Supplementary alphabetical reference list of articles included in the literature analysis

1. Afzal, S., A. Nair, J. Grainger, S. Latif, and A. U. Rehman. 2010. Spontaneous thrombosis of congenital extrahepatic portosystemic shunt (Abernethy malformation) simulating inguinal hernia incarceration. Vasc.Endovascular.Surg. 44:508-510.

2. Akahoshi, T., T. Nishizaki, K. Wakasugi, T. Mastuzaka, K. Kume, I. Yamamoto, and K. Sugimachi. 2000. Portal-systemic encephalopathy due to a congenital extrahepatic portosystemic shunt: three cases and literature review. Hepatogastroenterology 47:1113-1116.

3. Alewine, T. C., W. R. Carter, and M. J. Frew. 2007. Congenital absence of the portal vein in a patient with urolithiasis. AJR Am.J.Roentgenol. 189:W150-W152.

4. Ali, S., A. H. Stolpen, and W. N. Schmidt. 2010. Portosystemic encephalopathy due to mesoiliac shunt in a patient without cirrhosis. J.Clin.Gastroenterol. 44:381-383.

5. Alomari, A. I., G. Chaudry, V. L. Fox, S. J. Fishman, and T. L. Buchmiller. 2009. Atypical manifestation of patent ductus venosus in a child: intervening against a paradoxical presentation. J.Vasc.Interv.Radiol. 20:537-542.

6. Altavilla, G. and P. Cusatelli. 1998. Ultrastructural analysis of the liver with portal vein agenesis: a case report. Ultrastruct.Pathol. 22:477-483.

7. Altavilla, G. and G. Guariso. 1999. Focal nodular hyperplasia of the liver associated with portal vein agenesis: a morphological and immunohistochemical study of one case and review of the literature. Adv.Clin.Path. 3:139-145.

8. Alvarez, A. E., A. F. Ribeiro, G. Hessel, J. Baracat, and J. D. Ribeiro. 2002. Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr.Pulmonol. 34:391-394.

9. Andreani, P., P. Srinivasan, C. S. Ball, N. D. Heaton, and M. Rela. 2000. Congenital absence of the portal vein in liver transplantation for biliary atresia. Int.J.Surg.Investig. 2:81-84.

10. Appel, H., C. Loddenkemper, P. Schirmacher, H. P. Dienes, J. Sieper, M. Rudwaleit, W. Golder, and M. Zeitz. 2003. Congenital absence of the portal vein with splenomegaly and hypersplenism in a young woman. Digestion 67:105-110.

11. Araki, T., M. Kamada, Y. Okamoto, S. Arai, and O. Oba. 2003. Coil embolization of a patent ductus venosus in a 52-day-old girl with congenital heart disease. Ann.Thorac.Surg. 75:273-275.

12. Araki, T., T. Konishi, S. Yasuda, T. Osada, and T. Araki. 2003. Embolization of the patent ductus venosus in an adult patient. AJR Am.J.Roentgenol. 180:716-718.

13. Arana, E., L. Marti-Bonmati, V. Martinez, M. Hoyos, and H. Montes. 1997. Portal vein absence and nodular regenerative hyperplasia of the liver with giant inferior mesenteric vein. Abdom.Imaging 22:506-508.

14. Aydinli, M., I. K. Onal, O. Harmanci, O. Ersoy, F. Balkanci, and Y. Bayraktar. 2008. A case of patent ductus venosus complicated with tumor-like lesions of the liver. J.Natl.Med.Assoc. 100:108-111.

15. Barchetti, F., L. Pellegrino, N. Al Ansari, M. De, V, P. Scarpato, and P. Ialongo. 2011. Congenital absence of the portal vein in a middle-aged man. Surg.Radiol.Anat. 33:369-372.

16. Barjon, P., J. L. Lamarque, H. Michel, J. Fourcade, A. Mimran, and J. F. Ginestie. 1972. Persistent ductus venosus without portal hypertension in a young alcoholic man. Gut 13:982-985.

17. Barsky, M. F., R. N. Rankin, W. J. Wall, C. N. Ghent, and B. Garcia. 1989. Patent ductus venosus: problems in assessment and management. Can.J.Surg. 32:271-275.

18. Barton, J. W., III and M. S. Keller. 1989. Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein. Pediatr.Radiol. 20:113-114.

19. Bellah, R. D., J. Hayek, and R. L. Teele. 1989. Anomalous portal venous connection to the suprahepatic vena cava: sonographic demonstration. Pediatr.Radiol. 20:115-117.

20. Broere, D., W. G. van Gemert, C. M. Kneepkens, D. M. Neele, R. A. Manoliu, J. A. Rauwerda, and M. S. van der Knaap. 2000. A 6-year-old boy with hyperammonaemia: partial N-acetylglutamate synthase deficiency or portosystemic encephalopathy? Eur.J.Pediatr. 159:905-907.

21. Caruso, S., S. Riva, M. Spada, A. Luca, and B. Gridelli. 2010. Congenital extrahepatic portosystemic shunts (CEPS) type Ib: MDCT finding. Dig.Liver Dis. 42:593.

22. Chandler, T. M., M. K. Heran, S. D. Chang, A. Parvez, and A. C. Harris. 2011. Multiple focal nodular hyperplasia lesions of the liver associated with congenital absence of the portal vein. Magn Reson.Imaging 29:881-886.

23. Charre, L., F. Roggen, J. Lemaire, J. Mathijs, P. Goffette, E. Danse, and J. Lerut. 2004. Hematochezia and congenital extrahepatic portocaval shunt with absent portal vein: successful treatment by liver transplantation. Transplantation 78:1404-1406.

24. Cheung, K. M., C. Y. Lee, C. T. Wong, and A. K. Chan. 2005. Congenital absence of portal vein presenting as hepatopulmonary syndrome. J.Paediatr.Child Health 41:72-75.

25. Chiu, S. N., Y. H. Chien, M. H. Wu, J. K. Wang, and S. J. Chen. 2008. Transcatheter closure of portal-systemic shunt combining congenital double extrahepatic inferior vena cava with vascular plug. J.Pediatr. 153:723.

26. Cho, Y. K., N. K. Chang, and J. S. Ma. 2009. Successful transcatheter closure of a large patent ductus venosus with the Amplatzer vascular plug II. Pediatr.Cardiol. 30:540-542.

27. Collard, B., G. Maleux, S. Heye, M. Cool, D. Bielen, C. George, T. Roskams, and W. Van Steenbergen. 2006. Value of carbon dioxide wedged venography and transvenous liver biopsy in the definitive diagnosis of Abernethy malformation. Abdom.Imaging 31:315-319.

28. Crespin, J., A. Nemcek, G. Rehkemper, and A. T. Blei. 2000. Intrahepatic portal-hepatic venous anastomosis: a portal-systemic shunt with neurological repercussions. Am.J.Gastroenterol. 95:1568-1571.

29. da Rocha, A. J., F. T. Braga, C. J. da Silva, A. C. Maia, Jr., G. S. Mourao, and R. J. Gagliardi. 2004. Reversal of parkinsonism and portosystemic encephalopathy following embolization of a congenital intrahepatic venous shunt: brain MR imaging and 1H spectroscopic findings. AJNR Am.J.Neuroradiol. 25:1247-1250.

30. De Gaetano, A. M., B. Gui, G. Macis, R. Manfredi, and C. Di Stasi. 2004. 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 29:455-459.

31. Delle, C. L., P. Neuberger, and T. Von Kalle. 2008. Congenital intrahepatic portosystemic shunt: prenatal diagnosis and possible influence on fetal growth. Ultrasound Obstet.Gynecol. 32:233-235.

32. Digilio, M. C., R. Capolino, B. Marino, A. Sarkozy, and B. Dallapiccola. 2005. Congenital intrahepatic portosystemic venous shunt: an unusual feature in LEOPARD syndrome and in neurofibromatosis type 1. Am.J.Med.Genet.A 134:457-458.

33. Emre, S., R. Arnon, E. Cohen, R. A. Morotti, D. Vaysman, and B. L. Shneider. 2007. Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in Abernethy malformation. A case report. Liver Transpl. 13:1662-1668.

34. Eroglu, Y., J. Donaldson, L. G. Sorensen, R. L. Vogelzang, H. Melin-Aldana, J. Andersen, and P. F. Whitington. 2004. Improved neurocognitive function after radiologic closure of congenital portosystemic shunts. J.Pediatr.Gastroenterol.Nutr. 39:410-417.

35. Evans, W. N., A. Galindo, R. J. Acherman, A. Rothman, and D. P. Berthoty. 2009. Congenital portosystemic shunts and AMPLATZER vascular plug occlusion in newborns. Pediatr.Cardiol. 30:1083-1088.

36. Fanelli, F., G. Marcelli, M. Bezzi, S. F. Maria, M. Rossi, P. Rossi, and R. Passariello. 2003. Intrahepatic aneurysmal portohepatic venous shunt: embolization with a tissue adhesive solution. J.Endovasc.Ther. 10:147-153.

37. Farrant, P., H. B. Meire, and J. Karani. 1996. Ultrasound diagnosis of portocaval anastomosis in infants--a report of eight cases. Br.J.Radiol. 69:389-393.

38. Ferrero, G. B., F. Porta, E. Biamino, A. Mussa, E. Garelli, F. Chiappe, A. Veltri, M. C. Silengo, and F. Gennari. 2010. Remittent hyperammonemia in congenital portosystemic shunt. Eur.J.Pediatr. 169:369-372.

39. Florio, F., M. Nardella, S. Balzano, A. Giacobbe, and F. Perri. 1998. Congenital intrahepatic portosystemic shunt. Cardiovasc.Intervent.Radiol. 21:421-424.

40. Franchi-Abella, S., S. Branchereau, V. Lambert, M. Fabre, C. Steimberg, J. Losay, J. Y. Riou, D. Pariente, F. Gauthier, E. Jacquemin, and O. Bernard. 2010. Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J.Pediatr.Gastroenterol.Nutr. 51:322-330.

41. Gillespie, M. J., A. Golden, V. B. Sivarajan, and J. J. Rome. 2006. Transcatheter closure of patent ductus venosus with the Amplatzer vascular plug in twin brothers. Pediatr.Cardiol. 27:142-145.

42. Gitzelmann, R., I. Forster, and U. V. Willi. 1997. Hypergalactosaemia in a newborn: self-limiting intrahepatic portosystemic venous shunt. Eur.J.Pediatr. 156:719-722.

43. Gocmen, R., O. Akhan, and B. Talim. 2007. Congenital absence of the portal vein associated with congenital hepatic fibrosis. Pediatr.Radiol. 37:920-924.

44. Golewale, N., H. J. Paltiel, S. J. Fishman, and A. I. Alomari. 2010. Portal vascular anomalies in Down syndrome: spectrum of clinical presentation and management approach. J.Pediatr.Surg. 45:1676-1681.

45. Golli, M., S. Kriaa, M. Said, M. Belguith, M. Zbidi, J. Saad, A. Nouri, and A. Ganouni. 2000. Intrahepatic spontaneous portosystemic venous shunt: value of color and power Doppler sonography. J.Clin.Ultrasound 28:47-50.

46. Goo, H. W. 2007. Extrahepatic portosystemic shunt in congenital absence of the portal vein depicted by time-resolved contrast-enhanced MR angiography. Pediatr.Radiol. 37:706-709.

47. Grazioli, L., D. Alberti, L. Olivetti, W. Rigamonti, F. Codazzi, L. Matricardi, C. Fugazzola, and A. Chiesa. 2000. Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur.Radiol. 10:820-825.

48. Guariso, G., S. Fiorio, G. Altavilla, P. G. Gamba, T. Toffolutti, M. Chiesura-Corona, U. Tedeschi, and L. Zancan. 1998. Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and cystic dysplasia of the kidney. Eur.J.Pediatr. 157:287-290.

49. Gupta, V., N. Kalra, S. Vyas, K. S. Sodhi, B. R. Thapa, and N. Khandelwal. 2009. Embolization of congenital intrahepatic porto-systemic shunt by n-butyl cyanoacrylate. Indian J.Pediatr. 76:1059-1060.

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51. Hoover, W., V. Ackerman, M. Schamberger, M. Kumar, F. Marshalleck, and M. Hoyer. 2008. The congenital porto-caval fistula: a unique presentation and novel intervention. Pediatr.Pulmonol. 43:196-199.

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53. Howard, E. R. and M. Davenport. 1997. Congenital extrahepatic portocaval shunts--the Abernethy malformation. J.Pediatr.Surg. 32:494-497.

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56. Ikeda, S., Y. Sera, H. Ohshiro, S. Uchino, T. Uchino, and F. Endo. 1999. Surgical indications for patients with hyperammonemia. J.Pediatr.Surg. 34:1012-1015.

57. Ikeda, S., Y. Sera, M. Yoshida, T. Izaki, S. Uchino, F. Endo, M. Ohmuraya, and T. Beppu. 1999. Successful coil embolization in an infant with congenital intrahepatic portosystemic shunts. J.Pediatr.Surg. 34:1031-1032.

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59. Ikeda, S., Y. Yamaguchi, Y. Sera, H. Ohshiro, S. Uchino, and M. Ogawa. 1999. Surgical correction of patent ductus venosus in three brothers. Dig.Dis.Sci. 44:582-589.

60. Imamura, H., T. Momose, H. Kitabayashi, W. Takahashi, Y. Yazaki, H. Takenaka, M. Isobe, M. Sekiguchi, and K. Kubo. 2000. Pulmonary hypertension as a result of asymptomatic portosystemic shunt. Jpn.Circ.J. 64:471-473.

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63. Jacob, S., G. Farr, D. De Vun, H. Takiff, and A. Mason. 1999. Hepatic manifestations of familial patent ductus venosus in adults. Gut 45:442-445.

64. Joyce, A. D. and E. R. Howard. 1988. Rare congenital anomaly of the portal vein. Br.J.Surg. 75:1038-1039.

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