Abernethy Malformasyonu: Konjenital Ekstrahepatik Portokaval Şantın Nadir Nedeni

Yeliz Çağan Appak; Serdar Tarhan; Güzide Doğan; Erhun Kasırga

Case Report

Turk J Pediatr Dis 2016; 10(1): 53-56.

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The Abernethy Malformation: A Rare Cause of Congenital Extrahepatic Portacaval Shunt

Authors

Yeliz Çağan Appak

Serdar Tarhan

Güzide Doğan

Erhun Kasırga

Affiliations

Turk J Pediatr Dis 2016; 10(1): 53-56.

Published: April 1, 2016

Abstract

The Abernethy malformation is a rare anomaly of the splanchnic venous system with a widely variable clinical presentation. In this case report we presented a 5-month-old girl where liver masses and the Abernethy malformation were incidentally detected without clinical symptoms as a result of investigations. Clinical fi ndings of the malformation and the importance of potential complications are emphasized

Keywords: Child, Portal vein, Vasculer malformation

References

Abernethy J, Banks J. Account of two instances of the uncommon formation in the viscera of the human body. Philos Trans 1793; 83:59-66.
Mistinova J, Valacsai F, Varga I. Congenital absence of the portal vein: Case report and a review of literature. Clin Anat 2010; 23: 750-8.
Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classifi cation system for portasystemic vascular anomalies. J Pediatr Surg 1994; 29: 1239-41.
Gocmen R, Akhan O, Talim B. Congenital absence of the portal vein associated with congenital hepatic fi brosis. Pediatr Radiol 2007; 37: 920-4.
Gallego C, Velasco M, Marcuello P, Tejedor D, De Campo L, Friera A. Congenital and acquired anomalies of the portal venous system. Radiographics 2002;22:141-59.
Ratnasamy C, Kurbegov A, Swaminathan S. Cardiac anomalies in the setting of the Abernethy malformation of the portal vein. Cardiol Young 2007;17:212-4.
Hu GH, Shen LG, Yang J, Mei JH, Zhu YF. Insight into congenital absence of the portal vein: Is it rare?. World J Gastroenterol 2008;14:5969-79
Kim MJ, Ko JS, Seo JK, Yang HR, Chang JY, Kim GB, et al. Clinical features of congenital portosystemic shunt in children. Eur J Pediatr 2012;171:395-400.
Witters P, Maleux G, George C, Delcroix M, Hoffman I, Gewillig M, et al. Congenital veno-venous malformations of the liver: Widely variable clinical presentations. J Gastroenterol Hepatol 2008;23:390-4.
Franchi-Abella S, Branchereau S, Lambert V, Fabre M, Steimberg C, Losay J, et al. Complications of congenital portosystemic shunts in children: Therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 2010;51:322–30.
Singhal A, Srivastava A, Goyal N, Vij V, Wadhawan M, Bera M, et al. Successful living donor liver transplant in a child with Abernethy malformation with biliary atresia, ventricular septal defect and intrapulmonary shunting. Pediatr Transplant 2009; 13: 1041–7.
Passalacqua M, Lie KT, Yarmohammadi H. Congenital extrahepa- tic portosystemic shunt (Abernethy malformation) treated endovas- cularly with vascular plug shunt closure. Pediatr Surg Int 2012; 28:79-83.

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How to cite

Appak YÇ, Tarhan S, Doğan G, Kasırga E. The Abernethy Malformation: A Rare Cause of Congenital Extrahepatic Portacaval Shunt. Turk J Pediatr Dis [Internet]. 2016 Apr. 1 [cited 2025 May 24];10(1):53-6. Available from: https://turkjpediatrdis.org/article/view/418

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[ref1] Abernethy J, Banks J. Account of two instances of the uncommon formation in the viscera of the human body. Philos Trans 1793; 83:59-66.

[ref2] Mistinova J, Valacsai F, Varga I. Congenital absence of the portal vein: Case report and a review of literature. Clin Anat 2010; 23: 750-8.

[ref3] Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classifi cation system for portasystemic vascular anomalies. J Pediatr Surg 1994; 29: 1239-41.

[ref4] Gocmen R, Akhan O, Talim B. Congenital absence of the portal vein associated with congenital hepatic fi brosis. Pediatr Radiol 2007; 37: 920-4.

[ref5] Gallego C, Velasco M, Marcuello P, Tejedor D, De Campo L, Friera A. Congenital and acquired anomalies of the portal venous system. Radiographics 2002;22:141-59.

[ref6] Ratnasamy C, Kurbegov A, Swaminathan S. Cardiac anomalies in the setting of the Abernethy malformation of the portal vein. Cardiol Young 2007;17:212-4.

[ref7] Hu GH, Shen LG, Yang J, Mei JH, Zhu YF. Insight into congenital absence of the portal vein: Is it rare?. World J Gastroenterol 2008;14:5969-79

[ref8] Kim MJ, Ko JS, Seo JK, Yang HR, Chang JY, Kim GB, et al. Clinical features of congenital portosystemic shunt in children. Eur J Pediatr 2012;171:395-400.

[ref9] Witters P, Maleux G, George C, Delcroix M, Hoffman I, Gewillig M, et al. Congenital veno-venous malformations of the liver: Widely variable clinical presentations. J Gastroenterol Hepatol 2008;23:390-4.

[ref10] Franchi-Abella S, Branchereau S, Lambert V, Fabre M, Steimberg C, Losay J, et al. Complications of congenital portosystemic shunts in children: Therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 2010;51:322–30.

[ref11] Singhal A, Srivastava A, Goyal N, Vij V, Wadhawan M, Bera M, et al. Successful living donor liver transplant in a child with Abernethy malformation with biliary atresia, ventricular septal defect and intrapulmonary shunting. Pediatr Transplant 2009; 13: 1041–7.