenital extrahepatic portosystemic shunts. Hepatol Res. 2010;40(6):585–93.CrossRefPubMed

Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg. 1994;29(9):1239–41.CrossRefPubMed

Passalacqua M, Lie KT, Yarmohammadi H. Congenital extrahepatic portosystemic shunt (Abernethy malformation) treated endovascularly with vascular plug shunt closure. Pediatr Surg Int. 2012;28(1):79–83.CrossRefPubMed

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(6):675–81.CrossRefPubMed

Loomba RS, Telega GW, Gudausky TM. Type 2 Abernethy malformation presenting as a portal vein-coronary sinus fistula. J Pediatr Surg. 2012;47(5):E25–31.CrossRefPubMed

Gupta P, Sinha A, Sodhi KS, Lal A, Debi U, Thapa BR, Khandelwal N. Congenital extrahepatic portosystemic shunts: spectrum of findings on ultrasound, computed tomography, and magnetic resonance imaging. Radiol Res Pract. 2015;2015:181958.PubMedPubMedCentral

Horiguchi Y, Kitano T, Takagawa H, Imai H, Itoh M, Miyakawa S, Nakamura Y, Miura K, Itoh K. A large inferior mesenteric-caval shunt via the internal iliac vein. Gastroenterol Jpn. 1988;23(6):684–7.PubMedCrossRef

Hoseinzadeh S, Shafiei B, Salehian M, Neshandar Asli I, Ghodoosi I. Huge varicose inferior mesenteric vein: an unanticipated (99 m) Tc-labeled red blood cell scintigraphy finding. Nucl Med Mol Imaging. 2010;44(3):217–22.CrossRefPubMedPubMedCentral

10.

Lu J, Lin Z, Liu H, Liu Z. An unusual presentation of type II Abernethy malformation. Ann Vasc Surg. 2014;28(6):1567.e1-4.CrossRefPubMed

11.

Gong Y, Zhu H, Chen J, Chen Q, Ji M, Pa M, Zheng S, Qiao Z. Congenital portosystemic shunts with and without gastrointestinal bleeding—case series. Pediatr Radiol. 2015;45(13):1964–71.CrossRefPubMed

12.

Chocarro G, Amesty MV, Encinas JL, Vilanova Sánchez A, Hernandez F, Andres AM, Gamez M, Tovar JA, Lopez Santamaria M. Congenital portosystemic shunts: clinic heterogeneity requires an individual management of the patient. Eur J Pediatr Surg. 2016;26(1):74–80.PubMed

13.

Ghuman SS, Gupta S, Buxi TB, Rawat KS, Yadav A, Mehta N, Sud S. The Abernethy malformation-myriad imaging manifestations of a single entity. Indian J Radiol Imaging. 2016;26(3):364–72.CrossRefPubMedPubMedCentral

14.

Singhal M, Lal A, Thapa BR, Prakash M, Shanbhogue KP, Khandelwal N. Congenital atresia of portal vein with portocaval shunt associated with cardiac defects, skeletal deformities, and skin lesions in a boy. J Pediatr Surg. 2008;43(8):e25–8.CrossRefPubMed

15.

Nacif LS, Paranaguá-Vezozzo DC, Galvão FH, Rocha MS, Andraus W, Carrilho FJ, D’Albuquerque LC. Significance of CT scan and color Doppler duplex ultrasound in the assessment of Abernethy malformation. BMC Med Imaging. 2015;15:37.CrossRefPubMedPubMedCentral

16.

Gordon-Burroughs S, Balogh J, Weiner MA, Monsour HP Jr, Schwartz MR, Gaber AO, Ghobrial RM. Liver transplantation in an adult with adenomatosis and congenital absence of the portal vein: a case report. Transplant Proc. 2014;46(7):2418–21.CrossRefPubMed

17.

Jiang C, Ye W, Liu C, Wu W, Li Y. Surgical ligation of portosystemic shunt to resolve severe hematuria and hemafecia caused by type II Abernethy malformation. Ann Vasc Surg. 2015;29(5):1020.e11-16.CrossRef

18.

Guneyli S, Cinar C, Bozkaya H, Parildar M, Oran I, Akin Y. Successful transcatheter closure of a congenital high-flow portosystemic venous shunt with the Amplatzer vascular plug II. Perspect Vasc Surg Endovasc Ther. 2012;24(4):202–5.CrossRefPubMed

19.

Yamagami T, Yoshimatsu R, Matsumoto T, Terayama K, Nishiumra A, Maeda Y, Nishimura T. Successful embolization using interlocking detachable coils for a congenital extrahepatic portosystemic venous shunt in a child. J Pediatr Surg. 2007;42(11):1949–52.CrossRefPubMed

20.

Knirsch W, Benz DC, Bühr P, Quandt D, Weber R, Kellenberger C, Braegger CP, Kretschmar O. Catheter interventional treatment of congenital portosystemic venous shunts in childhood. Catheter Cardiovasc Interv. 2016;87(7):1281–92.CrossRefPubMed

21.

Franchi-Abella S, Branchereau S, Lambert V, Fabre M, Steimberg C, Losay J, Riou JY, Pariente D, Gauthier F, Jacquemin E, Bernard O. Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr. 2010;51(3):322–30.PubMed

22.

Takeuchi H, Takeda Y, Takahashi M, Hayashi S, Fukuzawa Y, Nakano T. A rare congenital extrahepatic portosystemic shunt affecting the inferior mesenteric vein, inferior vena cava, and left ovarian vein. Surg Radiol Anat. 2014;36(7):729–32.CrossRefPubMed

23.

Arana E, Martí-Bonmatí L, Martínez V, Hoyos M, Montes H. Portal vein absence and nodular regenerative hyperplasia of the liver with giant inferior mesenteric vein. Abdom Imaging. 1997;22(5):506–8.CrossRefPubMed

Title: Catheter Embolization of Type II Congenital Extrahepatic Portosystemic Shunt with Hematochezia: A Case Series and Review of the Literature
Authors: Bo Zhang
Ke-Tong Wu
Lin Li
Hai-Yang Lai
Publication date: 01-07-2018
Publisher: Springer US
Published in: CardioVascular and Interventional Radiology / Issue 7/2018
Print ISSN: 0174-1551
Electronic ISSN: 1432-086X
DOI: https://doi.org/10.1007/s00270-018-1972-6

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Catheter Embolization of Type II Congenital Extrahepatic Portosystemic Shunt with Hematochezia: A Case Series and Review of the Literature

Abstract

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