Li H, Ma Z, Xie Y, Tian F

Language: English
References: 24
Page: 460-464
PDF size: 294.83 Kb.

ABSTRACT

The Abernethy malformation is a rare congenital malformation defined by the presence of an extrahepatic portosystemic shunt. Although most patients are asymptomatic, clinical encephalopathy is present in 15% of cases. We present a patient with type 2 Abernethy malformation, hyperammonemia, and encephalopathy. Shunt closure was performed successfully using interventional angiography; however, hyperammonemia recurred 3 months later. The diagnosis of Abernethy malformation can be made easily, but the ideal patient management strategy has not yet been established. This is the first reported patient with recurrence of hyperammonemia after interventional treatment; we discuss the therapeutic options for Abernethy malformation.

REFERENCES

1. 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: 1239-41.

2. Howard ER, Davenport M. Congenital extrahepatic portocaval shunts: The Abernethy malformation. J Pediatr Surg 1997; 32: 494-7.

3. Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatr Radiol 2003; 33: 614-20.

4. Grazioli L, Alberti D, Olivetti L, Rigamonti W, Codazzi F, Matricardi L, Fugazzola C, et al. Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur Radiol 2000; 10: 820-5.

5. Witters P, Maleux G, George C, Delcroix M, Hoffman I, Gewillig M, Verslype C, et al. Congenital veno-venous malformations of the liver: widely variable clinical presentations. J Gastroenterol Hepatol 2008; 23: e390-4.

6. Lee WK, Chang SD, Duddalwar VA, Comin JM, Perera W, Lau Wing FE, Bekhit EK, et al. Imaging assessment of congenital and acquired abnormalities of the portal venous system. RadioGraphics 2011; 31: 905-26.

7. Asran MK, Loyer EM, Kaur H, Choi H. Case 177: congenital absence of the portal vein with hepatic adenomatosis. Radiology 2012; 262: 364-7.

8. Yabin H, Xu H, Xinyan Z. Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review. Oncol lett 2015; 9: 695-700.

9. Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet 2004; 363: 1461-8.

10. 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.

11. Collard B, Maleux G, Heye S, Cool M, Bielen D, George C, Roskams T, et al. Value of carbon dioxide wedged venography and transvenous liver biopsy in the definitive diagnosis of Abernethy malformation. Abdom Imaging 2006; 31: 315-9.

12. 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: 702-22.

13. Duprey J, Gouin B, Benazet MF, et al. Glucose intolerance and poststimulatory hypoglycemia secondary to a probably congenital intrahepatic portacaval anastomosis. Ann Med Interne 1985; 136: 655-8.

14. De Gaetano AM, Gui B, Macis G, Manfredi R, Di Stasi C. Congenital absence of the portal vein associated with focal nodular hyperplasia in the liver in an adult woman: imaging and review of the literature. Abdom Imaging 2004; 29: 455-9.

15. Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatr Radiol 2003; 33: 614-20.

16. 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: 675-81.

17. Crespin J, Nemcek A, Rehkemper G, Blei AT. Intrahepatic portal-hepatic venous anastomosis: a portal-systemic shunt with neurological repercussions. Am J Gastroenterol 2000; 95: 1568-71.

18. Singhal A, Srivastava A, Goyal N, Vij V, Wadhawan M, Bera M, Gupta S. 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.

19. KCS, Matsutani S. Maruyama H. Fukamachi T. Nomoto H. Akiike T. Ebara M, et al. Portal systemic encephalopathy in two patients without liver cirrhosis and portal hypertension. Hepatol Res 2002; 23: 122-9.

20. Orii T, Ohkohchi N, Kato H, Doi H, Hirano T, Sekiguchi S, Akamatsu Y, et al. Liver transplantation for severe hypoxemia caused by patent ductus venosus. J Pediatr Surg 1997; 32: 1795-7.

21. 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.

22. Venkateshwaran S, Krishnamoorthy KM, Sivasankaran S. Percutaneous Device Closure of Abernethy Malformation-A Treatable Cause of Hepatopulmonary Syndrome. Catheter Cardiovasc Interv 2014; 83: 968-70.

23. Kanazawa H, Nosaka S, Miyazaki O, Sakamoto S, Fukuda A, Shigeta T, Nakazawa A, et al. The classification based on intrahepatic portal system for congenital portosystemic shunts. J Pediatr Surg 2015; 50: 688-95.

24. Franchi-Abella S, Branchereau S, Lambert V, Fabre M, Steimberg C, Losay J, Riou JY, et al. Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 2010, 51: 322-30.