Korean J Radiol.  2010 Oct;11(5):583-586. 10.3348/kjr.2010.11.5.583.

Congenital Intrahepatic Portosystemic Venous Shunt and Liver Mass in a Child Patient: Successful Endovascular Treatment with an Amplatzer Vascular Plug (AVP)

Affiliations
  • 1Department of Radiology, Dankook University College of Medicine, Dankook University Hospital, Chungcheongnam-do 330-715, Korea. hae0820@naver.com
  • 2Department of Pediatrics, Dankook University College of Medicine, Dankook University Hospital, Chungcheongnam-do 330-715, Korea.

Abstract

A congenital intrahepatic portosystemic shunt is a rare anomaly; but, the number of diagnosed cases has increased with advanced imaging tools. Symptomatic portosystemic shunts, especially those that include hyperammonemia, should be treated; and various endovascular treatment methods other than surgery have been reported. Hepatic masses with either an intra- or extrahepatic shunt also have been reported, and the mass is another reason for treatment. Authors report a case of a congenital intrahepatic portosystemic shunt with a hepatic mass that was successfully treated using a percutaneous endovascular approach with vascular plugs. By the time the first short-term follow-up was conducted, the hepatic mass had disappeared.

Keyword

Portosystemic shunt, surgical; Liver neoplasm; Radiology, interventional

MeSH Terms

Child
Diagnosis, Differential
Hepatic Encephalopathy/*congenital/diagnosis/therapy
Humans
Liver Neoplasms/*congenital/diagnosis/therapy
Male
Portal Vein/*abnormalities
*Septal Occluder Device
Tomography, X-Ray Computed
Ultrasonography, Doppler, Color
Ultrasonography, Interventional

Figure

  • Fig. 1 Congenital intrahepatic portosystemic venous shunt and liver mass in child patient. A. Contrast-enhanced multidetector CT scan performed before embolization revealed 3.5 cm mass with heterogeneous enhancement in liver's right lobe. B. Right anterior oblique digital subtraction direct portogram demonstrated direct connection between dilated left portal vein and left hepatic vein by three variable shunt channels. Right portal vein was very small, compared to left portal vein. C. Right anterior oblique digital subtraction direct portogram immediately after embolization showed successful occlusion of intrahepatic portosystemic shunt and improvement of flow through right portal vein. D. Oblique coronal reformatted CT scan image one month after procedure showed markedly increased size of right portal veins, compared to B.


Reference

1. Chagnon SF, Vallee CA, Barge J, Chevalier LJ, Le Gal J, Blery MV. Aneurysmal portahepatic venous fistula: report of two cases. Radiology. 1986. 159:693–695.
2. Singh K, Kapoor A, Kapoor A, Gupta K, Mahajan G. Congenital intrahepatic portosystemic shunt--an incidental rare anomaly. Indian J Pediatr. 2006. 73:1122–1123.
3. Stringer MD. The clinical anatomy of congenital portosystemic venous shunts. Clin Anat. 2008. 21:147–157.
4. Kanamori Y, Hashizume K, Kitano Y, Sugiyama M, Motoi T, Tange T. Congenital extrahepatic portocaval shunt (Abernethy type 2), huge liver mass, and patent ductus arteriosus--a case report of its rare clinical presentation in a young girl. J Pediatr Surg. 2003. 38:E15.
5. Ikeda S, Sera Y, Yoshida M, Izaki T, Uchino S, Endo F, et al. Successful coil embolization in an infant with congenital intrahepatic portosystemic shunts. J Pediatr Surg. 1999. 34:1031–1032.
6. Yoon HK, Choo SW, Do YS, Choo IW, Han BK. Congenital intrahepatic portosystemic shunts in the neonate: coil embolization via the umbilical vein. J Vasc Interv Radiol. 1998. 9:509–511.
7. Fanelli F, Marcelli G, Bezzi M, Maria Salvatori F, Rossi M, Rossi P, et al. Intrahepatic aneurysmal portohepatic venous shunt: embolization with a tissue adhesive solution. J Endovasc Ther. 2003. 10:147–153.
8. Kudo M, Tomita S, Tochio H, Minowa K, Todo A. Intrahepatic portosystemic venous shunt: diagnosis by color Doppler imaging. Am J Gastroenterol. 1993. 88:723–729.
9. Park JH, Cha SH, Han JK, Han MC. Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol. 1990. 155:527–528.
10. Kerlan RK Jr, Sollenberger RD, Palubinskas AJ, Raskin NH, Callen PW, Ehrenfeld WK. Portal-systemic encephalopathy due to a congenital portocaval shunt. AJR Am J Roentgenol. 1982. 139:1013–1015.
11. Uchino T, Matsuda I, Endo F. The long-term prognosis of congenital portosystemic venous shunt. J Pediatr. 1999. 135:254–256.
12. Kim IO, Cheon JE, Kim WS, Chung JW, Yeon KM, Yoo SJ, et al. Congenital intrahepatic portohepatic venous shunt: treatment with coil embolisation. Pediatr Radiol. 2000. 30:336–338.
13. Okada Y, Endo T, Kusano S, Yoshida M. Multiple intrahepatic portohepatic venous shunts: treatment with steel-coil embolization. AJR Am J Roentgenol. 1991. 157:971–973.
14. Kessler J, Trerotola SO. Use of the Amplatzer Vascular Plug for embolization of a large retroperitoneal shunt during transjugular intrahepatic portosystemic shunt creation for gastric variceal bleeding. J Vasc Interv Radiol. 2006. 17:135–140.
15. Pattynama PM, Wils A, van der Linden E, van Dijk LC. Embolization with the Amplatzer Vascular Plug in TIPS patients. Cardiovasc Intervent Radiol. 2007. 30:1218–1221.
16. Crummy AB, McDermott JC, Wojtowycz M. A technique for embolization of biopsy tracts. AJR Am J Roentgenol. 1989. 153:67–68.
17. Lyon SM, Terhaar O, Given MF, O'Dwyer HM, McGrath FP, Lee MJ. Percutaneous embolization of transhepatic tracks for biliary intervention. Cardiovasc Intervent Radiol. 2006. 29:1011–1014.
18. Litvin S, Atar E, Knizhnik M, Bruckheimer E, Belenky A. Stent graft closure of a high flow splenorenal shunt after liver transplantation. Diagn Interv Radiol. 2009. [Epub ahead of print].
19. Yankes JR, Uglietta JP, Grant J, Braun SD. Percutaneous transhepatic recanalization and thrombolysis of the superior mesenteric vein. AJR Am J Roentgenol. 1988. 151:289–290.
20. Sawyer B, Dow C, Frank J, Lau E. Congenital portocaval shunt-associated liver lesions in a patient with cancer. ANZ J Surg. 2008. 78:613–614.
21. Kandpal H, Sharma R, Arora NK, Gupta SD. Congenital extrahepatic portosystemic venous shunt: imaging features. Singapore Med J. 2007. 48:E258–E261.
Full Text Links
  • KJR
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr