Congenital portosystemic shunts: diagnosis and treatment


Congenital portosystemic shunts (CPSS) are rare vascular malformations that create an abnormal connection between portal and systemic veins resulting in complete or partial diversion of the portal flow away from the liver to the systemic venous system. Different anatomic types exist and several classifications have been proposed. They can be associated with other malformations especially cardiac and heterotaxia. The main complications include hepatic encephalopathy, liver tumors, portopulmonary hypertension, and pulmonary arteriovenous shunts. Diagnosis relies on imaging, and prenatal diagnosis is possible. Spontaneous closure of the CPSS is possible in some anatomic forms during the first year of life. When the CPSS remains patent, radiologic or surgical closure of the CPSS may prevent, resolve, or stabilize complications. Interventional radiology plays a key role for both the preoperative evaluation with occlusion test to assess the exact anatomy and to measure portal pressure after occlusion of the CPSS. Endovascular closure is the first option for treatment when possible.

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  1. 1.

    Franchi-Abella S, Branchereau S, Lambert V, et al. (2010) Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 51:322–330.

    PubMed  Article  Google Scholar 

  2. 2.

    Blanc T, Guerin F, Franchi-Abella S, et al. (2014) Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy. Ann Surg 260:188–198.

    Article  PubMed  Google Scholar 

  3. 3.

    Mavrides E, Moscoso G, Carvalho JS, et al. (2001) The anatomy of the umbilical, portal and hepatic venous systems in the human fetus at 14-19 weeks of gestation. Ultrasound Obstet Gynecol 18:598–604.

    Article  PubMed  CAS  Google Scholar 

  4. 4.

    Hikspoors JPJM, Soffers JHM, Mekonen HK, et al. (2015) Development of the human infrahepatic inferior caval and azygos venous systems. J Anat 226:113–125.

    Article  PubMed  Google Scholar 

  5. 5.

    Hikspoors JPJM, Peeters MMJP, Kruepunga N, et al. (2017) Human liver segments: role of cryptic liver lobes and vascular physiology in the development of liver veins and left-right asymmetry. Sci Rep 7:17109.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  6. 6.

    Hikspoors JPJM, Peeters MMJP, Mekonen HK, et al. (2017) The fate of the vitelline and umbilical veins during the development of the human liver. J Anat 231:718–735.

    Article  PubMed  CAS  Google Scholar 

  7. 7.

    Collardeau-Frachon S, Scoazec J-Y (2008) Vascular development and differentiation during human liver organogenesis. Anat Rec 291:614–627.

    Article  Google Scholar 

  8. 8.

    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

    Article  PubMed  CAS  Google Scholar 

  9. 9.

    Howard ER, Davenport M (1997) Congenital extrahepatic portocaval shunts—the abernethy malformation. J Pediatr Surg 32:494–497

    Article  PubMed  CAS  Google Scholar 

  10. 10.

    Stringer MD (2008) The clinical anatomy of congenital portosystemic venous shunts. Clin Anat 21:147–157.

    Article  PubMed  Google Scholar 

  11. 11.

    Lautz TB, Tantemsapya N, Rowell E, Superina RA (2011) Management and classification of type II congenital portosystemic shunts. J Pediatr Surg 46:308–314.

    Article  PubMed  Google Scholar 

  12. 12.

    Kanazawa H, Nosaka S, Miyazaki O, et al. (2015) The classification based on intrahepatic portal system for congenital portosystemic shunts. J Pediatr Surg 50:688–695.

    Article  PubMed  Google Scholar 

  13. 13.

    Back SJ, Maya CL, Khwaja A (2017) Ultrasound of congenital and inherited disorders of the pediatric hepatobiliary system, pancreas and spleen. Pediatr Radiol 47:1069–1078.

    Article  PubMed  Google Scholar 

  14. 14.

    Bernard O, Franchi-Abella S, Branchereau S, et al. (2012) Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis 32:273–287.

    Article  PubMed  CAS  Google Scholar 

  15. 15.

    Cho Y, Tokuhara D, Shimono T, et al. (2014) Role of per-rectal portal scintigraphy in long-term follow-up of congenital portosystemic shunt. Pediatr Res 75:658–662.

    Article  PubMed  CAS  Google Scholar 

  16. 16.

    Shiomi S, Sasaki N, Ikeoka N, et al. (1998) Usefulness of per-rectal portal scintigraphy with Tc-99m pertechnetate for galactosemia in infants. Ann Nucl Med 12:375–378

    Article  PubMed  CAS  Google Scholar 

  17. 17.

    Uchino T, Matsuda I, Endo F (1999) The long-term prognosis of congenital portosystemic venous shunt. J Pediatr 135:254–256

    Article  PubMed  CAS  Google Scholar 

  18. 18.

    Chocarro G, Amesty MV, Encinas JL, et al. (2016) Congenital portosystemic shunts: clinic heterogeneity requires an individual management of the patient. Eur J Pediatr Surg 26:74–80.

    PubMed  Article  Google Scholar 

  19. 19.

    Achiron R, Kivilevitch Z (2016) Fetal umbilical-portal-systemic venous shunt: in utero classification and clinical significance. Ultrasound Obstet Gynecol 47:739–747.

    Article  PubMed  CAS  Google Scholar 

  20. 20.

    Loberant N, Herskovits M, Barak M, et al. (1999) Closure of the ductus venosus in premature infants: findings on real-time gray-scale, color-flow Doppler, and duplex Doppler sonography. AJR Am J Roentgenol 172:227–229.

    Article  PubMed  CAS  Google Scholar 

  21. 21.

    Loberant N, Barak M, Gaitini D, et al. (1992) Closure of the ductus venosus in neonates: findings on real-time gray-scale, color-flow Doppler, and duplex Doppler sonography. AJR Am J Roentgenol 159:1083–1085.

    Article  PubMed  CAS  Google Scholar 

  22. 22.

    Farrant P, Meire HB, Karani J (1996) Ultrasound diagnosis of portocaval anastomosis in infants—a report of eight cases. Br J Radiol 69:389–393.

    Article  PubMed  CAS  Google Scholar 

  23. 23.

    Ono H, Mawatari H, Mizoguchi N, et al. (1998) Clinical features and outcome of eight infants with intrahepatic porto-venous shunts detected in neonatal screening for galactosaemia. Acta Paediatr Oslo Nor 87:631–634

    Article  CAS  Google Scholar 

  24. 24.

    Francois B, Gottrand F, Lachaux A, et al. (2017) Outcome of intrahepatic portosystemic shunt diagnosed prenatally. Eur J Pediatr .

    Article  PubMed  Google Scholar 

  25. 25.

    Golewale N, Paltiel HJ, Fishman SJ, Alomari AI (2010) Portal vascular anomalies in Down syndrome: spectrum of clinical presentation and management approach. J Pediatr Surg 45:1676–1681.

    Article  PubMed  Google Scholar 

  26. 26.

    Tanya Chun P, Chun T, Files M, et al. (2013) Percutaneous embolization of congenital portosystemic venous fistula in an infant with down syndrome. Case Rep Vasc Med 2013:127023.

    PubMed  PubMed Central  Article  Google Scholar 

  27. 27.

    Yamaguchi H, Kosugiyama K, Honda S, et al. (2016) Down syndrome with patent ductus venosus and hepato-biliary-pancreatic abnormalities. Indian J Pediatr 83:78–80.

    Article  PubMed  Google Scholar 

  28. 28.

    Pérez-García C, Martín YR, Del Hoyo AA, et al. (2017) Adams-Oliver syndrome with unusual central nervous system findings and an extrahepatic portosystemic shunt. Pediatr Rep 9:7211.

    Article  PubMed  PubMed Central  Google Scholar 

  29. 29.

    Sokollik C, Bandsma RHJ, Gana JC, et al. (2013) Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr 56:675–681.

    Article  PubMed  Google Scholar 

  30. 30.

    Uchino T, Endo F, Ikeda S, et al. (1996) Three brothers with progressive hepatic dysfunction and severe hepatic steatosis due to a patent ductus venosus. Gastroenterology 110:1964–1968

    Article  PubMed  CAS  Google Scholar 

  31. 31.

    Shinkai M, Ohhama Y, Nishi T, et al. (2001) Congenital absence of the portal vein and role of liver transplantation in children. J Pediatr Surg 36:1026–1031.

    Article  PubMed  CAS  Google Scholar 

  32. 32.

    Cho Y, Shimono T, Morikawa H, et al. (2014) Hepatic focal nodular hyperplasia with congenital portosystemic shunt. Pediatr Int 56:e102–105.

    Article  PubMed  Google Scholar 

  33. 33.

    Gordon-Burroughs S, Balogh J, Weiner MA, et al. (2014) Liver transplantation in an adult with adenomatosis and congenital absence of the portal vein: a case report. Transplant Proc 46:2418–2421.

    Article  PubMed  CAS  Google Scholar 

  34. 34.

    Sanada Y, Mizuta K, Niki T, et al. (2015) Hepatocellular nodules resulting from congenital extrahepatic portosystemic shunts can differentiate into potentially malignant hepatocellular adenomas. J Hepato Biliary Pancreat Sci 22:746–756.

    Article  Google Scholar 

  35. 35.

    Benedict M, Rodriguez-Davalos M, Emre S, et al. (2017) Congenital extrahepatic portosystemic shunt (abernethy malformation type Ib) with associated hepatocellular carcinoma: case report and literature review. Pediatr Dev Pathol 20:354–362.

    Article  PubMed  Google Scholar 

  36. 36.

    Lautz TB, Shah SA, Superina RA (2016) Hepatoblastoma in children with congenital portosystemic shunts. J Pediatr Gastroenterol Nutr 62:542–545.

    Article  PubMed  Google Scholar 

  37. 37.

    Sharma R, Suddle A, Quaglia A, et al. (2015) Congenital extrahepatic portosystemic shunt complicated by the development of hepatocellular carcinoma. Hepatobiliary Pancreat Dis Int 14:552–557

    Article  PubMed  Google Scholar 

  38. 38.

    Takahashi S, Yoshida E, Sakanishi Y, et al. (2014) Congenital multiple intrahepatic portosystemic shunt: an autopsy case. Int J Clin Exp Pathol 7:425–431

    PubMed  Google Scholar 

  39. 39.

    Nardone R, Taylor AC, Höller Y, et al. (2016) Minimal hepatic encephalopathy: a review. Neurosci Res 111:1–12.

    Article  PubMed  Google Scholar 

  40. 40.

    Matsuura T, Takahashi Y, Yanagi Y, et al. (2016) Surgical strategy according to the anatomical types of congenital portosystemic shunts in children. J Pediatr Surg 51:2099–2104.

    Article  PubMed  Google Scholar 

  41. 41.

    Edula RGR, Pyrsopoulos NT (2015) New methods of testing and brain imaging in hepatic encephalopathy: a review. Clin Liver Dis 19:449–459.

    Article  PubMed  Google Scholar 

  42. 42.

    Kobayashi D, Edwards HD, Singh J, et al. (2011) Portopulmonary hypertension secondary to congenital extrahepatic portosystemic shunt with heterotaxy and polysplenia: a cause of sudden death in an infant. Pediatr Pulmonol 46:1041–1044.

    Article  PubMed  Google Scholar 

  43. 43.

    Lin W-C, Hsu T-W, Chen C-L, et al. (2014) Reestablishing brain networks in patients without overt hepatic encephalopathy after liver transplantation. J Cereb Blood Flow Metab 34:1877–1886.

    Article  PubMed  PubMed Central  Google Scholar 

  44. 44.

    da Rocha AJ, Braga FT, da Silva CJ, et al. (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

    PubMed  Google Scholar 

  45. 45.

    Ohno T, Muneuchi J, Ihara K, et al. (2008) Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association. Pediatrics 121:e892–899.

    Article  PubMed  Google Scholar 

  46. 46.

    Torigoe M, Maeshima K, Takeshita Y (2013) Congenital intrahepatic portosystemic venous shunt presenting with paraparesis as the initial symptom. Intern Med Tokyo Jpn 52:2439–2442

    Article  Google Scholar 

  47. 47.

    Fu L, Wang Q, Wu J, et al. (2016) Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome. Eur J Pediatr 175:195–201.

    Article  PubMed  Google Scholar 

  48. 48.

    Knirsch W, Benz DC, Bühr P, et al. (2016) Catheter interventional treatment of congenital portosystemic venous shunts in childhood. Catheter Cardiovasc Interv 87:1281–1292.

    Article  PubMed  Google Scholar 

  49. 49.

    Layangool T, Kojaranjit V, Promphan W, et al. (2014) An unusual cause of progressive cyanosis post Fontan operation: congenital extra-hepatic porto-systemic shunt. J Med Assoc Thail Chotmaihet Thangphaet 97(Suppl 6):S176–181

    Google Scholar 

  50. 50.

    Raghuram KA, Bijulal S, Krishnamoorthy KM, Tharakan JA (2013) Regression of pulmonary vascular disease after therapy of Abernethy malformation in visceral heterotaxy. Pediatr Cardiol 34:1882–1885.

    Article  PubMed  Google Scholar 

  51. 51.

    Newman B, Feinstein JA, Cohen RA, et al. (2010) Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia. Pediatr Radiol 40:1222–1230.

    Article  PubMed  Google Scholar 

  52. 52.

    Grimon G, André L, Bernard O, et al. (1994) Early radionuclide detection of intrapulmonary shunts in children with liver disease. J Nucl Med 35:1328–1332

    PubMed  CAS  Google Scholar 

  53. 53.

    Alvarez AE, Ribeiro AF, Hessel G, et al. (2002) Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol 34:391–394.

    Article  PubMed  Google Scholar 

  54. 54.

    Ohnishi Y, Ueda M, Doi H, et al. (2005) Successful liver transplantation for congenital absence of the portal vein complicated by intrapulmonary shunt and brain abscess. J Pediatr Surg 40:e1–3.

    Article  PubMed  Google Scholar 

  55. 55.

    Bellah RD, Hayek J, Teele RL (1989) Anomalous portal venous connection to the suprahepatic vena cava: sonographic demonstration. Pediatr Radiol 20:115–117

    Article  PubMed  CAS  Google Scholar 

  56. 56.

    McKie PM, McCully RB, Kamath PS, et al. (2012) Amelioration of high cardiac output and pulmonary hypertension by occlusion of congenital porto-systemic shunt. Circulation 126:2533–2534.

    Article  PubMed  Google Scholar 

  57. 57.

    Sumida W, Kaneko K, Ogura Y, et al. (2006) Living donor liver transplantation for congenital absence of the portal vein in a child with cardiac failure. J Pediatr Surg 41:e9–e12.

    Article  PubMed  Google Scholar 

  58. 58.

    Karashima S, Hattori S, Nakazato H, et al. (2000) Membranoproliferative glomerulonephritis in congenital portosystemic shunt without liver cirrhosis. Clin Nephrol 53:206–211

    PubMed  CAS  Google Scholar 

  59. 59.

    Lee SH, Lee D-G (2015) Macroscopic hematuria caused by congenital portosystemic shunt and concomitant nutcracker syndrome. Pediatr Int 57:e84–86.

    Article  PubMed  Google Scholar 

  60. 60.

    Gong Y, Zhu H, Chen J, et al. (2015) Congenital portosystemic shunts with and without gastrointestinal bleeding—case series. Pediatr Radiol 45:1964–1971.

    Article  PubMed  Google Scholar 

  61. 61.

    Bas S, Guran T, Atay Z, et al. (2015) Premature pubarche, hyperinsulinemia and hypothyroxinemia: novel manifestations of congenital portosystemic shunts (Abernethy malformation) in children. Horm Res Paediatr 83:282–287.

    Article  PubMed  CAS  Google Scholar 

  62. 62.

    Satoh M, Yokoya S, Hachiya Y, et al. (2001) Two hyperandrogenic adolescent girls with congenital portosystemic shunt. Eur J Pediatr 160:307–311

    Article  PubMed  CAS  Google Scholar 

  63. 63.

    Senniappan S, Pitt K, Shah P, et al. (2015) Postprandial hyperinsulinaemic hypoglycaemia secondary to a congenital portosystemic shunt. Horm Res Paediatr 83:217–220.

    Article  PubMed  CAS  Google Scholar 

  64. 64.

    Knisely AS (2014) Patent ductus venosus and acute liver failure in the neonate: consider neonatal hemochromatosis with liver scarring. Liver Transplant 20:124.

    Article  CAS  Google Scholar 

  65. 65.

    Scalabre A, Gorincour G, Hery G, et al. (2012) Evolution of congenital malformations of the umbilical-portal-hepatic venous system. J Pediatr Surg 47:1490–1495.

    Article  PubMed  Google Scholar 

  66. 66.

    Matsuura T, Yanagi Y, Saeki I, et al. (2011) Outcome of modified portal vein anastomosis for recipients with portal vein thrombosis or stenosis before living donor liver transplantation. J Pediatr Surg 46:2291–2295.

    Article  PubMed  Google Scholar 

  67. 67.

    Kuo MD, Miller FJ, Lavine JE, et al. (2010) Exploiting phenotypic plasticity for the treatment of hepatopulmonary shunting in Abernethy malformation. J Vasc Interv Radiol JVIR 21:917–922.

    Article  PubMed  Google Scholar 

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We would like to thank Professor Olivier Bernard and Professor Frédéric Gauthier for their major valuable contribution to the knowledge of portosystemic shunts.

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Correspondence to Stéphanie Franchi-Abella.

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Franchi-Abella, S., Gonzales, E., Ackermann, O. et al. Congenital portosystemic shunts: diagnosis and treatment. Abdom Radiol 43, 2023–2036 (2018).

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  • Portosystemic shunt
  • Hepatopulmonary syndrome
  • Portopulmonary syndrome
  • Liver tumors
  • Portosystemic encephalopathy