Abernethy Malformasyonu: Konjenital Ekstrahepatik Portokaval Şantın Nadir Nedeni

Yıl 2016, Cilt: 10 Sayı: 1, 53 - 56, 01.04.2016

Yeliz Çağan Appak Serdar Tarhan Güzide Doğan Erhun Kasırga

https://izlik.org/JA35DY64XC

Öz

Abernethy malformasyonu birçok farklı klinik bulgu ile görülebilen çok nadir bir splanknik venöz sistem anomalisidir. Bu olgu sunumunda klinik bulgusu olmadan tetkikler sonucunda rastlantısal olarak Abernethy malformasyonu saptanan ve karaciğerde kitleleri bulunan 5 aylık kız olgu sunulmaktadır. Malformasyonun klinik bulguları ve olası komplikasyonların önemi vurgulanmaktadır.

Anahtar Kelimeler

Çocuk , Portal ven , Vasküler malformasyon

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

https://izlik.org/JA35DY64XC

Öz

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

Anahtar Kelimeler

Child , Portal vein , Vasculer malformation

Kaynakça

• 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.