Abstract
A 3-year-old boy, who presented with progressive cyanosis and hypoxia, was diagnosed with a large congenital extrahepatic portosystemic shunt, interrupted IVC with azygos continuation, and multiple congenital anomalies. Traditionally open and laparoscopic surgical techniques have been used to treat this malformation. Endovascular repair using a 16-mm Amplatzer vascular plug (AGA Medical Corporation, Golden Valley, Minnesota, USA) was used to occlude the shunt. Immediate post-placement venography demonstrated cessation of flow within the shunt and increased portal venous flow. The patient’s hypoxia and cyanosis decreased significantly, and he was discharged on the 5th post-procedure day in stable clinical condition. Three months follow-up evaluation demonstrated the vascular plug in place, unchanged in position.
This is a preview of subscription content, log in via an institution to check access.
References
Abernethy J (1793) Account of two instances of the uncommon formation in the viscera of the human body. Philos Trans B83:59–66
Morgan G, Superina R (1994) Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg 29:1239–1241
Emre S, Arnon R, Cohen E, Morotti RA, Vaysman D, Shneider BL (2007) Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in Abernethy malformation. A case report. Liver Transpl 13(12):1662–1668
Howard ER, Davenport M (1997) Congenital extrahepatic portocaval shunts–the Abernethy malformation. J Pediatr Surg 32(3):494–497
Morikawa N, Honna T, Kuroda T, Kitano Y, Fuchimoto Y, Kawashima N, Kawasaki K (2008) Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation. J Pediatr Surg 43(2):e35–e38
Yagi H, Takada Y, Fujimoto Y, Ogura Y, Kozaki K, Ueda M, Tanaka K (2004) Successful surgical ligation under intraoperative portal vein pressure monitoring of a large portosystemic shunt presenting as an intrapulmonary shunt: report of a case. Surg Today 34(12):1049–1052
Kimura T, Soh H, Hasegawa T, Sasaki T, Kuroda S, Yuri E, Tomoda K, Fukazawa M (2004) Laparoscopic correction of congenital portosystemic shunt in children. Surg Laparosc Endosc Percutan Tech 14:285–288
Alvarez AE, Ribeiro AF, Hessel G, Baracat J, Ribeiro JD (2002) Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol 34:391–394
Kuo MD, Miller FJ, Lavine JE, Peterson M, Finch M (2010) Exploiting phenotypic plasticity for the treatment of hepatopulmonary shunting in abernethy malformation. J Vasc Interv Radiol 21(6):917–922 (Epub 2010 Apr 15)
Ikeda S, Sera Y, Ohshiro H, Uchino S, Uchino T, Endo F (1999) Surgical indications for patients with hyperammonemia. J Pediatr Surg 34:1012–1015
Ikeda S, Sera Y, Yoshida M et al (1999) Successful coil embolization in an infant with congenital intrahepatic portosystemic shunts. J Pediatr Surg 34:1031–1032
Yamagami T, Yoshimatsu R, Matsumoto T, Terayama K, Nishiumra A, Maeda Y, Nishimura T (2007) Successful embolization using interlocking detachable coils for a congenital extrahepatic portosystemic venous shunt in a child. J Pediatr Surg 42(11):1949–1952
Chiu SN, Chien YH, Wu MH, Wang JK, Chen SJ (2008) Transcatheter closure of portal-systemic shunt combining congenital double extrahepatic inferior vena cava with vascular plug. J Pediatr 153(5):723
Conflict of interest
Nothing to disclose.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Passalacqua, M., Lie, K.T. & Yarmohammadi, H. Congenital extrahepatic portosystemic shunt (Abernethy malformation) treated endovascularly with vascular plug shunt closure. Pediatr Surg Int 28, 79–83 (2012). https://doi.org/10.1007/s00383-011-2944-y
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00383-011-2944-y